Hairy cell leukemia is a chronic lymphoproliferative disorder characterized clinically by splenomegaly and cytopenias. Spontaneous remissions are rare and splenectomy is often performed when the blood counts worsen and cause symptoms. Three of our patients with hairy cell leukemia developed recurrent pancytopenia and transfusion-dependent anemia after splenectomy. Each subsequently acquired transfusion hepatitis and in two patients marked hematologic improvement was noted within 2 months. Complete peripheral blood remission occurred within 17 months in all patients although bone marrow infiltration with hairy cells persisted. One patient remains in remission for 12 years; the other two succumbed to infectious illnesses but with normal blood counts. The mechanism by which hepatitis virus induces hematologic recovery in patients with hairy cell leukemia is unknown but may involve augmentation of the interferon system.
A 50-year-old woman with typical acquired primary pure red cell aplasia (PRCA) was successfully treated with prednisone. A later relapse was preceded by a period of ineffective erythropoiesis characterized by a reticulocyte response inappropriately low for the degree of anemia, serum iron of 189 micrograms/dl, total iron-binding capacity (TIBC) of 213 micrograms/dl, and erythroid hyperplasia. In addition there was marked dyserythropoiesis and erythroblast-phagocytosis. One month later, bone marrow examination showed classic PRCA. This rarely reported evolutionary stage of PRCA has several implications: 1) it suggests antibody induced erythroblast cytotoxicity as one mechanism of PRCA; 2) at a particular time in the development of PRCA there is potential for misdiagnosis as primary refractory anemia (PRA); and 3) some cases of PRA with similar morphologic and laboratory findings may be pathogenetically related to PRCA and may benefit from evaluation for immune-mediated suppression of erythropoiesis.
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