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Pure red cell aplasia

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https://www.readbyqxmd.com/read/28256075/the-use-of-darbepoetin-to-stimulate-erythropoiesis-in-the-treatment-of-anemia-of-chronic-kidney-disease-in-dogs
#1
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 3, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28230570/refractory-pure-red-cell-aplasia-manifesting-as-deficiency-of-adenosine-deaminase-2
#2
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
#3
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
#4
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 14, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28191814/the-pathophysiologic-basis-of-anaemia-in-patients-with-malignant-diseases
#5
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
#6
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
#7
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)...
February 3, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28144099/thymoma-masquerading-as-transfusion-dependent-anemia
#8
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
https://www.readbyqxmd.com/read/28053696/invasive-thymoma-with-pure-red-cell-aplasia-and-amegakaryocytic-thrombocytopenia
#9
Takuya Onuki, Yusuke Kiyoki, Sho Ueda, Masatoshi Yamaoka, Seiich Shimizu, Masaharu Inagaki
We here describe a case involving a 67-yearold female patient who was referred to our hospital due to severe anemia (hemoglobin, 5.0 g/dL), thrombocytopenia (platelet count, 0.6 × 10(4)/μL), and a mediastinal shadow with calcification noted on X-ray. On admission, an anterior mediastinal tumor was detected, and bone marrow biopsy revealed few megakaryocytes and severely reduced numbers of erythroid cells. The diagnosis was thymoma with pure red cell aplasia (PRCA) and acquired amegakaryocytic thrombocytopenia (AAMT)...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/28042550/pure-red-cell-aplasia-due-to-antibody-against-erythropoietin-in-hemodialysis-patients
#10
Maryam Rahbar, Zahra Chitsazian, Firoozeh Abdoli, Seyed-Masoud Moeini Taba, Hosein Akbari
BACKGROUND: Anemia is a common complication of chronic renal failure due to reduce erythropoietin production by kidneys. Anemia treated with recombinant human erythropoietin (rHu-EPO). Pure red cell aplasia (PRCA) due to antibody productionagainst rHu-EPO is a rare but major complication of this drug. OBJECTIVES: The aim of this study was to determine the prevalence of PRCA due to antibodies in dialysis patients with resistant anemia who received erythropoietin...
January 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/27932518/epoetin-%C3%AE-induced-pure-red-cell-aplasia-an-unintended-consequence
#11
Muhammad Masoom Javaid, Priyanka Khatri, Srinivas Subramanian
Pure red cell aplasia is a rare condition associated with the use of recombinant human erythropoietin preparations. It has predominantly been associated with the subcutaneous use of a particular epoetin-α product, Eprex, and is rarely associated with intravenous use or with other commercially available products. Only a few cases of pure red cell aplasia secondary to epoetin-β have been reported. On account of its rarity, the condition can often be missed on initial presentation, leading to unnecessary investigations and delayed diagnosis...
March 2017: Postgraduate Medical Journal
https://www.readbyqxmd.com/read/27913462/pure-red-cell-aplasia
#12
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27882484/diagnostic-challenge-of-diamond-blackfan-anemia-in-mothers-and-children-by-whole-exome-sequencing
#13
Takuya Ichimura, Kenichi Yoshida, Yusuke Okuno, Toshiaki Yujiri, Kozo Nagai, Masanori Nishi, Yuichi Shiraishi, Hiroo Ueno, Tsutomu Toki, Kenichi Chiba, Hiroko Tanaka, Hideki Muramatsu, Toshiro Hara, Hitoshi Kanno, Seiji Kojima, Satoru Miyano, Etsuro Ito, Seishi Ogawa, Shouichi Ohga
Diamond-Blackfan anemia (DBA) is a pure red cell aplasia that arises from defective ribosomal proteins (RPs). Patients with this rare ribosomopathy present with neonatal anemia and occasional dysmorphism. Clinical heterogeneity and clusters of causative RP genes hamper the diagnosis and perinatal management. We report three mother-and-child pairs of anemia who were finally diagnosed by whole-exome sequencing. Each pair showed distinct disease severity and response to anemia treatment. Only one mother had the diagnostic dysmorphism, including short stature, webbed neck, and thenar hypoplasia...
April 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/27881371/pure-red-cell-aplasia
#14
REVIEW
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
November 24, 2016: Blood
https://www.readbyqxmd.com/read/27843612/tki-induced-pure-red-cell-aplasia-first-case-report-of-pure-red-cell-aplasia-with-both-imatinib-and-nilotinib
#15
Bishesh Sharma Poudyal, Sampurna Tuladhar, Bishal Gyawali
Tyrosine-kinase inhibitors (TKIs) represent the only hopes for long-term survival for patients with chronic myeloid leukaemia (CML) and gastrointestinal stromal tumours. Thus, uninterrupted use of TKIs is of importance in such patients. Pure red cell aplasia (PRCA) is a rare disorder, not previously known to be associated with TKIs. We present, to the best of our knowledge, the first case of a patient with CML who developed PRCA secondary to both imatinib and nilotinib. Although PRCA was controlled on withdrawal of TKI, TKI continuation in the patient with CML is important...
2016: ESMO Open
https://www.readbyqxmd.com/read/27756158/refractory-pure-red-cell-aplasia-associated-with-chronic-lymphocytic-leukemia-successfully-treated-with-ibrutinib
#16
Neta Goldschmidt, Deborah Rund
No abstract text is available yet for this article.
February 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27648265/pure-red-cell-aplasia-caused-by-parvovirus-b19-in-a-heart-transplant-recipient
#17
Rosangela Invernizzi, Raffaella Bastia, Federica Quaglia
A case of parvovirus B19-induced pure red cell aplasia occurring in a heart transplant recipient is reported. The diagnosis of this rare but clinically important complication can be suspected on the basis of the pathognomonic morphological features of the bone marrow.
September 2016: Clinical Case Reports
https://www.readbyqxmd.com/read/27642338/linezolid-induced-twice-pure-red-cell-aplasia-in-a-patient-with-central-nervous-system-infection-after-allogeneic-stem-cell-transplantation
#18
Wenqing Hu, Bing Shi, Lihui Liu, Shengke He, Liping Ye, DengMei Tian, Yongqing Zhang
Linezolid (LZD), severed as the first oxazolidinone antibiotic, was active against multidrug-resistant gram-positive strains. LZD can induce thrombocytopenia, anemia and leukocytopenia. Currently, reports on pure red cell aplasia (PRCA) cases induced by LZD are relatively rare (4-7). In this paper, we reported a patient with PRCA twice induced by LZD. A 37-year-old man was diagnosed with myelodysplatic syndrome (MDS) and underwent allo-HSCT from an unrelated donor with ABO blood type and leukocyte antigen (HLA)-matching...
2016: Iranian Journal of Pharmaceutical Research: IJPR
https://www.readbyqxmd.com/read/27601194/critical-diamond-blackfan-anemia-due-to-ribosomal-protein-s19-missense-mutation
#19
Shuichi Ozono, Miho Mitsuo, Maiko Noguchi, Shin-Ichiro Nakagawa, Koichiro Ueda, Hiroko Inada, Shouichi Ohga, Etsuro Ito
Diamond-Blackfan anemia (DBA) is a rare congenital disorder characterized by pure erythrocyte aplasia, and approximately 70% of patients carry mutations in the genes encoding ribosomal proteins (RP). Here, we report the case of a male infant with DBA who presented with anemic crisis (hemoglobin [Hb] concentration 1.5 g/dL) at 58 days after birth. On admission, the infant was pale and had tachypnea, but recovered with intensive care, including red blood cell transfusions, and prednisolone. Based on the clinical diagnosis of DBA, the father of the infant had cyclosporine-A-dependent anemia...
September 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27542218/methotrexate-therapy-of-t-cell-large-granular-lymphocytic-leukemia-impact-of-stat3-mutation
#20
Zhi-Yuan Qiu, Lei Fan, Rong Wang, Robert Peter Gale, Hua-Jin Liang, Man Wang, Li Wang, Yu-Jie Wu, Chun Qiao, Yao-Yu Chen, Wei Xu, Jun Qian, Jian-Yong Li
T-cell large granular lymphocytic leukemia (T-LGLL) is a rare haematologic neoplasm. Consequntly, there are no large prospective studies of therapy and no uniform therapy recommendations. We analyzed data from 36 subjects receiving methotrexate alone (N = 27) or with prednisone (N = 9) as initial therapy. 31 subjects responded (86%, 95% confidence interval [CI], 73, 95%) with 8 complete responses and 23 partial responses. Median time-to-response was 3 months (range, 1-5 months). Median response duration was 20 months (range, 2-55 months)...
September 20, 2016: Oncotarget
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