Prognosis for hairy cell leukemia
One form of chronic leukemia, which is manifested by the proliferation of leukocytes, was isolated from the general group of leukemia in connection with its specific morphology. This pathology is calledhairy cell leukemia(ON). This name was obtained because of the special structure of lymphoid cancer cells, which have hair-like processes of the cytoplasm.
What is dangerous for this disease?
The disease is rare, 6 - 7 cases per 1 million people per year. The average age of detection of the disease is 50 years, but recently this trend has been decreasing.
The danger of hairy cell leukemia is that the disease develops slowly, as a benign tumor, without any manifestations for many years. Then there is a sharp progression with an increasing deficit of blood cells, which leads to the death of the patient, primarily from anemic, hemorrhagic and infectious complications.
The exact cause of the disease, like most leukemias, is not established. Pathological B-lymphocytes are produced in the bone marrow and gradually fill the blood.
The early signs of the development of the disease are nonspecific, so patients do not attach much importance to them, explaining their condition with general fatigue and aging of the body:
- weakness, permanent fatigue;
- shortness of breath with minor physical exertion;
- slight increase in temperature;
- poor appetite and weight loss;
- heaviness in the hypochondrium;
- weakened immunity and frequent viral infections.
In the later stages such characteristic symptoms appear:
- propensity to hemorrhages (hemorrhages in the organs, mucous membranes, skin) and bleeding;
- pronounced splenomegaly ─ enlarged spleen;
- lymphadenopathy ─ enlarged lymph nodes;
- cytopenia ─ lack of one or more types of blood cells;
- severe infectious complications;
- secondary tumors.
What is included in the diagnosis?
According to the clinical picture and blood indices, ON is similar to other lymphoproliferative diseases (diseases in which pathological cells are lymphoid in nature). This fact requires a thorough differential diagnosis:
- General blood analysis─ a low number of platelets, 50% less than 100 × 109 liters, low white blood cell count, although 25% cases of leukopenia is not fixed, a decrease in hemoglobin and hematocrit (the percentage of blood cells).
- Cytochemistry─ explores the dynamics of chemical processes in cells. TRPK (tartrate-resistant acid phosphatase) ─ this marker is used when monitoring cancer of the bone system ─ a positive reaction. Markers of specific esterase and myeloperoxidase are used to detect acute leukemia ─ a negative reaction.
- A feature of the disease is the difficulty in obtaining material from the bone marrow whentrephine biopsy. In 50% of cases, the result of taking biomaterial is negative, or the so-called "dry puncture". This is due to the high content of connective reticular fibers (connective tissue from collagen) in the red brain.
Treatment and Essential Drugs
The choice of therapy depends on the severity of the disease. If GPL is detected at a young age, then after treatment relapses occur more often.
Optimum safety therapy for any manifestation of hairy cell leukemia is a consistent application of Interferon-Alpha and Cladribine. In young patients, special treatment is added to maintain remission.
"Interferon-Alpha" is a pharmaceutical preparation of immunomodulators. It is widely used in oncology for complex therapy of cellular leukemia, melanoma, Kaposi's sarcoma, follicular leukemia. Treatment of ON with one of these drugs in 10% of cases leads to complete recovery, in 70% - to partial remission, which significantly improves the course of the disease and prognosis. The shortcomings of this method include the duration of therapy, low rates of complete remissions, the need for lifelong maintenance of the body with drugs.
The use of pharmaceuticals ─ analogue purine ─ led to drastic changes in the effectiveness of treatment. Chemotherapy drugs 2-deoxycoformin ("Pentotatin "DCF") have the effect of pronounced suppression of lymphocyte proliferation. Their use in 60% of cases led to complete remission, in 25% ─ partial, including in patients with ineffective other therapy.
The most common drug in the treatment of GIT is Cladribine. It has a cytotoxic effect on non-dividing and dividing cells. The lymphoid cells are most sensitive to the drug. The effectiveness of therapy is expressed in such indicators: complete remission up to 85%, partial - 12%.
Even with the absolute disappearance of clinical and hematological symptoms, one can almost always find a minimal clone of cancer cells in the bone marrow, in a quarter of cases they lead to relapses.
"Cladribine" and "Pentostatin" have hematologic toxicity, cause deep and prolonged neutropenia. This creates a risk for the development of severe and even fatal complications. Also, they have toxic effects on the liver, kidneys, nervous system, strongly inhibit blood formation in the bone marrow. To avoid infectious complications, they are prescribed "Interferon-Alpha" before their application.
Depending on the severity of the pathology, a splenectomy is performed ─ removal of the spleen, in conjunction with the therapy "Interferon-Alpha" and "Cladribine."
With adequate treatment, the prognosis is favorable. In 95% of cases, the survival rate is more than 10 years. In elderly people after 70 years, survival rates are good only in the case of early diagnosis.
Every fourth case of the disease is patients younger than 40 years.
Ifhairy cell leukemianot treat, then the average life expectancy will not exceed 5 years. The cause of mortality in 25% of cases are complications..