OVERVIEW
Polycythemia vera is a special type of leukemia, which is prominently characterized by the presence of a large number of small villus-like projections on the surface of proliferating leukemia cells. It is a type of B-cell non-Hodgkin’s lymphoma, and patients are predominantly middle-aged and elderly.
Questions you may be concerned about
What is Hairy Cell Leukemia
Hairy cell leukemia belongs to a kind of B-cell non-Hodgkin’s lymphoma, and patients are mostly middle-aged and old, with a higher incidence in men than in women. The disease is characterized by proliferation of hair cells, increased circulating lymphocytes in the peripheral blood and infiltration of bone marrow with significant splenomegaly. Currently, the drugs of choice for this disease are nucleotide analogs.
The vast majority of patients with this disease are positive for the BRAFV600E mutation in tumor cells. Under the microscope, typical hairy cells are small lymphocytes slightly larger than normal lymphocytes, with oval, kidney-shaped or dumbbell-shaped nuclei, abundant cytoplasm and light blue-gray color, and finely distributed peripheral hairy protrusions on the cell membrane, and the cells strongly express CD19, CD103, CD25, CD22, and CD11.
The main clinical features of the disease are generally thrombocytopenia, marked splenomegaly but not lymph node enlargement, and specific cytoplasmic hairy “hirsute” B-lymphocytes in the peripheral blood and bone marrow, often accompanied by myelofibrosis.
In the mid-1980s, interferon replaced splenectomy as the mainstay of treatment for most patients with hirsute leukemia. In recent years, nucleotide analogs (e.g., cladribine) have begun to be used in clinical practice and have become the drugs of choice for the treatment of Hairy Cell Leukemia.
If the diagnosis of Hairy Cell Leukemia is confirmed, it is recommended to consult a doctor in time and standardize the treatment under the doctor’s guidance. The use of the above drugs should follow the doctor’s instructions, do not use without authorization, in order to avoid adverse consequences.
Etiology
The vast majority of patients are positive for BRAFV600E gene mutation in tumor cells, which is considered to be an important mechanism for the pathogenesis of this disease.
Symptoms
This disease has a chronic clinical course, and most of the patients are adults, with unexplained splenomegaly, especially huge spleen, no obvious lymph node enlargement, easy to secondary infection, accompanied by mild to moderate thrombocytopenia, bone marrow aspiration can be classified as “dry draw”, and globulin is increased in some cases.
Examination
1. Characteristic hairy cells can be found in blood film, bone marrow film, and puncture smear of spleen, liver and lymph nodes after Rachel’s staining.
2. Under the phase microscope, the polyhairy cells can be seen in the form of small villous protrusions with movable deformations.
3. Electron microscopy shows that the body of the polytrichous cells contains ribosomal platelet complexes in the form of tubular structure of inclusion bodies.
4. Cytochemical staining showed that the polychromatic cells were positive for acid phosphatase and were not inhibited by levulinic tartaric acid.
5. Flow cytometry is currently the main means of confirming the diagnosis. Polycythemia vera cells are almost exclusively CD19, CD20, CD123, HLA-DR, FMC7, CD200-positive B-cells with light chain restriction.
6. Genetic examination is an important means of confirming the diagnosis, the vast majority of patients are positive for BRAFV600E mutation by gene sequencing method; IGHV mutation detection can be seen in IgHV4-34 expression, etc.
Diagnosis
Diagnosis can be made on the basis of clinical manifestations and results of bone marrow imaging and electron microscopy.
Treatment
1. Splenectomy is the traditional treatment for this disease. With the application of interferon α and nucleoside analogs, the treatment of this disease has made great progress. At present, splenectomy is no longer the first choice of treatment for this disease, but splenectomy should still be chosen in cases of splenic rupture, lesions with predominantly splenomegaly, or significant thrombocytopenia.
2. Interferon alpha can lead to a significant decrease in the incidence of infection. The effect of treatment is fast, the hair cells in peripheral blood can be disappeared after a few weeks of treatment, and the platelet count, hemoglobin and neutrophil count can be normalized in 2 months, 4 months and 4~6 months respectively. The proportion of hair cells in the bone marrow may decrease significantly, but rarely disappears completely, and reticulocytes are still present. Therefore, it is not the treatment of choice.
3. Nucleoside analogs (e.g., cladribine) have been used in the treatment of this disease with good results, and may be close to a cure in some patients, and have now become the treatment of choice.
Prognosis
Life expectancy can be significantly prolonged with aggressive treatment.
Nursing care
Strengthen nutrition, moderate exercise, avoid crowded places, improve body resistance.