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Autoimmune hemolytic anemia

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https://www.readbyqxmd.com/read/29348906/an-ank1-ivs3-2a-c-mutation-causes-exon-4-skipping-in-two-patients-from-a-chinese-family-with-hereditary-spherocytosis
#1
Xiong Wang, Liyan Mao, Na Shen, Jing Peng, Yaowu Zhu, Qun Hu, Yanjun Lu
Hereditary spherocytosis (HS) is a congenital hemolytic anemia that affects the cell membrane of red blood cells and is characterized by the presence of spherical-shaped erythrocytes in the peripheral blood film. The clinical manifestation of HS ranges from asymptomatic to severe cases that require transfusion during early childhood. HS is caused by mutations in red blood cell membrane protein encoding genes, including ANK1, EPB42, SLC4A1, SPTA1, and SPTB. Mutations of the ANK1 gene account for 75% of all HS cases, and these particular mutations are typically inherited in an autosomal dominant manner...
December 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/29345206/successful-treatment-of-refractory-autoimmune-hemolytic-anemia-after-allogeneic-hematopoietic-stem-cell-transplantation-with-bortezomib
#2
Ling Cao, Liang Piu Koh, Yeh Ching Linn
No abstract text is available yet for this article.
January 18, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/29342998/-x-linked-immunodeficiency-with-magnesium-defect-epstein-barr-virus-infection-and-neoplasia-report-of-a-family-and-literature-review
#3
T Y He, Y Xia, C G Li, C R Li, Z X Qi, J Yang
Objective: To investigate the clinical features and genetic characteristics of cases with X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection, and neoplasia (XMEN). Methods: Characteristics of clinical material, immunological data and gene mutation of two cases with XMEN in the same family in China were retrospectively analyzed. The related reports literature were searched by using search terms'MAGT1 gene'or'XMEN'. Results: The proband, a 2-year-eight-month old boy, was admitted due to 'Urine with deepened color for two days and yellow stained skin for one day'...
January 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29340837/exacerbation-of-autoimmune-hemolytic-anemia-induced-by-the-first-dose-of-programmed-death-1-inhibitor-pembrolizumab-a-case-report
#4
Kenta Ogawa, Jiro Ito, Daichi Fujimoto, Mari Morita, Yuko Yoshizumi, Koichi Ariyoshi, Keisuke Tomii, Nobuyuki Katakami
Immune checkpoint inhibitors (ICIs) have demonstrated efficacy against various types of cancers. In addition to immune-related adverse events (irAEs) induced by ICIs, exacerbation of baseline autoimmune disease has been occasionally reported. This is the first report of autoimmune hemolytic anemia (AIHA) exacerbated by pembrolizumab. An 82-year-old Japanese male was diagnosed with lung adenocarcinoma 2 years ago. The patient had chronic anemia with positive direct and indirect Coombs test prior to initiating pembrolizumab therapy at a nearby hospital...
January 16, 2018: Investigational New Drugs
https://www.readbyqxmd.com/read/29335897/splenectomy-in-systemic-lupus-erythematosus-and-autoimmune-hematologic-disease-a-comparative-analysis
#5
Nahim Barron, Jesús Arenas-Osuna, Gabriela Medina, María Pilar Cruz-Dominguez, Fernando González-Romero, José Arturo Velásques-García, Ernesto Alonso Ayala-López, Luis J Jara
The objective of the study is to analyze the efficacy and safety of splenectomy in the management of refractory autoimmune thrombocytopenia (AT)/autoimmune hemolytic anemia (AIHA) associated or not with systemic lupus erythematosus. Thirty-four patients after splenectomy due to severe AT and/or AIHA were divided into group 1 (G1) 18 SLE/APS patients: 9 AT/SLE patients, 6 SLE/antiphospholipid syndrome (APS), and 3 primary APS. Group 2 (G2): 16 patients without SLE/APS: 2 Fisher-Evans syndrome and 14 AIHA. Surgery approach when (1) platelets ≤ 50,000/ml despite 2 weeks on medical therapy, (2) medically dependent, and (3) medically intolerant or after two hemolytic crises in AIHA patients...
January 16, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29334337/durable-responses-in-refractory-autoimmune-hemolytic-anemia-with-alemtuzumab
#6
Renee K McAlister, Mahsa S Talbott, Nishitha M Reddy
Autoimmune hemolytic anemia occurs due to an interaction of IgG antibodies with protein antigens expressed on red blood corpuscles. Glucocorticoids are the mainstay of treatment for autoimmune hemolytic anemia. For patients not responding to initial therapy, other agents such as rituximab, immunosuppressive therapy, or splenectomy are considered. When refractory to these treatment options, alemtuzumab is an alternative agent. However, long-term outcomes of patients supporting its use are lacking. We present three patients with refractory autoimmune hemolytic anemia treated with alemtuzumab...
January 1, 2018: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/29330115/pediatric-onset-evans-syndrome-heterogeneous-presentation-and-high-frequency-of-monogenic-disorders-including-lrba-and-ctla4-mutations
#7
Caroline Besnard, Eva Levy, Nathalie Aladjidi, Marie-Claude Stolzenberg, Aude Magerus-Chatinet, Olivier Alibeu, Patrick Nitschke, Stéphane Blanche, Olivier Hermine, Eric Jeziorski, Judith Landman-Parker, Guy Leverger, Nizar Mahlaoui, Gérard Michel, Isabelle Pellier, Felipe Suarez, Isabelle Thuret, Geneviève de Saint-Basile, Capucine Picard, Alain Fischer, Bénédicte Neven, Frédéric Rieux-Laucat, Pierre Quartier
Evans syndrome (ES) is defined by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. Clinical presentation includes manifestations of immune dysregulation, found in primary immune deficiencies, autoimmune lymphoproliferative syndrome with FAS (ALPS-FAS), Cytotoxic T Lymphocyte Antigen-4 (CTLA-4) and Lipopolysaccharide-Responsive vesicle trafficking Beige-like and Anchor protein (LRBA) defects. We report the clinical history and genetic results of 18 children with ES after excluding ALPS-FAS...
January 9, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29296731/human-and-murine-splenic-neutrophils-are-potent-phagocytes-of-igg-opsonized-red-blood-cells
#8
Sanne M Meinderts, Per-Arne Oldenborg, Boukje M Beuger, Thomas R L Klei, Johanna Johansson, Taco W Kuijpers, Takashi Matozaki, Elise J Huisman, Masja de Haas, Timo K van den Berg, Robin van Bruggen
Red blood cell (RBC) clearance is known to occur primarily in the spleen, and is presumed to be executed by red pulp macrophages. Erythrophagocytosis in the spleen takes place as part of the homeostatic turnover of RBCs to remove old RBCs. It can be strongly promoted by immunoglobulin G (IgG) opsonization of RBCs, a condition that can occur as a consequence of autoantibody or alloantibody formation. The purpose of our study was to investigate which phagocytes are involved in IgG-mediated RBC clearance in the human spleen...
June 13, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296728/cold-agglutinin-disease-burden-a-longitudinal-analysis-of-anemia-medications-transfusions-and-health-care-utilization
#9
Megan Mullins, Xiaohui Jiang, Lauren C Bylsma, Jon P Fryzek, Heidi Reichert, Evan C Chen, Shivaani Kummar, Adam Rosenthal
Cold agglutinin disease (CAD), a rare disease and subtype of autoimmune hemolytic anemia, is characterized by autoantibodies that bind to red blood cells at low temperatures. There is no established standard of care for CAD treatment and CAD cohort studies are limited by the rarity of the condition. The objectives of this study are to present the longitudinal experience of a CAD cohort from the United States, with a focus on anemia severity, use of medications and transfusions, and health care resource utilization...
May 23, 2017: Blood Advances
https://www.readbyqxmd.com/read/29283149/unilateral-isolated-superior-ophthalmic-vein-thrombosis
#10
Raksha Rao, Yasmin Ali, Chinmay P Nagesh, Unnikrishnan Nair
Superior ophthalmic vein (SOV) thrombosis is an uncommon orbital pathology that can present with sudden onset proptosis, conjunctival injection, and visual disturbance. SOV thrombosis is frequently secondary to a cavernous sinus pathology. A 32-year-old female with a known history of autoimmune hemolytic anemia presented with sudden painful proptosis left eye, and on imaging, she was found to have SOV thrombosis without cavernous sinus involvement. She was diagnosed with unilateral isolated SOV thrombosis and was managed conservatively...
January 2018: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/29281948/a-patient-with-oxaliplatin-immune-induced-syndrome-oiis-who-also-developed-leucovorin-and-palonosetron-associated-thrombocytopenia
#11
S A Curtis, B R Curtis, A I Lee, J E Hendrickson, J Lacy, N A Podoltsev
OBJECTIVES: We report a case of an 83 year old man who developed oxaliplatin immune-induced syndrome (OIIS) after his 19th cycle of FOLFOX (5FU, leucovorin, oxaliplatin). When oxaliplatin was omitted from his next cycle of chemotherapy he continues to show signs of drug-induced immune thrombocytopenia (DITP) and was found to have drug-dependent, platelet-reactive antibodies (DDPA) to leucovorin and palonosetron as well as oxaliplatin. METHODS: The patient was admitted for monitoring but required no transfusions and thrombocytopenia resolved without treatment during his first admission...
December 28, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29280474/anemia-in-pregnancy-a-pragmatic-approach
#12
Dongmei Sun, Anne McLeod, Shital Gandhi, Ann Kinga Malinowski, Nadine Shehata
Importance: Anemia is common in pregnancy, ranging from 5.4% in developed countries to more than 80% in developing countries. Anemia in pregnancy has been associated with prematurity, low birth weight, and adverse pregnancy outcomes. Objective: This review uses clinical vignettes to illustrate the clinical presentations, approach to diagnosis, maternal and fetal implications, and treatment for the common etiologies of anemia in pregnancy. Evidence Acquisition: Literature review...
December 2017: Obstetrical & Gynecological Survey
https://www.readbyqxmd.com/read/29277896/c5ar-activation-in-the-absence-of-c5a-a-new-disease-mechanism-of-autoimmune-hemolytic-anemia-in-mice
#13
Shahzad N Syed, Eduard Rau, Mareen Ziegelmann, Georgios Sogkas, Bernhard Brüne, Reinhold E Schmidt
IgG Fc receptors (FcγRs) and the C5a anaphylatoxin receptor (C5aR) were identified as key regulators of type II autoimmune injury in mice. However, and with respect to C5aR, the relative importance of C5a for IgG autoantibody-induced cellular destruction remained unclear. Using an experimental model of autoimmune hemolytic anemia (AIHA), we here report marked differences in the development of AIHA between mice lacking C5aR and C5-deficient (Hc0 ) strain, indicating a limited role of C5 in this type of C5aR-regulated disease...
December 25, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/29277816/paraneoplastic-auto-immune-hemolytic-anemia-an-unusual-sequela-of-enteric-duplication-cyst
#14
Arsha Sreedhar, Ranjit Nair, William Scialla
Enteric duplication (ED) cysts are rare congenital anomalies of the alimentary canal that present in childhood. Although benign in most case, ED cysts have the potential to cause complications including a rare association with secondary carcinomas. Autoimmune hemolytic anemia presenting as a paraneoplastic syndrome secondary to solid tumors is an unusual phenomenon. Here we report a patient case with ED cyst described in association with intestinal adenocarcinoma and warm-auto immune hemolytic anemia, with resolution of the hemolysis upon its surgical resection...
January 2018: Anticancer Research
https://www.readbyqxmd.com/read/29277513/risk-factors-treatment-and-immune-dysregulation-in-autoimmune-cytopenia-following-allogeneic-hematopoietic-stem-cell-transplantation-in-pediatric-patients
#15
Matthijs D Kruizinga, Maarten J D van Tol, Vincent Bekker, Tanja Netelenbos, Frans J Smiers, Dorine Bresters, Anja M Jansen-Hoogendijk, Monique M van Ostaijen-Ten Dam, Wouter J W Kollen, Jaap J Zwaginga, Arjan C Lankester, Robbert G M Bredius
Auto- or alloimmune cytopenia (AIC) is a known rare complication of hematopoietic stem cell transplantation (SCT). AIC after SCT is considered difficult to treat and associated with high morbidity and mortality. In this retrospective study in pediatric patients we evaluated incidence, outcome, potential risk factors and current treatment strategies. A nested matched case-control study was performed to search for biomarkers associated with AIC. Of 531 consecutive SCTs at our center between 2000 and 2016, 26 were complicated by the development of AIC (cumulative incidence 5...
December 19, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29261629/false-positive-heat-damaged-rbc-scan-for-splenosis-case-of-retroperitoneal-extramedullary-hematopoiesis
#16
Joseph S Donald, Delphine L Chen, Jessica Peterson, Mark Perry
Extramedullary hematopoiesis (EMH) is a well-known compensatory response to severe anemia, most commonly due to a chronic hemolytic anemia such as thalassemia or sickle cell disease. We present a case of a 72-year-old man with autoimmune hemolytic anemia refractory to steroids and splenectomy. An incidental retroperitoneal mass on CT was suggestive of ectopic splenic tissue. However, an alternative diagnosis of EMH was considered, given an atypical appearance on heat-damaged Tc-RBC scan, and was confirmed with biopsy...
December 19, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29247540/efficacy-and-safety-of-rituximab-for-systemic-lupus-erythematosus-associated-immune-cytopenias-a-multicenter-retrospective-cohort-study-of-71-adults
#17
Alexandra Serris, Zahir Amoura, Florence Canouï-Poitrine, Benjamin Terrier, Eric Hachulla, Nathalie Costedoat-Chalumeau, Thomas Papo, Olivier Lambotte, David Saadoun, Miguel Hié, Philippe Blanche, Bertrand Lioger, Jacques-Eric Gottenberg, Bertrand Godeau, Marc Michel
The aim of the study was to assess the efficacy and safety of rituximab (RTX) for treating systemic lupus erythematosus (SLE)-associated immune cytopenias. This multicenter retrospective cohort study of adults from French referral centers and networks for adult immune cytopenias and SLE involved patients ≥18 years old with a definite diagnosis of SLE treated with RTX specifically for SLE-associated immune cytopenia from 2005 to 2015. Response assessment was based on standard definitions. In total, 71 patients, 61 women (85...
December 16, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29245301/copper-associated-hepatitis-in-a-patient-with-chronic-myeloid-leukemia-following-hematopoietic-stem-cell-transplantation-a-case-report
#18
Ching-Fen Lee, Chi-Hua Chen, Yu-Chuan Wen, Tsung-Yen Chang, Ming-Wei Lai, Tang-Her Jaing
RATIONALE: We report a complicated case of cholestatic hepatitis with suspected autoimmune hemolytic anemia (AIHA) and copper toxicity syndrome after HSCT and donor lymphocyte infusion (DLI). PATIENT CONCERNS: A 19-year-1-month-old girl presented with a history of CML. She underwent matched unrelated donor HSCT and donor lymphocyte infusion subsequently. Three months later, yellowish discoloration of the skin was found, which was accompanied by progressive itchy skin, easy fatigability, insomnia, and dark urine output...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29214674/neutrophil-extracellular-traps-in-plasma-from-dogs-with-immune-mediated-hemolytic-anemia
#19
C Lawson, S A Smith, M O'Brien, M McMichael
BACKGROUND: Neutrophil extracellular traps (NETs) are part of the innate immune response and are essential in local pathogen control, but are associated with pathological inflammation, organ damage, autoimmunity, and thrombosis. Immune-mediated hemolytic anemia (IMHA) is a pro-inflammatory, prothrombotic disease associated with high mortality. HYPOTHESIS/OBJECTIVES: Neutrophil extracellular traps (NETs) are a feature of the inflammatory process in dogs with IMHA...
December 7, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/29164157/inflammatory-breast-cancer-and-warm-antibody-autoimmune-hemolytic-anemia-a-rare-paraneoplastic-syndrome
#20
Nene Ugoeke, Chidinma Onweni, Jennifer Treece, Vandana Pai, Sowminya Arikapudi, Evan Kulbacki, Kailash Bajaj
Autoimmune hemolytic anemia (AIHA) is a disease process that involves the destruction of red blood cells mediated by the humoral immune system. It can be characterized as a cold agglutinin syndrome, paroxysmal cold hemoglobinuria, and warm, mixed type, and drug-induced AIHA. Although a well-established relationship exists between the presence of AIHA and lymphoproliferative malignancy, AIHA rarely presents in association with solid malignancies. An analysis of the limited number of published cases of AIHA in association with solid malignancies performed showed that AIHA may present before the diagnosis of a solid malignancy, concurrently with the presence of a solid malignancy, or even on resolution of a solid malignancy...
October 2017: Journal of Investigative Medicine High Impact Case Reports
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