2,594 results match your criteria Pure Red Cell Aplasia


Diamond Blackfan Anemia: Genetics, Pathogenesis, Diagnosis and Treatment.

EJIFCC 2019 Mar 1;30(1):67-81. Epub 2019 Mar 1.

Department of Hematology & Immunohematology, School of Biomedical and Laboratory Sciences, College of Medicine and Health Sciences, University of Gondar, Ethiopia.

Diamond Blackfan Anaemia (DBA) is a sporadic inherited anemia with broad spectrum of anomalies that are presented soon after delivery. It is inherited mainly in autosomal dominant inheritance manner and caused by mutations and deletions in either large or small ribosomal protein genes that results in an imbalance between the biosynthesis of rRNA and ribosomal proteins, eventually the activation and stabilization of p53. Diagnosing DBA is usually problematic due to a partial phenotype and its wide inconsistency in its clinical expression; however, molecular studies have identified a heterozygous mutated gene in up to 50% of the DBA cases and corticosteroid drugs are the backbone treatment options of DBA. Read More

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Pure Red Cell Aplasia (PRCA) and Cerebellar Hypoplasia as Atypical Features of Polyglandular Autoimmune Syndrome Type I (APS-1): Two Sisters With the Same AIRE Mutation but Different Phenotypes.

Front Pediatr 2019 26;7:51. Epub 2019 Feb 26.

Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.

The polyglandular autoimmune syndrome type I is a rare hereditary autosomal recessive disease. We describe a child with the classic triad of the disease and her sister with pure red cell aplasia and cerebellar hypoplasia. The latter received two haematopoietic stem cell transplantations, complicated by an acute disseminated encephalomyelitis. Read More

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https://www.frontiersin.org/article/10.3389/fped.2019.00051/
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http://dx.doi.org/10.3389/fped.2019.00051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399394PMC
February 2019
6 Reads

Occurrence of acute pulmonary embolism induced by recombinant erythropoietin during treatment of pure red cell aplasia associated with thymoma: A case report.

Medicine (Baltimore) 2019 Mar;98(10):e14789

Department of Respiratory and Critical Care Medicine, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China.

Rationale: Thymoma is a type of rare tumor in the thymus gland, and among patients with thymoma, less than 10% will develop pure red cell aplasia (PRCA), whereas less than 5% of patients with PRCA have a thymoma. The optimal approach for PRCA in thymoma is immunosuppressive therapy, such as steroids, cyclosporine, and human antithymocyte globulin.

Patient Concerns: A sixty-one-year-old male was diagnosed with thymoma with PRCA after he complained fatigue, tinnitus, and weakness for 1 month, he received therapy with recombinant erythropoietin (rhEPO) for 1 month after the tumor was totally resected and readmitted with pulmonary embolism and received anticoagulation therapy with enoxaparin for 3 months. Read More

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http://dx.doi.org/10.1097/MD.0000000000014789DOI Listing
March 2019
1 Read

Pure Red Cell Aplasia After Kidney Transplantation: Parvovirus B19 Culprit or Coincidence?

Ann Transplant 2019 Mar 5;24:123-131. Epub 2019 Mar 5.

Department of Nephrology Transplantation and Internal Medicine, Pomeranian Medical University, Szczecin, Poland.

BACKGROUND Anemia is present even in long-term observation after kidney transplantation. Observational study results indicate the presence of chronic post-transplantation anemia in 1 in 3 recipients. An extreme form of erythroid line dysfunction is pure red cell aplasia (PRCA). Read More

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https://www.annalsoftransplantation.com/abstract/index/idArt
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http://dx.doi.org/10.12659/AOT.913663DOI Listing
March 2019
3 Reads
1.430 Impact Factor

Hematologic Complications of Immune Checkpoint Inhibitors.

Oncologist 2019 Feb 28. Epub 2019 Feb 28.

Division of Hematology/Oncology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

Immune checkpoint inhibitors have improved outcomes for patients with numerous hematological and solid cancers. Hematologic toxicities have been described, but the spectrum, timing, and clinical presentation of these complications are not well understood. We used the World Health Organization's pharmacovigilance database of individual-case-safety-reports (ICSRs) of adverse drug reactions, VigiBase, to identify cases of hematologic toxicities complicating immune checkpoint inhibitor therapy. Read More

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http://dx.doi.org/10.1634/theoncologist.2018-0574DOI Listing
February 2019
1 Read

Transient Erythroblastopenia of Childhood: A Review for the Pediatric Emergency Medicine Physician.

Pediatr Emerg Care 2019 Mar;35(3):237-240

Assisstant Professor (Burns), Department of Pediatrics, Division of Pediatric Emergency Medicine, University of Washington and Seattle Children's Hospital, Seattle, WA; Professor of Pediatrics (Woodward), Division Chief of Emergency Medicine, Department of Pediatrics, University of Washington and Seattle Children's Hospital, Seattle, WA.

Transient erythroblastopenia of childhood is a form of pure red cell aplasia that is self-limited and occurs in children 4 years old and younger. It is characterized by an absence or a significantly reduced quantity of erythroblasts in the bone marrow without underlying congenital red blood cell abnormalities. Transient erythroblastopenia of childhood should be considered in previously healthy children who present with normocytic anemia and lack of reticulocytosis without evidence of blood loss, hemolysis, or other causes of bone marrow suppression. Read More

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http://dx.doi.org/10.1097/PEC.0000000000001760DOI Listing

Recurrence of Pure Red Cell Aplasia in a Kidney Transplant Recipient Due to Reactivation of Parvovirus B19 Infection Despite Two Cycles of Intravenous Immunoglobulin Therapy.

Exp Clin Transplant 2019 01;17(Suppl 1):195-197

From the Department of Nephrology, Cukurova University Faculty of Medicine, Adana, Turkey.

Parvovirus B19 is a single-stranded DNA virus that typically has an affinity for erythroid progenitor cells in bone marrow and leads to pure red cell aplasia. This is a common pathogen in humans, and the expression of the infection depends on the host's hematologic and immunologic status. Here, we report a female patient who developed severe and persistent anemia after kidney transplant while being on immunosuppressive therapy. Read More

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http://dx.doi.org/10.6002/ect.MESOT2018.P63DOI Listing
January 2019
1 Read

ABO Blood Grouping Mismatch in Hematopoietic Stem Cell Transplantation and Clinical Guides.

Int J Hematol Oncol Stem Cell Res 2018 Oct;12(4):322-328

Diagnostic Laboratory Sciences and Technology Research Center, School of Paramedical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran.

Hematopoietic stem cell transplantation (HSCT) is a useful treatment. In contrast to solid organ transplantations, the use of ABO blood group mismatch is acceptable in HSCT. Immediate or late hemolytic reactions, pure red cell aplasia, delayed red blood cell recovery, and graft-versus -host disease are the results of this situation. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375375PMC
October 2018
1 Read

A case of immune thrombocytopaenia induced by pembrolizumab in a metastatic melanoma patient with a history of immune-mediated pure red cell aplasia.

Eur J Cancer 2019 Mar 8;110:49-52. Epub 2019 Feb 8.

Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Service de Dermatologie, Pierre-Bénite, France. Electronic address:

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http://dx.doi.org/10.1016/j.ejca.2019.01.011DOI Listing
March 2019
1 Read

Chronic Lymphocytic Leukemia Presenting as a Subcortical Watershed Infarct.

Case Rep Hematol 2019 9;2019:2089359. Epub 2019 Jan 9.

Internal Medicine, Monmouth Medical Center, Long Branch, New Jersey, USA.

Internal watershed infarcts (WI) involve white matter between deep and superficial arterial systems of middle cerebral artery. These infarcts are considered to be either from low blood flow or microembolism. Anemia is an extremely rare cause of watershed infarcts. Read More

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https://www.hindawi.com/journals/crihem/2019/2089359/
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http://dx.doi.org/10.1155/2019/2089359DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343168PMC
January 2019
11 Reads

[Successful treatment of pure red cell aplasia with cyclosporin in a patient with T-cell large granular lymphocytic leukemia harboring the STAT3 D661V mutation].

Rinsho Ketsueki 2019 ;60(1):39-45

Pathology and Tumor Biology, Graduate School of Medicine, Kyoto University.

T-cell large granular lymphocyte (T-LGL) leukemia is a chronic T-cell monoclonal disease that is occasionally associated with pure red cell aplasia (PRCA). A 71-year-old previously healthy man complained of physical fatigue and exhibited anemia (hemoglobin, 10.5 g/dl) with lymphocytosis (76%) showing LGL. Read More

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http://dx.doi.org/10.11406/rinketsu.60.39DOI Listing
January 2019
1 Read

Regulation of globin-heme balance in Diamond-Blackfan anemia by HSP70/GATA1: Implications for disease severity.

Blood 2019 Jan 30. Epub 2019 Jan 30.

Inserm U1149 / ERL 8252, Inflammation Research Center (CRI), France;

Diamond-Blackfan anemia is a congenital erythroblastopenia characterized by blockade in erythroid differentiation related to impaired ribosome biogenesis. DBA phenotype and genotype are highly heterogeneous. We have previously identified two in vitro erythroid cell growth phenotypes for primary CD34+ cells from DBA patients and following shRNA knockdown of RPS19, RPL5 and RPL11 expression in normal human CD34+ cells. Read More

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http://dx.doi.org/10.1182/blood-2018-09-875674DOI Listing
January 2019
1 Read

Clinical impact & pathogenic mechanisms of human parvovirus B19: A multiorgan disease inflictor incognito.

Indian J Med Res 2018 Oct;148(4):373-384

Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India.

Human parvovirus B19 (B19V) causes myriads of clinical diseases; however, owing to lack of awareness and undetermined clinical impact, it has failed to become a virus pathogen of global concern. Cryptically, B19V causes significant morbidity and mortality. Half of the world population and 60 per cent of Indians are known to be serologically naive and are at risk of acquiring B19V infections. Read More

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http://www.ijmr.org.in/text.asp?2018/148/4/373/250539
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http://dx.doi.org/10.4103/ijmr.IJMR_533_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362725PMC
October 2018
9 Reads

Atraumatic splenic rupture following IVIg for parvovirus B19 pure red cell aplasia post renal transplant.

Transpl Infect Dis 2018 Dec 26:e13045. Epub 2018 Dec 26.

Department of Anatomical Pathology, Alfred Health, Melbourne, Victoria, Australia.

Parvovirus B19 (PB19) associated pure red cell aplasia (PRCA) is an uncommon but well described complication of immunosuppression post solid organ transplantation. We report a unique case of a renal transplant patient with PB19 associated PRCA who developed a spontaneous splenic rupture after receiving IVIg for persistent anemia. He subsequently required splenectomy. Read More

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http://dx.doi.org/10.1111/tid.13045DOI Listing
December 2018
5 Reads

Non-motor Comorbidity of Myasthenia Gravis: Myasthenia Gravis as a Systemic Immunological Disorder Involving Non-motor Systems.

Intern Med 2018 Dec 18. Epub 2018 Dec 18.

Department of Neurology, Brain Science Centre, Sapporo City General Hospital, Japan.

To explore non-motor comorbidities of myasthenia gravis (MG), we present two cases of thymoma-associated MG patients. Alopecia, pure red cell aplasia, and thymoma- associated multiorgan autoimmunity were observed in Case 1, and alopecia, thrombocytopenia, hypogammaglobulinemia and nephrotic syndrome were observed in Case 2. In both cases, autoreactive T lymphocytes inappropriately stimulated by thymus tissue may have played key roles in generating the various autoimmune-associated symptoms. Read More

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http://dx.doi.org/10.2169/internalmedicine.1990-18DOI Listing
December 2018
2 Reads

Complete remission of pure white cell aplasia associated with thymoma after thymectomy and cyclosporine administration.

Int J Hematol 2019 Mar 9;109(3):346-350. Epub 2018 Dec 9.

Department of Haematology, Atomic Bomb Disease and Hibakusha Medicine Unit, Atomic Disease Institute, Nagasaki University, 1-12-4 Sakamoto, Nagasaki, 852-8523, Japan.

We present the case of a 63-year-old male with pure white cell aplasia (PWCA), a rare complication of thymoma, who was successfully treated with cyclosporine A (CyA) and thymectomy. The patient presented with high fever and agranulocytosis. Complete blood count revealed a white blood cell count of 0. Read More

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http://dx.doi.org/10.1007/s12185-018-02573-yDOI Listing
March 2019
3 Reads

[Pure red cell aplasia following the rapid reduction and discontinuation of cyclosporine for mixed chimerism after allogeneic bone marrow transplantation].

Rinsho Ketsueki 2018;59(11):2408-2412

Department of Pediatrics, Kyoto Prefectural University of Medicine.

A 19-year-old male with therapy-related myelodysplastic syndrome underwent allogeneic bone marrow transplantation with reduced-intensity conditioning from his HLA-identical sibling whose ABO blood type exhibited major incompatibility with the patient. After post-transplantation 1 month, chimerism analysis of the bone marrow revealed mixed chimerism with 30% of recipient cells, and after post-transplantation 3 months, complete remission was maintained; however, recipient granulocytes were elevated up to 50% per the chimerism analysis. Next, pancytopenia developed following the rapid discontinuation of the immunosuppressive agent. Read More

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http://dx.doi.org/10.11406/rinketsu.59.2408DOI Listing
January 2018
2 Reads

Haematological immune-related adverse events induced by anti-PD-1 or anti-PD-L1 immunotherapy: a descriptive observational study.

Lancet Haematol 2019 Jan 4;6(1):e48-e57. Epub 2018 Dec 4.

Service de Médecine Interne and Immunologie Clinique, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France; Université Paris Sud, Centre de Recherche en Immunologie des Infections Virales et des Maladies Auto-Immunes, INSERM, Le Kremlin-Bicêtre, France; Division d'Immunovirologie, Commissariat à l'Energie Atomique et aux Energies Alternatives, Fontenay-aux-Roses, France.

Background: Anti-programmed cell death 1 (PD-1) and anti-programmed cell death ligand 1 (PD-L1) antibodies are novel immunotherapies for cancer that can induce immune-related adverse events (irAEs). These adverse events can involve all organs, including the haemopoietic system. Thus far, haematological irAEs (haem-irAEs) have not been extensively characterised. Read More

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http://dx.doi.org/10.1016/S2352-3026(18)30175-3DOI Listing
January 2019
8 Reads

Donor-type fresh frozen plasma is effective in preventing hemolytic reaction in major ABO incompatible allogeneic stem cell transplant.

Transfusion 2019 Jan 22;59(1):335-339. Epub 2018 Nov 22.

Department of Hematology, Singapore General Hospital, Singapore.

Background: Hemolysis at the time of graft infusion is one of the immediate complications in major ABO-incompatible allogeneic hematopoietic stem cell transplants (HSCTs). We conducted a retrospective analysis to evaluate the efficacy of donor-type fresh frozen plasma (FFP) in reducing isohemagglutinin titer and preventing hemolysis, as well as its effect on delayed red cell engraftment.

Materials And Methods: This is a single-center study on a series of 380 allogeneic HSCT between 2005 and 2015; of which 99 were either major (n = 74) or bidirectional (n = 25) ABO mismatched. Read More

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http://dx.doi.org/10.1111/trf.15053DOI Listing
January 2019
17 Reads

Vasculopathy, Immunodeficiency, and Bone Marrow Failure: The Intriguing Syndrome Caused by Deficiency of Adenosine Deaminase 2.

Authors:
Pui Y Lee

Front Pediatr 2018 18;6:282. Epub 2018 Oct 18.

Division of Allergy, Immunology and Rheumatology, Boston Children's Hospital, Boston, MA, United States.

Deficiency of adenosine deaminase 2 (DADA2) is a monogenic form of systemic vasculopathy that often presents during early childhood. Linked to biallelic mutations in (previously ), DADA2 was initially described as a syndrome of recurrent fever, livedo racemosa, early-onset strokes, and peripheral vasculopathy that resembles polyarteritis nodosum. However, the wide spectrum of clinical findings and heterogeneity of disease, even among family members with identical mutations, is increasingly recognized. Read More

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https://www.frontiersin.org/article/10.3389/fped.2018.00282/
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http://dx.doi.org/10.3389/fped.2018.00282DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6200955PMC
October 2018
5 Reads

Daratumumab for Delayed Red-Cell Engraftment after Allogeneic Transplantation.

N Engl J Med 2018 11;379(19):1846-1850

From the Dana-Farber Cancer Institute at St. Elizabeth's Medical Center (C.I.C.), Blood Bank, Department of Pathology (R.M.K.), and the Division of Hematology (J.M.C.), Brigham and Women's Hospital, and the Department of Medical Oncology, Dana-Farber Cancer Institute (E.P.A.) - all in Boston; and the Department of Hematology and Oncology, University Medical Center, Göttingen, Germany (C.I.C.).

Daratumumab, a human IgG1κ monoclonal antibody targeting CD38, is used to treat multiple myeloma. We describe successful treatment with daratumumab in a case of treatment-refractory pure red-cell aplasia after ABO-mismatched allogeneic stem-cell transplantation. The patient was a 72-year-old man with the myelodysplastic syndrome who received a transplant from an HLA-matched, unrelated donor with a major ABO incompatibility (blood group A in the donor and blood group O in the recipient). Read More

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http://dx.doi.org/10.1056/NEJMoa1807438DOI Listing
November 2018
3 Reads

Pure Red Cell Aplasia Associated with Thymolipoma: Complete Anaemia Resolution following Thymectomy.

Case Rep Hematol 2018 9;2018:8627145. Epub 2018 Oct 9.

Haematology Department, Haematology Staff Specialist, John Hunter Hospital, Lookout Rd., New Lambton Heights, NSW 2305, Australia.

Pure red cell aplasia is an uncommon cause of anaemia rarely associated with thymoma. A combination of immunosuppressive therapy and thymectomy offers a potential cure. Thymectomy alone rarely results in anaemia resolution. Read More

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http://dx.doi.org/10.1155/2018/8627145DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6198544PMC
October 2018
3 Reads

Frequent mutations in CD8 T cells from patients with pure red cell aplasia.

Blood Adv 2018 Oct;2(20):2704-2712

Division of Hematology, Department of Internal Medicine, School of Medicine.

Dysregulation of T-cell-mediated immunity is responsible for acquired pure red cell aplasia (PRCA). Although mutations are frequently detected in patients with T-cell large granular lymphocytic leukemia (T-LGLL), which is often complicated by PRCA and which is also reported to be associated with acquired aplastic anemia (AA) and myelodysplastic syndrome (MDS), whether -mutated T cells are involved in the pathophysiology of PRCA and other types of bone marrow failure remains unknown. We performed mutation analyses of the peripheral blood mononuclear cells from PRCA patients (n = 42), AA (n = 54), AA-paroxysmal nocturnal hemoglobinuria (AA-PNH; n = 7), and MDS (n = 21) using an allele-specific polymerase chain reaction and amplicon sequencing. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018022723DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6199660PMC
October 2018
16 Reads

Isoniazid-induced pure red cell aplasia.

BMJ Case Rep 2018 Oct 7;2018. Epub 2018 Oct 7.

Department of Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Mangalore, Karnataka, India.

The pure red cell aplasia (PRCA) is an uncommon side effect of isoniazid. We describe a 28-year-old man who developed a severe anaemia caused by PRCA. The patient received antituberculous therapy including isoniazid for his pulmonary tuberculosis. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22613
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http://dx.doi.org/10.1136/bcr-2018-226134DOI Listing
October 2018
4 Reads

Linezolid-induced pure red cell aplasia: a case report and literature review.

J Int Med Res 2018 Nov 1;46(11):4837-4844. Epub 2018 Oct 1.

1 Department of Respiratory Diseases, Ningbo First Hospital, Zhejiang, China.

Linezolid (LZD) is the first oxazolidinone with excellent safety and efficacy profiles against refractory infections caused by gram-positive organisms. Hematological toxicities such as thrombocytopenia, anemia, and leukocytopenia are common in LZD therapy; however, LZD-induced pure red cell aplasia (PRCA) is rare. An 83-year-old man diagnosed with pleural empyema caused by Staphylococcus aureus received LZD after developing resistance to multiple antibiotics. Read More

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http://dx.doi.org/10.1177/0300060518800126DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6259366PMC
November 2018
8 Reads

Pure red cell aplasia associated with thymoma: a report of a single-center experience.

J Thorac Dis 2018 Aug;10(8):5066-5072

Department of Oncology, Immunology and Surgery, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.

Background: Pure red cell aplasia (PRCA) associated with thymoma is relatively rare, and relevant reports are limited. We investigated the clinical features and outcomes of PRCA associated with thymoma in this study.

Methods: A retrospective review of all PRCA patients who underwent surgical resection of thymoma from April 1, 2004, to December 31, 2015, was performed. Read More

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http://dx.doi.org/10.21037/jtd.2018.07.14DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6129890PMC
August 2018
4 Reads

Clinical and laboratory features of seventy-eight UK patients with Good's syndrome (thymoma and hypogammaglobulinaemia).

Clin Exp Immunol 2019 Jan 21;195(1):132-138. Epub 2018 Oct 21.

Immunology, University of Manchester, Manchester University Hospitals NHS Trust, Manchester, UK.

Good's syndrome (thymoma and hypogammaglobulinaemia) is a rare secondary immunodeficiency disease, previously reported in the published literature as mainly individual cases or small case series. We use the national UK-Primary Immune Deficiency (UKPID) registry to identify a large cohort of patients in the UK with this PID to review its clinical course, natural history and prognosis. Clinical information, laboratory data, treatment and outcome were collated and analysed. Read More

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http://dx.doi.org/10.1111/cei.13216DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6300645PMC
January 2019
13 Reads

Acquired Pure Red Cell Aplasia and Acquired Amegakaryocytic Thrombocytopenia Associated With Clonal Expansion of T-Cell Large Granular Lymphocytes in a Patient With Lipopolysaccharide-responsive Beige-like Anchor (LRBA) Protein Deficiency.

J Pediatr Hematol Oncol 2018 Sep 4. Epub 2018 Sep 4.

Carman and Ann Adams Department of Pediatrics, Wayne State University.

Acquired pure red cell aplasia and acquired amegakaryocytic thrombocytopenic purpura are rare in children. Similarly, clonal expansion of T-cell large granular lymphocytes is infrequently seen in pediatrics. Lipopolysaccharide-responsive beige-like anchor (LRBA) protein deficiency is a recently described immunodeficiency syndrome that has been associated with inflammatory bowel disease and autoimmune phenomena such as Evans syndrome. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001292DOI Listing
September 2018
4 Reads

Investigating Serious Adverse Drug Reactions in Patients Receiving Erythropoiesis-Stimulating Agents: A Root Cause Analysis Using the "ANTICIPATE" Framework.

Am J Ther 2018 Nov/Dec;25(6):e670-e674

The Southern Network on Adverse Reactions (SONAR) Program, University of South Carolina College of Pharmacy, Columbia, SC.

Background: Unexpected serious adverse drug reactions (sADRs) affecting patients with chronic kidney disease (CKD) who received erythropoiesis-stimulating agents were identified by study co-authors. These included pure red cell aplasia (PRCA) after administration of the Eprex formulation of epoetin or the epoetin biosimilar HX575 and fatal anaphylaxis associated with peginesatide, an erythropoietin receptor agonist. We developed and applied a structured framework to describe these sADRs, including root cause analyses and eradication efforts. Read More

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http://Insights.ovid.com/crossref?an=00045391-900000000-9857
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http://dx.doi.org/10.1097/MJT.0000000000000768DOI Listing
March 2019
16 Reads

Pure red cell aplasia and anti-erythropoietin antibodies in patients on hemodialysis: a report of two cases and a literature review.

J Bras Nefrol 2018 Aug 23. Epub 2018 Aug 23.

Serviço de Nefrologia, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brasil.

Introduction: Anemia is a frequent multifactorial complication of CKD seen in patients on dialysis derived mainly from impaired erythropoietin (EPO) production. A less common cause of anemia in individuals with CKD is pure red cell aplasia (PRCA) secondary to the production of anti-EPO antibodies.

Objective: This paper aimed two describe two cases of PRCA secondary to the production of anti-EPO antibodies including choice of treatment, patient progression, and a literature review. Read More

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http://dx.doi.org/10.1590/2175-8239-jbn-2018-0054DOI Listing
August 2018
10 Reads

Pure red cell aplasia caused by azathioprine: case report and review of the literature.

Cardiovasc Hematol Disord Drug Targets 2018 Aug 28. Epub 2018 Aug 28.

Evangelismos General Hospital, Athens. Greece.

Pure red cell aplasia (PRCA) is a clinical entity comprising severe normochromic normocytic anemia, reticulocytopenia, erythroblastopenia in the bone marrow, with normal leukocyte and platelets count. PRCA can be classified into congenital and acquired, with the latter characterized as idiopathic or secondary to various infections, hematological malignancies, collagen vascular diseases, thymoma, and exposure to a variety of drugs and other chemical substances. Herein, we present a female patient, who presented with PRCA due to azathioprine treatment. Read More

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http://dx.doi.org/10.2174/1871529X18666180828145818DOI Listing
August 2018
17 Reads

Autoimmune cytopenias in patients with chronic lymphocytic leukaemia treated with ibrutinib in routine clinical practice at an academic medical centre.

Br J Haematol 2018 Nov 16;183(3):421-427. Epub 2018 Aug 16.

Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA.

The effects of ibrutinib on the natural history of autoimmune cytopenias (AIC) among chronic lymphocytic leukaemia (CLL) patients treated in routine clinical practice require further investigation. Using the Mayo Clinical CLL Database, 193 CLL patients treated with ibrutinib between November 2013 and January 2017 outside the context of a clinical trial were identified; complete review of their medical records was performed for details of past history of AIC and treatment-emergent AIC. We identified 29/193 (15%) patients with history of AIC prior to ibrutinib start. Read More

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http://dx.doi.org/10.1111/bjh.15545DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234062PMC
November 2018
6 Reads
1 Citation
4.711 Impact Factor

Aggressive Systemic Mastocytosis in Association with Pure Red Cell Aplasia.

Case Rep Hematol 2018 8;2018:6928571. Epub 2018 Jul 8.

Rosalind Franklin University, 3333 Green Bay Road, North Chicago, IL 60064, USA.

Aggressive systemic mastocytosis (ASM) is characterized by mast cell accumulation in systemic organs. Though ASM may be associated with other hematological disorders, the association with pure red cell aplasia (PRCA) is rare and has not been reported. Pure red cell aplasia (PRCA) is a syndrome, characterized by normochromic normocytic anemia, reticulocytopenia, and severe erythroid hypoplasia. Read More

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http://dx.doi.org/10.1155/2018/6928571DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6057401PMC
July 2018
3 Reads

Progressive peripheral CD8 T lymphocytosis complicated by pure red cell aplasia following immunosuppressive therapy for thymoma-associated myasthenia gravis.

Int Immunopharmacol 2018 Oct 26;63:14-18. Epub 2018 Jul 26.

Department of Diagnostic Pathology, Dokkyo Medical University School of Medicine, Tochigi, Japan.

We herein report a unique case of type B2 thymoma-associated myasthenia gravis which was ameliorated by immunosuppressive therapy in combination with chemotherapy. However, the patient subsequently developed pure red cell aplasia and marked lymphocytosis after additional chemotherapy aimed at improvement of thymoma. While a separate immunosuppressive regimen was effective for anemia, lymphocytosis was exacerbated. Read More

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http://dx.doi.org/10.1016/j.intimp.2018.07.030DOI Listing
October 2018
6 Reads

Critical Issues in Diamond-Blackfan Anemia and Prospects for Novel Treatment.

Hematol Oncol Clin North Am 2018 Aug 5;32(4):701-712. Epub 2018 Jun 5.

Division of Hematology/Oncology, Dana Farber and Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, MA 02215, USA. Electronic address:

Diamond-Blackfan anemia (DBA) is a severe congenital hypoplastic anemia caused by mutation in a ribosomal protein gene. Major clinical issues concern the optimal management of patients resistant to steroids, the first-line therapy. Hematopoietic stem cell transplantation is indicated in young patients with an HLA-matched unaffected sibling donor, and recent results with matched unrelated donor transplants indicate that these patients also do well. Read More

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http://dx.doi.org/10.1016/j.hoc.2018.04.005DOI Listing
August 2018
2 Reads

Preventing or Eradicating Factor VIII Antibody Formation in Patients with Hemophilia A: What Can We Learn from Other Disorders?

Semin Thromb Hemost 2018 Sep 25;44(6):531-543. Epub 2018 Jul 25.

Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands.

Eradication of factor VIII (FVIII) specific neutralizing antibodies (also known as inhibitors) by the traditional method of immune tolerance induction (ITI) is costly and unsuccessful in one out of three patients. Furthermore, effective inhibitor prevention strategies are presently lacking. An overview is given in this narrative review of antidrug antibody prevention or eradication strategies that have been used in disorders beyond hemophilia A, with the aim of analyzing what we can learn from these strategies for hemophilia A. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1666823
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http://dx.doi.org/10.1055/s-0038-1666823DOI Listing
September 2018
23 Reads

Secondary pure red cell aplasia in multiple myeloma treated with lenalidomide.

Leuk Res Rep 2018 7;10:4-6. Epub 2018 Jul 7.

First Department of Internal Medicine, Kansai Medical University, 2-5-1, Shin-machi, Hirakata, Osaka 573-1010, Japan.

Pure red cell aplasia (PRCA) is a rare disorder characterized by marked erythroid hypoplasia with maturation arrest in the bone marrow. Secondary acquired PRCA may be associated with hematologic disorders. A few case reports have described PRCA associated with multiple myeloma (MM). Read More

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http://dx.doi.org/10.1016/j.lrr.2018.06.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6037886PMC
July 2018
3 Reads

Successful treatment of refractory/relapsed acquired pure red cell aplasia with sirolimus.

Ann Hematol 2018 Nov 7;97(11):2047-2054. Epub 2018 Jul 7.

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.

Acquired pure red cell aplasia (aPRCA) is a kind of anemia characterized by severe reticulocytopenia and obvious bone marrow erythroblastic cells decreased. Some patients are refractory or intolerant to the first-line therapy (cyclosporine A with/without steroids). The effects of the second-line therapy are not satisfactory and sometimes not available. Read More

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http://dx.doi.org/10.1007/s00277-018-3431-5DOI Listing
November 2018
25 Reads

Del(5q) myelodysplastic syndrome combined with pure red cell aplasia.

Authors:
Joowon Park

Blood Res 2018 Jun 25;53(2):104. Epub 2018 Jun 25.

Department of Laboratory Medicine, Dankook University Hospital, Cheonan, Korea.

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http://dx.doi.org/10.5045/br.2018.53.2.104DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6021577PMC

Deficiency of Adenosine Deaminase 2 (DADA2): Updates on the Phenotype, Genetics, Pathogenesis, and Treatment.

J Clin Immunol 2018 Jul 27;38(5):569-578. Epub 2018 Jun 27.

Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, USA.

Deficiency of ADA2 (DADA2) is the first molecularly described monogenic vasculitis syndrome. DADA2 is caused by biallelic hypomorphic mutations in the ADA2 gene that encodes the adenosine deaminase 2 (ADA2) protein. Over 60 disease-associated mutations have been identified in all domains of ADA2 affecting the catalytic activity, protein dimerization, and secretion. Read More

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http://dx.doi.org/10.1007/s10875-018-0525-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6061100PMC
July 2018
21 Reads

Autoimmune cytopenias in chronic lymphocytic leukemia: a concise review and treatment recommendations.

Expert Rev Hematol 2018 08 14;11(8):613-624. Epub 2018 Jul 14.

a Department of Hematology and Lymphoma and Myeloma Center (LYMMCARE) , Academic Medical Center, University of Amsterdam , Amsterdam , The Netherlands.

Introduction: Chronic lymphocytic leukemia (CLL) is frequently complicated by cytopenias, either due to bone marrow infiltration or autoimmunity, resulting in autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), pure red cell aplasia (PRCA), or autoimmune neutropenia (AIN). Morbidity due to autoimmune cytopenias (AIC) can be substantial; in addition, infection risk increases and pre-existing infections might deteriorate due to immunosuppressive medication. In the aging population, CLL occurs more frequently and AIC related to CLL represent a growing clinical challenge. Read More

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http://dx.doi.org/10.1080/17474086.2018.1489720DOI Listing
August 2018
21 Reads

Intravenous Epoetin Alfa-epbx versus Epoetin Alfa for Treatment of Anemia in End-Stage Kidney Disease.

Clin J Am Soc Nephrol 2018 Aug 19;13(8):1204-1214. Epub 2018 Jun 19.

Global Clinical Development, Hospira Inc., a Pfizer company, Lake Forest, Illinois.

Background And Objectives: This study was conducted to compare the safety and efficacy of intravenous epoetin alfa-epbx, an epoetin alfa biosimilar, to epoetin alfa in patients on hemodialysis with ESKD and anemia.

Design, Setting, Participants, & Measurements: In this 24-week, multicenter, double-blind comparative efficacy and safety study, 612 patients on hemodialysis with ESKD and anemia who had stable hemoglobin and were receiving stable doses of intravenous epoetin alfa were randomized (1:1) to intravenous epoetin alfa or epoetin alfa-epbx. Dosing was adjusted according to the epoetin alfa prescribing information. Read More

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http://dx.doi.org/10.2215/CJN.11631017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6086700PMC
August 2018
15 Reads

Pure red cell aplasia with t-cell large granular lymphocytic leukemia.

J Biol Regul Homeost Agents 2018 May-Jun;32(3):589-597

Department of Anesthesia, China-Japan Union Hospital of Jilin University.

Pure red cell aplasia (PRCA) develops as a result of erythroid precursors failing to reach maturity in the bone marrow, which eventually leads to anemia. Here we present a case of a 64-year-old Asian male with a medical history of colorectal adenocarcinoma who had been treated with 6 cycles of oxaliplatin and capecitabine four years ago. The patient was diagnosed with PRCA and T-cell large granular lymphocyte leukemia. Read More

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August 2018
1 Read

Recovery of Pure Red Cell Aplasia Following Hematopoietic Stem Cell Transplantation Associated with Interleukin (IL)-6 Elevation Caused by Odontogenic Infection.

Intern Med 2018 Nov 6;57(21):3175-3177. Epub 2018 Jun 6.

Department of Hematology, Gifu University Graduate School of Medicine, Japan.

We herein report a case of long-lasting pure red cell aplasia (PRCA) after major ABO-incompatible allogeneic stem cell transplantation (SCT) for acute lymphoblastic leukemia. The patient needed red blood cell (RBC) transfusion every week after SCT. On day 236, he was diagnosed with odontogenic infection, and the serum levels of Interleukin (IL)-6 were elevated to 12. Read More

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http://dx.doi.org/10.2169/internalmedicine.0869-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6262699PMC
November 2018
4 Reads

Development of an anti-EPO antibody detection kit based on lab-on-a-chip and bridging antibody technologies.

Biologicals 2018 Jul 17;54:8-12. Epub 2018 May 17.

Department of Pharmacy, Integrated Research Institute of Pharmaceutical Sciences, and BK21 PLUS Team for Creative Leader Program for Pharmacomics-based Future Pharmacy, College of Pharmacy, The Catholic University of Korea, 43 Jibong-ro, Wonmi-gu, Bucheon-si, Gyeonggi-do, 14662, Republic of Korea; ILAb Inc., NP513, College of Pharmacy, The Catholic University of Korea, Bucheon, 420-743, Republic of Korea. Electronic address:

Immunogenicity is a major concern in the use of biological drugs. In particular, antibody-mediated pure red cell aplasia (PRCA) is a rare condition that is caused by administration of recombinant erythropoietin. There are numerous assay platforms for detect EPO anti-drug antibody (ADA), and most have appropriate assay sensitivity, but in need of improvement in terms of assay turnaround time and user accessibility. Read More

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http://dx.doi.org/10.1016/j.biologicals.2018.05.005DOI Listing
July 2018
17 Reads

[Effect of sirolimus on erythropoiesis of K562 cell line and patients with pure red cell aplasia ].

Zhonghua Xue Ye Xue Za Zhi 2018 Apr;39(4):310-313

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.

To understand the effect of sirolimus on the erythropoiesis of K562 cell line and bone marrow cells from pure red cell aplasia (PRCA) patients and normal controls. Different concentrations (10, 100, 1 000 nmol/L) of sirolimus were added to the K562 cell line or bone marrow cells from PRCA patients or normal controls and cultured 14 days for BFU-E formation. Meanwhile, sirolimus was also added to the serum treated PRCA bone marrow cells to cultivate for the same priod of time. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2018.04.011DOI Listing
April 2018
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Identification of mutations in patients with acquired pure red cell aplasia.

Acta Biochim Biophys Sin (Shanghai) 2018 Jul;50(7):685-692

Pathology Center, Shanghai General Hospital/Faculty of Basic Medicine, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Idiopathic acquired pure red cell aplasia (PRCA) is a rare, autoimmune-related disease. This study aimed to describe the previously unidentified DNA alterations associated with PRCA. Here, next generation sequencing using a panel containing 295 critical genes was applied to detect potentially pathogenic mutations in four patients with PRCA. Read More

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http://dx.doi.org/10.1093/abbs/gmy052DOI Listing
July 2018
4 Reads

Parvovirus B19 Induced Red Cell Aplasia in a Heart Transplant Patient Diagnosed on Pleural Fluid.

Transplantation 2018 08;102(8):e367-e368

Queensland Heart-Lung Transplant Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia.

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http://dx.doi.org/10.1097/TP.0000000000002277DOI Listing
August 2018
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The clinical characteristics and therapy response of patients with acquired pure red cell aplasia.

Hematology 2018 Oct 12;23(9):639-645. Epub 2018 May 12.

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

Objective: To summarize the clinical characteristics of acquired pure red cell aplasia (PRCA) patients diagnosed in our hospital in the last 10 years.

Method: The clinical features, immune state and treatment response of acquired PRCA patients diagnosed in our hospital from January 2007 to January 2017 were retrospectively analyzed.

Results: The results showed that thymoma (13. Read More

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http://dx.doi.org/10.1080/10245332.2018.1470068DOI Listing
October 2018
5 Reads