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hyperplasia of reticuloendothelial tissue.
leukemic reticuloendotheliosis hairy cell leukemia.
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