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Chronic Lymphocytic Leukaemia
Overview
Chronic lymphocytic leukemia is a disease from the group of non-Hodgkin's lymphomas . Accompanied by an increase in the number of morphologically mature, but defective B-lymphocytes. Chronic lymphocytic leukemia is the most common form of hemoblastoses , accounting for one third of all leukemias diagnosed in the US and Europe. Men are affected more often than women. The peak incidence occurs at the age of 50-70 years, in this period about 70% of the total number of chronic lymphocytic leukemias are detected.
Young patients rarely suffer, up to 40 years the first symptom of the disease occurs in only 10% of patients. In recent years, experts have noted some "rejuvenation" of the pathology. The clinical course of chronic lymphocytic leukemia is very variable, ranging from a prolonged absence of progression to an extremely aggressive variant with a fatal outcome within 2-3 years after diagnosis. There are a number of factors that can predict the course of the disease. Treatment is carried out by specialists in the field of oncology and hematology.
Symptoms
At the initial stage, the pathology is asymptomatic and can only be detected by blood tests. Within a few months or years, a patient with chronic lymphocytic leukemia has 40-50% lymphocytosis. The number of leukocytes is close to the upper limit of the norm. In the normal state, peripheral and visceral lymph nodes are not enlarged. During the period of infectious diseases, the lymph nodes can temporarily increase, and after recovery they decrease again. The first sign of the progression of chronic lymphocytic leukemia is a stable increase in lymph nodes , often in combination with hepatomegaly and splenomegaly .
First, the cervical and axillary lymph nodes are affected, then the nodes in the mediastinum and abdominal cavity, then in the inguinal region. Palpation reveals mobile, painless, densely elastic formations that are not soldered to the skin and nearby tissues. The diameter of the nodes in chronic lymphocytic leukemia can range from 0.5 to 5 or more centimeters. Large peripheral lymph nodes may swell with a visible cosmetic defect. With a significant increase in the liver, spleen and visceral lymph nodes, compression of the internal organs can be observed, accompanied by various functional disorders.
Patients with chronic lymphocytic leukemia complain of weakness, unreasonable fatigue and decreased ability to work. According to blood tests, there is an increase in lymphocytosis up to 80-90%. The number of erythrocytes and platelets usually remains within the normal range, in some patients minor thrombocytopenia is detected . In the later stages of chronic lymphocytic leukemia, weight loss, night sweats and fever to subfebrile figures are noted. Immune disorders are characteristic. Patients often suffer from colds, cystitis and urethritis . There is a tendency to suppuration of wounds and the frequent formation of abscesses in the subcutaneous fatty tissue.
The cause of death in chronic lymphocytic leukemia is often severe infectious diseases. Possible inflammation of the lungs, accompanied by a collapse of the lung tissue and gross violations of ventilation. Some patients develop exudative pleurisy , which may be complicated by rupture or compression of the thoracic lymphatic duct. Another common manifestation of advanced chronic lymphocytic leukemia is herpes zoster , which in severe cases becomes generalized, capturing the entire surface of the skin, and sometimes the mucous membranes. Similar lesions can be seen in herpes and chicken pox .
Causes
The causes of occurrence have not been fully elucidated. Chronic lymphocytic leukemia is considered the only leukemia with an unconfirmed relationship between the development of the disease and adverse environmental factors (ionizing radiation, contact with carcinogens). Experts believe that the main factor contributing to the development of chronic lymphocytic leukemia is hereditary predisposition. Typical chromosomal mutations that cause damage to oncogenes at the initial stage of the disease have not yet been identified, but studies confirm the mutagenic nature of the disease.
Treatment
At the initial stages of chronic lymphocytic leukemia, expectant management is used. Patients are scheduled for examination every 3-6 months. In the absence of signs of progression, they are limited to observation. An indication for active treatment is an increase in the number of leukocytes by a factor of two or more within six months. The main treatment for chronic lymphocytic leukemia is chemotherapy . The combination of rituximab, cyclophosphamide, and fludarabine is usually the most effective drug combination.
In the persistent course of chronic lymphocytic leukemia, large doses of corticosteroids are prescribed, bone marrow transplantation is performed . In elderly patients with severe somatic pathology, the use of intensive chemotherapy and bone marrow transplantation may be difficult. In such cases, monochemotherapy with chlorambucil is carried out or this drug is used in combination with rituximab.
In chronic lymphocytic leukemia with autoimmune cytopenia, prednisolone is prescribed. Treatment is carried out until the patient's condition improves, while the duration of the course of therapy is at least 8-12 months. After a stable improvement in the patient's condition, treatment is stopped. The indication for resuming therapy is clinical and laboratory symptoms, indicating the progression of the disease.
Prognosis:
Chronic lymphocytic leukemia is considered as a practically incurable long-term disease with a relatively satisfactory prognosis. In 15% of cases, an aggressive course is observed with a rapid increase in leukocytosis and the progression of clinical symptoms. Lethal outcome in this form of chronic lymphocytic leukemia occurs within 2-3 years. In other cases, slow progression is noted, the average life expectancy from the moment of diagnosis ranges from 5 to 10 years. With a benign course, the life span can be several decades. After the course of treatment, improvement is observed in 40-70% of patients with chronic lymphocytic leukemia, but complete remissions are rarely detected.
Tests Required for Diagnosis
In half of the cases, the pathology is discovered by chance, during examination for other diseases or during a routine examination. When making a diagnosis, complaints, anamnesis, objective examination data, results of blood tests and immunophenotyping are taken into account. The diagnostic criterion for chronic lymphocytic leukemia is an increase in the number of leukocytes in the blood test up to 5×109/l in combination with characteristic changes in the immunophenotype of lymphocytes. Microscopic examination of a blood smear reveals small B-lymphocytes and Gumprecht shadows, possibly in combination with atypical or large lymphocytes. Immunophenotyping confirms the presence of cells with an aberrant immunophenotype and clonality.
The determination of the stage of chronic lymphocytic leukemia is carried out on the basis of the clinical manifestations of the disease and the results of an objective examination of the peripheral lymph nodes. To draw up a treatment plan and assess the prognosis for chronic lymphocytic leukemia, cytogenetic studies are carried out. If you suspect Richter's syndrome, a biopsy is prescribed. To determine the causes of cytopenia, a sternal puncture of the bone marrow is performed, followed by a microscopic examination of the punctate.
