Remission Of Pure Red Cell Aplasia Research Articles

Antigen-Specific Immunoadsorption With the Glycosorb® ABO Immunoadsorption System as a Novel Treatment Modality in Pure Red Cell Aplasia Following Major and Bidirectional ABO-Incompatible Allogeneic Hematopoietic Stem Cell Transplantation.

Pure red cell aplasia (PRCA) after ABO-incompatible allogeneic hematopoietic stem cell transplantation (HSCT) is caused by persisting host-derived isohemagglutinins directed against donor red blood cell (RBC) antigens. ABO antigen-specific immunoadsorption (ABO-IA) with Glycosorb®, commonly used for desensitization therapy in ABO-incompatible living donor renal transplantation, specifically eliminates circulating isohemagglutinins and might represent a novel treatment option for post-HSCT PRCA. In this prospective observational (n = 3) and retrospective (n = 3) analysis of six adult HSCT-recipients with PRCA, ABO-IA was initiated at 159 (range: 104–186) days following HSCT. The median treatment frequency was 4.5 (range: 3.9–5.5) sessions/week. ABO-IA-treatment led to a continuous decrease in isohemagglutinin titers. Reticulocytes increased to ≥30 G/L after 17.5 (range: 4–37) immunoadsorption sessions over 28.5 (range: 6–49) days and continued to rise after that. By the end of the 3-month follow-up period after discontinuation of ABO-IA, all patients showed a sustained remission of PRCA and were independent of erythropoietin-stimulating agents and transfusions. No case of infection or graft-versus-host disease was observed. After a median follow-up of 22.03 (range: 6.08–149.00) months after ABO-IA-treatment, all patients were alive and showed a stable RBC engraftment of the donor blood group. Our data provide the first evidence for ABO-IA as an effective treatment for post-HSCT PRCA.

1901P Immunomodulatory role of multi-agent therapy in thymic epithelial tumours and severe immunological dysregulation

The association of different parathymic entities is common in thymic epithelial tumors (TETs), although only anecdotal case reports of TET patients exhibiting both pure red cell aplasia (PRCA) and Good Syndrome (GS) were described to date. We prospectively evaluated the outcomes of TET patients with PRCA and GS treated with combination therapy allowing prolonged control of TET, autoimmunity (PRCA) and immunodeficiency (GS). From 2013 consecutive TET patients, treated at Rare Tumors Reference Center of University Federico II, underwent diagnostic work-up for GS and PRCA, in the presence of suspicious symptoms. Subjects with GS who developed also PRCA were treated with a multi-agent therapy, including long acting octreotide 30 mg i.m. every 4 weeks, prednisone 0.6-1 mg/kg/daily (tapered when reaching Hb > 13 g/dl), cyclosporine escalation dose 50 to 200 mg/daily, darbepoietin 150 mcg weekly, intravenous immunoglobulin at 500 mg/kg per day for 5 days/monthly (tapered to 30 gr every 4 weeks), cotrimoxazolo antibiotic prophylaxis. Reticulocytes, serum immunoglobulin, C reactive protein (CRP), and Hb levels were closely monitored. Immunophenotype was assessed on whole blood by 8-colour immunophenotyping kit and Treg detection kit (CD4/CD25/CD127). TET Time to progression (TET-TTP), PRCA remission (PRCA-R), as well as occurrence of life treating infections, were assessed. 16 patients were included in this study. Median age was 44 years (range 33-65); male/female ratio was 1/1; the most frequent histotype was B2 thymoma (57%). Prolonged PRCA-R was observed in all but one patient, with median time to PRCA-R of 40 days. Correlation between increase of reticulocytes and circulating Treg was detected (p = 0.0003). At the time of the last follow-up, 15 patients were alive; 2 had PRCA recurrence after a median of 2 years from the initial diagnosis, one patient died for fatal medullary aplasia and 2 were hospitalized for staphylococcus pneumonitis. Median TET-TTP of 11 months was registered. This is the largest series of TET patients with PRCA and GS ever reported. The proposed multi-agent therapy achieved fast and prolonged PRCA-R, prolonged TET control and low rate of life treating infectious complications.

Thymoma associated with pure red cell aplasia: a case report and literature review.

Pure red cell aplasia (PRCA) is a rare hematological complication associated with thymoma. Remission of PRCA occurs after thymectomy but can reappear after a latency period even without recurrence of thymoma. We report a case and we have reviewed the literature of thymoma associated with PRCA. Because of less number of reported cases, there have been no clinical trials investigating the immunosuppression regimen following thymectomy for thymoma associated with PRCA. After reviewing the literature, we came to a conclusion that thymectomy followed by immumosuppression with the cyclosporin-containing regimen is currently the most effective treatment.

Pure red cell aplasia caused by ribavirin and interferon treatment.

Anemia is a common complication of hepatitisC (HCV), and antiviral treatment can further increase this risk. We present the case of a 59-year-old man with HCV treated with ribavirin and pegylated interferon alpha (INF-α) who presented with severe anemia. Two months after initiating treatment his hemoglobin dropped from 14.2 to 5.0g/dL. There was no evidence of bleeding or hemolysis, and a bone marrow biopsy revealed pure red cell aplasia (PRCA). Evaluations for acute cytomegalovirus and parvovirus B19 were negative. There was no evidence of malignancy or thymoma. The INF-α and ribavirin treatment were determined to have caused the PRCA, and withdrawal of the medications led to PRCA remission. INF-α and ribavirin have become the standard treatment for HCV. While these medications offer a potential cure, they are often poorly tolerated due to frequent side effects including anemia. Patients who are receiving treatment with ribavirin and INF-α warrant close monitoring for development of anemia, and PRCA should be considered in all patients in whom reticulocytopenic anemia develops.

Thymoma With Pure Red Cell Aplasia and Good's Syndrome

Thymoma patients with pure red cell aplasia (PRCA) or hypogammaglobulinemia (Good's syndrome) are rare, whereas those with both PRCA and Good's syndrome are even rarer. Here we present the case report of a 70-year-old woman with invasive thymoma and simultaneous PRCA and Good's syndrome, who achieved complete PRCA remission after thymectomy.

Antiepoetin antibody-related pure red cell aplasia: successful remission with cessation of recombinant erythropoietin alone

An elderly patient with pure red cell aplasia (PRCA) with antierythropoietin (anti-EPO) antibodies is described. PRCA due to alloimmunization is a rare and severe complication of recombinant human erythropoietin (rHu-EPO) therapy. Most reported patients with PRCA were cured primarily by immunosuppressive drug therapy. The patient in this case, however, did not want to receive any immunosuppressive drugs. Therefore, rHu-EPO injection was simply discontinued, the severe anemia gradually improved, and the hemoglobin approached normal range. This case is very rare and significant in that there have been few such elderly patients with rHu-EPO-induced PRCA in whom PRCA remission was achieved, with decreasing antibody titers, after cessation of rHu-EPO alone. Further cases are needed to assess how PRCA should be treated in patients with anti-EPO antibodies.

Thymectomy Resulted in Complete Remission of Pure Red Cell Aplasia When Associated with Thymoma

Patients with thymoma associated with pure red cell aplasia (PRCA) are rare and the relevant literature is limited. Prognostic evaluations into the appropriate surgical management of this rare coincidence of diseases are controversial, with some authors believing that none of their cases experienced remission of anemia after thymectomy. Despite the extreme rare concomitance of thymoma and PRCA, we report three consecutive cases, who experienced long-term complete remission of PRCA after extended thymectomy as their only therapy over a 6-year period. Possible features leading to the favorable outcome are discussed.

Successful Treatment of Pure Red Cell Aplasia after Major ABO-Incompatible Allogeneic Hematopoietic Stem Cell Transplantation for Acute Myeloid Leukemia with Repeated Doses of the Single-Agent Rituximab: A Case Report.

Background: ABO incompatibility occurs in up to 20–40% of HLA matched allogeneic hematopoietic stem cell transplantation (HSCT). Pure red cell aplasia (PRCA) following ABO-incompatible allogeneic HSCT is a rare complication associated with interaction of recipient anti-A or anti-B isoagglutinins, produced by residual B cells, with donor erythrocytes precursors expressing A and/or B antigens. The best treatment approach for PRCA is not established. Plasma exchange and immunoadsorption are regarded as a first-line treatment strategies. Other therapeutic approaches include: erythropoietn (EPO), donor-derived leukocyte infusion (DLI), steroids or induction of graft versus host disease (GVHD) by immunosuppression withdraw. Moreover, recovery or erythropoiesis is often incomplete and may induce futher complications. Recent reports describe successful treatment of PRCA and others cytopenias after HSCT using Rituximab (Mabthera®; Roche; Switzerland). This drug is a chimeric IgG1 monoclonal antibody directed against the CD20 surface antigen expressed by most human B lymphocytes. Its exact mechanism of action is not known.Case Report: We treat a 47-year-old woman with PRCA after a major ABO-mismatched (Donor A Rh-; Recipient O Rh-) allogeneic HSCT after a second remission of a acute myeloid leukemia. Allogeneic HSCT was performed in November 2005, from a HLA-matched older sister, using bone marrow graft (Mn cell dose 1,72x108/kg) following a myeloablative conditioning with busulfan (16mg/kg) and melfalan (140mg/m2). GVHD prophylaxis included cyclosporine A and methotrexate. Baseline anti-A isoagglutinin IgM titers of the paient before transplantation was 1:128 and after immunoadsorption with plasma A for 48 hours decreased to 1:32. One plasmapheresis session was performed to remove ABO antibodies from the patient and her anti-A IgM titer dropped to 1:4 before bone marrow infusion. We also removed red blood cells from the donor's marrow product to prevent risk of hemolysis. After bone marrow transplantation, the patient developed a severe anemia that required weekly red blood cells transfusion over six months. Reticulocyte counts were extremely low during this period, bone marrow aspirate was normal, except for decreased erythrocyte precursors (only 1% of total cells). In August 2006, the diagnosis of PRCA was set after exclusion of red blood cells alloantibodies, hemolysis and viral or bacterial infection. Donor chimerism (STR) was totally completed and the recipient anti-A IgM titer was 1:16 and the patient showed no evidence of GVHD. In October 2006, she was treated with rituximab 375mg/m2 administered once weekly for four weeks. In the next two months, after the treatment with rituximab, her hemoglobin (Hb) increased from 5.5g/dl to 12g/dl. Up to her last medical avaluation (July 2006), she was in PRCA remission, her Hb was 12g/dl and she had no detectable anti-A titer. Conclusion: Rituximab can be a very effective treatment to PRCA secondary to major ABO-incompatible allogeneic stem cell transplantation. The best dose schedule and long term effects must be establish.

Thymoma Associated with Pure Red Cell Aplasia

We report a 55-year-old female who presented with concurrent thymoma and pure red cell aplasia (PRCA). The initial presentation was typical of severe anemia. Differential diagnoses were based on reticulocyte counts in the peripheral blood, an anterior mediastinal mass on chest roentgenography and a CT scan of the mediastinum. No erythroblasts in bone marrow aspiration were found and PRCA was diagnosed. The patient underwent extended thymectomy which led to a rapid postoperative remission of PRCA. Thymoma associated with PRCA is very rare. The literature was reviewed to document the etiology, adequate therapy, and prognosis. ( Mid Taiwan J Med 2004;9:119-23 )

Durable remission of pure red cell aplasia after treatment with high‐dose intravenous gammaglobulin and prednisone

Pure red cell aplasia (PRCA) is a rare disorder of erythrocyte production believed to have an autoimmune basis in most cases. Responses to a variety of immunosuppressive regimens are regularly observed, but toxicity is considerable. Brief responses to high-dose intravenous immunoglobulin (IVIg) have been previously reported. This case demonstrates a durable clinical remission of PRCA treated with IVIg and glucocorticoids.

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

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.