2,809 results match your criteria Pure Red Cell Aplasia


Monoclonal IgM gammopathy in adult acquired pure red cell aplasia: culprit or innocent bystander?

Blood Cells Mol Dis 2021 Jul 14;91:102595. Epub 2021 Jul 14.

Translational Hematology and Oncology Research Department, Taussig Cancer Center, Cleveland Clinic, USA; Department of Hematology and Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA.

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Characterization of the clinical and genetic spectrum of autoimmune polyendocrine syndrome type 1 in Chinese case series.

Orphanet J Rare Dis 2021 Jul 3;16(1):296. Epub 2021 Jul 3.

Department of Endocrinology, Key Laboratory of Endocrinology of the Ministry of Health, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Dongcheng District, Shuaifuyuan No.1, Beijing, 100730, China.

Background: Autoimmune polyendocrine syndrome type 1 (APS1) is a hereditary disease caused by mutations in the AIRE gene with both endocrine and non-endocrine organ involvement. The existing data from China are limited, and this study aims to describe the phenotypes and genetic characterization in Chinese APS1 patients. In this single-center, retrospective, observational study, comprehensive endocrine and extra-endocrine manifestations were collected, and genetic analysis in AIRE was conducted in patients with APS1 between the years of 1984 and 2018 at Peking Union Medical College Hospital. Read More

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Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia.

Hematology 2021 Dec;26(1):444-446

Department of Hematology, General Hospital, Tianjin Medical University, Tianjin, People's Republic of China.

Objectives: Our aim is to investigate the clinical characteristics of low- and intermediate-risk myelodysplastic syndrome (MDS) with pure red cell aplasia (PRCA).

Methods: We retrospectively reviewed the patients of low- and intermediate-risk MDS patients who had been diagnosed with PRCA in our hospital between January 2010 and December 2019.

Results: There were 6 low- and intermediate-risk MDS patients with PRCA in our study, 1 male and 5 females, with a median age of 63. Read More

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December 2021

Roxadustat on anti-erythropoietin antibody-related pure red cell aplasia in the patient with end-stage renal disease.

Semin Dial 2021 Jul 15;34(4):319-322. Epub 2021 Jun 15.

Department of Nephrology, The First Affiliated Hospital of Shantou University Medical College, Shantou, China.

Anti-erythropoietin antibody-related pure red cell aplasia (anti-EPO PRCA) is a severe complication in patients who receive erythropoiesis-stimulating agents for nephrogenic anemia. The standard therapy is withdrawl of EPO and immunosuppression. Here, we present successful treatment of anti-EPO PRCA with roxadustat, a hypoxia-inducible factor prolyl hydroxylase inhibitor. Read More

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Concomitant autoimmune hemolytic anemia and pure red cell aplasia in a patient with chronic lymphocytic leukemia successfully treated with ibrutinib.

Ann Hematol 2021 Jun 10. Epub 2021 Jun 10.

Department of Internal Medicine, General Hospital of Šibenik-Knin County, Stjepana Radića 83, 22000, Šibenik, Croatia.

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Computational Studies of the Structural Basis of Human RPS19 Mutations Associated With Diamond-Blackfan Anemia.

Front Genet 2021 24;12:650897. Epub 2021 May 24.

State Key Laboratory of Chemical Oncogenomics, Peking University Shenzhen Graduate School, Shenzhen, China.

Diamond-Blackfan Anemia (DBA) is an inherited rare disease characterized with severe pure red cell aplasia, and it is caused by the defective ribosome biogenesis stemming from the impairment of ribosomal proteins. Among all DBA-associated ribosomal proteins, RPS19 affects most patients and carries most DBA mutations. Revealing how these mutations lead to the impairment of RPS19 is highly demanded for understanding the pathogenesis of DBA, but a systematic study is currently lacking. Read More

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Management of thymoma associated autoimmune pure red cell aplasia: Case report and systematic review of the literature.

Lung Cancer 2021 07 11;157:131-146. Epub 2021 May 11.

Department of Medical Oncology, Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium; Laboratoire Facultaire de Médecine Factuelle de l'Université Libre de Bruxelles, Belgium. Electronic address:

Pure red cell aplasia (PRCA) is a rare paraneoplastic syndrome observed in 2-5 % of thymomas. Literature reports great variability in its management. Based on an illustrative clinical case, we present a systematic literature review whose main objective is to evaluate the therapeutic management of PRCA. Read More

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Selective ABO immunoadsorption in hematopoietic stem cell transplantation with major ABO incompatibility.

Eur J Haematol 2021 May 22. Epub 2021 May 22.

Department of Hematology, Oncology, Hemostaseology and Stem Cell Transplantation, Medical Faculty, RWTH Aachen University, Aachen, Germany.

Objective: ABO mismatch between donor and recipient occurs in 40% of allogeneic hematopoietic stem cell transplantations (HCT). Different strategies have been described to reduce isohemagglutinins (IHA) before HCT. We describe the effect of selective ABO immunoadsorption (ABO IA) on erythrocyte transfusion rate and the development of post-transplant pure red cell aplasia (ptPRCA). Read More

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Good treatment-free survival of monoclonal gammopathy of undetermined significance associated pure red cell aplasia after bortezomib plus dexamethasone.

Blood Cells Mol Dis 2021 07 27;89:102573. Epub 2021 Apr 27.

Regenerative Medicine Clinic, State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China. Electronic address:

Pure red cell aplasia (PRCA) is a rare syndrome characterized by severe anemia and absence of erythroid precursors. PRCA associated to monoclonal gammopathy of undetermined significance (MGUS) is a scarce condition with less than five cases reported so far. There is no agreement on the treatment of MGUS associated PRCA and treatment- free survival (TFS) is an unmet clinical need. Read More

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The N-terminal 5-68 amino acids domain of the minor capsid protein VP1 of human parvovirus B19 enters human erythroid progenitors and inhibits B19 infection.

J Virol 2021 May 5. Epub 2021 May 5.

Department of Microbiology, Molecular Genetics and Immunology, University of Kansas Medical Center, Kansas City, Kansas, USA

Parvovirus B19 (B19V) infection causes diseases in humans ranging from the mild to severe hematological disorders. The unique region of the minor structural protein VP1 (VP1u) of 227 amino acids harbors strong neutralizing epitopes which elicit dominant immune responses in patients. Recent studies have shown that the VP1u selectively binds to and enters B19V permissive cells through an unknown cellular proteinaceous receptor. Read More

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Pure Red Cell Aplasia following ABO-Mismatched Allogeneic Hematopoietic Stem Cell Transplantation: Resolution with Daratumumab Treatment.

Acta Haematol 2021 Apr 22:1-5. Epub 2021 Apr 22.

Department of Hematology and Bone Marrow Transplantation, Rambam Health Care Campus, Haifa, Israel.

Pure red cell aplasia (PRCA) can potentially occur after allogeneic hematopoietic stem cell transplantation (allo-HSCT) if recipient and donor ABO blood groups are mismatched, with the recipient having isoagglutinins against the donor blood group. Patient plasma cells that survive transplant conditioning produce anti-ABO isoagglutinins targeting donor erythroid precursors in the bone marrow and thus causing red cell aplasia. Therapeutic options include steroids, discontinuation of immunosuppression, plasmapheresis, donor lymphocyte infusion, rituximab, and bortezomib, all with limited benefit. Read More

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Pure red cell aplasia in systemic lupus erythematosus, a nationwide retrospective cohort and review of the literature.

Rheumatology (Oxford) 2021 Apr 19. Epub 2021 Apr 19.

Service de Médecine Interne, Centre National de Référence des Cytopénies Auto-Immunes de l'Adulte, Centre Hospitalier Universitaire Henri-Mondor, Assistance Publique-Hôpitaux de Paris, Université Paris Est Créteil, Créteil, France.

Objectives: To characterize the clinical and biological course, management and response to treatment in Systemic Lupus Erythematosus (SLE)-associated Pure Red Cell Aplasia (PRCA).

Methods: Nationwide multicentre retrospective cohort study. From 2006 to 2018, we included adults with a diagnosis of PRCA supported by bone-marrow examination and SLE or biologic manifestations of SLE after ruling out parvovirus B19 infection. Read More

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Suspected primary pure red cell aplasia in a 4-month-old intact male mixed breed Bernese mountain dog.

Can Vet J 2021 04;62(4):408-412

VCA Canada 404 Veterinary Emergency and Referral Hospital, 510 Harry Walker Parkway South, Newmarket, Ontario L3Y 0B3.

A 4-month-old, 31-kg intact male mixed-breed Bernese mountain dog was presented for evaluation of severe non-regenerative anemia after several days of lethargy, inappetence and pale mucous membranes. Bone marrow evaluation and complete response to immunosuppressive therapy were suggestive of primary pure red cell aplasia (PRCA). Primary PRCA is a rare immune-mediated non-regenerative anemia that is overrepresented in middle-aged to older spayed female dogs and has not previously been described in an intact male puppy. Read More

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Pure red blood cell aplasia: patient management pitfalls in major ABO-incompatible haematopoietic cell transplantation.

Br J Haematol 2021 May 12;193(4):701-702. Epub 2021 Apr 12.

Department of Hematology and Medical Oncology, Winship Cancer Institute of Emory University, Atlanta, GA, USA.

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Treatment for pure red cell aplasia after major ABO-incompatible allogeneic stem cell transplantation: a multicentre study.

Br J Haematol 2021 May 12;193(4):814-826. Epub 2021 Apr 12.

Haematology Transplant Unit, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.

Pure red cell aplasia (PRCA) following allogeneic haematopoietic stem cell transplantation (aHSCT) with major ABO incompatibility is responsible for transfusion dependent anaemia, impaired quality of life and iron overload. We conducted a retrospective study, over a 10-year period, which included all consecutive patients who received a major ABO mismatched aHSCT, to assess the impact of specific treatment on PRCA. We did not observe any PRCA in the 57 aHSCT issued from cord blood. Read More

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Autoimmune Complications in Hematologic Neoplasms.

Cancers (Basel) 2021 Mar 26;13(7). Epub 2021 Mar 26.

Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Autoimmune cytopenias (AICy) and autoimmune diseases (AID) can complicate both lymphoid and myeloid neoplasms, and often represent a diagnostic and therapeutic challenge. While autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are well known, other rarer AICy (autoimmune neutropenia, aplastic anemia, and pure red cell aplasia) and AID (systemic lupus erythematosus, rheumatoid arthritis, vasculitis, thyroiditis, and others) are poorly recognized. This review analyses the available literature of the last 30 years regarding the occurrence of AICy/AID in different onco-hematologic conditions. Read More

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Eltrombopag Improves Erythroid Differentiation in a Human Induced Pluripotent Stem Cell Model of Diamond Blackfan Anemia.

Cells 2021 Mar 26;10(4). Epub 2021 Mar 26.

Cellular and Molecular Therapeutics Branch, National Heart, Lung and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, MD 20892, USA.

Diamond Blackfan Anemia (DBA) is a congenital macrocytic anemia associated with ribosomal protein haploinsufficiency. Ribosomal dysfunction delays globin synthesis, resulting in excess toxic free heme in erythroid progenitors, early differentiation arrest, and pure red cell aplasia. In this study, DBA induced pluripotent stem cell (iPSC) lines were generated from blood mononuclear cells of DBA patients with inactivating mutations in RPS19 and subjected to hematopoietic differentiation to model disease phenotypes. Read More

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Expanding spectrum of DADA2: a review of phenotypes, genetics, pathogenesis and treatment.

Clin Rheumatol 2021 Mar 31. Epub 2021 Mar 31.

Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, 160012, India.

Deficiency of adenosine deaminase 2 (DADA2) is a monogenic disease caused by biallelic mutations in ADA2 gene (previously CECR1). The aim of this review was to describe the clinical phenotypes, genetics, pathogenesis and treatment of DADA2. ADA2 is highly expressed on myeloid cells and deficiency leads to polarisation of macrophages to an M1 inflammatory type and activation of neutrophils. Read More

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Human parvovirus B19-associated early postoperative acquired pure red cell aplasia in simultaneous pancreas-kidney transplantation: A case report.

World J Clin Cases 2021 Mar;9(8):1968-1975

Department of Kidney and Pancreas Transplant, Tianjin First Central Hospital, School of Medicine, Nankai University, Tianjin 300192, China.

Background: Acquired pure red cell aplasia (aPRCA) related to human parvovirus B19 (HPV B19) is rarely reported in simultaneous pancreas-kidney transplantation (SPKT) recipients; there has yet to be a case report of early postoperative infection. In this current study, we report the case of a Chinese patient who experienced the disease in the early postoperative period.

Case Summary: A 63-year-old man, with type 2 diabetes and end-stage renal disease, received a brain dead donor-derived SPKT. Read More

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[Expression of iron-regulating erythroid factors in different types of erythropoiesis disorders].

Zhonghua Xue Ye Xue Za Zhi 2021 Jan;42(1):52-57

State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China.

To investigate the expression of iron-regulating erythroid factors in different types of erythropoiesis disorders. From January 2016 to November 2019, the plasma concentrations of iron-regulating erythroid factors were measured by ELISA methods in 47 patients with different types of erythropoiesis disorders. The adaptation orientation of iron-regulating erythroid factor expression with bone marrow erythropoiesis activities (represented by bone marrow-nucleated erythrocytes ratio) was analyzed. Read More

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January 2021

How I manage acquired pure red cell aplasia in adults.

Blood 2021 Apr;137(15):2001-2009

Translational Hematology and Oncology Research Department, Taussig Cancer Center, Cleveland Clinic, Cleveland, OH; and.

Pure red cell aplasia (PRCA) is a rare hematological disorder with multiple etiologies. The multifaceted nature of this disease is emphasized by the variety of concomitant clinical features. Classic idiopathic presentation aside, prompt recognition of pathogenetic clues is important because of their diagnostic and therapeutic implications. Read More

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Therapeutic drug monitoring of intravenous busulfan in Thai children undergoing hematopoietic stem cell transplantation: A pilot study.

Pediatr Hematol Oncol 2021 May 3;38(4):346-357. Epub 2021 Mar 3.

Department of Pharmacy, Faculty of Pharmacy, Mahidol University, Bangkok, Thailand.

Busulfan (Bu) is commonly used in myeloablative conditioning regimens for children undergoing hematopoietic stem cell transplantation. The standard target area under the concentration-time curve (AUC) of Bu is approximately 900-1500 µM min. In previous studies using five fixed doses (0. Read More

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Eltrombopag restores erythropoiesis in refractory adult acquired pure red cell aplasia.

Int J Hematol 2021 Jul 21;114(1):124-128. Epub 2021 Feb 21.

Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, 210029, China.

Acquired pure red cell aplasia is a rare condition characterized by normocytic normochromic anemia with severe reticulocytopenia. In refractory acquired pure red cell aplasia, the low response rate of immunosuppressive therapy also constitutes a challenge. We herein report the case of a 58-year-old male with refractory acquired pure red cell aplasia that was successfully treated by eltrombopag at a dose of 75 mg/day. Read More

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Atezolizumab-induced pure red cell aplasia.

Br J Haematol 2021 04 14;193(1):10. Epub 2021 Feb 14.

Department of Pathology and Laboratory Medicine, Auckland City Hospital, Auckland, New Zealand.

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[Treatment-refractory anaemia in a 35-year-old heart transplant recipient on chronic hemodialysis].

Internist (Berl) 2021 Jul 12;62(7):768-771. Epub 2021 Feb 12.

Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Carl-Neuberg-Straße 1, 30625, Hannover, Deutschland.

This article presents a case of pure red cell aplasia in a 35-year-old heart transplant recipient on chronic hemodialysis. Elevated parvovirus B19 immunoglobulin M blood levels were detected along with a high viral load of 80 billion IU/ml quantified by polymerase chain reaction. Bone marrow examination revealed giant proerythroblasts confirming parvovirus B19 infection. Read More

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A case of recurrent anemia due to chronic parvovirus B19 infection in a kidney transplant recipient. Can everolimus make a difference?

CEN Case Rep 2021 08 4;10(3):388-392. Epub 2021 Feb 4.

Department of Nephrology and Kidney Transplantation, Hospital Clinic, 170 Villarroel Street, 08036, Barcelona, Spain.

Parvovirus B19 (PB19) is a common infection among solid transplant recipients. Usually, it is asymptomatic, but sometimes it can become a real therapeutic challenge. We report a case of a kidney transplant recipient with relapsing pure red cell aplasia due to PB19 infection. Read More

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Daratumumab for delayed RBC engraftment following major ABO mismatched haploidentical bone marrow transplantation.

Transfusion 2021 04 2;61(4):1041-1046. Epub 2021 Feb 2.

Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.

Background: Recent case reports have described the efficacy of daratumumab to treat refractory pure red cell aplasia (PRCA) following major ABO mismatched allogeneic hematopoietic stem cell transplantation (HSCT). In this report, we describe the use of daratumumab as a first-line agent for treatment of delayed red blood cell (RBC) engraftment following a major ABO mismatched pediatric HSCT and provide a review of the literature.

Study Design And Materials: We report on a 14-year-old with DOCK8 deficiency who underwent a myeloablative, haploidentical bone marrow transplant from her major ABO mismatched sister (recipient O+, donor A+) for treatment of her primary immunodeficiency. Read More

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Pure red-cell aplasia secondary to pregnancy: Case report and review of the literature.

Rev Colomb Obstet Ginecol 2020 12;71(4):365-373

Médico, ginecobstetra, Hospital Universitario de La Samaritana, Bogotá (Colombia).

Objective: To report a case of pure red-cell aplasia secondary to pregnancy and to conduct a review of the literature regarding diagnosis and treatment, as well as maternal and perinatal prognosis.

Methods: This is the case of a 24-year-old patient at 34 weeks of gestation, referred to a regional public referral hospital due to anemia. Bone marrow biopsy was performed, leading to the diagnosis of pregnancy-related pure red-cell aplasia. Read More

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December 2020

Clonal hematopoiesis in adult pure red cell aplasia.

Sci Rep 2021 Jan 26;11(1):2253. Epub 2021 Jan 26.

Department of General Internal Medicine and Clinical Laboratory Medicine, Akita University Graduate School of Medicine, Akita, Japan.

Idiopathic pure red cell aplasia (PRCA) and secondary PRCA associated with thymoma and large granular lymphocyte leukemia are generally considered to be immune-mediated. The PRCA2004/2006 study showed that poor responses to immunosuppression and anemia relapse were associated with death. PRCA may represent the prodrome to MDS. Read More

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January 2021