Leukemic reticuloendotheliosis (hairy cell leukemia)

Abstract

Leukemic reticuloendotheliosis (hairy cell leukemia) is a well-established independent clinical and pathologic entity. This study will report the clinical, hematologic, and histologic findings in 82 patients who have been followed for over 20 yr. The diagnosis must be confirmed by the finding in the peripheral blood and/or bone marrow of the pathognomonic mononuclear cell of leukemic reticuloendotheliosis, the so-called hairy cell, which is best characterized by the use of supravital stain, phase-contrast microscopy, slide-cultures, and electron microscopy. The most common physical finding is splenomegaly, which was present in 93% of our patients. The course of the disease is, in general, chronic. The most frequent complication is infection. Spontaneous remissions can occur in leukemic reticuloendotheliosis. Transition to other forms of leukemia or lymphoma has not been recorded. Splenic irradiation is usually followed by temporary improvement. Chemotherapy is not indicated initially. However, in patients who have life-threatening disease with a predominance of hairy cells in the peripheral blood, marked thrombocytopenia, neutropenia, and anemia due to extensive infiltration of the marrow by the malignant cells, aggressive chemotherapy should be initiated. Splenectomy is indicated in cases of frank hypersplenism, and the results, in the majority of patients, have been excellent and long-lasting.