Hairy cell leukemia is a mature B cell neoplasm. It is usually classified as a sub-type of chronic lymphoid leukemia for convenience. It is uncommon, representing about 2% of all leukemias, or less than a total of 2000 new cases diagnosed each year in North America and Western Europe combined.
Originally known as histiocytic leukemia, malignant reticulosis, or lymphoid myelofibrosis in publications dating back to the 1920s, this disease was formally named leukemic reticuloendotheliosis and its characterization significantly advanced by Bertha Bouroncle, M.D. and her colleagues at the Ohio State University College of Medicine in 1958. Its common name, which was coined in 1966, is derived from the appearance of the cells under a microscope.
In hairy cell leukemia, the broken "hairy cells" build up in the bone marrow, which means that the bone marrow has difficulty producing enough normal cells: white blood cells to fight infections, red blood cells to carry oxygen, and platelets to stop bleeding. Consequently, patients usually present with infection, anemia-related fatigue, and/or easy bleeding.
Most symptoms are often vague, such as "persistent fatigue" or "not feeling well." Some of the leukemic cells may gather in the spleen and cause it to swell; this can have the side effect of making the person feel full even when he or she has not eaten much.
Hairy cell leukemia is commonly diagnosed after a routine blood count shows unexpectedly low numbers for one or more kinds of blood cells, or after unexplained bruises or unexplained infections, such as repeated bouts of pneumonia in an otherwise apparently healthy patient.
Platelet function may be somewhat impaired in HCL patients, although this does not appear to have any significant practical effect. It may result in somewhat more mild bruises than would otherwise be expected for a given platelet count or a mildly increased bleeding time for a minor cut. It is likely the result of producing slightly abnormal platelets in the overstressed bone marrow tissue.
Patients with a high tumor burden may also have somewhat reduced levels of cholesterol, especially in patients with an enlarged spleen. Cholesterol levels return to more normal values with successful treatment of HCL.
The cause is unknown, but it is generally accepted that it is not caused by tobacco, ionizing radiation, or industrial chemicals other than possibly diesel. Farming and gardening appear to increase the risk in some studies. The possibility that HCL is caused by a random accident during routine cell division can not be ruled out.