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Hairy Cell Leukemia

Overview :

Hairy cell leukemia (HCL) is a rare cancer. It was first described in 1958 as leukemic reticuloendotheliosis, erroneously referring to a red blood cell because researchers were unsure of the cell of origin. It became more easily identifiable in the 1970s. There are approximately 600 new cases diagnosed every year in the United States, making up about 2% of the adult cases of leukemia each year.

HCL is found in cells located in the blood. There are three types of cells found in the blood: the red blood cells that carry oxygen to all the parts of the body; the white blood cells that are responsible for fighting infection and protecting the body from diseases; and the platelets that help in the clotting of blood. Hairy cell leukemia affects a type of white blood cell called the lymphocyte. Lymphocytes are made in the bone marrow, spleen, lymph nodes, and other organs. It specifically affects B-lymphocytes, which mature in the bone marrow. However, extremely rare variants of HCL have been discovered developing from T-lymphocytes, which mature in the thymus.

When hairy cell leukemia develops, the white blood cells become abnormal both in the way they appear (by acquiring hairy projections) and in the way they act (by proliferating without the normal control mechanisms). Further, the cells tend to accumulate in the spleen, causing it to become enlarged. The cells may also collect in the bone marrow and prevent it from producing normal blood cells. As a result, there may not be enough normal white blood cells in the blood to fight infection.

The median age at which people develop HCL is 52 years. Though it occurs in all ages, HCL more commonly develops in the older population. Men are four times more likely to develop HCL than women. There have been reports of familial aggregation of disease, with higher occurrences in Ashkenazi Jewish men. A potential genetic link is undergoing further investigation.

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