Abstract
Prolymphocyte leukemia is a very rare, complex, and often aggressive, mature lymphoid hemopathy. The mean age of onset is 65 years with a predominance of males. We report here the case of a patient with splenomegaly and severe lymphocytosis in whom the smear showed an invasion of 90% of prolymphocytes suggesting Prolymphocyte leukemia confirmed by immunophenotyping. Both purine analogues and monoclonal antibodies have shown promoting results, however, allogeneic hematopoietic stem cell transplantation remains the only therapeutic means allowing a lasting response. However, it is reserved for young patients with compatible donors. A good understanding of the pathogenesis and a better integration of the molecular data of this hemopathy open the way to the use of new targeted therapies.
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