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hemolytic anemias

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https://www.readbyqxmd.com/read/28078984/rasburicase-induced-methemoglobinemia-in-a-patient-with-glucose-6-phosphate-dehydrogenase-deficiency
#1
Maliha Khan, Shilpa Paul, Saad Farooq, Thein Hlaing Oo, Priya Ramshesh, Nitin Jain
BACKGROUND: Rasburicase is commonly used in patients with hematologic malignancies for tumor lysis syndrome prophylaxis and management. Methemoglobinemia is a serious rare adverse effect of rasburicase, more common in patients with G6PD deficiency. Prompt diagnosis and appropriate management of this condition can make the difference between successful recovery and significant morbidity. Here we discuss the link of rasburicase with methemoglobinemia and the pathophysiology behind increased incidence of this side effect in G6PD deficient patients...
January 11, 2017: Current Drug Safety
https://www.readbyqxmd.com/read/28073728/biermer-anemia-hematologic-characteristics-of-66-patients-in-a-clinical-hematology-unit-at-senegal
#2
F Seynabou, N Fatou Samba Diago, D Oulimata Diop, S Abibatou Fall, D Nafissatou
Hematological manifestations can lead to diagnosis of pernicious anemia, also known as Biermer disease and Biermer anemia. This disease has been little studied among black Africans. Our aim is to describe its diagnostic and therapeutic aspects and outcome in our practice. This descriptive study retrospectively examined the records of 66 patients with pernicious anemia seen at the Clinical Hematology Unit of Le Dantec Hospital in Senegal from January 1, 2000, to June 30, 2014. Symptoms were anemic syndrome (40 cases), hemolytic anemia (13), anemic heart failure (7), isolated pallor of the mucous membranes (5), and venous thrombosis (2)...
November 1, 2016: Médecine et Santé Tropicales
https://www.readbyqxmd.com/read/28072956/-clinical-and-immunological-analysis-of-the-patient-with-autoimmunity-due-to-germline-stat3-gain-of-function-mutation
#3
Y Ding, Y Zhang, Y P Wang, H Y Zhao, X M Chen, X H Xue, X M Bai, Y F An, Z Y Zhang, X M Tang, X D Zhao
Objective: To investigate the clinical and immunological laboratory features and gene mutation in a female patient who carried a germline gain-of-function mutation in STAT3. Method: A patient with lymphadenopathy and pancytopenia, visited the Department of Rheumatology and Immunology of Children's Hospital of Chongqing Medical University in May 2016. The clinical and laboratory characteristics, results of immunophenotyping and exome sequencing were analyzed retrospectively and related literature was reviewed...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28070879/a-review-of-pharmacogenetics-of-antimalarials-and-associated-clinical-implications
#4
REVIEW
Hazem Elewa, Kyle John Wilby
Genetic variability in drug-metabolizing enzymes and drug transporters is known to influence the pharmacokinetics of many drugs. Antimalarial drugs are a class of agents known to utilize metabolic and elimination pathways prone to genetic variation. This paper aims to review the genetic variants affecting antimalarial medications and discuss their clinical implications. Data were identified for the genes coding for the cytochrome P450 (CYP) enzymes: CYP2C8, CYP2C19, CYP2A6, CYP2D6, CYP2B6, and the P-glycoprotein drug transporter...
January 9, 2017: European Journal of Drug Metabolism and Pharmacokinetics
https://www.readbyqxmd.com/read/28069451/determination-of-extinction-coefficients-of-human-hemoglobin-in-various-redox-states
#5
Fantao Meng, Abdu I Alayash
The role of hemoglobin (Hb) redox forms in tissue and organ toxicities remain ambiguous despite the well-documented contribution of Hb redox reactivity to cellular and subcellular oxidative changes. Moreover, several recent studies, in which Hb toxicity were investigated, have shown conflicting outcomes. Uncertainties over the potential role of these species may in part be due to the protein preparation method of choice, the use of published extinction coefficients and the lack of suitable controls for Hb oxidation and heme loss...
January 6, 2017: Analytical Biochemistry
https://www.readbyqxmd.com/read/28063954/autoimmune-cytopenias-associated-with-inflammatory-bowel-diseases-insights-from-a-multicenter-retrospective-cohort
#6
Mathieu Uzzan, Lionel Galicier, Jean-Marc Gornet, Eric Oksenhendler, Claire Fieschi, Matthieu Allez, Yoram Bouhnik, Julien Kirchgesner, David Boutboul, Xavier Treton, Laurence Gérard, Matthieu Mahévas, Jacques Cosnes, Aurélien Amiot
INTRODUCTION: Autoimmune cytopenias (AIC) including autoimmune hemolytic anemia (AIHA) and immunologic thrombocytopenia (ITP) are rare immunologic disorders, scarcely reported in inflammatory bowel diseases (IBD). We conducted a multicentric retrospective study, including a case-control analysis, that aimed to describe the characteristics and outcomes of patients affected by AIC and IBD. METHOD: Forty cases were recruited from 4 IBD centers and 2 AIC tertiary centers...
December 23, 2016: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28060122/coinheritance-of-hereditary-elliptocytosis-and-deletional-hemoglobin-h-disease
#7
Pimlak Charoenkwan, Rungrote Natesirinilkul, Worawut Choeyprasert, Natchanon Kulsumritpon, Orapan Sangiamporn
Hereditary elliptocytosis is an inherited red blood cell membrane disorder characterized by typical peripheral blood smear findings of elliptocytes or rod-like red blood cells. Hemoglobin H disease is a form of α-thalassemia disease resulting in mild to moderate hemolytic anemia. The authors report 1 case of a girl who was diagnosed with oculo-auriculo-vertebral spectrum and a coinheritance of hereditary elliptocytosis and deletional hemoglobin H disease. She had moderate, non-transfusion-dependent anemia...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28060108/diagnostic-utility-of-complement-immunohistochemical-studies-in-post-stem-cell-transplant-intestinal-thrombotic-microangiopathy-case-report
#8
Jenna E Rossoff, Jennifer Schneiderman, Sonali Chaudhury, Nicoleta C Arva
Thrombotic complications are a significant source of morbidity and mortality following hematopoietic stem cell transplants. Among them, transplant-associated thrombotic microangiopathy (TA-TMA) is a well-recognized syndrome that can affect various organ systems. Its etiology is related to endothelial injury accompanied by complement activation. As many of the signs and symptoms of the disease are also encountered in other complications following hematopoietic stem cell transplant, it can often be difficult to establish the diagnosis based on clinical data alone...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28059001/possible-association-of-3-utr-357-a-g-ivs11-nt-93-t-c-c-1311-c-t-polymorphism-with-g6pd-deficiency
#9
Mahmoud M Sirdah, Mohammad E Shubair, Mustafa S Al-Kahlout, Jamal M Al-Tayeb, Josef T Prchal, N Scott Reading
BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common X-linked inherited enzymopathic disorder affecting more than 500 million people worldwide. It has so far been linked to 217 distinct genetic variants in the exons and exon-intron boundaries of the G6PD gene, giving rise to a wide range of biochemical heterogeneity and clinical manifestations. OBJECTIVES: Reports from different settings suggested the association of intronic and other mutations outside the reading frame of the G6PD gene with reduced enzyme activity and presenting clinical symptoms...
January 6, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28045688/pseudo-thrombotic-thrombocytopenic-purpura-due-to-severe-vitamin-b12-deficiency
#10
Paul A Trubin, Justin A Edward, Monica Hand
As a subset of microangiopathic hemolytic anemia, thrombotic thrombocytopenic purpura can present with a constellation of symptoms similar to the hemolytic anemia attributed to severe vitamin B12 deficiency. There have been few case reports in the medical literature concerning the development of a clinical syndrome consistent with microangiopathic hemolytic anemia attributable to B12 deficiency. We report a case of B12 deficiency leading to microangiopathic hemolytic anemia that was corrected solely with a ten-day course of intramuscular cobalamin replacement...
November 2016: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28028749/oral-cyclophosphamide-was-effective-for-coombs-negative-autoimmune-hemolytic-anemia-in-cd16-cd56-chronic-lymphoproliferative-disorder-of-nk-cells
#11
Nodoka Sekiguchi, Sayaka Nishina, Toru Kawakami, Hitoshi Sakai, Noriko Senoo, Yasushi Senoo, Toshiro Ito, Hiroshi Saito, Hideyuki Nakazawa, Tomonobu Koizumi, Fumihiro Ishida
An 84-year-old woman was referred to our hospital presenting anemia. Her hemoglobin level was 5.8 g/dL, and white blood cell count was 9400/μL, consisting of 82% lymphocytes. Given the lymphocyte phenotype (CD2+, CD3-, CD16+, and CD56-) and negative whole blood EBV viral load, we made a diagnosis of chronic lymphoproliferative disorder of NK cells (CLPD-NK). We suspected hemolytic anemia because of the high levels of reticulocytes in the peripheral blood and the low haptoglobin value. Although the direct Coombs test was negative and there was no cold agglutination, we examined her red-blood-cell-bound IgG (RBC-IgG), which was elevated...
December 27, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/28024505/-comparison-of-therapeutic-efficacy-for-neonatal-abo-hemolytic-disease-treated-with-intravenous-immunoglobin-g-by-different-modes-of-administration
#12
Yun-Feng Liu, Chao-Chun Zou, Hua-Qin Yang, Li-Jiang Lou
OBJECTIVE: To compare the therapeutic efficacy of patients with neonatal ABO hemolytic disease treated with introvenous immunoglobin G (IVIG) by different modes of administration. METHODS: Ninety-three in patients with neonatal ABO hemolytic disease treated in our hospital were divided into group A (31 cases), B(31 cases) and C (31 cases). Based on basic treatment, the patients in group A were treated by a single high dose of IVIG (1 g/kg), patients in group B were treated by multiple low-dose of IVIG (0...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28018448/a-2-month-old-boy-with-hemolytic-anemia-and-reticulocytopenia-following-intravenous-immunoglobulin-therapy-for-kawasaki-disease-a-case-report-and-literature-review
#13
Na Yeon Kim, Joon Hwan Kim, Jin Suk Park, Soo Hyun Kim, Yeon Kyung Cho, Dong Hyun Cha, Ki Eun Kim, Myung Suh Kang, Kyung Ah Lim, Youn Ho Sheen
Herein, we report a rare case of hemolytic anemia with reticulocytopenia following intravenous immunoglobulin therapy in a young infant treated for Kawasaki disease. A 2-month-old boy presented with fever lasting 3 days, conjunctival injection, strawberry tongue, erythematous edema of the hands, and macular rash, symptoms and signs suggestive of incomplete Kawasaki disease. His fever resolved 8 days after treatment with aspirin and high dose infusion of intravenous immunoglobulin. The hemoglobin and hematocrit decreased from 9...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28013299/autoimmunity-in-primary-antibody-deficiencies
#14
Gholamreza Azizi, Moslem Ahmadi, Hassan Abolhassani, Reza Yazdani, Hamed Mohammadi, Abbas Mirshafiey, Nima Rezaei, Asghar Aghamohammadi
Primary antibody deficiencies (PADs) are the most common inherited primary immunodeficiencies in humans, characterized by hypogammaglobulinemia, an inability to produce specific antibodies, and recurrent infections mainly caused by encapsulated bacteria. However, it has been shown that inflammatory disorders, granulomatous lesions, lymphoproliferative diseases, cancer, and autoimmunity are associated with the various types of PAD. Both systemic and organ-specific autoimmune diseases could be attributed to B-cell defects in PAD patients...
December 24, 2016: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28011677/n-acetylcysteine-in-preclinical-mouse-and-baboon-models-of-thrombotic-thrombocytopenic-purpura
#15
Claudia Tersteeg, Jan Roodt, Walter J Van Rensburg, Charlotte Dekimpe, Nele Vandeputte, Inge Pareyn, Aline Vandenbulcke, Barbara Plaimauer, Seb Lamprecht, Hans Deckmyn, José A Lopez, Simon F De Meyer, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic disorder diagnosed by thrombocytopenia and hemolytic anemia, associated with a deficiency in von Willebrand factor (VWF) cleaving protease ADAMTS13. Current treatment is based on plasma infusion for congenital TTP, or plasma exchange, often in combination with immunosuppressive agents, for acquired TTP. These treatment methods are however not always effective and therefore new treatment methods are highly necessary. N-acetylcysteine (NAC), an FDA-approved anti-mucolytic agent, could be a possible new treatment strategy for TTP as it was demonstrated to reduce disulfide bonds in VWF, thereby decreasing VWF multimer size and hence its prothrombotic potential...
December 23, 2016: Blood
https://www.readbyqxmd.com/read/28009956/new-onset-hemolytic-anemia-after-percutaneous-paravalvular-leak-closure
#16
Luís Almeida-Morais, António Fiarresga, Duarte Cacela, Lídia de Sousa, Ana Galrinho, Rui Rodrigues, Lurdes Ferreira, Rui Ferreira
BACKGROUND: Despite being usually clinically silent, paravalvular leak can present with congestive heart failure (CHF) or haemolytic anemia. Here, the case is reported of a paravalvular leak presenting with CHF, complicated by new-onset severe hemolytic anemia after percutaneous closure with a large ventricular septal duct (VSD) occlude device. METHODS: A 57-year-old patient presented with infectious endocarditis of a native mitral valve with major mitral regurgitation and was submitted for mitral valvuloplasty...
July 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28006845/howell-jolly-bodies-in-acute-hemolytic-anemia
#17
Barbara J Bain
No abstract text is available yet for this article.
December 22, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27999774/hyal-2-wwox-smad4-signaling-in-cell-death-and-anticancer-response
#18
REVIEW
Li-Jin Hsu, Ming-Fu Chiang, Chun-I Sze, Wan-Pei Su, Ye Vone Yap, I-Ting Lee, Hsiang-Ling Kuo, Nan-Shan Chang
Hyaluronidase HYAL-2 is a membrane-anchored protein and also localizes, in part, in the lysosome. Recent study from animal models revealed that both HYAL-1 and HYAL-2 are essential for the metabolism of hyaluronan (HA). Hyal-2 deficiency is associated with chronic thrombotic microangiopathy with hemolytic anemia in mice due to over accumulation of high molecular size HA. HYAL-2 is essential for platelet generation. Membrane HYAL-2 degrades HA bound by co-receptor CD44. Also, in a non-canonical signal pathway, HYAL-2 serves as a receptor for transforming growth factor beta (TGF-β) to signal with downstream tumor suppressors WWOX and SMAD4 to control gene transcription...
2016: Frontiers in Cell and Developmental Biology
https://www.readbyqxmd.com/read/27995893/-recent-progresses-of-autoimmune-hemolytic-anemia
#19
Q Y Gao, F K Zhang
No abstract text is available yet for this article.
November 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/27989485/acute-oxaliplatin-induced-hemolytic-anemia-thrombocytopenia-and-renal-failure-case-report-and-a-literature-review
#20
Pooja Phull, Karen Quillen, Kevan L Hartshorn
No abstract text is available yet for this article.
November 23, 2016: Clinical Colorectal Cancer
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