Cyclosporin‐a for the treatment of pure red cell aplasia in a patient with chronic lymphocytic leukemia

Abstract

A 62-year-old man with B-cell chronic lymphocytic leukemia had three separate episodes of pure red cell aplasia (PRCA). The last episode was treated with cyclosporin-A (CyA) and prednisone. After the patient was on the therapy for 2 weeks, erythropoietic recovery was observed and with continued therapy the hematocrit (Hct) became normal. The PRCA remission was associated with a fall in the blood lymphocyte count, and a reduction in the spleen and lymph node size and bone marrow lymphocyte density. At diagnosis of PRCA the blood T-cells bearing IgG Fc receptors (T gamma cells) were increased, and the marrow contained very few or no late-stage erythroid progenitors. After remission of PRCA the T gamma cell fraction decreased, and the marrow erythroid progenitor's number became normal. We speculate that therapy with CyA and prednisone inhibited the production of interleukins-1 and -2 from monocytes and T-cells, respectively, and was responsible for the reduction of the T gamma cell fraction and B-cell leukemic mass in this patient. Further, we believe that normalization of T gamma cells in association with the therapy was responsible for the PRCA remission.

Cyclosporine and prednisone therapy for pure red cell aplasia in patients with chronic lymphocytic leukemia

“…Since then three other cases have been published showing induction of remission of their PRCA by CYA [6-81. We describe here results of six successfully treated episodes of PRCA (including our previously reported case [ 5 ] ) with CYA and prednisone in four B cell CLL patients. Results of therapy with CYA plus prednisone are compared with results of conven-0 1992 Wiley-Liss, Inc.…”

“…In several patients an abnormal T cell population was described to be responsible for the pathogenesis of PRCA [3,4]. We previously reported the successful induction of remission from PRCA by administration of immunosuppressive agents cyclosporine (CYA) and prednisone in a patient with CLL [5]. Since then three other cases have been published showing induction of remission of their PRCA by CYA [6-81.…”

Recovery of erythropoiesis following allogeneic bone marrow transplantation for chronic lymphocytic leukaemia-associated pure red cell aplasia

“…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.…”

Effect of Cyclosporin A in a Patient with Refractory Nephrotic Syndrome Associated with B Chronic Lymphocytic Leukemia

“…Only few reports deal with the action of CsA in the treatment of B-CLL [14][15][16][17], Of the 4 patients reported (2 with pure red cell aplasia, 1 refractory to conventional treatment, 1 with autoimmune thrombocytopenic purpu ra), all showed good responses with reduction of tumor mass [14][15][16] or control of disease progression [17], In vitro studies support the application of CsA in B-CLL demonstrating an effective reduction of tumor cells of the B cell phenotype [18], CsA is known to inhibit T cell acti vation at an early stage, leading to a transcription failure of early genes [19], such as those encoding interleukin-2 (IL-2) and other cytokines. IL-2 is necessary for T cell growth, induces proliferation and differentiation of B cells and enhances natural killer cell activity [20], The fact that the majority of B-CLL cells express the receptor for IL-2, release this cytokine and may respond to IL-2 expo sure with a proliferative signal [21] suggests that IL-2 plays a role in regulating the progression of B-CLL [22].…”