Resolution of pure red cell aplasia and lymphoma: Response to intravenous gammaglobulin and combination chemotherapy

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Abstract
Pure red cell aplasia (PRCA) rarely occurs in nonthymic lymphoproliferative disorders. This report describes the clinical course and therapy of a patient with a history of Sjogren syndrome who developed well‐differentiated lymphocytic lymphoma associated with PRCA and severe hemolytic anemia. Life‐threatening hemolytic anemia combined with the presence of multiple antibodies and lack of erythroid precursors was treated successfully with a single dose of intravenous gammaglobulin. A sustained, complete remission and normalization of the bone marrow was achieved following six courses of an aggressive chemotherapy regimen. Thus, occasionally low‐grade lymphomas can produce life‐threatening complications, requiring a more aggressive therapeutic intervention than those routinely applied.