leukemic reticuloendotheliosis


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reticuloendotheliosis

 [rĕ-tik″u-lo-en″do-the″le-o´sis]
hyperplasia of reticuloendothelial tissue.
leukemic reticuloendotheliosis hairy cell leukemia.
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