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May 1976

Leukemic Reticuloendotheliosis: Report of a Case With Prolonged Remission Following Intensive Chemotherapy

Author Affiliations

From the Oncology Center, the Johns Hopkins University School of Medicine and the Johns Hopkins Hospital, and the Division of Hematology, Department of Medicine, Baltimore City Hospitals, Baltimore, and the Division of Clinical Oncology, University of Wisconsin, University Hospital, Madison, Wis (Dr Davis).

Arch Intern Med. 1976;136(5):620-622. doi:10.1001/archinte.1976.03630050094016

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

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