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

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.