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Hemolytic anemia

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https://www.readbyqxmd.com/read/27920704/autoimmune-hemolytic-anemia-as-a-complication-of-nivolumab-therapy
#1
Amruth R Palla, Devin Kennedy, Hossain Mosharraf, Donald Doll
Recently, immunotherapeutic drugs, including PD-1 inhibitors (nivolumab, pembrolizumab), PD-L1 inhibitors (atezolizumab, avelumab), and CTLA4 inhibitors (ipiliumumab), have emerged as important additions to the armamentarium against certain malignancies and have been incorporated into therapeutic protocols for first-, second-, or third-line agents for these metastatic cancers. Immune checkpoint inhibitor nivolumab is currently FDA approved for the treatment of patients with metastatic malignant melanoma [Redman et al...
September 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27919500/favorable-outcome-of-interferon-beta-associated-thrombotic-microangiopathy-following-treatment-with-corticosteroids-plasma-exchange-and-rituximab-a-case-report
#2
Lea M Gerischer, Eberhard Siebert, Oliver Janke, Gerhard Jan Jungehuelsing, Klemens Ruprecht
Thrombotic microangiopathy (TMA) is a rare but increasingly recognized complication of interferon-beta therapy, which can be associated with serious sequelae. We report on a 53-year-old woman with a longstanding history of relapsing-remitting multiple sclerosis, who developed TMA after 15 years of high-dose treatment with subcutaneous interferon-beta-1a. The patient presented with headaches, an epileptic seizure, confusion, and arterial hypertension. Laboratory findings included thrombocytopenia and hemolytic anemia...
November 2016: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/27918350/a-case-of-idiopathic-pulmonary-hemosiderosis-presenting-with-signs-and-symptoms-mimicking-hemolytic-anemia
#3
Dilek Doğruel, Ayşe Erbay, Nalan Yazici, Alev Arslan, Bermal Hasbay Biçen
Idiopathic pulmonary hemosiderosis is primarily a disorder of childhood, which is characterized by hemoptysis, iron deficiency anemia, and diffuse parenchymal infiltrates on chest x-ray secondary to recurrent attacks of alveolar hemorrhage. It can be diagnosed by showing hemosiderin laden macrophages in bronchoalveolar lavage fluid after other specific causes of diffuse alveolar hemorrhage are definitely excluded. A 5-year-old male patient was admitted to our clinic with sudden-onset pallor during iron therapy given for anemia...
December 1, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27917465/warm-reactive-immunoglobulin-g-autoantibodies-and-laboratory-testing-best-practices-review-of-the-literature-and-survey-of-current-practice
#4
REVIEW
Alyssa Ziman, Claudia Cohn, Patricia M Carey, Nancy M Dunbar, Mark K Fung, Andreas Greinacher, Simon Stanworth, Nancy M Heddle, Meghan Delaney
BACKGROUND: Warm-reactive autoantibodies (WAAs) are the most common cause of autoimmune hemolytic anemia (AIHA) and can also be present without clinically significant hemolysis. WAAs complicate immunohematological testing, yet there is no commonly accepted approach to laboratory evaluation and red blood cell (RBC) selection. STUDY DESIGN AND METHODS: We searched PubMed/Cochrane Central for articles that described testing methodology and blood selection for patients with WAAs...
December 4, 2016: Transfusion
https://www.readbyqxmd.com/read/27914961/expanding-the-clinical-and-genetic-spectrum-of-g6pd-deficiency-the-occurrence-of-bcgitis-and-novel-missense-mutation
#5
Taj Ali Khan, Humaira Mazhar, Mehboob Nawaz, Kalsoom Kalsoom, Muhammad Ishfaq, Huma Asif, Hazir Rahman, Muhammad Qasim, Farkhanda Naz, Mubashir Hussain, Baharullah Khattak, Waheed Ullah, Otavio Cabral-Marques, Jawad Butt, Asif Iqbal
Glucose-6-phosphate dehydrogenase (G6PD) is a key enzyme in the pentose phosphate pathway that ensures sufficient production of coenzyme nicotinamide adenine dinucleotide phosphate (NADPH) by catalyzing the reduction of NADP+ to NADPH. Noteworthy, the latter mediates the production of reactive oxygen species (ROS) by phagocytic cells such as neutrophils and monocytes. Therefore, patients with severe forms of G6PD deficiency may present impaired NADPH oxidase activity and become susceptible to recurrent infections...
November 30, 2016: Microbial Pathogenesis
https://www.readbyqxmd.com/read/27914600/polymorphisms-in-nat2-n-acetyltransferase-2-gene-in-patients-with-systemic-lupus-erythematosus
#6
Elaine Cristina Lima Dos Santos, Amanda Chaves Pinto, Evandro Mendes Klumb, Jacyara Maria Brito Macedo
OBJECTIVE: To investigate potential associations of four substitutions in NAT2 gene and of acetylator phenotype of NAT2 with systemic lupus erythematosus (SLE) and clinical phenotypes. METHODS: Molecular analysis of 481C>T, 590G>A, 857G>A, and 191G>A substitutions in the NAT2 gene was performed by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique, from DNA extracted from peripheral blood samples obtained from patients with SLE (n=91) and controls (n=97)...
November 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27913548/warm-antibody-autoimmune-hemolytic-anemia
#7
Theodosia A Kalfa
Autoimmune hemolytic anemia (AIHA) is a rare and heterogeneous disease that affects 1 to 3/100 000 patients per year. AIHA caused by warm autoantibodies (w-AIHA), ie, antibodies that react with their antigens on the red blood cell optimally at 37°C, is the most common type, comprising ∼70% to 80% of all adult cases and ∼50% of pediatric cases. About half of the w-AIHA cases are called primary because no specific etiology can be found, whereas the rest are secondary to other recognizable underlying disorders...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913484/cold-agglutinin-disease
#8
Sigbjørn Berentsen
Primary chronic cold agglutinin disease (CAD) is a well-defined clinicopathologic entity in which a specific, clonal lymphoproliferative B-cell bone marrow disorder results in autoimmune hemolytic anemia. The immune hemolysis is entirely complement-dependent, predominantly mediated by activation of the classical pathway and phagocytosis of erythrocytes opsonized with complement protein C3b. Typical clinical features in CAD have diagnostic and therapeutic implications. Pharmacologic treatment should be offered to patients with symptom-producing anemia or disabling circulatory symptoms...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913482/update-on-the-diagnosis-and-management-of-paroxysmal-nocturnal-hemoglobinuria
#9
Charles J Parker
Once suspected, the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) is straightforward when flow cytometric analysis of the peripheral blood reveals a population of glycosyl phosphatidylinositol anchor protein-deficient cells. But PNH is clinically heterogeneous, with some patients having a disease process characterized by florid intravascular, complement-mediated hemolysis, whereas in others, bone marrow failure dominates the clinical picture with modest or even no evidence of hemolysis observed. The clinical heterogeneity is due to the close, though incompletely understood, relationship between PNH and immune-mediated bone marrow failure, and that PNH is an acquired, nonmalignant clonal disease of the hematopoietic stem cells...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27906107/previously-undiagnosed-hereditary-spherocytosis-in-a-patient-with-jaundice-and-pyelonephritis-a-case-report
#10
Yuki Tateno, Ryoji Suzuki, Yukihiro Kitamura
BACKGROUND: Hereditary spherocytosis is autosomal dominant inherited extravascular hemolytic disorder and is the commonest cause of inherited hemolysis in northern Europe and the United States. The classical clinical features of hereditary spherocytosis are anemia, jaundice, and splenomegaly. However, all of these classical features are not always revealed in the case of mild hemolysis or when hemolysis is well compensated. Patients with hereditary spherocytosis may remain undiagnosed for years if their hemolysis is mild...
December 1, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#11
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27902998/autoimmune-cytopenias-diagnosis-management
#12
Christian P Nixon, Joseph D Sweeney
The autoimmune cytopenias are a related group of disorders in which differentiated hematopoietic cells are destroyed by the immune system. Single lineage disease is characterized by the production of autoantibodies against red cells (autoimmune hemolytic anemia [AIHA]), platelets (autoimmune thrombocytopenia [ITP]) and neutrophils (autoimmune neutropenia [AIN]) whereas multilineage disease may include various combinations of these conditions. Central to the genesis of this disease is the breakdown of central and/or peripheral tolerance, and the subsequent production of autoantibodies by both tissue and circulating self-reactive B lymphocytes with support from T helper lymphocytes...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27898514/a-rare-combination-of-thrombotic-thrombocytopenic-purpura-and-antiphospholipid-syndrome
#13
Maya Viner, Irina Murakhovskaya
Thrombocytopenia, in the setting of microangiopathic hemolytic anemia and thrombotic events, is characteristic of both thrombotic thrombocytopenic purpura and primary antiphospholipid syndrome. Clinically, it is difficult to distinguish between these two syndromes. We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies. She had clinical manifestations of antiphospholipid syndrome without meeting laboratory criteria of the Sydney classification system...
November 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27898421/stimulation-of-erythrocyte-cell-membrane-scrambling-by-quinine
#14
Morena Mischitelli, Mohamed Jemaà, Mustafa Almasry, Caterina Faggio, Florian Lang
BACKGROUND/AIMS: The analkaloid drug quinine is utilized mainly for the chemoprophylaxis of malaria. The multiple side effects of quinine include hemolytic anemia and hemolytic uremic syndrome, disorders involving suicidal erythrocyte death or eryptosis, which is characterized by cell shrinkage and cell membrane scrambling with phosphatidylserine translocation to the erythrocyte surface. Signaling contributing to stimulation of eryptosis include increase of cytosolic Ca2+ activity ([Ca2+]i), oxidative stress, ceramide and D4476 sensitive casein kinase...
November 30, 2016: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/27885153/karl-otto-landsteiner-1868-1943-physician-biochemist-immunologist
#15
Armond S Goldman, Frank C Schmalsteig
Karl Landsteiner applied the sciences of biochemistry, pathology, microbiology, and immunology in medical research to great success during the first half of the 20th century. Although he is principally known for elucidating the major blood group antigens A and B and their isoantibodies for which he was awarded the Nobel Prize in Physiology or Medicine, Landsteiner made many other important medical discoveries. In that respect, he ascertained that paralytic poliomyelitis was due to a virus, the pancreas was damaged in cystic fibrosis, simple chemicals called haptens were able to combine with antibodies, and the Rh antigen that was later found to be the principal cause of hemolytic anemia of the newborn was found in most humans...
November 24, 2016: Journal of Medical Biography
https://www.readbyqxmd.com/read/27882637/transplantation-associated-thrombotic-microangiopathy-isolated-to-a-congenital-anomaly-of-the-lung
#16
Rebecca T Kummen, Geoffrey D E Cuvelier, Camelia Stefanovici, Anamarija M Perry, Rick Higgins, Rochelle Yanofsky, Suyin A Lum Min, Donna A Wall
TA-TMA is a post-hematopoietic stem cell transplant complication with clinical features of hemolytic anemia and thrombocytopenia. A 26-month-old child who had had an allogeneic transplant for treatment of DBA developed severe TA-TMA with heavy red blood cell and platelet transfusion dependence. Incidentally, he was found to have a lung sequestration. TA-TMA resolved and transfusion dependence resolved after resection of the sequestration. The finding suggests the malformation vasculature was selectively vulnerable to the trigger of TA-TMA-raising perhaps a clue to basic pathophysiology of TA-TMA and/or vascular malformations...
November 24, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27879544/immune-hemolytic-anemia-paroxysmal-cold-hemoglobinuria-preceding-burkitt-lymphoma-in-a-12-year-old-child
#17
Majd T Ghanim, Brian Berman
Autoimmune hemolytic anemia (AIHA) in childhood, including paroxysmal cold hemoglobinuria, is an uncommon, potentially life-threatening disorder. AIHA is a recognized complication of several varieties of lymphoproliferative disorders, including high-grade B-cell lymphoma, but it has not been associated with Burkitt lymphoma in people without an underlying immunodeficiency. When AIHA occurs in association with lymphoproliferative disorders, it may precede or accompany the diagnosis of malignant disease or herald relapse...
November 22, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27879540/severe-hemolytic-anemia-following-intravenous-immunoglobulin-in-an-infant-with-kawasaki-disease
#18
Vlad Tocan, Akari Inaba, Tamami Kurano, Motoshi Sonoda, Keiji Soebijanto, Hideki Nakayama
Severe hemolytic anemia (HA) is an uncommon adverse reaction of intravenous immunoglobulin (IVIg) administration. Previous reports assume that antibodies contained in IVIg preparations are the cause of hemolysis. We report a 10-month-old infant with Kawasaki disease who was treated with high-dose IVIg and developed severe HA. The patient's Rh blood type was D+C+c+E-e+. He developed anti-C and anti-e antibodies following treatment with IVIg, and, after considering all possible causes of hemolysis, we concluded that this was a case of autoimmune HA induced by immunoglobulin treatment...
November 22, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27879189/gemcitabine-induced-hemolytic-uremic-syndrome-treated-with-eculizumab-or-plasmapheresis-two-case-reports%C3%A2
#19
María Esperanza López Rubio, Raquel Rodado Martínez, María Luisa Illescas, Encarnación Mateo Bosch, Mercedes Martínez Díaz, Lourdes de la Vara Inesta, Basilio Cabezuelo, María Elisa Morales Albuja, Eladio Lucas Guillén, Luisa Jimeno García
BACKGROUND: Drug-induced hemolytic-uremic syndrome (HUS) has shown good response to eculizumab (ECU). We present 2 cases of patients with gemcitabine-induced HUS (GEM-HUS), one of whom was treated with ECU and the other with conventional treatment. Patient 1: A 74-year-old male with resected adenocarcinoma of the pancreas started adjuvant treatment with GEM, but after 5 months GEM was discontinued due to acute kidney injury and severe hypertension. Laboratory analyses identified microangiopathic hemolytic anemia (MHA) and thrombocytopenia...
November 23, 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27871907/prognostic-value-of-t786c-and-g894t-enos-polymorphisms-in-sickle-cell-disease
#20
Iakovos Armenis, Vassiliki Kalotychou, Revekka Tzanetea, Panagoula Kollia, Zoi Kontogeorgiou, Dimitra Anastasopoulou, Marina Mantzourani, Michael Samarkos, Konstantinos Pantos, Kostas Konstantopoulos, Ioannis Rombos
Endothelial Nitric Oxide Synthase (eNOS) is crucial for vascular homeostasis. Polymorphisms T786C and G894T are reportedly affecting eNOS regulation and have been related to various diseases. Sickle Cell Disease (SCD), a clinically diverse chronic hemolytic anemia, implies impaired nitric oxide bioavailability. Aim of this study was to determine eNOS genotype for T786C and G894T polymorphisms in Greek patients with SCD and to elucidate its functional consequences and possible effects on clinical phenotype. Seventy nine steady state cases, most of them compound heterozygous for Sickle cell anemia/beta thalassemia and 48 controls were enrolled...
November 18, 2016: Nitric Oxide: Biology and Chemistry
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