Hairy cell leukemia is a rare form of chronic leukemia. This blood disorder affects adults and manifests by fatigue, pale complexion, increased spleen and liver. It is accompanied by a general weakening of the immune system (monocytopenia). The disease affects more men than women, and occurs most often in middle age individuals or older adults. Average age of onset is 55 years.
Hairy cell leukemia is an uncommon form of leukemia which is characterized by deficiency of monocytesand the presence of abnormal B -cells in bone marrow, spleen and peripheral blood cells. This is a slowly progressive chronic lymphocytic leukemia. Its name comes from the shape of abnormal lymphocytes, which have hair-like extensions. Hairy cell leukemia represents 2% of all cases of leukemia and its annual incidence has been estimated at 1 out 500, 000.
Hairy Cell Leukemia Symptoms and Complications
Hairy cell leukemia Symptoms are linked to the abnormal production of blood cells: low production of red blood cells causes anemia, low white blood cell production leads to an increased risk of infections, and low platelet count is subject to bleeding or bruising. Abdominal discomfort is often due to hepatosplenomegaly [simultaneous enlargement of both the liver (hepatomegaly) and the spleen (splenomegaly)]. Indeed,enlargement of the spleen is present in almost all cases; it is in more than 80% of cases; while the hepatomegaly with mild hepatic dysfunction is encountered in 20% of cases, and lymphadenopathy are found in 10 % of cases. Complications of Hairy cell leukemia include recurrent infections, bleeding, anemia and splenic rupture.
Hairy Cell Leukemia Causes and Diagnostics
The real causes of Hairy cell leukemia are still unknown. It is, however, theorized that family history of certain malignancies (Non-cancerous), Ashkenazi Jews (also known as Ashkenazic Jews or simply Ashkenazim), personal history of certain cancers, prolonged or unprotected exposure to radiation, chemicals or sawdust are risk factors that lead to the disease.
Diagnosis is based on the results of the physical examination, and often includes blood tests, bone marrow biopsy and CT.
Hairy Cell Leukemia Treatment
In general, Hairy cell leukemia is easily controlled by health professionals. However treatment given to the patient depends on the characteristic of the symptoms, low blood cell counts and more frequent infections, or any sign that indicates that the tumor is worsening.
Hairy cell leukemia can be treated by chemotherapy drugs: 2-chlorodeoxyadenosine (cladribine),Pentostatin (Nipent®); or biotherapy: interferon alpha, Rituximab (Rituxan). A complete or partial remission is obtained with chemotherapy in approximately 90% of patients. Splenectomy may be useful in patients with difficulty to control infections. In some cases, the disease is mild enough and changes slowly enough so that patients do not need treatment and remain stable for several years. Most patients live ten or more years with the disease.
Chemotherapy – Chemotherapy consists of using anti-cancer (cytotoxic) drugs to treat malignant tumors (cancer). It is usually a systemic treatment that circulates throughout the body and destroys cancer cells, including those that could escape from the primary tumor. Some of the chemo agents used to treat Hairy cell leukemia include:
- 2-chlorodeoxyadenosine (cladribine)
Biological Therapy – this therapy Is designed to strengthen or restore the body’s immune response to work as it should. It is commonly used in Hairy cell leukemia treatment. It is also called biotherapy or biological response modifiers (BRMs). Biological therapy uses natural or synthetic substances to destroy or control cancer cells or to change their behavior. Different types of biological agents act differently.
Biological agents most commonly administered to treat a progressive Hairy cell leukemia include:
- alpha interferon ( Intron A Injection, Roferon A Injection)
- rituximab (Rituxan ).
Splenectomy – For most people with Hairy cell leukemia, surgically removing the spleen (splenectomy) will allow the number of blood cells to partially or completely back to normal. However, given splenectomy produce little or no change in the bone marrow, the disease becomes progressive in almost all cases. However, this surgical procedure is rarely used to treat the disease due to effectiveness of the chemotherapeutic and biological agents.
Splenectomy is usually recommended in cases of the following factors:
- uncontrolled infection
- pain from an enlarged spleen that is uncontrollable by the other therapeutic methods
- abnormal number of obvious blood cells
- refractory disease