Splenic lymphoma presenting as warm autoimmune hemolytic anemia associated with pure red cell aplasia - demonstration of a humoral factor suppressing bone marrow erythropoiesis

A Zeidman MD, Z Fradin MD, Y Barac PhD, D Bendayan MD, M Mittelman MD, J Orlin MD .

Rabin Medical Center, Hasharon Hospital, Petah-Tikva, ISRAEL Israel

Background and Aims: Warm-autoimmune hemolytic anemia (AIHA) is a process in which peripheral red blood cell (RBC) destruction is induced by the presence of an autoantibody. Pure red cell aplasia (PRCA) represents an isolated process of decreased erythropoiesis. The combination of both is quite rare, with a very poor prognosis. We describe a patient with isolated splenic lymphoma whose presentation was a combination of AIHA and PRCA. The patient was resistant to all treatment. Materials and Methods: Erythroid colony assays were performed comparing the effect of the patient's serum on colonies with that of a normal control. A red cell eluate revealed the presence of a warm autoantibody. Results: The patient's serum significantly suppressed normal erythroid colony growth. Conclusions: The patient's serum contained warm autoantiantibody responsible for peripheral RBC destruction and a humoral factor, perhaps the warm autoantibody, which suppressed bone marrow erythropoiesis. Establishing an early diagnosis, and treatment of the underlying disease might result in a better prognosis. Summary: A rare presentation of isolated splenic lymphoma with AIHA and PRCA is reported. The erythroid colony assay data, the treatment options and the poor outcome of the patient is described.

KEY WORDS: Autoimmune hemolytic anemia (AIHA), Pure red cell aplasia (PRCA), Red blood cell (RBC), Bone marrow (BM).

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Paper presented at the International Symposium on Predictive Oncology and Intervention Strategies; Paris, France; February 9 - 12, 2002; in the section on Autoimmunity.