Refractory Anemia With Dysproteinemia: Long-term Therapy With Low-Dose Corticosteroids

Abstract

Abstract Nine elderly patients with a refractory anemia associated with a high blood sedimentation rate and with minor serum protein abnormalities, especially elevation of the alpha2 globulin fraction, have been studied. The anemia was characterized by red cell hypochromia, reduced serum iron, and normal or reduced iron binding capacity concentrations, elevated erythrocyte protoporphyrin levels, and normoblastic erythropoiesis with reticuloendothelial iron present on Prussian-blue staining of bone-marrow preparations; no evidence of hemolysis was elicited by radiochromium red cell survival measurements, and plasma clearance and red cell utilization of intravenously injected radioiron were normal. The mechanism of the anemia was thus compatible with a block in reticuloendothelial release of iron derived from hemoglobing catabolism. Five patients complained of musculoskeletal pains and could thus be classified as polymyalgia rheumatica. Four patients had no pain and anemia dominated the clinical presentation. Carcinoma, myelomatosis, lymphoid neoplasm, leukemia, chronic infection, renal and hepatic failure, and auto-immune disease were excluded by investigation, and prolonged follow-up for 3-11 years has failed to reveal underlying disease. All patients responded clinically and hematologically to prednisolone, with a rapid increase in general well-being, a fall in sedimentation rate, and a return of the hemoglobin level to normal; remission has been maintained for 3-11 yr on minimal steroid dosage. Steroid dependence was illustrated in two cases, where withdrawal of therapy resulted in a rapid relapse of symptoms, a rise in sedimentation rate, and a return of the anemia. While five patients had clinical manifestations suggestive of polymyalgia rheumatica, four had no pain. In view of the excellent therapeutic response to steroids, the recognition of this pain-free group is important.