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Pure red cell aplasia

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https://www.readbyqxmd.com/read/27932518/epoetin-%C3%AE-induced-pure-red-cell-aplasia-an-unintended-consequence
#1
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...
December 8, 2016: Postgraduate Medical Journal
https://www.readbyqxmd.com/read/27913462/pure-red-cell-aplasia
#2
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
#3
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...
November 23, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27881371/pure-red-cell-aplasia
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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)...
August 17, 2016: Oncotarget
https://www.readbyqxmd.com/read/27514238/extending-the-clinical-phenotype-of-adenosine-deaminase-2-deficiency
#11
Tal Ben-Ami, Shoshana Revel-Vilk, Rebecca Brooks, Avraham Shaag, Michael S Hershfield, Susan J Kelly, Nancy J Ganson, Shlomit Kfir-Erenfeld, Michael Weintraub, Orly Elpeleg, Yackov Berkun, Polina Stepensky
Adenosine deaminase 2 deficiency is an autoinflammatory disease, characterized by various forms of vasculitis. We describe 5 patients with adenosine deaminase 2 deficiency with various hematologic manifestations, including pure red cell aplasia, with no evidence for vasculitis.
October 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27512715/dapsone-induced-pure-red-cell-aplasia-and-cholestatic-jaundice-a-new-experience-for-diagnosis-and-management
#12
Kamal Kumar Sawlani, Shyam Chand Chaudhary, Jitendra Singh, Deep Chandh Raja, Sanjay Mishra, Madhu Mati Goel
Dapsone (4,4'- diaminodiphenylsulfone) is the parent compound of the sulfones, and it has potent antiparasitic, anti-inflammatory, and immunomodulatory effects. It is used in the treatment of leprosy, dermatitis herpetiformis, and prophylactically to prevent Pneumocystis pneumonia and toxoplasmosis in patients unable to tolerate trimethoprim with sulfamethoxazole. We hereby report a case of dapsone toxicity who developed pure red cell aplasia and cholestatic jaundice in a suspected case of dermatitis herpetiformis...
July 2016: Journal of Research in Pharmacy Practice
https://www.readbyqxmd.com/read/27478605/post-renal-transplant-pure-red-cell-aplasia-is-tacrolimus-a-culprit
#13
Malagouda R Patil, Arpita Roy Choudhury, Manong Chohwanglim, Smita Divyaveer, Chetan Mahajan, Rajendra Pandey
Anemia is not uncommon in the post-renal transplant period and has been reported in up to 40% of renal transplant recipients. It is commonly due to drugs and infections. While post-transplantation anemia is usually due to graft dysfunction and drugs such as mycophenolate and cotrimoxazole, tacrolimus is an uncommon cause. Tacrolimus is usually not believed to be significantly myelosuppressive, but it can cause anemia due to thrombotic microangiopathy. A literature review shows a very small number of reported cases of pure red cell aplasia (PRCA) where tacrolimus seemed to be a causative agent...
August 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27478604/pure-red-cell-aplasia-induced-by-epoetin-zeta
#14
Vincenzo Panichi, Guido Ricchiuti, Alessia Scatena, Lucia Del Vecchio, Francesco Locatelli
Pure red cell aplasia (PRCA) may develop in patients with chronic kidney disease receiving erythropoiesis-stimulating agents (ESA). We report on a 72-year-old patient who developed hypo-proliferative anaemia unresponsive to ESA following the administration of epoetin zeta subcutaneously for 7 months. On the basis of severe isolated hypoplasia of the erythroid line in the bone marrow and high-titre neutralizing anti-erythropoietin antibodies (Ab), a diagnosis of Ab-mediated PRCA was made. Epoetin zeta was discontinued and the patient was given steroids...
August 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27457977/chronic-preclinical-safety-evaluation-of-epo-018b-a-pegylated-peptidic-erythropoiesis-stimulating-agent-in-monkeys-and-rats
#15
Xue-Lian Gong, Xiao-Lei Gu, Yong-Chun Chen, Hai Zhu, Zhen-Na Xia, Jian-Zhong Li, Guo-Cai Lu
EPO-018B, a synthetic peptide-based erythropoiesis stimulating agent (ESA), is mainly designed for treatment of anemia caused by chronic renal failure and chemotherapy against cancer. It overcomes the deficiencies of currently approved ESA, including the frequent administration of temperature-sensitive recombinant protein and anti-EPO antibody-mediated pure red cell aplasia (PRCA). This study was designed to evaluate the potential chronic toxicity of EPO-018B. Subcutaneous administration doses were designed as 0, 0...
September 15, 2016: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/27408396/thymoma-with-concomitant-pure-red-cell-aplasia-good-s-syndrome-and-myasthenia-gravis-responding-to-rituximab
#16
Ahmad I Antar, Zaher K Otrock, Mohamed A Kharfan-Dabaja, Rami A Mahfouz, Raafat S Alameddine, Nadim M W El-Majzoub, Ziad M Salem
Thymomas are often associated with a variety of autoimmune diseases, mostly myasthenia gravis. The association of thymomas with both pure red cell aplasia (PRCA) and Good's syndrome is exceedingly rare. To the best of our knowledge, the combination of a thymoma with manifestations of myasthenia gravis, PRCA, and Good's syndrome, as in our case herein, has not been described before in the medical literature. We present a 90-year-old man initially diagnosed with an asymptomatic thymoma. Later, he developed generalized muscle weakness and was found to have severe anemia...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27384857/successful-high-dose-dexamethasone-treatment-of-acquired-pure-red-cell-aplasia-following-abo-major-mismatched-allogeneic-hematopoietic-stem-cell-transplantation
#17
Yuki Hiroshima, Yousuke Shiono, Ikuko Suzuki, Masakazu Yamamoto, Yuichi Kato, Katsushi Tajima, Takeo Kato
A 46-year-old man was diagnosed with acute myelomonocytic leukemia involving inv(16)(p13.1q22) in August 2007. He received a human leukocyte antigen-identical, ABO major-mismatched (donor: A, recipient: O) bone marrow transplantation from an unrelated donor in June 2009. Cyclosporin A (CsA) and short-course methotrexate were used for graft versus host disease prophylaxis. Although granulocyte and platelet engraftment were achieved, the patient exhibited persistent anemia and was dependent on red blood cell (RBC) transfusions...
June 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27382560/primary-acquired-chronic-pure-red-cell-aplasia-refractory-to-standard-treatments-remission-with-rituximab
#18
Andrea Tendas, Pasquale Niscola, Laura Scaramucci, Luca Cupelli, Alessio Pio Perrotti, Paolo de Fabritiis
No abstract text is available yet for this article.
June 2016: Blood Research
https://www.readbyqxmd.com/read/27348021/a-cluster-of-epoetin-associated-pure-red-cell-aplasia-clinical-features-and-the-possible-association-of-hla-drb1-12-02
#19
Chuen Wen Tan, Wei-Chuen Tan-Koi, Jennifer Ng, Choong Meng Chan, William Ying Khee Hwang
Antibody-mediated pure red cell aplasia is a rare but serious complication in chronic kidney disease patients receiving recombinant human erythropoietin (r-HuEpo). Between April 2012 and May 2013, eight such cases were reported in our institution. Their clinical features were reviewed and their HLA alleles were compared with those of healthy controls. All patients were exposed to epoetin alfa (Eprex(®)) with polysorbate-80 as stabilizer via subcutaneous route with a mean age of 61.9 years and mean exposure of 11...
June 27, 2016: Pharmacogenomics
https://www.readbyqxmd.com/read/27338269/pure-red-cell-aplasia-induced-by-anti-erythropoietin-antibodies-well-controlled-with-tacrolimus
#20
Koji Hashimoto, Makoto Harada, Yuji Kamijo
Anti-erythropoietin (anti-EPO) antibody-related pure red cell aplasia (PRCA) is a rare but serious complication that can occur during the administration of erythropoiesis-stimulating agents. Treatment with the calcineurin inhibitor cyclosporine has shown benefits in patients with anti-EPO PRCA. The efficacy of tacrolimus, another calcineurin inhibitor, in patients with anti-EPO PRCA has not been determined. The present report is the first our knowledge to describe the successful treatment of a patient with anti-EPO PRCA using tacrolimus...
October 2016: International Journal of Hematology
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