Leukemic Reticuloendotheliosis

Abstract

Leukemic reticuloendotheliosis (LRE) is a rare neoplastic disease of the hematopoietic system that is characterized clinically by a chronic course, splenomegaly in the absence of substantial lymphadenopathy, and pancytopenia. The distinctive morphologic feature of the disease is the presence of unique circulating mononuclear cells with many cytoplasmic projections, which have been referred to frequently as "hairy cells."^1-5 Most of the recent reports that have dealt with LRE have focused on the morphologic features and cytogenesis of the characteristic cells or have delineated their unique cytochemistry.^6-9 Splenectomy, while not curative, has been emphasized as the most beneficial treatment for those patients with massive splenomegaly and hypersplenism.^10-12 Published reports have tended to support the concept that chemotherapy is of little benefit in the treatment of LRE and may, in fact, worsen the clinical picture by the production of severe marrow suppression.^6,8,12 In this report, we present a patient