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Pure Red-Cell Aplasia

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Suppression of erythropoiesis with little or no abnormality of leukocyte or platelet production.

Also Known As:

Red-Cell Aplasia, Pure; Aplasia, Erythrocyte; Aplasia, Pure Red-Cell; Aplasias, Erythrocyte; Aplasias, Pure Red-Cell; Erythrocyte Aplasias; Pure Red Cell Aplasia; Pure Red-Cell Aplasias; Red Cell Aplasia, Pure; Red-Cell Aplasias, Pure; Erythrocyte Aplasia

Networked: 770 relevant articles (62 outcomes, 16 trials/studies)

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Disease Context: Research Results

Hemic and Lymphatic Diseases
- Hematologic Diseases: 1962
  - Anemia: 22925
    - Pure Red-Cell Aplasia: 770
      - Diamond-Blackfan Anemia: 260

Related Diseases

1.	Thymoma (Thymic Carcinoma)
2.	Autoimmune Hemolytic Anemia (Cold Agglutinin Disease)
3.	Infection
4.	Aplastic Anemia (Anemia, Hypoplastic)
5.	Anemia

Experts

1.	Casadevall, Nicole: 25 articles (03/2015 - 01/2002)
2.	Schellekens, Huub: 12 articles (05/2014 - 01/2003)
3.	Macdougall, Iain C: 11 articles (03/2015 - 11/2003)
4.	Eckardt, Kai-Uwe: 8 articles (11/2009 - 09/2002)
5.	Locatelli, Francesco: 7 articles (03/2015 - 07/2003)
6.	Rossert, Jerome: 7 articles (11/2009 - 02/2004)
7.	Praditpornsilpa, Kearkiat: 5 articles (05/2014 - 03/2005)
8.	Bennett, Charles L: 5 articles (08/2008 - 09/2004)
9.	Hirokawa, Makoto: 4 articles (03/2015 - 08/2007)
10.	Eiam-Ong, Somchai: 4 articles (05/2014 - 03/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pure Red-Cell Aplasia:

1.	rituximab (Mabthera)FDA Link 10/01/2013 - "Pure red cell aplasia associated with CD20+ myeloma: complete remission with rituximab." 11/01/2015 - "Rituximab: A viable treatment option for epoetin-induced pure red cell aplasia." 03/01/2009 - "We report four patients (mean age 65 yr; range 40-77 yr) affected by acquired pure red cell aplasia (PRCA) complicating chronic lymphoid disorders and treated with anti-CD20 monoclonal antibody rituximab. " 11/19/2005 - "[Pure red cell aplasia associated with chronic lymphocytic leukaemic successfully treated with rituximab]." 09/01/2002 - "Successful response to rituximab in a patient with pure red cell aplasia complicating chronic lymphocytic leukaemia."
2.	Prednisone (Sone)FDA LinkGeneric 11/01/2005 - "Successful reintroduction of a different erythropoiesis-stimulating agent after pure red cell aplasia: relapse after successful therapy with prednisone." 02/01/1988 - "A 50-year-old woman with typical acquired primary pure red cell aplasia (PRCA) was successfully treated with prednisone. " 07/01/1982 - "Acquired pure red cell aplasia: case report of a patient responding to prednisone." 10/01/1989 - "Durable remission of pure red cell aplasia after treatment with high-dose intravenous gammaglobulin and prednisone." 11/01/1993 - "A patient with pure red cell aplasia associated with hepatitis A showed a dramatic and rapid recovery after initiation of oral prednisone therapy. "
3.	Erythropoietin FDA Link 11/01/2010 - "Improvement in erythropoieis-stimulating agent-induced pure red-cell aplasia by introduction of darbepoetin-α when the anti-erythropoietin antibody titer declines spontaneously." 09/01/1991 - "Steroid-resistant acquired pure red cell aplasia: a partial remission induced by recombinant human erythropoietin." 03/01/2015 - "Incidence of erythropoietin antibody-mediated pure red cell aplasia: the Prospective Immunogenicity Surveillance Registry (PRIMS)." 09/01/2014 - "Antibody-mediated pure red cell aplasia (PRCA) is a rare complication of erythropoietin (EPO) therapy. " 05/01/2014 - "The high prevalence of pure red cell aplasia in Thailand has been associated with the sharp increase in number of recombinant human erythropoietin (rhEPO) copy products, based on a classical generic regulatory pathway, which have entered the market. "
4.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 11/01/2013 - "Response of pure red cell aplasia to cyclophosphamide after failure of mycofenolate mofetil in a patient with polyglandular syndrome type I." 05/01/1997 - "Oral cyclophosphamide monotherapy can successfully treat the pure red cell aplasia associated with T-cell-lineage GLPD." 05/01/1997 - "Cyclophosphamide therapy for pure red cell aplasia associated with granular lymphocyte-proliferative disorders." 12/01/1986 - "Action of cyclophosphamide on the pure red cell aplasia suggested the immune origin of this disease. " 04/01/1984 - "Is antilymphocytic globulin a better immunosuppressant than cyclophosphamide for pure red cell aplasia?"
5.	Cyclosporine (Ciclosporin)FDA LinkGeneric 10/15/2001 - "Anecdotal reports suggest that cyclosporin A (CsA) may be beneficial for patients with CLL-associated pure red cell aplasia. " 03/01/1995 - "The efficacy of cyclosporine for patients with pure red-cell aplasia was 60.0% at 16 weeks treatment. " 06/01/2015 - "She was diagnosed with pure red cell aplasia (PRCA) by bone marrow examination and was successfully treated with cyclosporin A (CsA). " 03/01/2015 - "Cyclosporine dependent pure red cell aplasia: a case presentation." 12/01/2012 - "The pharmacokinetic analysis of cyclosporine is essential for the management of refractory pure red cell aplasia."
6.	Antilymphocyte Serum (Anti-Thymocyte Globulin)IBA 07/01/1993 - "Pure red cell aplasia--antilymphocyte globulin-mediated remission." 01/01/1984 - "Successful treatment of chronic refractory pure red cell aplasia with antithymocyte globulin: correlation with in vitro erythroid culture studies." 11/01/2001 - "Clonality of acquired primary pure red cell aplasia: effectiveness of antithymocyte globulin." 11/01/2001 - "[Successful treatment of idiopathic pure red cell aplasia with antithymocyte globulin]." 12/01/1986 - "Pure red cell aplasia: response to therapy with anti-thymocyte globulin."
7.	alemtuzumab (Campath)FDA Link 10/01/2005 - "Alemtuzumab induced complete remission of therapy-resistant pure red cell aplasia." 12/01/2013 - "Low-dose subcutaneous alemtuzumab is a safe and effective treatment for pure red cell aplasia and further trials should be conducted to compare the long-term effectiveness of this treatment with conventional therapy. " 12/01/2013 - "Low-dose subcutaneous alemtuzumab is a safe and effective treatment for chronic acquired pure red cell aplasia." 07/01/2007 - "Campath-1H induced pure red cell aplasia in a patient with chronic lymphatic leukaemia." 05/01/2003 - "Treatment of pure red cell aplasia and autoimmune haemolytic anaemia in chronic lymphocytic leukaemia with Campath-1H."
8.	Immunoglobulins (Immunoglobulin)IBA 10/01/2009 - "The findings of a bone marrow biopsy suggested pure red cell aplasia (PRCA), and the serum levels of all immunoglobulins were extremely low. " 11/01/1994 - "Chronic idiopathic pure red cell aplasia: successful treatment during pregnancy and durable response to intravenous immunoglobulin." 04/01/1993 - "Idiopathic pure red-cell aplasia was successfully treated by high-dose intravenous immunoglobulin infusions (HDivIg) in 2 patients; 1 of these patients was refractory to other immunosuppressive drugs. " 07/01/1992 - "Pure red cell aplasia: treatment with intravenous immunoglobulin concentrate." 10/01/1991 - "A B cell line from a patient with pure red cell aplasia produces an immunoglobulin that suppresses erythropoiesis."
9.	AntibodiesIBA 05/01/2012 - "Neutralizing anti-EPO antibodies can cause pure red cell aplasia (PRCA). " 10/01/2010 - "An elderly patient with pure red cell aplasia (PRCA) with antierythropoietin (anti-EPO) antibodies is described. " 01/01/2010 - "[Pure red cell aplasia (PRCA) induced by anti-EPO antibodies: epidemiology, diagnosis and treatment]." 11/01/2007 - "The diagnosis of classical erythropoiesis-stimulating agent induced pure red cell aplasia is made by a constellation of clinical features, including severe transfusion-dependent anaemia, reticulocytopenia, low or absent erythroblasts in the bone marrow, and the presence of circulating antierythropoietin antibodies. " 10/01/2006 - "Although in many cases the presence of antibodies may have little clinical consequence, the upsurge of pure red cell aplasia cases further increased concerns about potential clinical consequences of extensive use of biopharmaceuticals and biosimilars. "
10.	Epoetin Alfa (Epogen)FDA Link 11/01/2007 - "During the period of 1998 to 2002, there was an increase in the incidence of antibody-positive pure red cell aplasia (PRCA) in patients receiving subcutaneous administration of EPREX (epoetinum alfa). " 01/01/2007 - "Lesson learned from Eprex-associated pure red cell aplasia." 06/01/2006 - "Eprex-associated pure red cell aplasia and leachates." 06/01/2005 - "An upsurge in the incidence of pure red cell aplasia (PRCA) was linked to the epoetin-alpha product Eprex/Erypo in Europe, and an increase in PRCA cases of the same magnitude was not seen in patients taking other epoetin products s.c. " 06/01/2005 - "The increased incidence of pure red cell aplasia with an Eprex formulation in uncoated rubber stopper syringes."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/1987 - "A 4 year old boy with acute lymphoblastic leukaemia developed a pure red cell aplasia 13 months after entering remission and while on maintenance chemotherapy. " 01/01/2005 - "We report here the case of a patient with DLBL-TH who developed overt pure red cell aplasia (PRCA) following chemotherapy and autologous peripheral blood stem cell transplantation. " 03/01/2014 - "Effective treatment of Waldenström macroglobulinemia associated pure red cell aplasia with standard antilymphomatous chemotherapy." 06/01/2006 - "Metastatic basal cell carcinoma: complete response to chemotherapy and associated pure red cell aplasia." 10/01/1996 - "Pure red-cell aplasia requiring cytotoxic chemotherapy: presence of clonal T-lymphocytes without characteristics of chronic lymphocytic leukemia."
2.	Transplants (Transplant) 09/01/2007 - "We report a case of 64-year-old patient with pure red cell aplasia (PRCA) who was intolerant of conventional immunosuppressive therapies but achieved a complete long-term remission following autologous hematologic stem cell transplant (HSCT). " 02/01/2015 - "A case of parvovirus B19-induced pure red cell aplasia in a child following heart transplant." 08/01/2011 - "A kidney transplant patient with pure red cell aplasia: first things first!" 01/01/2011 - "Parvovirus B19 is recognized as a rare cause of pure red cell aplasia (PRCA) in allogeneic stem cell (SCT) and solid organ transplant patients. " 07/01/2008 - "We describe a case of pure red cell aplasia developing within two months after kidney transplant in a patient who was actively receiving immunosuppression. "
3.	Splenectomy 12/01/1981 - "[A case of pure red cell aplasia of which remission was obtained by splenectomy alone (author's transl)]." 08/19/1978 - "[Remission of pure red cell aplasia obtained by splenectomy after failure of thymomectomy and immunosuppressive treatment]." 08/01/1975 - "This study indicates that in some cases the anemia associated with abundant marrow proerythroblasts and the absence of mature erythroblasts has the same pathogenesis as pure red cell aplasia and that splenectomy may be beneficial when there is a lack of response to immunosuppressive drugs." 07/01/1978 - "Complete or near complete remission of acquired pure red cell aplasia, not associated with thymoma, occurred in response to prednisone (six occasions), prednisone and cyclophosphamide (twice), cyclophosphamide alone (once) and splenectomy (once). "
4.	Thymectomy 06/01/2010 - "One year after the diagnosis of pure red cell aplasia and seven months after thymectomy, bone marrow aspiration and biopsy showed an absence of megakaryocytes, marked erythroid hypoplasia with normal myeloid series. " 04/01/2006 - "Pure red cell aplasia regressed after gluten-free diet, corticoid therapy and thymectomy. " 06/01/2002 - "The possible onset of pure red-cell aplasia after thymectomy should be kept in mind during follow-up." 08/01/2001 - "Concomitant thymectomy and cardiac operation in a patient with pure red cell aplasia." 11/01/1991 - "[A case of pure red cell aplasia appearing after thymectomy]."
5.	Plasma Exchange 01/01/1986 - "A 57-year-old man with idiopathic pure red cell aplasia went into remission after plasma exchange. " 01/01/1986 - "Long-term remission of pure red cell aplasia after plasma exchange and lymphocytapheresis." 01/01/1985 - "Remission of acquired pure red cell aplasia following plasma exchanges." 01/01/1985 - "The place of plasma exchange in the management of pure red cell aplasia and its mode of action will be discussed." 03/01/2004 - "Pure red cell aplasia after ABO major-mismatched allogeneic peripheral blood stem cell transplantation successfully treated with plasma exchange and low-dose steroid: two case reports."