Hairy cell leukemia

Hairy cell leukemia: Description, Causes and Risk Factors: Abbreviation: HCL. ICD-10: C91.4 Hairy cell leukemiaHairy cell leukemia is an uncommon but distinct form of chronic lymphoproliferative disorder characterized by an indolent course. Peripheral cytopenias and splenomegaly due to presence of characteristics neoplastic B lymphocytes in the blood, bone marrow, and organs of the classic reticuloendothelial system associated with reticulin-fibrosis of the marrow. Hairy cell leukaemia is rare. It occurs mostly in people aged 40-60 and is more common in men than in women. HCL usually develops very slowly, so the term 'chronic' is used to describe it. In hairy cell leukaemia, the abnormal white blood cells also build up in the spleen and cause it to grow bigger. An enlarged spleen may remove normal blood cells from the bloodstream. This can also reduce the number of red cells and normal white cells in the blood. The causes of hairy cell leukaemia are unknown. Hairy cell leukaemia is not infectious and cannot be passed on to other people. Doctors know that cancer occurs when cells develop errors in their DNA. In the case of hairy cell leukemia, mutations in the DNA cause your bone marrow stem cells to create too many white blood cells that do not work properly. Risk Factors: Exposure to radiation: People exposed to radiation, such as those who work around X-ray machines or those who received radiation treatment for cancer, may have a higher risk of developing hairy cell leukemia.
  • Exposure to chemicals: Industrial and agricultural chemicals could play a role in hairy cell leukemia development. However, some studies have found this not to be the case.
  • Exposure to sawdust: Some studies have found a link between working with wood and sawdust and an increased risk of hairy cell leukemia. But this connection has not been proven conclusively.
Symptoms: Because HCL usually develops slowly, it may not cause any symptoms for a long time. It is sometimes discovered by chance when a blood test is taken for another reason, such as part of a routine health check. Hairy cell leukemia can cause symptoms, such as weakness, weight loss, frequent infections and breathlessness.Some people see their doctor because they feel tired or look pale due to a lack of red blood cells (anemia). Some people get repeated infections because they have a shortage of healthy white blood cells that normally fight off infections. People who have an enlarged spleen may feel a tender lump on the left side of their abdomen. These symptoms can be caused by other conditions, but should always be checked by your doctor. Diagnosis: The diagnosis is based upon recognition of the characteristics hairy nature of the leukemic lymphoid cells in the peripheral blood smears and the typical appearance and pattern of infiltration in the bone marrow biopsies in associations with increased reticulin and eventual fibrosis of the marrow. Additional special features include presence of tartrate-resistant acid phosphatase (TRAP) positivity within the cells associated with a specific HCL immunophenotypic profile on flow cytometry and elevated levels of soluble interleukin 2 receptors in the peripheral circulation, which are all important features in establishing the diagnosis. Tests may include: Blood tests.
  • Bone marrow biopsy —removal of a sample of bone marrow tissue to test for cancer cells.
  • CT scan —a type of x-ray that uses a computer to make pictures of the spleen and the liver.
Treatment: Hairy cell leukemia (HCL) tends to be slow growing. Patients without symptoms often do not need to be treated right away, but they do need to have careful follow-up exams. These are done every few months to check for disease progression and appearance of symptoms. Some patients with HCL live for many years without having any symptoms or receiving any treatment. Treatment may be advised for HCL patients with low blood cell counts, recurrent infections, or an enlarged spleen or lymph nodes. Treatment is most often with one of the purine analog drugs -- either cladribine (2-CdA) or pentostatin. Up to 80% to 90% of patients respond to these drugs, and the responses last more than 5 years in most patients. Even if HCL recurs, many cases will respond to a second treatment with these drugs. Giving rituximab after these drugs may get rid of any remaining disease in people who have not fully responded. Because this is a fairly rare disease, too few people have been treated with rituximab to know if it will make a long-term difference. In rare cases, HCL may not respond to chemotherapy. Rituximab or interferon-alfa, a type of biologic therapy, may be helpful. If a patient is uncomfortable because of an enlarged spleen, removing the spleen by surgery (splenectomy) can often help relieve pain or other symptoms. Surgery: Surgery to remove your spleen (splenectomy) might be an option if your spleen ruptures or if it is enlarged and causing pain. Though removing your spleen cannot cure hairy cell leukemia, it can usually restore normal blood counts. Splenectomy is not commonly used to treat hairy cell leukemia, but it may be helpful in certain situations. All surgery carries a risk of bleeding and infection. NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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