What happens in the body
A tumor clone of B-lymphocytes arises due to accumulated genetic damage in the precursor cell. Such lymphocytes hardly divide, but they also do not die in time, so their number is steadily growing. They fill the lymph nodes, spleen and bone marrow, gradually displacing normal hematopoiesis, hence anemia and a decrease in platelets in the later stages. At the same time, the immune system suffers: the production of protective antibodies drops, and the person begins to get sick often. Autoimmune complications often develop when the body's own immune system destroys red blood cells or platelets.
- Accumulation of defective B-lymphocytes
- Enlarged lymph nodes and spleen
- Displacement of normal hematopoiesis
- Decreased production of protective antibodies
- Autoimmune anemia and thrombocytopenia
- Slow course in most patients
Symptoms
In the early stages there are usually no complaints, and lymphocytosis turns out to be an incidental finding. Later, painless dense elastic lymph nodes appear in the neck, armpits and groin, symmetrical on both sides. A person notices heaviness in the left hypochondrium due to an enlarged spleen and rapid saturation. The so-called general symptoms are possible: causeless fever, night sweats, weight loss, severe fatigue. Characteristic symptoms include frequent and persistent respiratory tract infections, shingles, and unusually severe reactions to insect bites.
- Painlessly enlarged lymph nodes
- Heaviness in the left hypochondrium
- Fatigue and weakness
- Night sweats and low-grade fever
- Weight loss
- Frequent infections and shingles
- Pallor and bruising in later stages
Diagnostics
Suspicion arises when there is a persistent increase in lymphocytes that persists for several months. Blood immunophenotyping using flow cytometry confirms the diagnosis: it shows a characteristic set of tumor B-cell markers, and a bone marrow biopsy is usually not required. The blood smear shows fragile cells that are destroyed during preparation of the drug. Next, the prevalence is assessed by stage, ultrasound of the lymph nodes, spleen and liver is performed, the level of immunoglobulins, beta-2-microglobulin and a direct Coombs test are determined. Cytogenetic and molecular tests influence treatment decisions.
- General blood test with leukoformula over time
- Immunophenotyping by flow cytometry
- Ultrasound of lymph nodes, spleen and liver
- Immunoglobulin level
- Beta-2-microglobulin
- Direct Coombs test
- Cytogenetic and molecular studies
When to start treatment
Early initiation of therapy in asymptomatic cases does not prolong life, so many patients are offered observation with regular examinations and tests. Treatment begins when signs of active disease appear: increasing anemia or thrombocytopenia, rapid enlargement of the lymph nodes and spleen, severe general symptoms, rapid doubling of the number of lymphocytes, autoimmune complications that respond poorly to treatment. This tactic avoids unnecessary side effects. A high lymphocyte count in itself without other signs is not a reason for therapy.
- Decreased hemoglobin and platelets due to illness
- Rapid growth of lymph nodes and spleen
- Fever, sweating and weight loss
- Rapid doubling of lymphocytes
- Autoimmune complications
- Significant deterioration in health
Treatment and prevention of infections
Current treatment is based on targeted drugs that block the signaling pathways of tumor cells and monoclonal antibodies; classical chemotherapy is used less frequently and for specific indications. The regimen is selected by a hematologist taking into account age, concomitant diseases and molecular characteristics of the tumor. Protection against infections is no less important: routine vaccination against influenza, pneumococcus and, on the recommendation of a doctor, against other pathogens, early treatment at the first signs of infection, and in case of low antibody levels - immunoglobulin replacement therapy. Live vaccines are not used for this disease.
- Targeted drugs as the basis of modern therapy
- Monoclonal antibodies
- Chemotherapy for selected indications
- Vaccination against influenza and pneumococcus
- Immunoglobulin replacement therapy for low antibodies
- Early treatment of any infection
- Regular monitoring of tests during observation
