How chronic lymphocytic leukemia manifests itself, why it occurs, methods for its diagnosis and treatment. Chronic lymphocytic leukemia: about its diagnosis and treatment Chronic lymphocytic leukemia is characterized by

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2015

Chronic lymphocytic leukemia (C91.1)

Oncohematology

general information

Short description

Recommended
Expert Council
RSE on REM "Republican Center
health development"
Ministry of Health
and social development
Republic of Kazakhstan
dated July 9, 2015
Protocol #6

Chronic lymphocytic leukemia/small lymphocyte lymphoma- a tumor disease of the blood system, characterized by the proliferation and accumulation in the blood, bone marrow and lymphoid organs of morphologically mature and immunologically incompetent B-lymphocytes with a characteristic immunophenotype (co-expression of CD5 and CD23).
Chronic lymphocytic leukemia (CLL) and small lymphocyte lymphoma are different manifestations of the same disease. In both cases, clonal small B-lymphocytes are the main substrate. The only difference is that in CLL the bulk of tumor lymphocytes are concentrated in the bone marrow and peripheral blood, and in lymphoma from small lymphocytes in the lymph nodes.

Protocol code:

ICD code -10:
C91.1 - Chronic lymphocytic leukemia

Protocol development date: 2015

Abbreviations used in the protocol:
* - drugs purchased as part of a single import
CLL - chronic lymphocytic leukemia
NCCN - National Comprehensive Cancer Network
HSC - hematopoietic stem cells
MRD - minimal residual (residual) disease
PCT - polychemotherapy
TKI - tyrosine kinase inhibitors
TCM - bone marrow stem cell transplant
FISH - fluorescent in situ hybridization
HLA - human leukocyte antigen system
AH - arterial hypertension
BP - blood pressure
ALAT - alanine aminotransferase
ASAT - aspartate aminotransferase
HIV - human immunodeficiency virus
ELISA - enzyme immunoassay
CT - computed tomography
LDH - lactate dehydrogenase
MDS - myelodysplastic syndrome
MPO - myeloperoxidase
NE - naphthylesterase
KLA - complete blood count
PCR - polymerase chain reaction
ESR - erythrocyte sedimentation rate
UZDG - ultrasonic dopplerography
Ultrasound - ultrasonography
EF - ejection fraction
FGDS - fibrogastroduodenoscopy
RR - respiratory rate
HR - heart rate
ECG - electrocardiography
EchoCG - echocardiography
NMRI - nuclear magnetic resonance imaging
PET/CT - positron emission tomography/computed tomography

Protocol Users: therapists, general practitioners, oncologists, hematologists.

Level of Evidence Scale

Classification

Clinical classification

Table 1. Classification of CLL stages according to K. Rai. [quoted from 2]

Table 2. Classification of stages of CLL according to J. Binet. [quoted from 2]

Clinical picture

Symptoms, course

Diagnostic Criteria for Making a Diagnosis :
Absolute monoclonal B-lymphocytosis (lymphocytes ≥5×109/l) in peripheral blood for at least 3 months;
· Cytological characteristics of peripheral blood lymphocytes: small narrow cytoplasmic lymphocytes with condensed chromatin of the nucleus that do not contain nucleoli.
· Confirmation of B-lymphocyte clonality by light chains (λ or κ) and detection of aberrant immunophenotype (CD19+/CD5+/CD23+/CD20dim+/CD79βdim+) by flow cytometry.
· If the diagnosis of chronic lymphocytic leukemia is confirmed by flow cytometry of peripheral blood lymphocytes, cytological and histological/immunohistochemical studies of the bone marrow and lymph nodes are not necessary.

Complaints about:
· weakness;
· sweating;
· fatigue;
subfebrile condition;
· chilling;
pain in the bones or joints;
Decrease in body weight;
hemorrhagic rashes in the form of petechiae and ecchymosis on the skin;
epistaxis;
menorrhagia;
increased bleeding
swollen lymph nodes
pain and heaviness in the left upper abdomen (enlarged spleen);
heaviness in the right hypochondrium.

Anamnesis attention should be paid to:
Long-lasting weakness
fast fatigue;
frequent infectious diseases;
Increased bleeding
the appearance of hemorrhagic rashes on the skin and mucous membranes;
Enlargement of lymph nodes, liver, spleen.

Physical examination:
pallor of the skin;
hemorrhagic rashes - petechiae, ecchymosis;
shortness of breath
· tachycardia;
Enlargement of the liver
Enlargement of the spleen
enlargement of lymph nodes;
swelling of the neck, face, hands - appear with pressure from enlarged intrathoracic lymph nodes of the superior vena cava (a vessel that brings blood to the heart from the upper half of the body).

Diagnostics

The list of basic and additional diagnostic measures:

The main (mandatory) diagnostic examinations carried out at the outpatient level:



Ultrasound of peripheral lymph nodes, abdominal organs, incl. spleen.

Additional diagnostic examinations performed at the outpatient level:
myelogram;





ELISA for HIV markers;
ELISA for markers of herpes group viruses;
β2 microglobulin;
direct Coombs test, haptoglobin
Reberg-Tareev test;
· general urine analysis;
· coagulogram;

· HLA typing;
ECG;
Echocardiography;
· Whole-body PET/CT for suspected Richter's syndrome to determine the preferred lymph node for biopsy;
CT scan of the thoracic and abdominal segments with contrast.

The minimum list of examinations that must be carried out when referring to planned hospitalization:
KLA (calculation of leukoformula, platelets in a smear);
blood type and Rh factor;
biochemical blood test (total protein, albumin, globulins, IgA, IgM, IgG, uric acid, creatinine, urea, LDH, ALT, AST, total and direct bilirubin);
Ultrasound of the abdominal organs and spleen, peripheral lymph nodes;
X-ray of the chest organs.

The main (mandatory) diagnostic examinations carried out at the hospital level:
KLA (with counting platelets and reticulocytes);
· OAM;
Immunophenotyping of peripheral blood on a flow cytometer (CD3, CD5, CD10, CD20, CD23, cyclinD1, light chains, IgM);
biochemical blood test (total protein, albumin, globulins, IgA, IgM, IgG, uric acid, creatinine, urea, LDH, ALT, AST, total and direct bilirubin);
Ultrasound examination of peripheral lymph nodes, abdominal organs, incl. spleen;
x-ray of the chest;
myelogram;
Cytogenetic study of the bone marrow;
bone marrow examination by FISH (t(11;14), t(11q,v);+12; del(11q); del(13q); del(17p));
· molecular genetic study: mutational status of genes of variable regions of heavy chains of immunoglobulins (IGHV);
Immunochemical study of blood serum and urine (free light chains of blood serum, electrophoresis with immunofixation of blood serum and daily urine). In the absence of the possibility of conducting an immunochemical study - electrophoresis of serum proteins;
ELISA and PCR for markers of viral hepatitis;
ELISA for HIV markers;
β2 microglobulin;
Direct Coombs test, haptoglobin;
ECG;
echocardiography;
Reberg-Tareev test;
· coagulogram;
blood type and Rh factor;
· HLA typing.

Additional diagnostic examinations carried out at the hospital level:
pro-BNP (atrial natriuretic peptide) in blood serum;
bacteriological examination of biological material;
cytological examination of biological material;
The immunogram
Histological examination of the biopsy specimen (lymph node, iliac crest);
PCR for viral infections (viral hepatitis, cytomegalovirus, herpes simplex virus, Epstein-Barr virus, Varicella / Zoster virus);
radiography of the paranasal sinuses;
radiography of bones and joints;
FGDS;
· Ultrasound of blood vessels;
bronchoscopy;
colonoscopy;
daily monitoring of blood pressure;
daily ECG monitoring;
spirography.

Diagnostic measures taken at the stage of emergency medical care:
collection of complaints and anamnesis of the disease;
physical examination (determination of respiratory rate, heart rate, assessment of the skin, determination of the size of the liver, spleen, peripheral lymph nodes).

12.4 Instrumental Research:
· Ultrasound of the abdominal organs, lymph nodes: an increase in the size of the liver, spleen, peripheral lymphadenopathy.
· CT scan of the thoracic segment: to detect enlarged intrathoracic lymph nodes.
· ECG: violation of the conduction of impulses in the heart muscle.
· EchoCG: to exclude heart defects, arrhythmias and other diseases in patients, accompanied by damage to the heart.
· FGDS: leukemic infiltration of the mucous membrane of the gastrointestinal tract, which can cause ulcerative lesions of the stomach, duodenum 12, gastrointestinal bleeding.
· Bronchoscopy: detection of the source of bleeding.

Indications for consultation of narrow specialists:
Doctor for X-ray endovascular diagnostics and treatment - installation of a central venous catheter from a peripheral access (PICC);
hepatologist - for the diagnosis and treatment of viral hepatitis;
· gynecologist - pregnancy, metrorrhagia, menorrhagia, consultation when prescribing combined oral contraceptives;
Dermatovenereologist - skin syndrome;
infectious disease specialist - suspicion of viral infections;
cardiologist - uncontrolled hypertension, chronic heart failure, cardiac arrhythmia and conduction disturbances;
· neuropathologist acute cerebrovascular accident, meningitis, encephalitis, neuroleukemia;
neurosurgeon - acute cerebrovascular accident, dislocation syndrome;
nephrologist (efferentologist) - renal failure;
oncologist - suspicion of solid tumors;
otorhinolaryngologist - for the diagnosis and treatment of inflammatory diseases of the paranasal sinuses and middle ear;
Ophthalmologist - visual impairment, inflammatory diseases of the eye and appendages;
proctologist - anal fissure, paraproctitis;
psychiatrist - psychoses;
psychologist - depression, anorexia, etc.;
· resuscitator - treatment of severe sepsis, septic shock, acute lung injury syndrome in differentiation syndrome and terminal states, installation of central venous catheters.
rheumatologist - Sweet's syndrome;
Thoracic surgeon - exudative pleurisy, pneumothorax, pulmonary zygomycosis;
· transfusiologist - for the selection of transfusion media in case of a positive indirect mantiglobulin test, transfusion failure, acute massive blood loss;
Urologist - infectious and inflammatory diseases of the urinary system;
phthisiatrician - suspicion of tuberculosis;
surgeon - surgical complications (infectious, hemorrhagic);
· maxillofacial surgeon - infectious and inflammatory diseases of the dento-jaw system.

Laboratory diagnostics

Laboratory research:

• General blood analysis: leukocytes, erythrocytes and platelets are counted. This analysis is one of the first in patients with suspected blood disease. This analysis can reveal the presence in the peripheral blood of at least 5.0x10/9 l of small, morphologically mature lymphocytes, the presence of which during differential diagnosis cannot be due to other diseases that occur with lymphocytosis. During the initial treatment at an early stage of the disease, the number of leukocytes can vary between 10-20x10 / l, the bulk (over 60%) of which are small lymphocytes with a small content of their transitional forms (lymphoblasts, prolymphocytes).
• Blood chemistry: there is an increase in LDH activity, hypogammaglobulinemia, signs of hemolysis.
• Morphological study: in a bone marrow aspirate, lymphocyte infiltration should be at least 30%.
• Immunophenotyping: lymphoid cells in CLL are mainly monoclonal and B-lymphocytes expressing both CD19, CD20, CD23 and CD5, while maintaining a low level of slg on the cell surface. T-cell antigen (eg, CD2, CD3) are absent.

Differential Diagnosis

differential diagnosis.
Using the phenotypic characteristics of cells in CLL, it is possible to make a differential diagnosis with other diseases that occur with an increased number of circulating atypical lymphocytes (plasma cell, prolymphocytic, hairy cell and variant hairy cell leukemia, as well as non-Hodgkin's lymphoma in the stage of leukemization).
· prolymphocytic leukemia. The morphological substrate is represented by cells with a large round nucleus and prominent nucleoli. In PPL, most peripheral blood mononuclear cells have the morphological characteristics of prolymphocytes; in PPL transformed from CLL, a polymorphic population of lymphocytes is present. The cells of PLL patients carry immunoglobulins that are different from the immunoglobulins of B-CLL. They may be CD5 and express the CD20 antigen. A high frequency of somatic mutation of the V(H) gene has been described in PLL.
· Hairy cell leukemia. Patients with HCL are characterized by the presence of cells with villous cytoplasm, thrombocytopenia (less than 100 x 109 /l), anemia, neutropenia (<0,5х 10/ 9). Ворсинчатые клетки имеют эксцентричное бобообразное ядро, характерные выросты цитоплазмы. Ворсинчатые клетки имеют В-клеточное происхождение, экспрессируют CD19, CD20 и моноцитарный антиген CD11с. Возможно, наиболее специфичным маркером для ворсинчатых клеток является антиген CD 103. Наличие мутации BRAFV600E при классической форме ВКЛ и ее отсутствие — при вариантной форме заболевания. В связи с этим в настоящее время выявление мутации BRAFV600E можно рассматривать как критерий диагностики типичной формы ВКЛ .
· Lymphoplasmacytic lymphoma. The tumor is represented by diffuse proliferation of small and plasmacytoid lymphocytes and plasma cells with a different number of immunoblasts. The volume of infiltration is usually less than in B-CLL and contains plasma and plasmacytoid cells in addition to small lymphocytes. Tumor cells have surface and cytoplasmic immunoglobulins, usually of the IgM class, less IgD, and necessarily express antigens that characterize B cells (CD 19, CB20, CD22, CD79a). CD5 cells are negative and do not contain CD10, CD23, CD43+ "~; CD25 or CDllc in some cases. The absence of CD5 and CD23, high levels of slg and CD20, the presence of cytoplasmic immunoglobulins are used for differential diagnosis with CLL. When combined with B-small cell infiltration of bone brain and IgM-monoclonal gammopathy with any concentration of monoclonal protein confirms the diagnosis of lymphoplasmacytic lymphoma.
· Lymphoma from marginal zone cells. Extranodal B-cell marginal zone lymphoma is defined as extranodal lymphoma of heterogeneous small B-lymphocytes containing cells (centrocyte-like) from the marginal zone, monocytoid cells, small lymphocytes in various ratios, scattered immunoblast-, centroblast-like and plasma cells (40%). Tumor cells express slg (IgM>IgG>IgA), to a lesser extent - IgD and from 40 to 60% cytoplasmic Ig, indicating plasmacytoid differentiation. The cells carry B cell antigens (CD19, CD20, CD22, CD79a) and are CD5 and CDlO negative. Immunophenotypic studies are usually performed to confirm the tumor and rule out B-CLL (CD5+), mantle zone lymphoma (CD5+), and follicle center lymphoma (CD1O, CD43, CD11c, and clg).
· Lymphoma from the cells of the mantle zone. Tumor-forming cells are composed of small to medium-sized lymphocytes, whose nuclei are irregularly shaped with a poorly visible nucleolus, and define a narrow rim of pale cytoplasm. Among the tumor cells, centroblasts or immunoblasts are detected. Tumor cells from the mantle zone are considered CD5, CD19, CD20, CD22, CD43 positive, carry surface immunoglobulins (slg+), but CD10 and CD23 are negative. In 50-82% of patients with lymphoma from the cells of the mantle zone, infiltration of the bone marrow by tumor cells is observed, which can be nodular, paratrabecular or interstitial in nature. Cytogenetic changes in tumor cells from the mantle zone are characterized by the presence of the t(ll;14)(ql3;q32) translocation.
· Follicular lymphoma. FL is composed of cells that are morphologically and immunophenotypically similar to normal germinal center cells and are one of the most common lymphoma variants. The histological picture of the lymph node is characterized by a nodular or follicular type of growth of tumor cells. The presence of diffuse infiltration of the lymph node worsens the prognosis of the disease.

Treatment abroad

Get treatment in Korea, Israel, Germany, USA

Treatment abroad

Get advice on medical tourism

Treatment

Treatment goals:
Achieving and maintaining remission.

Treatment tactics:

Non-drug treatment:
Mode: general protection.
Diet: neutropenic patients are advised not to follow a specific diet ( level of evidence B).

Medical treatment

Indications for starting treatment:

The presence of B-symptoms that worsen the quality of life;
Anemia and/or thrombocytopenia due to infiltration of the bone marrow with leukemia cells (advanced stage of the disease: C according to Binet, III-IV according to Rai);
Massive lymphadenopathy or splenomegaly causing compression problems
Doubling the absolute number of lymphocytes in the blood in less than 6 months (only in patients with lymphocytosis more than 30 × 109 / l);
Autoimmune hemolytic anemia or thrombocytopenia refractory to standard therapy.
Indications for initiation of therapy should be critically assessed.
In case of autoimmune complications (hemolytic anemia, thrombocytopenia), if there are no additional indications for the start of CLL therapy, treatment is carried out according to the protocols for the treatment of autoimmune hemolytic anemia and autoimmune thrombocytopenia.

Treatment of early stages of CLL without signs of progression (Binet stages A and B, Rai stages 0-II with symptoms, Rai stages III-IV).

Treatment of early stages of CLL does not improve survival. The standard tactic in the early stages is the "watch and wait" strategy. A follow-up clinical and laboratory examination with a mandatory study of a deployed UAC should be carried out every 3-6-12 months.

Treatment of advanced stages of CLL stage A and B according to Binet with signs of activity, stage C according to Binet; Rai stages 0-II with symptoms, Rai stages III-IV (level of evidence B).

In this group, patients have indications for chemotherapy. The choice of treatment depends on the somatic status of the patient and the presence of concomitant diseases.
In patients younger than 70 years of age without comorbidities, FCR (Fludarabine + Cyclophosphamide + Rituximab), BR (Bendamustine + Rituximab) are the first-line therapy. Pentostatin and cladribine can be used as first-line therapy in CLL, but the FCR combination is preferred. The use of Bendamustine as first-line therapy is a less toxic treatment option compared to FCR, more effective than Chlorambucil (median event-free survival 21.6 months vs 8.3 months; p<0,0001) и может быть рекомендовано при наличии противопоказаний к Флударабину.
In patients over 70 years of age and/or with severe comorbidities, Chlorambucil is the standard first-line therapy. Bendamustine, rituximab monotherapy, or reduced dose cycles of purine analogs may be the most common alternatives.

Drugs (active substances) used in the treatment

Groups of drugs according to ATC used in the treatment

Hospitalization

Indications for hospitalization:

Indications for emergency hospitalization:
infectious complications;
autoimmune hemolysis;
hemorrhagic syndrome.

Indications for planned hospitalization:
to verify the diagnosis

Prevention

Preventive actions: no.

Further management:
The effectiveness of consolidation or maintenance therapy in CLL has not been proven. Carrying out any maintenance therapy for CLL is possible only within the framework of clinical trials.

Information

Sources and literature

1. Minutes of the meetings of the Expert Council of the RCHD MHSD RK, 2015
   1. References: 1. Scottish Intercollegiate Guidelines Network (SIGN). SIGN 50: a guideline developer's handbook. Edinburgh: SIGN; 2014. (SIGN publication no. 50). . Available from URL: http://www.sign.ac.uk. 2. NCCN Clinical Practice Guidelines in Oncology: Non-Hodgkin's Lymphomas, 2014 (http://www.nccn.org). 3. Eichhorst B., HallekM., DreylingM. Chronic lymphocytic leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up Ann Oncol (2010) 21 (suppl 5): v162-v164 4. Parker A., ​​Bain B., Devereux S. et al. Best Practice in Lymphoma Diagnosis and Reporting, 2012. 5. Cheng MM , Goulart B, Veenstra DL, Blough DK, Devine EB A network meta-analysis of therapies for previously untreated chronic lymphocytic leukemia Cancer Treat Rev. 2012 Dec;38(8):1004-11. 7. Moody K, Finlay J, Mancuso C, Charlson M. Feasibility and safety of a pilot randomized trial of infection rate: neutropenic diet versus standard food safety guidelines J Pediatr Hematol Oncol 2006 Mar;28(3):126-33 8 Gardner A, Mattiuzzi G, Faderl S, Borthakur G, Garcia-Manero G, Pierce S, Brandt M, Estey E. Randomized comparison of cooked and noncooked diets in patients undergoing remission induction therapy for acute myeloid leukemia. J Clin Oncol. 2008 Dec 10;26(35):5684-8. 9. Carr SE, Halliday V. Investigating the use of the neutropenic diet: a survey of UK dietitians. J Hum Nutr Diet. 2014 Aug 28. 10. Boeckh M. Neutropenic diet--good practice or myth? Biol Blood Marrow Transplant. 2012 Sep;18(9):1318-9. 11. Trifilio, S., Helenowski, I., Giel, M. et al. Questioning the role of a neutropenic diet following hematopoetic stem cell transplantation. Biol Blood Marrow Transplant. 2012; 18:1387–1392. 12. DeMille, D., Deming, P., Lupinacci, P., and Jacobs, L.A. The effect of the neutropenic diet in the outpatient setting: a pilot study. Oncol Nurs Forum. 2006; 33:337–343. 13. Raul C. Ribeiro and Eduardo Rego Management of APL in Developing Countries: Epidemiology, Challenges and Opportunitiesfor International Collaboration Hematology 2006: 162-168. 14. Shanshal M, Haddad RY. Chronic lymphocytic leukemia Dis Mon. 2012 Apr;58(4):153-67. 15. NCCN Clinical Practice Guidelines in Oncology: Non-Hodgkin's Lymphomas, 2014 16. http://www.nccn.org/about/nhl.pdf 17. Bruton's tyrosine kinase inhibitors and their clinical potential in the treatment of B-cell malignancies: focus on ibrutinib. 18. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4212313/ 19. A Phase 3 Study of Ibrutinib (PCI-32765) Versus Ofatumumab in Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia (RESONATE™) https://clinicaltrials.gov/ct2/show/NCT01578707.

Information

List of protocol developers with qualification data:
1) Kemaikin Vadim Matveyevich - Candidate of Medical Sciences, JSC "National Scientific Center of Oncology and Transplantation", Head of the Department of Oncohematology and Bone Marrow Transplantation.
2) Klodzinsky Anton Anatolyevich - Candidate of Medical Sciences, JSC "National Scientific Center of Oncology and Transplantation", Hematologist, Department of Oncohematology and Bone Marrow Transplantation.
3) Ramazanova Raigul Mukhambetovna - doctor of medical sciences, professor of JSC "Kazakh Medical University of Continuing Education", head of the course of hematology.
4) Gabbasova Saule Telembaevna - RSE on REM "Kazakh Research Institute of Oncology and Radiology", head of the department of hematological malignancies.
5) Karakulov Roman Karakulovich - Doctor of Medical Sciences, Professor, Academician of the MAI RSE on REM "Kazakh Research Institute of Oncology and Radiology", Chief Researcher of the Department of Hemoblastoses.
6) Tabarov Adlet Berikbolovich - Head of the Innovation Management Department of the RSE on the REM "Hospital of the Medical Center Administration of the President of the Republic of Kazakhstan", clinical pharmacologist, pediatrician.

Indication of no conflict of interest: missing.

Reviewers:
1) Afanasiev Boris Vladimirovich - Doctor of Medical Sciences, Director of the Scientific Research Institute of Pediatric Oncology, Hematology and Transplantation named after R.M. Gorbacheva, Head of the Department of Hematology, Transfusiology and Transplantology of the State Budgetary General Educational Institution of Higher Professional Education of the First St. Petersburg State Medical University. I.P. Pavlova.
2) Rakhimbekova Gulnara Aibekovna - Doctor of Medical Sciences, Professor, JSC "National Scientific Medical Center", Head of Department.
3) Pivovarova Irina Alekseevna - Medicinae Doctor, Master of Business Administration, Chief Freelance Hematologist of the Ministry of Health and Social Development of the Republic of Kazakhstan.

Indication of the conditions for revising the protocol: revision of the protocol after 3 years and / or when new methods of diagnosis and / or treatment with a higher level of evidence appear.

Attached files

Attention!

• By self-medicating, you can cause irreparable harm to your health.
• The information posted on the MedElement website cannot and should not replace an in-person medical consultation. Be sure to contact medical facilities if you have any diseases or symptoms that bother you.
• The choice of drugs and their dosage should be discussed with a specialist. Only a doctor can prescribe the right medicine and its dosage, taking into account the disease and the condition of the patient's body.
• The MedElement website is an information and reference resource only. The information posted on this site should not be used to arbitrarily change the doctor's prescriptions.
• The editors of MedElement are not responsible for any damage to health or material damage resulting from the use of this site.

Oncological diseases are usually very difficult. Any manifestations of cancer have a complex negative impact on the body and human well-being. Tumor diseases of the blood can affect any organ of the human body. Chronic lymphocytic leukemia (CLL) occurs in the blood cells of lymphocytes and causes malignant lesions of the lymphatic tissue. Today there is no treatment that would guarantee the patient a complete recovery, but modern medicine has all available means to slow down the course of the disease and prolong life.

The reasons

The specificity of blood diseases indicates a certain pathological process that leads to a change and degeneration of cells. Chronic lymphocytic leukemia is caused by changes in white blood cells, lymphocytes. This disease in its acute form affects immature leukocyte cells, chronic forms destroy mature lymphocytes. Until now, medicine does not know the exact causes of this disease. Knowledge about the order of development and spread of the disease is based on medical observations and statistical studies.

Among the reasons that provoke the development of chronic lymphocytic leukemia, physicians call the following.

• hereditary factor. This is one of the main reasons that can be traced by examining the medical history of a particular family. If there have been cases of blood tumors in the past, this increases the likelihood of developing lymphocytic leukemia in future generations.
• Congenital diseases and pathologies. A number of medical studies have found that certain types of pathological conditions significantly increase the risk of developing cancer. Chronic lymphocytic leukemia is more likely to appear in a person suffering from Down syndrome, Wiskott-Aldrich, etc.
• The effect of viruses on the body. In the course of medical studies on animals, the negative effect of viruses on DNA and RNA was confirmed. Thus, this gives the right to assume that some severe viral diseases can provoke chronic lymphocytic leukemia. For example, the Epstein-Barr virus, which is also known as the herpes virus type 4.
• consequences of irradiation. At low doses of radiation, as a rule, the body does not receive significant damage. At the same time, with a serious influence of radiation, radiation therapy, there is a risk of blood diseases. About 10% of patients undergoing radiation therapy subsequently develop lymphocytic leukemia.

To date, scientists have not come to a consensus about the factors provoking the development of chronic lymphocytic leukemia. One of the main theories is the hereditary factor. However, studies have been conducted in which a clear relationship has not been established between genetic material and the likelihood of a blood tumor. Other researchers refute the influence of carcinogens and toxic substances. The causes of the development of the disease are visible in statistical observations, but require confirmation.

Symptoms of the disease

Before the start of diagnosis, any disease manifests itself with specific signs that worsen the state of human health. Chronic lymphocytic leukemia develops gradually. The symptoms of this type of cancer also develop slowly. The following signs of the disease are distinguished.

• General weakness and a feeling of fatigue that accompany a person throughout the day. This symptom is often confused with normal fatigue. Most often, a person really gets tired due to physical or nervous tension, but if the ailment lasts longer than a week, you should consult a doctor.
• Enlarged lymph nodes.
• Chronic lymphocytic leukemia causes severe sweating in a person, especially during a night's sleep.
• With the development of a blood tumor, an increase in the liver and spleen is observed. As a result, a person may feel soreness and a feeling of heaviness in the abdomen, more often on the left side.
• During physical exertion, even minor, shortness of breath is observed.
• In chronic lymphocytic leukemia, the symptoms are supplemented by loss of appetite.
• A blood test usually detects a decrease in the concentration of platelets in the patient's blood.
• Chronic lymphocytic leukemia leads to a decrease in neutrophils. This is due to changes in granulocyte cells in the blood, in particular those cells that mature in the bone marrow.
• Patients are often exposed to the manifestation of allergies.
• Chronic lymphocytic leukemia reduces the overall immune system of the body. A person begins to get sick more often, especially infectious and viral diseases (ARVI, influenza, etc.).

One or two of these symptoms are unlikely to indicate that the patient is developing leukemia or leukemia, but if a person has several forms of malaise, you should immediately consult a doctor. Only an examination by a qualified specialist and the subsequent delivery of the necessary tests can confirm or refute the development of the disease.

Diagnostics

Most tests and studies in the diagnosis of any disease begin with a general or clinical blood test. Chronic lymphocytic leukemia is no exception. The diagnostic process is not difficult for a qualified doctor. The main tests that the doctor can prescribe are the following.

• General blood analysis. This type of study of the autoimmune form of the disease is aimed at identifying the number of leukocytes and lymphocytes in the patient's blood. With an increase in the concentration of lymphocytic cells by more than 5×10 9 g/l, lymphoblastic leukemia is diagnosed.
• Biochemistry of blood. Biochemical research allows you to determine the deviations in the work of the body caused by a weakened immune system. According to the general indicators, the doctor can judge which organs were affected. At the initial stage of the development of lymphocytic leukemia, biochemistry does not reveal any violations.
• Myelogram. This is a special type of study for lymphocytic leukemia, which allows you to determine the replacement of red bone marrow cells with lymph tissue. In the initial stages of the disease, the concentration of lymphatic cells does not exceed 50%. With the development of cancer, the number of lymphocytes reaches 98%.
• Immunophenotyping. A specific study aimed at searching for oncological markers of lymphocytic leukemia.
• Biopsy of lymphatic tissue. This type of diagnosis is usually accompanied by a cytological examination, ultrasound, computed tomography and a number of other procedures. It is carried out to confirm the diagnosis, as well as to determine the stage of the disease and the degree of damage to the body.

Disease classification and prognosis

Today, in world medicine, two forms are used to reflect the severity of chronic lymphocytic leukemia. The first was developed by the American scientist Rai in 1975. Later, this methodology was supplemented and revised. The development of the disease according to Rai has 5 stages from 0 to IV. The initial form of lymphocytic leukemia is considered to be a zero mark, in which there are no symptoms, and the life of the patient belonging to the treatment exceeds a decade.

In Europe, as well as in domestic medicine, the division into stages developed by French scientists in 1981 is used. The scale is known as the Binet stages. For staging, a blood test is used that determines the patient's hemoglobin and platelet levels. Damage to the lymph nodes of the main zones is also taken into account: axillary and inguinal regions, neck, spleen, liver and head. Depending on the data obtained, lymphocytic leukemia is divided into 3 stages.

1. Stage A. In the human body, the disease affects less than 3 main zones. At the same time, the hemoglobin level is not lower than 100 g/l, and the platelet concentration exceeds 100×10 9 g/l. At this stage, doctors make the most optimistic prognosis for the patient, the life expectancy exceeds 10 years.
2. Stage B. The second stage of severity of lymphocytic leukemia is diagnosed when 3 or more major areas of the lymph nodes are affected. This state corresponds to blood parameters: hemoglobin more than 100 g/l, platelets more than 100×10 9 g/l. The prognosis for such a lesion of the body is on average about 6-7 years of life.
3. Stage C. The third most severe stage of the disease is characterized by blood tests with a hemoglobin content of less than 100 g/l, and platelets less than 100×10 9 g/l. This means that such damage to the body is almost irreversible. Any number of affected areas of the lymph nodes can be observed. On average, survival at this stage of the disease is about one and a half years.

Treatment

The development of modern medicine and science, supported by the technological equipment of medical institutions, gives doctors the opportunity to treat many diseases. However, treatment for chronic lymphocytic leukemia is supportive. This disease cannot be completely cured.

Every year, new means and methods of influencing the disease are developed.

At the initial stages of special exposure to medications is not required. Medicine knows many cases when the course of chronic lymphocytic leukemia is so slow that it does not cause inconvenience to a person. Therapy is prescribed for the progressive development of cancer. A significant increase in the concentration of lymphocytes in the blood, as well as a deterioration in the functioning of the spleen and liver, are an indication for the appointment of special drugs.

• For chronic lymphocytic leukemia, treatment is always complex. The most effective and common form of drug exposure includes intravenous fludarabine, intravenous cyclophosphamide, and ruthiximab. Depending on the individual characteristics of the patient, other drugs or combinations of drugs may be prescribed.
• In the absence of the effectiveness of drug treatment, as well as in the later stages of the disease, radiation therapy can be used. As a rule, at this stage, there is a significant increase in the lymph nodes and the penetration of lymphatic tissue into the nerve trunks, internal organs and human systems.
• If the spleen is severely enlarged, surgery may be performed to remove it. This method is considered insufficiently effective for combating chronic lymphocytic leukemia and increasing the number of lymphocytes. However, it is still used in medicine.

No matter how terrible the disease may seem, you should definitely resort to professional medical help. The active course of the disease without treatment of lymphocytic leukemia leads to damage to the body and death of the patient. At the same time, exposure to medication in 70% of cases leads to remission and prolongs life.

In contact with

For most people, a frightening diagnosis of chronic lymphocytic leukemia, with a long life expectancy, becomes a death sentence. Blood cancer is just a terrifying expression. Over the past 20 years, medicine has found a lot of ways to deal with the disease and stocked up in its arsenal a number of super-powerful drugs. The effectiveness of drugs helps to achieve conditional recovery and long-term remission, while drugs of the pharmacological group are completely canceled.

Causes of chronic lymphocytic leukemia

The defeat of leukocytes, bone marrow, peripheral blood with the involvement of lymphoid organs is called chronic lymphocytic leukemia.

How long do people with this disease live? Chronic lymphocytic leukemia (CLL) is an insidious but sluggish disease. The tumor includes exclusively mature lymphocytes. The disease has several features that affect the course of the disease, in particular, and life expectancy. More often, the disease occurs in elderly people and proceeds rather slowly over decades.

Scientists around the world believe that the causes of blood cancer lie in the human genetic background. The predisposition to the disease at the generic level takes on a pronounced character. It is generally accepted that children have a family predisposition to the disease is very high. It is important to note that the gene itself, which generates the development of the disease, has not been identified.

America and Western Europe are in first place in terms of the number of patients with blood cancer. Asia and Japan have few such patients. Such observations led to an unequivocal conclusion: the environment and its factors cannot become causative agents of the disease.

Chronic lymphocytic leukemia may be a consequence of the treatment of tumor diseases with ionizing radiation.

There are suggestions that the so-called changes in the chromosomes of the fetus (Down syndrome, etc.) can cause the development of the disease throughout life.

How to recognize the disease?

CLL is characterized by the following symptoms:

• the liver, spleen and peripheral nodes are markedly enlarged;
• erythrocytes are damaged;
• there is general muscle weakness and pain in the bones;
• increased sweating;
• skin rashes appear, an increased body temperature is noted;

• appetite decreases, a person loses weight dramatically and suffers from general weakness;
• traces of blood appear in the urine, bleeding occurs;
• new tumors are formed.

The disease does not have any special and distinctive symptoms. When the disease is actively progressing, the patient usually feels great.

Going to the doctor is associated with an infectious disease that the body's immune system cannot cope with.

As a rule, the manifestations of cancer are recognized in the analysis of blood, which contains a lot of abnormal white blood cells. As the disease progresses, the number of leukocytes slowly increases.

If the diagnosis of chronic lymphocytic leukemia is made at an early stage, therapy is not required. Everything is explained by the sluggish nature of the course of the disease, which does not affect the general well-being of a person. However, as soon as the disease enters the stage of intensive development, chemotherapy is inevitable.

Stages of development of the disease and methods of diagnosis

The stage of the disease is determined by blood counts and depends on the number of lymph nodes involved in the process of pathological changes:

1. Group A stage. It is not extensive and covers from 1 to 2 areas. Pathological changes in the number of lymphocytes in the peripheral blood are pronounced. A person can live with this stage for more than 15 years.
2. Group B stage. 4 areas are affected. Lymphocytosis is dangerous. Risks are assessed as medium. Survival of a person is not more than 10 years.
3. Group C stage. The entire lymphatic system is affected. The number of lymphocytes is many times higher than the norm. The level of platelets in the red bone marrow is significantly underestimated. There is anemia. The risks are high, a person lives no more than 4 years.

No matter how frightening the life expectancy figures may sound, infectious complications are the main cause of death in this disease.

To correctly diagnose, specialists use the following examinations:

1. The general research method is a general clinical blood test for the ratio of all types of white cells.
2. Characterization of cells using monoclonal antibodies is a diagnostic that allows you to accurately determine their type and functionality, which will make it possible to predict the further course of the disease.
3. Examination for the presence of tumor cells by bone marrow trepanobiopsy is a diagnostic manipulation that allows you to take a complete piece of tissue.
4. Research on the relationship between the hereditary factor and the structure of the cell, by microscopic examination.
5. Diagnosis of the gene background, genetic fingerprinting, PCR analysis of hepatitis C. Allows you to recognize the disease at an early stage and prescribe effective treatment.
6. Collection of blood and urine for analysis using an immunochemical study. Helps to determine the amount of leukocytes.

Future development of the disease and life expectancy

With a disease such as chronic lymphocytic leukemia, the prognosis for a full recovery is disappointing. It is considered normal when each cell of the human body has a specific structure that characterizes the area for which it functions. As soon as the cellular background undergoes pathological changes, a healthy cell turns into a cancerous structural unit of the human body. According to statistics, the field of oncology is in second place in terms of the number of deaths. However, chronic lymphocytic leukemia has minimal rates.

If a patient's lymphocytes have immunoglobulin antibody genes with pronounced resistance to healing, he can live long enough.

The median overall life expectancy in these patients reaches about 30 years. But patients with immunoglobulin genes who have not undergone a mutation live no more than 9 years.

The effectiveness of the fight against the disease depends entirely on the duration of the remission.

• Patients are advised to abandon any physical activity, reduce labor activity and adhere to a rest regimen.
• As for nutrition, the menu should contain a lot of animal protein, organic matter and vitamins.

• The diet of the patient should consist mainly of fresh vegetables and fruits.

During the treatment of the disease, signs of other complications in the body may appear. The immune system can self-destruct blood cells, and malignant neoplasms will begin to develop intensively. With any manifestation of such symptoms, it is important to immediately contact a specialist for the timely diagnosis of the disease and the appointment of the most effective treatment.

As it became clear, chronic lymphocytic leukemia is inherent in people of advanced age. According to long-term observations, the pattern of disease development is directly related to human aging. The longer a person has lived, the higher the risk of disease. The peak of the probability of the disease occurs at the age of 65 and mostly in men. The reason for the sexual separation is still unknown.

The chronic form is not subject to treatment, however, survival forecasts are made for several decades in 80% of cases. It is important that for all these years the disease may not reappear.

Properly selected treatment guarantees a stable course of the disease, which means that deterioration in well-being is definitely not expected.

Lymphocytic leukemia is a malignant lesion that occurs in the lymphatic tissue. It is characterized by the accumulation of tumor lymphocytes in the lymph nodes, in the peripheral blood and in the bone marrow. The acute form of lymphocytic leukemia has recently been classified as a “childhood” disease due to its exposure mainly to patients aged two to four years. Today, lymphocytic leukemia, the symptoms of which are characterized by their own specificity, is observed more often among adults.

general description

The specificity of malignant neoplasms as a whole is reduced to a pathology accompanied by the formation of cells, whose division occurs in an uncontrolled manner with the subsequent ability to invade (that is, to invade) the tissues adjacent to them. At the same time, they also have the possibility of metastasizing (or moving) to organs located at a certain distance from them. This pathology is directly related to both tissue proliferation and cell division that arose due to one or another type of genetic disorder.

With regard specifically to lymphocytic leukemia, as we have already noted, it is a malignant disease, while the growth of lymphoid tissue occurs in the lymph nodes, in the bone marrow, in the liver, in the spleen, and also in some other types of organs. Mostly the diagnosis of pathology is noted in the Caucasian race, and for every one hundred thousand people annually there are about three cases of the disease. Basically, the defeat of the disease occurs among the elderly, while the male sex is twice as likely to be affected by lymphocytic leukemia than the female. In addition, the predisposition to the disease is also determined by the influence of the hereditary factor.

The existing classification, which determines the course and specifics of the disease, distinguishes two forms of lymphocytic leukemia: acute (lymphoblastic) leukemia and chronic leukemia (lymphocytic leukemia).

Acute lymphocytic leukemia: symptoms

To diagnose this form of the disease, peripheral blood is used, in which characteristic blasts are found in about 98% of the total number of cases. A blood smear is characterized by a "leukemic dip" (or "gaping"), that is, there are only mature cells and blasts, there are no intermediate stages. The acute form of lymphocytic leukemia is characterized by normochromic anemia, as well as. Somewhat less common are other signs of an acute form of lymphocytic leukemia, namely leukopenia and leukocytosis.

In some cases, consideration of the overall blood picture in combination with symptoms suggests the relevance of acute lymphocytic leukemia, however, diagnostic accuracy is possible only when conducting a study that affects the bone marrow, in particular, to characterize its blasts histologically, cytogenetically and cytochemically.

The main symptoms of the acute form of leukocytosis are as follows:

• Complaints of patients on general malaise, weakness;
• Loss of appetite;
• Change (decrease) in weight;
• Unmotivated rise in temperature;
• Anemia, provoking pallor of the skin;
• Shortness of breath, cough (dry);
• Stomach ache;
• Nausea;
• Headache;
• The state of general intoxication in the widest variety of manifestations. Intoxication defines this type of condition in which there is a violation of the normal functioning of the body due to the penetration or formation of toxic substances in it. In other words, this is a general poisoning of the body, and depending on the degree of its damage against this background, the symptoms of intoxication are determined, which, as noted, can be very different: nausea and vomiting, headache, diarrhea, abdominal pain - a disorder of the gastrointestinal tract; symptoms of cardiac arrhythmia (arrhythmia, tachycardia, etc.); symptoms of dysfunction of the central nervous system (dizziness, depression, hallucinations, impaired visual acuity), etc. ;
• Pain in the spine and limbs;
• Irritability;
• An increase in the development of the disease of peripheral lymph nodes. In some cases - mediastinal lymph nodes. The mediastinal lymph nodes, in turn, are divided into 4 main groups: the lymph nodes of the upper mediastinum to the site of the bifurcation of the trachea; retrosternal lymph nodes (in the area behind the sternum); bifurcation lymph nodes (lymph nodes of the lower tracheobroncheal region); lymph nodes of the region of the lower posterior mediastinum .;
• About half of the total number of cases of the disease is characterized by the development of hemorrhagic syndrome with its characteristic hemorrhages - these are petechiae. Petechiae are a small type of hemorrhage, focusing mainly on the skin, in some cases on the mucous membranes, their sizes can be different, from a pinhead to the size of a pea;
• The formation of foci of extramedullary lesions in the central nervous system provokes the development of neuroleukemia;
• In rare cases, testicular infiltration occurs - such a lesion in which they increase in size, predominantly such an increase is unilateral (respectively, the leukemic nature of the occurrence is diagnosed in about 1-3% of cases).

Chronic lymphocytic leukemia: symptoms

In this case, we are talking about an oncological disease of the lymphatic tissue, for which a characteristic manifestation is the accumulation of tumor lymphocytes in the peripheral blood. When compared with the acute form of lymphocytic leukemia, it can be noted that the chronic form is characterized by a slower course. As for violations of hematopoiesis, they occur only at a late stage of the course of the disease.

Modern oncologists use several types of approaches that allow you to determine the accuracy of compliance with a specific stage of chronic lymphocytic leukemia. Life expectancy among patients suffering from this disease depends directly on two factors. In particular, these include the degree of disturbance in the bone marrow of the process of hematopoiesis and the degree of prevalence, which is characteristic of a malignant neoplasm. Chronic lymphocytic leukemia, in accordance with the general symptoms, is divided into the following stages:

• Initial stage (A). It is characterized by a slight increase in the area of ​​the lymph nodes of one or two groups. For a long time, the trend in blood leukocytosis does not increase. Patients remain under medical supervision, without the need for cytostatic therapy. Thrombocytopenia and anemia are absent.
• Expanded stage (B). In this case, leukocytosis takes on an increasing form, the lymph nodes increase on a progressive or generalized scale. Recurrent infections develop. For the advanced stage of the disease, appropriate active therapy is required. Thrombocytopenia and anemia are also absent.
• Terminal stage (C). This includes cases in which malignant transformation of the chronic form of leukocytosis occurs. Thrombocytopenia occurs and, regardless of the susceptibility to the defeat of a particular group of lymph nodes.

The letter designation is often displayed using Roman numerals, which also determine the specifics of the disease and the presence of certain of its signs in the patient in a particular case:

• I - in this case, the figure indicates the presence of lymphadenopathy (that is, an increase in lymph nodes);
• II - an indication of an increase in the size of the spleen;
• III - an indication of the presence of anemia;
• IV - an indication of the presence of thrombocytopenia.

Let us dwell in more detail on the main symptoms that characterize chronic lymphocytic leukemia. Here, the following manifestations become relevant, the development of which is gradual and slow:

• General weakness and malaise (asthenia);
• Feeling of heaviness that occurs in the abdomen (especially from the left hypochondrium);
• Sudden weight loss;
• Enlarged lymph nodes;
• Increased susceptibility to various types of infections;
• excessive sweating;
• Decreased appetite;
• Enlargement of the liver (hepatomegaly);
• Enlargement of the spleen (splenomegaly);
• Anemia;
• Thrombocytopenia (a symptom characterized by a decrease in the concentration of platelets in the blood below a certain norm);
• Neutropenia. In this case, we mean a symptom characterized by a decrease in the blood of neutrophilic granulocytes. Neutropenia, which in this case acts as a symptom of the underlying disease (lymphocytic leukemia itself), is a disease accompanied by a change (decrease) in the number of neutrophils (neutrophilic granulocytes) in the blood. Neutrophils in particular are blood cells that mature in the bone marrow within a period of two weeks. Due to these cells, the subsequent destruction of foreign agents that may be in the circulatory system occurs. Thus, against the background of a decrease in the number of neutrophils in the blood, our body becomes more susceptible to the development of certain infectious diseases. Similarly, this symptom is attached to lymphocytic leukemia;
• The occurrence of frequently manifested allergic reactions.

Chronic lymphocytic leukemia: forms of the disease

Morphological and clinical signs of the disease determine the detailed classification of chronic lymphocytic leukemia, which also indicates the appropriate response to the treatment being performed. The main forms of chronic lymphocytic leukemia include:

• Benign form;
• Classical (progressive) form;
• Tumor form;
• Splenomegalic form (with enlargement of the spleen);
• Bone marrow form;
• A form of chronic lymphocytic leukemia with a complication in the form of cytolysis;
• Prolymphocytic form;
• Leukemia hairy cell;
• T-cell form.

Benign form. It provokes a slow and noticeable increase only over the years in the blood of lymphocytosis, which is also accompanied by an increase in the number of leukocytes in it. It is noteworthy that in this form the disease can last for a considerable time, up to decades. The ability to work is not impaired. In most cases, when patients are under observation, sternal puncture and histological examination of the lymph nodes are not performed. These studies have a significant effect on the psyche, while neither they nor cytostatic medications may, due to similar features of the course of the disease, be needed at all until the end of the patient's life.

Classical (progressive) form. It begins by analogy with the form of the previous one, however, the number of leukocytes increases from month to month, and the growth of lymph nodes is also observed, which can be dough-like in consistency, slightly elastic and soft. The appointment of cytostatic therapy is carried out in case of a noticeable increase in the manifestations of the disease, as well as in the case of growth of lymph nodes and leukocytosis.

tumor form. Here, the peculiarity lies in the significant increase in the consistency and density of the lymph nodes, while leukocytosis is low. There is an increase in the tonsils almost to their closure with each other. The spleen enlarges to moderate levels, in some cases the increase can be significant, up to a protrusion within a few centimeters in the hypochondrium. Intoxication in this case has a mild character.

Bone form. It is characterized by rapidly progressive pancytopenia, partial or total replacement by mature lymphocytes in their diffusely growing bone marrow stage. There is no enlargement of the lymph nodes; in the vast majority of cases, the spleen is not enlarged, as is the liver. As for morphological changes, they are characterized by the homogeneity of the structure that nuclear chromatin acquires, in some cases pictonicity is observed in it, structural elements are rarely determined. It is noteworthy that earlier this form was fatal, with a life expectancy with the disease of up to 2 years.

Prolymphocytic form. The difference lies primarily in the morphology of lymphocytes. Clinical features are characterized by the rapid development of this form with a significant increase in the spleen, as well as with a moderate increase in peripheral lymph nodes.

Chronic lymphocytic leukemia with paraproteinemia. The clinical picture has the usual features of the forms listed above, accompanied by monoclonal gammopathy of the G- or M- type.

Hairy cell form. In this case, the name defines the structural features of lymphocytes, which represent the development of the process of chronic lymphocytic leukemia in this form. The clinical picture has characteristic features, which consist in cytopenia in one form or another (moderation / severity). The spleen is enlarged, the lymph nodes are of normal size. The course of the disease in this form is different, up to the complete absence of signs of progression for many years. There is granulocytopenia, in some cases provoking the occurrence of fatal complications of an infectious nature, as well as thrombocytopenia, characterized by the presence of a hemorrhagic syndrome.

T-shape. This form accounts for about 5% of cases. Infiltration affects mainly skin tissue and deep layers of the dermis. Blood is characterized by leukocytosis in varying degrees of severity, neutropenia, anemia occurs.

Lymphocytic leukemia: treatment of the disease

The peculiarity of the treatment of lymphocytic leukemia is that experts agree on the inappropriateness of its implementation at an early stage. This is due to the fact that most patients during the initial stages of the course of the disease carry it in a "smoldering" form. Accordingly, for a long time you can do without the need to take medications, as well as live without any restrictions, while being in a relatively good condition.

Therapy is performed for chronic lymphocytic leukemia, and only if there are grounds for this in the form of characteristic and striking manifestations of the disease. Thus, the expediency of treatment arises if there is a rapid increase in the number of lymphocytes, as well as with the progression of an increase in lymph nodes, a rapid and significant increase in the spleen, an increase in anemia and thrombocytopenia.

Treatment is also necessary if signs characteristic of tumor intoxication occur. They consist in increased sweating at night, in rapid weight loss, constant weakness and fever.

Today, it is actively used for treatment chemotherapy. Until recently, chlorbutin was used for procedures, but now the most effective treatment is achieved using purine analogues. The current solution is bioimmunotherapy, the method of which involves the use of antibodies of the monoclonal type. Their introduction provokes the selective destruction of tumor cells, while damage to healthy tissues does not occur.

In the absence of the desired effect in the use of these methods, the doctor prescribes high-dose chemotherapy, which involves the subsequent transplantation of hematopoietic stem cells. In the presence of a significant tumor mass in a patient, it is used radiation therapy, serving as adjuvant therapy in treatment.

A severe enlargement of the spleen may require the complete removal of this organ.

Diagnosis of the disease requires contact with specialists such as a general practitioner and a hematologist.

Chronic lymphocytic leukemia is a benign tumor consisting of mature atypical lymphocytes that accumulate not only in the blood, but also in the bone marrow and lymph nodes.

The disease belonging to the group of non-Hodgkin's lymphomas accounts for about a third of all leukemias. According to statistics, chronic lymphocytic leukemia is more common in men aged 50-70 years, young people rarely suffer from it.

Causes of chronic lymphocytic leukemia

At the moment, the true causes of the development of the disease are unknown. Scientists could not even prove the dependence of lymphocytic leukemia on aggressive environmental factors. The only confirmed point is hereditary predisposition.

Classification of chronic lymphocytic leukemia

Depending on the signs of the disease, examination data and the response of the human body to the therapy, the following variants of chronic lymphocytic leukemia are distinguished.

Chronic lymphocytic leukemia with a benign course

The most favorable form of the disease, the progression is very slow, can last for several years. The level of leukocytes increases slowly, the lymph nodes remain normal, and the patient maintains his usual lifestyle, work and activity.

Progressive chronic lymphocytic leukemia

A rapid increase in the level of leukocytes in the blood and an increase in lymph nodes. The prognosis of the disease in this form is unfavorable, complications and death can develop quite quickly.

Tumor form

A significant increase in lymph nodes is accompanied by a slight increase in the level of leukocytes in the blood. Lymph nodes, as a rule, do not cause pain when palpated, and only when they reach a large size can cause aesthetic discomfort.

Bone marrow form

The liver, spleen and lymph nodes remain unaffected, only changes in the blood are observed.

Chronic lymphocytic leukemia with enlarged spleen

For such leukemia, as the name implies, an enlarged spleen is characteristic.

Prelymphocytic form chronic lymphocytic leukemia

A distinctive feature of this form is the presence of lymphocytes containing nucleoli in blood and bone marrow smears, tissue samples of the spleen and lymph nodes.

Hairy cell leukemia

This form of the disease got its name due to the fact that tumor cells with “hairs” or “villi” are found under a microscope. Cytopenia is noted, that is, a decrease in the level of basic cells or blood cells, and an increase in the spleen. The lymph nodes remain unaffected.

T-cell form of chronic lymphocytic leukemia

One of the rare forms of the disease, prone to rapid progression.

Symptoms of chronic lymphocytic leukemia

The disease proceeds in three successive stages: the initial stage, the stage of advanced clinical manifestations and the terminal stage.

Symptoms of the initial stage

At this stage, the disease in most cases is latent, that is, asymptomatic. The number of leukocytes in the general blood test is close to normal, and the level of lymphocytes does not cross the 50% mark.

The first real sign of the disease is a persistent enlargement of the lymph nodes, liver and spleen.

The axillary and cervical lymph nodes are usually affected first, and nodes in the abdominal cavity and in the groin area are gradually involved.

Large lymph nodes, as a rule, are painless on palpation and do not cause severe discomfort, except for aesthetic (with large sizes). The enlarged liver and spleen can compress the internal organs, disrupting digestion, urination, and a number of other problems.

Symptoms of the stage of developed clinical manifestations

At this stage of chronic lymphocytic leukemia, fatigue and weakness, apathy and decreased ability to work can be observed. Patients complain of profuse night sweats, chills, a slight increase in body temperature and causeless weight loss.

The level of lymphocytes is steadily increasing and already reaches 80-90%, while the number of other blood cells remains unchanged, in some cases platelets decrease.

End stage symptoms

As a result of a progressive decrease in immunity, patients often suffer from colds, infections of the genitourinary system and pustules on the skin.

Severe inflammation of the lungs, accompanied by respiratory failure, generalized herpes infection, renal failure - this is not a complete list of complications caused by chronic lymphocytic leukemia.

As a rule, it is severe, multiple diseases that cause death in chronic lymphocytic leukemia. Other causes of death include malnutrition, severe kidney failure, and bleeding.

Complications of chronic lymphocytic leukemia

In the terminal stage of the disease, infiltration of the auditory nerve is observed, leading to hearing impairment and constant tinnitus, as well as damage to the meninges and nerves.

In some cases, chronic lymphocytic leukemia passes into another form - Richter's syndrome. The disease is characterized by rapid progression and the formation of pathological foci outside the lymphatic system.

Diagnosis of chronic lymphocytic leukemia

In 50% of cases, the disease is discovered by chance during a blood test. After that, the patient is referred for a consultation with a hematologist and a specialized examination.

As the disease progresses, a blood smear analysis becomes informative, in which the so-called “crushed leukocytes”, or Botkin-Gumprecht shadows (Botkin-Gumprecht bodies), are visualized.

A biopsy of the lymph nodes is also carried out, followed by cytology of the obtained material, and immunotyping of lymphocytes. The detection of pathological antigens CD5, CD19 and CD23 is considered a reliable sign of the disease.

The degree of enlargement of the liver and spleen on ultrasound helps the doctor determine the stage of development of chronic lymphocytic leukemia.

Treatment of chronic lymphocytic leukemia

Chronic lymphocytic leukemia is a systemic disease, and therefore radiation therapy is not used in its treatment. Drug therapy includes the use of several groups of drugs.

Hormones corticosteroids

Corticosteroids inhibit the development of lymphocytes, therefore, they can be involved in the complex therapy of chronic lymphocytic leukemia. But at present they are rarely used, due to the large number of serious complications that cast doubt on the appropriateness of their use.

Alkylating drugs

Among alkylating agents, Cyclophosphamide is the most popular in the treatment of chronic lymphocytic leukemia. It has shown good efficacy, but can also provoke serious complications. The use of the drug often leads to a sharp decrease in the level of red blood cells and platelets, which is fraught with severe anemia and bleeding.

Vinca alkaloid preparations

The main drug from this group is Vincristine, which blocks the division of cancer cells. The drug has a number of side effects, such as neuralgia, headaches, increased blood pressure, hallucinations, sleep disturbances and loss of sensitivity. In severe cases, muscle spasms or paralysis occur.

Anthracyclines

Anthracyclines are drugs with a dual mechanism of action. On the one hand, they destroy the DNA of cancer cells, causing their death. On the other hand, they form free radicals that do the same. Such active influence, as a rule, helps to achieve good results.

However, the use of drugs in this group often causes complications from the cardiovascular system in the form of rhythm disturbances, insufficiency, and even myocardial infarction.

Purine analogs

Purine analogues are antimetabolites, which, integrating into metabolic processes, disrupt their normal course.

In the case of cancer, they block the formation of DNA in tumor cells, and therefore inhibit the processes of growth and reproduction.

The most important advantage of this group of drugs is their relatively easy tolerability. The treatment usually gives a good effect, while the patient does not suffer from serious side effects.

Monoclonal antibodies

Drugs belonging to the group of "monoclonal antibodies" are currently considered the most effective means for the treatment of chronic lymphocytic leukemia.

The mechanism of their action is that when the antigen and antibody bind, the cell receives a signal to die and dies.

The only danger is the side effects, the most severe of which is a decrease in immunity. This creates a high risk of infections, up to generalized forms in the form of sepsis. Such treatment should be carried out only in specialized clinics where sterile rooms are equipped and the risk of infection is minimal. In such conditions, the patient is recommended to be not only directly during therapy, but also within two months after its completion.