hairy cell leukaemia

A low-grade B-cell leukaemia comprising 2% of adult leukaemia, commonly affecting men—male:female ratio, 4:1—age 50-55, leading to progressive pancytopenia; 10% have platelet counts of < 20 x 109/L—US: < 20,000/mm3—20% have thrombocytosis; 1-80% of nucleated RBCs are hairy.
The ‘classic’ or Japanese form—which comprises most cases of HCL—responds better to therapy than the non-classic or variant form
Lab Increased acid phosphatase, especially isoenzyme 5—increased in bone metastases and in Gaucher’s disease
Management First-line therapy: purine analogues 2’-deoxycoformycin (pentostatin) and 2-chlorodeoxyadenosine (cladribine)
Cause of death Infections, gram-negative bacteria, atypical mycobacteria, fungi

hairy cell leukaemia

A rare disease featuring overgrowth of B .LYMPHOCYTES usually in men between 40 and 60 years of age. The lymphocytes show prominent hair-like projections. There is a profound drop in the numbers of all blood cells and the spleen, which is the site of the bulk of the abnormal lymphocytes, is enlarged. Treatment is by spleen removal (splenectomy) and the use of drugs such as alpha-INTERFERON and deoxycoformycin.

单词	hairy cell leukaemia
释义	EncyclopediaSeeleukemic reticuloendotheliosis hairy cell leukaemia A low-grade B-cell leukaemia comprising 2% of adult leukaemia, commonly affecting men—male:female ratio, 4:1—age 50-55, leading to progressive pancytopenia; 10% have platelet counts of < 20 x 109/L—US: < 20,000/mm3—20% have thrombocytosis; 1-80% of nucleated RBCs are hairy. The ‘classic’ or Japanese form—which comprises most cases of HCL—responds better to therapy than the non-classic or variant form Lab Increased acid phosphatase, especially isoenzyme 5—increased in bone metastases and in Gaucher’s disease Management First-line therapy: purine analogues 2’-deoxycoformycin (pentostatin) and 2-chlorodeoxyadenosine (cladribine) Cause of death Infections, gram-negative bacteria, atypical mycobacteria, fungi hairy cell leukaemia A rare disease featuring overgrowth of B .LYMPHOCYTES usually in men between 40 and 60 years of age. The lymphocytes show prominent hair-like projections. There is a profound drop in the numbers of all blood cells and the spleen, which is the site of the bulk of the abnormal lymphocytes, is enlarged. Treatment is by spleen removal (splenectomy) and the use of drugs such as alpha-INTERFERON and deoxycoformycin.
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