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https://www.readbyqxmd.com/read/27913548/warm-antibody-autoimmune-hemolytic-anemia
#1
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
#2
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/27902998/autoimmune-cytopenias-diagnosis-management
#3
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/27882637/transplantation-associated-thrombotic-microangiopathy-isolated-to-a-congenital-anomaly-of-the-lung
#4
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/27879540/severe-hemolytic-anemia-following-intravenous-immunoglobulin-in-an-infant-with-kawasaki-disease
#5
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
#6
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/27868334/consensus-on-the-standardization-of-terminology-in-thrombotic-thrombocytopenic-purpura-and-related-thrombotic-microangiopathies
#7
M Scully, S Cataland, P Coppo, J de la Rubia, K D Friedman, J Kremer Hovinga, B Lämmle, M Matsumoto, K Pavenski, E Sadler, R Sarode, H Wu
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic Microangiopathy is a broad pathophysiological process that leads to microangiopathic hemolytic anemia, thrombocytopenia and involves capillary and small vessel platelet aggregates. The most common cause being disseminated intravascular coagulation (DIC), which may be differentiated by abnormal coagulation. Clinically, a number of conditions present with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT), including cancer, infection, transplantation, drugs, autoimmune disease and pre-eclampsia and HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome in pregnancy...
November 21, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27864296/a-mechanistic-investigation-of-thrombotic-microangiopathy-associated-with-intravenous-abuse-of-opana-er
#8
Ryan Hunt, Ayla Yalamanoglu, James Tumlin, Tal Schiller, Jin Hyen Baek, Andrew Wu, Agnes B Fogo, Haichun Yang, Edward Wong, Peter Miller, Paul W Buehler, Chava Kimchi-Sarfaty
Since 2012, a number of case reports have described the occurrence of thrombotic microangiopathy (TMA) following intravenous abuse of extended release oxymorphone hydrochloride (Opana ER), an oral opioid for long-term treatment of chronic pain. Here, we present unique clinical features of three patients and investigate intravenous exposure to the tablet's inert ingredients as a possible causal mechanism. Guinea pigs were used as an animal model to understand the hematopathologic and nephrotoxic potential of the inert ingredient mixture (termed here as PEO+) which primarily contains high molecular weight polyethylene oxide (HMW PEO)...
November 18, 2016: Blood
https://www.readbyqxmd.com/read/27861376/risk-factor-analysis-of-autoimmune-hemolytic-anemia-after-allogeneic-hematopoietic-stem-cell-transplantation-in-children
#9
Tsung-Yen Chang, Tang-Her Jaing, Yu-Chuan Wen, I-Anne Huang, Shih-Hsiang Chen, Pei-Kwei Tsay
Autoimmune hemolytic anemia (AIHA) is a clinically relevant complication after allogeneic hematopoietic stem cell transplantation (HSCT). Currently, there is no established consensus regarding the optimal therapeutic approach. Whether AIHA contributes to increased mortality is still somewhat controversial.We investigated the incidence, risk factors, and outcome of post-transplant AIHA in 265 consecutive pediatric patients undergoing allo-HSCT over a 17-year period. Onset of AIHA was calculated from the first documented detection of AIHA by either clinical symptoms or positive direct agglutinin test...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27852481/mitral-paravalvular-leak-closure
#10
REVIEW
Paul Sorajja
For patients with paravalvular mitral prosthetic regurgitation, percutaneous repair is an established therapy for the treatment of symptoms of heart failure or hemolytic anemia. Percutaneous repair of paravalvular mitral regurgitation is a complex procedure with unique technical challenges, even when performed in experienced centers. Herein, the author discusses patient selection, catheter-based techniques for repair, and clinical outcomes of percutaneous repair for paravalvular mitral regurgitation.
January 2016: Interv Cardiol Clin
https://www.readbyqxmd.com/read/27833958/clinical-importance-of-pharmacogenetics-in-the-treatment-of-hepatitis-c-virus-infection
#11
Adina Maria Kamal, Paul MitruŢ, Kamal Constantin Kamal, Oana Sorina Tica, Mihaela Niculescu, Dragoş Ovidiu Alexandru, Andrei Adrian Tica
Globally, over 4% of the world population is affected by hepatitis C virus (HCV) infection. The current standard of care for hepatitis C infection is combination therapy with pegylated interferon and ribavirin for 48 weeks, which yield a sustained virological response in only a little over half of the patients with genotype 1 HCV. We investigated the clinical importance of pharmacogenetics in treatment efficacy and prediction of hematotoxicity. A total of 148 patients infected with HCV were enrolled. All patients were treated for a period of 48 weeks or less with pegylated interferon and ribavirin...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27818359/delayed-haemolysis-secondary-to-treatment-of-severe-malaria-with-intravenous-artesunate-report-on-the-experience-of-a-referral-centre-for-tropical-infections-in-spain
#12
Teresa Aldámiz-Echevarría Lois, Ana López-Polín, Francesca F Norman, Begoña Monge-Maillo, Rogelio López-Vélez, Jose Antonio Pérez-Molina
BACKGROUND: Post-artesunate delayed haemolysis is described as hemolytic anemia presenting days after malaria treatment in hyperparasitemic patients. Physiopathological mechanisms and clinical manifestations have not been thoroughly characterised. METHODS: We conducted a retrospective study of hospitalised malaria patients who received artemisinin derivatives from January 1, 2010 to December 31, 2015. RESULTS: 21 patients were included in the study: 11 travellers, 8 travellers visiting friends and relatives and 2 immigrants...
November 3, 2016: Travel Medicine and Infectious Disease
https://www.readbyqxmd.com/read/27817114/nitrogen-containing-bisphosphonate-induces-a-newly-discovered-hematopoietic-structure-in-the-omentum-of-an-anemic-mouse-model-by-stimulating-g-csf-production
#13
Hirotada Otsuka, Hideki Yagi, Yasuo Endo, Satoshi Soeta, Naoko Nonaka, Masanori Nakamura
We previously reported that the injection of nitrogen-containing bisphosphonate (NBP) induced the site of erythropoiesis to shift from the bone marrow (BM) to the spleen. Our previous study established a severely anemic mouse model that was treated with a combination of NBP with phenylhydrazine (PHZ), which induced newly discovered hematopoietic organs in the omentum. No reports have shown that new hematopoietic organs form under any condition. We characterized the structures and factors related to the formation of these new organs...
November 5, 2016: Cell and Tissue Research
https://www.readbyqxmd.com/read/27778386/case-of-pediatric-bullous-systemic-lupus-erythematosus-treated-with-intravenous-immunoglobulin
#14
Margit Juhász, Megan Rogge, Mary Chen, Annette Czernik, Soo Jung Kim, Lauren Geller
This is a case report of a 16-year-old girl recently diagnosed with systemic lupus erythematosus (SLE) who presented with multiple blisters on the face, hands, arms, legs, trunk, and vaginal and oral mucosa. Skin biopsy was consistent with bullous SLE (BSLE). Dapsone is often the first-line treatment option for BSLE, but the patient's history of anemia and leukopenia and long-term immunosuppression requirement for her systemic symptoms raised concerns about dapsone and bone marrow toxicity, especially hemolytic anemia and agranulocytosis...
October 25, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27777394/systemic-lupus-erythematosus-presenting-as-refractory-thrombotic-thrombocytopenic-purpura-a-diagnostic-and-management-challenge-a-case-report-and-concise-review-of-the-literature
#15
Mohammad Abu-Hishmeh, Alamgir Sattar, Fnu Zarlasht, Mohamed Ramadan, Aisha Abdel-Rahman, Shante Hinson, Caroline Hwang
BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE...
October 25, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27776934/the-antiphospholipid-syndrome-in-patients-with-systemic-lupus-erythematosus
#16
Guillermo J Pons-Estel, Laura Andreoli, Francesco Scanzi, Ricard Cervera, Angela Tincani
The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL). APS may be associated with other diseases, mainly systemic lupus erythematosus (SLE). The presence or absence of SLE might modify the clinical or serological expression of APS. Apart from the classical manifestations, APS patients with associated SLE more frequently display a clinical profile with arthralgias, arthritis, autoimmune hemolytic anemia, livedo reticularis, epilepsy, glomerular thrombosis, and myocardial infarction...
October 21, 2016: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27768015/hemolytic-uremic-syndrome-in-children
#17
Valentina Talarico, Monica Aloe, Alice Monzani, Roberto Miniero, Gianni Bona
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non-immune microangiopathic hemolytic anemia and acute renal failure. HUS is typically classified into two primary types: 1) HUS due to infections, often associated with diarrhea (D+HUS, Shiga toxin-producing Escherichia Coli-HUS), with the rare exception of HUS due to a severe disseminated infection caused by Streptococcus; 2) HUS related to complement, such HUS is also known as "atypical HUS" and is not diarrhea associated (D-HUS, aHUS); but recent studies have shown other forms of HUS, that can occur in the course of systemic diseases or physiopathological conditions such as pregnancy, after transplantation or after drug assumption...
December 2016: Minerva Pediatrica
https://www.readbyqxmd.com/read/27766045/clinical-evaluation-of-thrombotic-microangiopathy-identification-of-patients-with-suspected-atypical-hemolytic-uremic-syndrome
#18
Yu-Min Shen
Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by defective complement regulation resulting in thrombotic microangiopathy (TMA). Patients can present as children or adults. The syndrome consists of hemolytic anemia with schistocytosis, thrombocytopenia, significant renal damage, and/or other organ system dysfunction(s). Patients with aHUS may succumb to the complications of the disease with the very first manifestation; surviving patients often suffer from progressive organ dysfunction with significant morbidity and mortality despite plasma infusion or plasma exchange...
2016: Thrombosis Journal
https://www.readbyqxmd.com/read/27765664/differentiation-of-pernicious-anemia-from-thrombotic-thrombocytopenic-purpura-the-clinical-value-of-subtle-pathologic-findings
#19
Daniel W Abbott, Kenneth D Friedman, Matthew S Karafin
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic hemolytic anemia that requires emergent treatment with plasma exchange and is one of the most important conditions for which apheresis service professionals are consulted. Careful interpretation of initial laboratory values and the peripheral blood smear is a critical first step to determining the need for plasma exchange because other conditions can show deceptively similar red cell morphology, and ADAMTS13 levels are often not rapidly available...
September 28, 2016: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/27760006/autoimmune-phenomena-in-patients-with-solid-tumors
#20
Ivanka S Nenova, Mariana Y Valcheva, Elina A Beleva, Dora Y Tumbeva, Marianna P Yaneva, Emilia L Rancheva, Zhanet G Grudeva-Popova
INTRODUCTION: Autoimmune disorders have been documented in solid tumors and malignant hematological disorders. They are very common and well studied in lymphomas which are associated with immune imbalance. They are less common in solid tumors and are categorized as paraneoplastic syndromes with unclear pathogenesis. AIM: The aim of the present study was to find the frequency of autoimmune phenomena in solid tumors of various origin, location and status of the tumor...
September 1, 2016: Folia Medica
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