Summary:Pure red cell aplasia is a rare condition, that can be either idiopathic or associated with a lymphoproliferative disorder. The latter is considered to result from T cell-mediated suppression of haematopoiesis, and usually responds well to treatment with immunosuppressive medication. We describe a patient with B-CLL-associated pure red cell aplasia who did not respond to several courses of immunosuppressive treatment. Erythropoiesis was finally restored after allogeneic bone marrow transplantation. Bone Marrow Transplantation (2001) 27, 771-773. Keywords: pure red cell aplasia; allo-BMT; CLL First described by Kazsnelson, pure red cell aplasia (PRCA) is a rare haematopoietic disorder characterised by selective aplasia of the erythroid cell line in the bone marrow.1,2 The syndrome can be either congenital (Diamond-Blackfan anaemia) or acquired. Most cases of acquired PRCA are idiopathic, but a large number of associated illnesses have been described, most notably thymomas and lymphoid malignancies. 3 The association of PRCA with B cell chronic lymphocytic leukaemia (CLL) is well known, and appears to be based on the presence of T␥ suppressor cells. 4,5 Several studies report good results using immunosuppressive medication, in particular cyclosporin A, for the treatment of CLL-associated PRCA. 6,7 We describe a patient with B-CLL-associated PRCA, who did not respond to several courses of immunosuppressive treatment. Erythropoiesis was finally restored after T cell-depleted allogeneic bone marrow transplantation.
Case reportA 43-year-old Caucasian man was first diagnosed with PRCA in 1997, when he presented with signs and symp- /l, as were levels of vitamin B12 and folic acid. There was no serologic evidence of recent CMV or EBV infection, serologic and DNA studies for parvovirus B19 infection were negative and diagnostic imaging studies showed no evidence of thymoma. Bone marrow examination was remarkable for the near absence of mature erythroid precursors with paratrabecular clusters of mature lymphocytes on biopsy. Immunophenotyping showed a monoclonal B cell population in blood and bone marrow (CD5 ϩ , CD10 Ϫ , CD19 ϩ , CD20 ϩ , CD23 ϩ , weak expression of surface Ig), comprising approximately 35% of the lymphocyte population. Southern blot analysis of IgH genes confirmed the presence of a monoclonal B cell population, whereas no clonal T cell receptor gene rearrangement was detected. Cytogenetic analysis revealed a normal XY karyotype. In vitro colony forming assays showed normal BFU-E and CFU-GM from bone marrow nucleated cells as well as from T cell-depleted bone marrow mononuclear cells. Peripheral blood erythropoietin levels were elevated, but no formal studies were done to exclude antibodies to erythropoietin or erythropoietin receptors. A diagnosis of B-CLL, RAI stage 0 with accompanying PRCA was made.Initial treatment with chlorambucil and prednisone followed by treatment with cyclosporin A and prednisone had no effect. Evaluation 1 year after diagnosis showed persistent B-CLL (approximately 30%...