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Pure red cell aplasia

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https://www.readbyqxmd.com/read/28644950/hematopoietic-cell-transplantation-in-fanconi-anemia-and-dyskeratosis-congenita-a-minireview
#1
REVIEW
Mouhab Ayas
Bone marrow failure syndrome is an epithet of bone marrow failure (all or single-cell lineage) that is attributable to an underlying genetic aberration usually with a constellation of somatic abnormalities. Multiple inheritance patterns have been described in these disorders; many are transmitted in an autosomal recessive pattern, which may consequently lead to a higher prevalence of such illnesses in homogeneous societies such as Saudi Arabia, where consanguineous marriages are not uncommon. At King Faisal Specialist Hospital and Research Center, the most common entity referred for allogeneic hematopoietic cell transplantation (HCT) is Fanconi anemia, followed by pure red aplasia, and, less commonly, dyskeratosis congenita, congenital neutropenia, and others...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28637614/classical-inherited-bone-marrow-failure-syndromes-with-high-risk-for-myelodysplastic-syndrome-and-acute-myelogenous-leukemia
#2
REVIEW
Sharon A Savage, Carlo Dufour
The inherited marrow failure syndromes (IBMFS) are a heterogeneous group of diseases characterized by failure in the production of one or more blood lineage. The clinical manifestations of the IBMFS vary according to the type and number of blood cell lines involved, including different combinations of anemia, leukopenia, and thrombocytopenia. In some IBMFS, systemic non-hematologic manifestations, including congenital malformations, mucocutaneous abnormalities, developmental delay, and other medical complications, may be present...
April 2017: Seminars in Hematology
https://www.readbyqxmd.com/read/28598573/successful-treatment-of-tacrolimus-related-pure-red-cell-aplasia-and-autoimmune-hemolytic-anemia-with-rituximab-in-a-pediatric-cardiac-transplant-patient
#3
Chenue Abongwa, Ghada Abusin, Ayman El-Sheikh
Acquired pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) are rare complications of immunosuppression in pediatric solid organ transplant patients. We report a 14-month-old female child who developed Coombs positive hemolytic anemia and reticulocytopenia while on tacrolimus after cardiac transplantation. She was successfully treated with rituximab after failing treatment with corticosteroids and intravenous immunoglobulins. Clinicians should consider PRCA differential diagnosis in a patient presenting with reticulocytopenia and hemolysis...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28582594/histologic-and-cytologic-bone-marrow-findings-in-dogs-with-suspected-precursor-targeted-immune-mediated-anemia-and-associated-phagocytosis-of-erythroid-precursors
#4
Cynthia de A Lucidi, Christian L E de Rezende, L Ari Jutkowitz, Michael A Scott
BACKGROUND: Precursor-targeted immune-mediated anemia (PIMA) has been suspected in dogs with nonregenerative anemia and bone marrow findings varying from erythroid hyperplasia to pure red cell aplasia. Phagocytosis of erythroid precursors/rubriphagocytosis (RP) reported in some affected dogs suggests a destructive component to the pathogenesis of PIMA. OBJECTIVES: The purpose of the study was to characterize laboratory and clinical findings in dogs with suspected PIMA and RP, with emphasis on cytologic and histologic bone marrow findings...
June 5, 2017: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/28543448/successful-treatment-of-pure-red-cell-aplasia-because-of-abo-major-mismatched-stem-cell-transplant
#5
Katie Sackett, Claudia S Cohn, Kayla Fahey-Ahrndt, Angela R Smith, Andrew D Johnson
BACKGROUND: Pure red cell aplasia (PRCA) is a well-documented potential side effect of ABO major mismatched allogeneic hematopoietic stem cell transplants. This side effect may be self-limiting, but is sometimes treated using modalities such as steroids, antithymocyte globulin, donor lymphocyte infusions, rituximab, or plasma exchanges. Another well-documented cause of pure red cell aplasia is a chronic parvovirus B19 infection, which may be seen in immunocompromised hosts. The treatment of this cause of PRCA includes removal of immunosuppression, intravenous immunoglobulin (IVIg), or rituximab; however, this condition may also be self-limiting...
May 24, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28525589/bone-marrow-involvement-in-systemic-lupus-erythematosus
#6
Emilie Chalayer, Nathalie Costedoat-Chalumeau, Odile Beyne-Rauzy, Jacques Ninet, Stephane Durupt, Jacques Tebib, Bouchra Asli, Olivier Lambotte, Martine Ffrench, Christian Vasselon, Pascal Cathébras
BACKGROUND: Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia, and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a target organ in SLE. Our objective was to describe this bone marrow involvement. METHODS: This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome...
May 19, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28488966/haemolysis-pure-red-cell-aplasia-and-red-cell-antibody-formation-associated-with-major-and-bidirectional-abo-incompatible-haematopoietic-stem-cell-transplantation
#7
Gordana Tomac, Ines Bojanić, Sanja Mazić, Ivana Vidović, Mirela Raos, Branka Golubić Ćepulić, Ranka Serventi Seiwerth, Jadranka Kelečić, Boris Labar
BACKGROUND: Acute and delayed haemolysis, alloimmunisation and pure red cell aplasia (PRCA) are potential complications after ABO incompatible haematopoietic stem cell transplantation (HSCT). The aims of this study were to investigate acute and delayed red blood cell (RBC) antibody-associated complications, including haemolysis, PRCA and alloimmunisation in major and bidirectional ABO incompatible HSCT. MATERIALS AND METHODS: We retrospectively examined the transplant courses of 36 recipients of bone marrow or peripheral blood stem cells from ABO incompatible donors and evaluated the current practice of performing plasmapheresis in patients with higher isoagglutinin titres...
April 19, 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28473943/cyclosporine-is-a-potential-curative-treatment-option-for-advanced-thymoma
#8
Yusuke Isshiki, Hiroaki Tanaka, Yoshio Suzuki, Yukihiro Yoshida
BACKGROUND: Thymectomy can effectively cure most thymoma patients; however, patients with advanced thymoma typically require chemotherapy, which is associated with limited efficacy in this context. Here we provide the first report of a patient with recurrent thymoma who achieved complete remission (CR) using cyclosporine therapy. CASE PRESENTATION: A 63-year-old woman who had undergone resection surgery for recurrent type B1 thymoma developed pure red cell aplasia (PRCA), and CT findings revealed thymoma recurrence...
2017: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/28450687/thymoma-with-immunodeficiency-good-syndrome-associated-with-myasthenia-gravis
#9
Shunsuke Takai, Asako Tagawa, Tomoko Ogawa, Hiroyuki Kato, Noriko Saito, Shinya Okada
Good syndrome is a rare condition in which thymoma is associated with hypogammaglobulinemia; it is characterized by repeated respiratory or systemic infections caused by bacteria, viruses, and fungi, as well as with various autoimmune disorders such as pure red cell aplasia. A 65-year-old woman was admitted to our hospital with ptosis and abdominal muscle weakness. Based on the presence of anti-acetylcholine receptor (Ach-R) antibodies, she was diagnosed with myasthenia gravis (MG). At that time, invasive thymoma of Masaoka stage IVa was also detected...
April 27, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28425751/development-of-red-blood-cell-autoantibodies-following-treatment-with-checkpoint-inhibitors-a-new-class-of-anti-neoplastic-immunotherapeutic-agents-associated-with-immune-dysregulation
#10
Laura L Cooling, John Sherbeck, Jonathon C Mowers, Sheri L Hugan
Ipilimumab, nivolumab, and pembrolizumab represent a new class of immunotherapeutic drugs for treating patients with advanced cancer. Known as checkpoint inhibitors, these drugs act to upregulate the cellular and humoral immune response to tumor antigens by inhibiting T-cell autoregulation. As a consequence, they can be associated with immune-related adverse events (irAEs) due to loss of self-tolerance, including rare cases of immune-related cytopenias. We performed a retrospective clinical chart review, including serologic, hematology, and chemistry laboratory results, of two patients who developed red blood cell (RBC) autoantibodies during treatment with a checkpoint inhibitor...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28387173/pure-red-cell-aplasia-due-to-parvovirus-b19-erythropoietin-resistant-anemia-in-a-pediatric-kidney-recipient
#11
Baris Malbora, Serdar Saritas, Eda Ataseven, Burcu Belen, Caner Alparslan, Onder Yavascan, Dilek Yilmaz Ciftdogan, Berna Atabay, Meral Turker
No abstract text is available yet for this article.
June 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28382272/pure-red-cell-aplasia-associated-with-good-syndrome
#12
Masayuki Okui, Takashi Yamamichi, Ayaka Asakawa, Masahiko Harada, Hirotoshi Horio
Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.
April 2017: Korean Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28256075/the-use-of-darbepoetin-to-stimulate-erythropoiesis-in-the-treatment-of-anemia-of-chronic-kidney-disease-in-dogs
#13
E H Fiocchi, L D Cowgill, D C Brown, J E Markovich, S Tucker, M A Labato, M B Callan
BACKGROUND: Darbepoetin alfa (darbepoetin) is an erythropoiesis-stimulating agent used for the treatment of anemia secondary to chronic kidney disease (CKD) in dogs, but reports describing response are lacking. HYPOTHESIS/OBJECTIVES: To evaluate the effectiveness of darbepoetin in dogs with anemia secondary to CKD, dosing protocols, and adverse events. ANIMALS: Thirty-three client-owned dogs with naturally occurring CKD, including 26 with comorbidities...
March 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28230570/refractory-pure-red-cell-aplasia-manifesting-as-deficiency-of-adenosine-deaminase-2
#14
Hasan Hashem, Rachel Egler, Jignesh Dalal
Primary progress has been made in the last 2 years, particularly in finding novel disease-causing genes for a number of autoinflammatory diseases and primary immunodeficiencies. Whole-exome sequencing has dramatically increased the pace at which causative genes are being discovered. CECR1 (Cat eye syndrome chromosome region, candidate 1) gene encodes adenosine deaminase 2 (ADA2) protein. Patients who carry CECR1 mutation(s) suffer from deficiency of ADA2 (DADA2). Here, we describe a patient with pure red cell aplasia discovered to have DADA2...
February 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28217621/refractory-anemia-in-human-immunodeficiency-virus-expect-the-unexpected
#15
Sumeet Prakash Mirgh, Vikas A Mishra, Virti D Shah, Jehangir Soli Sorabjee
Pure red cell aplasia (PRCA) is an uncommon hematological disorder affecting selectively the erythroid cell lines. PRCA is defined as anemia with normal leukocyte and platelet counts, a corrected reticulocyte count <1%, <5% erythroid precursors in the bone marrow and an absence of hemolysis. We describe a case of Zidovudine (AZT) induced PRCA causing severe anemia in a patient taking antiretroviral therapy (ART) after 4 months of starting therapy and in whom all other causes were excluded. The hematological abnormalities resolved after AZT was replaced with tenofovir and the patient remained transfusion independent thereafter...
July 2016: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/28197963/a-concise-review-of-autoimmune-cytopenias-in-chronic-lymphocytic-leukemia
#16
REVIEW
Mazie Tsang, Sameer A Parikh
Chronic lymphocytic leukemia (CLL) is frequently associated with autoimmune complications such as autoimmune hemolytic anemia, immune thrombocytopenia, pure red cell aplasia, and autoimmune granulocytopenia. It is critical to diagnose cytopenias from these secondary complications of CLL accurately, since prognosis and therapy are substantially different from patients who have cytopenias due to extensive bone marrow infiltration by CLL. The pathogenesis of autoimmune cytopenias in CLL is complex; and it involves antigen presentation by CLL cells to polyclonal B cells resulting in production of autoantibody, and alteration of the T cell milieu tilting the balance in favor of an autoimmune response...
February 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28191814/the-pathophysiologic-basis-of-anaemia-in-patients-with-malignant-diseases
#17
Umma A Ibrahim, Aminu A Yusuf, Sagir G Ahmed
Cancer patients frequently present with anaemia that may result from the direct or indirect effects of the tumor or its treatment. Anaemia is an independent adverse prognostic factor that exerts negative influence on quality of life and survival of cancer patients. Anaemia in malignant disorders often arises from an interplay of multiple aetiological and pathophysiologic mechanisms. Understanding these mechanisms will help the oncologist identify and treat specific causes of the anaemia thereby minimizing the use of blood transfusion, which is associated with many adverse effects...
September 2016: Gulf Journal of Oncology
https://www.readbyqxmd.com/read/28182355/pure-red-cell-aplasia-associated-with-monoclonal-gammopathy-of-undetermined-significance-and-literature-review
#18
Hyunjung Gu, Woo-In Lee, You La Jeon, So Young Kang, Myeong Hee Kim, Tae Sung Park
BACKGROUND: Pure red cell aplasia (PRCA) is an uncommon disease which involves an almost complete absence of the erythroid lineage in bone marrow (BM) and causes severe anemia. Cases due to monoclonal gammopathy occurring in plasma cell disorder have been infrequently reported. Here we report a case of PRCA associated plasma cell disorder, especially monoclonal gammopathy of undetermined significance (MGUS). METHODS: A 55-year-old male visited the ER due to general weakness...
February 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28160319/higher-anti-a-b-isoagglutinin-titers-of-igg-class-but-not-of-igm-are-associated-with-increased-red-blood-cell-transfusion-requirements-in-bone-marrow-transplantation-with-major-abo-mismatch
#19
Gil Cunha De Santis, Aline Cristina Garcia-Silva, Giuliana Martinelli Dotoli, Pamela Tinti de Castro, Belinda Pinto Simões, Dimas Tadeu Covas
BACKGROUND: Major ABO mismatch between donor and recipient in bone marrow transplantation (BMT) may cause hemolysis, delayed red blood cell (RBC) engraftment and pure red cell aplasia (PRCA), which result in increased transfusion needs. High pretransplant anti-A/B antibody titers have been associated with increased risk of PRCA. Herein, we studied the impact of anti-A/B titers on transfusion needs after BMT with major ABO mismatch. METHODS: We reviewed the medical charts of 27 patients who underwent to BMT with major ABO mismatch and categorized them into two groups according to anti-A/B titers of IgG (≤16 and ≥32)...
April 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28144099/thymoma-masquerading-as-transfusion-dependent-anemia
#20
Javvid Muzamil, Aejaz Aziz Shiekh, Gull Mohammad Bhat, Abdul Rashid Lone, Shuaeb Bhat, Firdousa Nabi
Pure red cell aplasia (PRCA) is a known entity in clinical medicine. Patients are often transfusion dependent for their whole life. Ascertaining its etiology is always a herculean task. We received a similar transfusion-dependent patient, who on evaluation was found to have thymoma as an etiological factor. Thymoma presenting as PRCA is seen in 2%-5% patients and evaluating PRCA for thymoma is seen in 5%-13% patient. As per the WHO histopathological classification, thymoma has six types and Type A is associated with PRCA and Type B is associated with myasthenia gravis...
October 2016: Indian Journal of Medical and Paediatric Oncology
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