Splenic Lymphoma Presenting as Warm Autoimmune Hemolytic Anemia Associated with Pure Red Cell Aplasia

Abstract

Background and Objective: Warm autoimmune hemolytic anemia (AIHA) is a condition 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, in order to compare the effect of the patient's serum on colonies with that of a normal control. Results: The patient's serum significantly suppressed normal erythroid colony growth. A red cell eluate revealed the presence of a warm autoantibody. Conclusions: The patient's serum contained warm autoantibody 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.

Autoimmune haemolytic anaemia associated with mantle cell lymphoma

“…A number of cases in the literature have reported co-existence of AIHA and red cell aplasia within the same patient, usually in association with systemic autoimmune disease (e.g. systemic lupus erythematosus and Sjögrens syndrome) [7,8] or non-Hodgkin lymphoma [9,10]. In most cases, this dual phenomenon is thought to be due to the presence of autoantibodies directed against erythroid progenitors as well as mature erythrocytes, although one case report identified an anti-erythropoietin autoantibody in the patient's serum with in vitro and in vivo evidence that this was the primary cause of the anaemia [7].…”

Successful treatment of pure red cell aplasia and autoimmune cytopenia with cyclosporine and prednisolone in a patient with Sj�gren?s syndrome

“…The combination of AIHA and PRCA has rarely been reported. 12,13 Ziedman et al 12 reported that a patient's serum had suppressed normal erythroid colony growth, which was considered to be a warm autoantibody-mediated mechanism. On the other hand, Tohda et al 13 reported that the patient's serum did not suppress normal colony formation, while the patient's mononuclear cells did suppressed it.…”