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hemolytic anemias

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https://www.readbyqxmd.com/read/28641660/-comparison-of-laboratory-indexes-and-therapeutic-efficacy-between-two-kinds-of-different-rbc-transfusion-in-aiha-patients
#1
Jing-Jing Gao, Xiong-Peng Zhu, Ming-Quan Wang
OBJECTIVE: To investigate the effect of 2 kinds of red blood cells (RBC) on the laboratorial indexes and therapeutic efficacy of patients with autoimmune hemolytic anemia (AIHA). METHODS: The clinical data of 120 patients with AIHA from June 2015 to June 2016 were analyzed retrospectively. These 120 patients were divided into A goup and B group. The patients in A group (60 cases) were infused with washed RBC, while the patients in B group (60 cases) were infused with WBC-deplated RBC...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28639307/anti-d-in-a-mother-hemizygous-for-the-variant-rhd-dnb-gene-associated-with-hemolytic-disease-of-the-fetus-and-newborn
#2
Kelli M Quantock, Genghis H Lopez, Catherine A Hyland, Yew-Wah Liew, Robert L Flower, Frans J Niemann, Arthur Joyce
BACKGROUND: Individuals with the partial D phenotype when exposed to D+ red blood cells (RBCs) carrying the epitopes they lack may develop anti-D specific for the missing epitopes. DNB is the most common partial D in Caucasians and the clinical significance for anti-D in these individuals is unknown. STUDY DESIGN AND METHODS: This article describes the serologic genotyping results and clinical manifestations in two group D+ babies of a mother presenting as group O, D+ with alloanti-D...
June 21, 2017: Transfusion
https://www.readbyqxmd.com/read/28636757/babesiosis-an-unusual-cause-of-sepsis-after-kidney-transplantation-and-review-of-the-literature
#3
Imtiaz Ather, Negiin Pourafshar, Denise Schain, Asmita Gupte, Michael J Casey
We report a unique case of babesiosis presenting as sepsis after kidney transplantation. A 70-year-old female kidney transplant recipient presented with fever, hemolytic anemia, and acute kidney injury, and met three of four systemic inflammatory response syndrome (SIRS) criteria. Serology was positive for Babesia microti, confirmed by polymerase chain reaction. The patient was treated with atovaquone and azithromycin and made a full recovery. Reports of babesiosis after solid organ transplantation are rare, with only four prior cases reported in the literature...
June 21, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28630094/type-i-ifn-is-necessary-and-sufficient-for-inflammation-induced-red-blood-cell-alloimmunization-in-mice
#4
David R Gibb, Jingchun Liu, Prabitha Natarajan, Manjula Santhanakrishnan, David J Madrid, Stephanie C Eisenbarth, James C Zimring, Akiko Iwasaki, Jeanne E Hendrickson
During RBC transfusion, production of alloantibodies against RBC non-ABO Ags can cause hemolytic transfusion reactions and limit availability of compatible blood products, resulting in anemia-associated morbidity and mortality. Multiple studies have established that certain inflammatory disorders and inflammatory stimuli promote alloimmune responses to RBC Ags. However, the molecular mechanisms underlying these findings are poorly understood. Type I IFNs (IFN-α/β) are induced in inflammatory conditions associated with increased alloimmunization...
June 19, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28626544/atypical-hemolytic-uremic-syndrome-a-brief-review
#5
REVIEW
Kuixing Zhang, Yuxin Lu, Kevin T Harley, Minh-Ha Tran
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28626212/a-huge-subcutaneous-hematoma-in-an-adult-with-kasabach-merritt-syndrome
#6
Kuan-Lin Wu, Chiung-Ying Liao, Chen-Kuang Chang, Shang-Yun Ho, Yeu-Sheng Tyan, Yuan-Chun Huang
BACKGROUND Kasabach-Merritt syndrome is a potentially fatal disease that consists of hemangioma(s) with thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy. Extensive hemangiomatosis is rare. We present the radiological features and treatment strategy of a young adult suffering from Kasabach-Merritt syndrome with widespread hemangiomas and an infected huge hematoma in the right thigh. CASE REPORT A 33-year-old Taiwanese male presented with a painful 20-cm mass over his right thigh and gross hematuria for 2 days...
June 19, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28621877/autoimmune-hemolytic-anemia-in-solid-organ-transplantation-the-role-of-immunosuppression
#7
REVIEW
Theoni Kanellopoulou
Hemolysis after solid organ transplantation can be caused by both immune and non-immune mediated mechanisms and the evaluation must take into account issues distinctive to the post-transplant period. Autoimmune hemolytic anemia usually occurs within the first year and has been attributed to immunosuppressive treatment, infections or underlying post-transplant lymphoproliferative disorder. Review of the literature revealed 59 cases with autoimmune hemolytic anemia mostly in children after liver transplantation...
June 16, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#8
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28619392/platelet-satellitism-in-autoimmune-hemolytic-anemia
#9
REVIEW
I Ghariani, N Braham, S Hamzaoui, L Bekir
Platelet satellitism is a rare phenomenon observed in blood smears obtained from blood anticoagulated with EDTA with an estimated frequency at 0.008%. It is characterized by platelet rosetting around neutrophils and in rare cases around other blood cells, which may causes pseudothrombocytopenia. Our case involves a 26-year-old female patient with autoimmune hemolytic anemia (AIHA). Blood smear with EDTA as an anticoagulant had platelet satellitism whereas the phenomenon was not observed in tubes with different anticoagulants (citrate, heparin) and capillary blood...
June 12, 2017: Current Research in Translational Medicine
https://www.readbyqxmd.com/read/28612003/discontinuation-of-peritoneal-dialysis-after-late-initiation-of-eculizumab-in-a-case-of-familial-atypical-hemolytic-uremic-syndrome-a-case-report
#10
Rafael Alonso Valente, Giannina Elena García Rodríguez, Yanina García Marcote, Manuel Fidalgo Díaz, Vanesa Becerra Mosquera, Daniel Novoa García, Teresa Cordal Martínez, Cándido Díaz Rodríguez
BACKGROUND: Atypical hemolytic-uremic syndrome is caused by a thrombotic microangiopathy and manifests itself with hemolytic anemia, thrombocytopenia, and organ ischemia. Its etiology is a mutation affecting the genes encoding for proteins of the complement system. Early treatment with eculizumab (8.6 months from the moment of presentation), a humanized monoclonal antibody against complement, is shown to be effective in controlling symptoms and reversing organ damage. We present a patient with a mutation not previously described in the literature...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28607779/thrombotic-microangiopathy-secondary-to-intravenous-abuse-of-opana%C3%A2-er
#11
Kamia Thakur, Vaibhav Agrawal, Ashley Kass, Lauren M Dimarino, R Patrick Dorion, Joseph Vadakara
Opana ER (oxymorphone) is an opioid drug available throughout the United States, and intravenous abuse of the crushed oral formulation has been associated with drug-induced thrombotic microangiopathy. In this abstract, we describe two young patients who lived together and used Opana ER intravenously. Both presented with microangiopathic hemolytic anemia that mimicked thrombotic thrombocytopenic purpura (TTP). Treating this condition poses a clinical challenge, as it is difficult to distinguish it from TTP. The role for plasma exchange is not clear but can be used while awaiting the results of the ADAMTS-13 activity, but ultimately supportive care with drug discontinuation is the recommended therapy of choice...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28605161/the-use-of-therapeutic-plasma-exchange-to-reduce-serum-bilirubin-in-a-dog-with-kernicterus
#12
Tricia Tovar, Sarah Deitschel, Christine Guenther
OBJECTIVE: To describe the use of a manual method of therapeutic plasma exchange to reduce total serum bilirubin, manage kernicterus, and halt progression of neurological dysfunction in a dog with immune-mediated hemolytic anemia (IMHA). CASE SUMMARY: A 5-year-old male neutered Lhasa Apso diagnosed with IMHA developed acute onset neurologic signs consistent with kernicterus. Manual therapeutic plasma exchange was performed in an attempt to reduce total serum bilirubin...
June 12, 2017: Journal of Veterinary Emergency and Critical Care
https://www.readbyqxmd.com/read/28600963/ultra-performance-liquid-chromatography-tandem-mass-spectrometry-based-multiplex-enzyme-assay-for-six-enzymes-associated-with-hereditary-hemolytic-anemia
#13
Chul Min Park, Kyunghoon Lee, Sun-Hee Jun, Sang Hoon Song, Junghan Song
Deficiencies in erythrocyte metabolic enzymes are associated with hereditary hemolytic anemia. Here, we report the development of a novel multiplex enzyme assay for six major enzymes, namely glucose-6-phosphate dehydrogenase, pyruvate kinase, pyrimidine 5'-nucleotidase, hexokinase, triosephosphate isomerase, and adenosine deaminase, deficiencies in which are implicated in erythrocyte enzymopathies. To overcome the drawbacks of traditional spectrophotometric enzyme assays, the present assay was based on ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS)...
June 3, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/28600054/prenatal-treatment-of-severe-fetal-hemolytic-disease-due-to-anti-m-alloimmunization-by-serial-intrauterine-transfusions
#14
Lin Li, Linhuan Huang, Guangping Luo, Yanmin Luo, Qun Fang
OBJECTIVE: Fetal hemolytic disease is a common cause of fetal hydrops and fetal morbidity and mortality. Despite its relatively low frequency, the anti-M IgG antibody is one of the causes of severe fetal anemia and intrauterine death; only a few cases have been reported. CASE REPORT: This is a case of a pregnant woman with a history of three intrauterine deaths. A diagnosis of severe fetal anemia attributed to anti-M alloimmunization was confirmed in her fifth pregnancy...
June 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28599278/identification-of-a-novel-de-novo-ank1-r1426-nonsense-mutation-in-a-chinese-family-with-hereditary-spherocytosis-by-ngs
#15
Xiong Wang, Bin Yi, Ketao Mu, Na Shen, Yaowu Zhu, Qun Hu, Yanjun Lu
Hereditary spherocytosis (HS) is an inherited heterogeneous hemolytic anemia, characterized by the presence of spherical-shaped erythrocytes on the peripheral blood smear, and the clinical manifestation ranges from asymptomatic to severely anemic, and transfusion-dependent patients. Mutations in at least five genes (ANK1, EPB42, SLC4A1, SPTA1, and SPTB) have been identified so far, and mutations of ANK1 gene are responsible for the majority of all HS cases. In this study, targeted next generation sequencing (NGS) was applied to identify a novel de novo ANK1 c...
May 27, 2017: Oncotarget
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
#16
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/28597685/a-gain-of-function-mutation-of-stat1-a-novel-genetic-factor-contributing-to-chronic-mucocutaneous-candidiasis
#17
Narges Eslami, Marzieh Tavakol, Mehrnaz Mesdaghi, Mohammad Gharegozlou, Jean-Laurent Casanova, Anne Puel, Satoshi Okada, Saba Arshi, Mohammad Hassan Bemanian, Morteza Fallahpour, Rasool Molatefi, Farhad Seif, Samaneh Zoghi, Nima Rezaei, Mohammad Nabavi
Heterozygous gain-of-function (GOF) mutations in the signal transducer and activator of transcription 1 (STAT1) have increasingly been identified as a genetic cause of autosomal-dominant (AD) chronic mucocutaneous candidiasis (CMC). In this article, we describe a 33-year-old man who experienced chronic refractory candidiasis, recurrent otitis media, and pneumonia resulting in bronchiectasis, severe oral and esophageal candidiases with strictures associated with hypothyroidism and immune hemolytic anemia. His son also suffered from persistent candidiasis, chronic diarrhea, poor weight gain, and pneumonia that resulted in his demise because of sepsis...
June 9, 2017: Acta Microbiologica et Immunologica Hungarica
https://www.readbyqxmd.com/read/28596671/autologous-blood-transfusion-as-a-life-saving-measure-for-a-trauma-patient-with-fracture-femur-and-drug-induced-hemolytic-anemia-a-case-report
#18
Sumit Vishwakarma, Rahul Chaurasia, Arulselvi Subramanian, Vivek Trikha, Kabita Chatterjee
A positive direct antiglobulin test has been reported in 1:1000 to 1:14,000 blood donors and 1-15 % of hospital patients. Drugs may cause a positive direct antiglobulin test result and/or immune-mediated haemolysis with an incidence of approximately 1 in a 1 million population. Our aim is to highlight the importance of following strict transfusion protocols and management insight in a direct antiglobulin test positive patient showing incompatibility with multiple units possibly due to drug induced immune haemolytic anaemia (DIIHA)...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28596226/autoimmune-hemolytic-anemia-associated-with-trimethoprim-sulfamethoxazole-use
#19
Jillian Frieder, Jason Aboudi Mouabbi, Rami Zein, Tariq Hadid
PURPOSE: A case report of drug-induced immune hemolytic anemia (DIIHA) triggered by exposure to trimethoprim-sulfamethoxazole is presented along with a brief review of the pathophysiology of DIIHA and diagnostic considerations. SUMMARY: A 58-year-old woman recently initiated on trimethoprim-sulfamethoxazole for treatment of a urinary tract infection presented to the emergency department with generalized weakness and fatigue. Initial laboratory studies were significant for the following values: hemoglobin concentration, 5...
June 15, 2017: American Journal of Health-system Pharmacy: AJHP
https://www.readbyqxmd.com/read/28595636/two-novel-c-terminal-frameshift-mutations-in-the-%C3%AE-globin-gene-lead-to-rapid-mrna-decay
#20
Katarzyna Rawa, Roman J Szczesny, Ewelina P Owczarek, Anna Adamowicz-Salach, Anna Klukowska, Urszula Demkow, Danuta Plochocka, Pawel Szczesny, Monika Gora, Andrzej Dziembowski, Beata Burzynska
BACKGROUND: The thalassemia syndromes are classified according to the globin chain or chains whose production is affected. β-thalassemias are caused by point mutations or, more rarely, deletions or insertions of a few nucleotides in the β-globin gene or its immediate flanking sequences. These mutations interfere with the gene function either at the transcriptional, translational or posttranslational level. METHODS: Two cases of Polish patients with hereditary hemolytic anemia suspected of thalassemia were studied...
June 8, 2017: BMC Medical Genetics
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