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Autoimmune hemolytic anemia

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
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 PCR-RFLP technique, using DNA extracted from peripheral blood samples obtained from patients with SLE (n=91) and controls (n=97). RESULTS AND CONCLUSIONS: The 857GA genotype was more prevalent among nonwhite SLE patients (OR=4...
September 28, 2016: Revista Brasileira de Reumatologia
Filiz Kizilirmak, Gultekin Gunhan Demir, Beytullah Cakal, Hüseyin Saffet Bekoz, Fatih Erkam Olgun
Evans syndrome (ES) is a rare hematological disease characterized by autoimmune hemolytic anemia, immune thrombocytopenia, and/or neutropenia, all of which may be seen simultaneously or subsequently. Thrombotic events in ES are uncommon. Furthermore, non-ST segment-elevation myocardial infarction (NSTEMI) during ES is a very rare condition. Here, we describe a case of a 69-year-old female patient presenting with NSTEMI and ES. Revascularization via percutaneous coronary intervention (PCI) was scheduled and performed...
September 2016: Indian Heart Journal
Vandana Pradhan, Pallavi Pandit, Anjali Rajadhyaksha, Manisha Patwardhan, Prathamesh Surve, Pradnya Kamble, Maxime Lecerf, Jagadeesh Bayry, Srinivas Kaveri, K Ghosh, Milind Y Nadkar
OBJECTIVE: To identify the hematological manifestations and its association with serum ferritin levels in SLE patients from Western India. METHODS: Ninety clinically diagnosed SLE patients fulfilling ACR criteria were included. Disease activity was assessed at the time of evaluation using Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Sera were tested for serum ferritin levels by ELISA (Calbiotech, USA). Autoantibodies such as ANA, anti-dsDNA by indirect immunofluorescence test (IFA- Bio-Rad, USA) and anti-cardiolipin antibodies (ACA) to IgG and IgM isotypes and Anti-β2 GP antibodies to IgG and IgM isotypes were detected by ELISA using commercially available kits (Euroimmun, Lubeck, Germany)...
May 2016: Journal of the Association of Physicians of India
Kazuyo Fujimoto, Takatoshi Nakashima, Kazunari Sasaki, Kenichi Hayashi, Masao Hanafusa, Shiei Yoshida, Satoshi Myojo, Shun-Ichi Yoshida, Shigeaki Sawai, Nobuya Sano
A 48-year-old woman with a history of autoimmune hemolytic anemia and taking long-term corticosteroid therapy presented with a 3-month history of general fatigue, abdominal distension, and pigmentation. A computed tomography scan of the abdomen showed a tumor in the sigmoid colon and multiple metastatic nodules in the liver. A colonoscopy revealed an obstructing mass with the presence of an irregular ulcer in the sigmoid colon. Following biopsy and histopathological analysis, the patient was diagnosed with neuroendocrine carcinoma (NEC) of the colon...
2016: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
Ulla Derhaschnig, Jim Gilbert, Ulrich Jäger, Georg Böhmig, Georg Stingl, Bernd Jilma
BACKGROUND: Innovative trial designs are sought to streamline drug development in rare diseases. Basket- and integrated protocol designs are two of these new strategies and have been applied in a handful oncologic trials. We have taken the concept outside the realm of oncology and report about a first-in-human integrated protocol design that facilitates the transition from phase Ia in healthy volunteers to phase Ib in patients with rare complement-mediated disorders driven by the classical pathway...
October 4, 2016: Orphanet Journal of Rare Diseases
Amanda L Richards, Linda M Kapp, Xiaohong Wang, Heather L Howie, Krystalyn E Hudson
Autoimmune hemolytic anemia (AIHA) occurs when pathogenic autoantibodies against red blood cell (RBC) antigens are generated. While the basic disease pathology of AIHA is well studied, the underlying mechanism(s) behind the failure in tolerance to RBC autoantigens are poorly understood. Thus, to investigate the tolerance mechanisms required for the establishment and maintenance of tolerance to RBC antigens, we developed a novel murine model. With this model, we evaluated the role of regulatory T cells (Tregs) in tolerance to RBC-specific antigens...
2016: Frontiers in Immunology
Marc Michel, Louis Terriou, Francoise Roudot-Thoraval, Mohamed Hamidou, Mikael Ebbo, Guillaume Le Guenno, Lionel Galicier, Sylvain Audia, Bruno Royer, Anne Sophie Morin, Jean Marie Michot, Arnaud Jaccard, Laurent Frenzel, Mehdi Khellaf, Bertrand Godeau
This phase 3 multicentre randomized double-blind and placebo-controlled trial aimed to compare the efficacy and safety of rituximab (RTX) to placebo for treating newly diagnosed warm autoimmune hemolytic anemia (wAIHA) in adults receiving prednisone. Adults with a confirmed diagnosis of wAIHA who previously received corticosteroids for less than 6 weeks could be included. At inclusion, all patients received prednisone at a daily dose of 1 mg/kg for 2 weeks, then tapered according to a pre-defined recommended reduction scheme...
October 3, 2016: American Journal of Hematology
Shahrzad Bakhtiar, Frank Ruemmele, Fabienne Charbit-Henrion, Eva Lévy, Frédéric Rieux-Laucat, Nadine Cerf-Bensussan, Peter Bader, Ulrich Paetow
Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type 1 diabetes mellitus, hypothyroidism, adrenal insufficiency, and vitiligo. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency was described in 2012 as a novel primary immunodeficiency, predominantly causing immune dysregulation and early onset enteropathy. We describe the heterogeneous clinical course of LRBA deficiency in two siblings, mimicking an autoimmune polyendocrine disorder in one of them in presence of the same underlying genetic mutation...
2016: Frontiers in Pediatrics
Jun He, Mihua Liu, Zhufeng Ye, Tianping Tan, Xinghui Liu, Xiaoxing You, Yanhua Zeng, Yimou Wu
Mycoplasma are the smallest prokaryotic microbes present in nature. These wall‑less, malleable organisms can pass through cell filters, and grow and propagate under cell‑free conditions in vitro. Of the pathogenic Mycoplasma Mycoplasma pneumoniae has been examined the most. In addition to primary atypical pneumonia and community‑acquired pneumonia with predominantly respiratory symptoms, M. pneumoniae can also induce autoimmune hemolytic anemia and other diseases in the blood, cardiovascular system, gastrointestinal tract and skin, and can induce pericarditis, myocarditis, nephritis and meningitis...
September 23, 2016: Molecular Medicine Reports
R Patejdl, M Wittstock, U K Zettl, K Jost, A Grossmann, J Prudlo
INTRODUCTION: Recently defined consensus criteria for the diagnosis of neuromyelitis optica spectrum disorders (NMOSD) allow establishing the diagnosis in patients without elevated AQP4-Ab and optic nerve involvement. According to the new extended definition, NMOSD is closely associated with extensive spinal cord inflammation occurring in the course of systemic autoimmune diseases as sarcoidosis or lupus erythematodes. NMOSD occurring in the course of hematological disease have not yet been reported in the literature...
September 2016: Multiple Sclerosis and related Disorders
Taro Yoneda, Hayato Koba, Kota Tanimura, Naohiko Ogawa, Satoshi Watanabe, Johsuke Hara, Miki Abo, Takashi Sone, Hideharu Kimura, Kazuo Kasahara
A 50-year-old man presented to our hospital in 1995. Invasive thymoma was diagnosed and extended thymectomy and left upper lobe partial resection were performed. In 2013, he complained of dyspnea. Chest computed tomography showed postoperative recurrence of invasive thymoma. Several chemotherapies were administered. Severe anemia and an increase in the total bilirubin level were observed with chemotherapies. In additional, an examination showed that the direct Coombs test was positive. Cold agglutinin was also high...
2016: Internal Medicine
Arun Panigrahi, Amy Clark, John Myers, Ashok Raj
BACKGROUND: Successful treatment of both pediatric autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP), specifically those that are refractory to first-line therapies, remains unsatisfactory in terms of long-term remission and medication side effects. Here, we propose a novel combination therapy of mycophenolate mofetil (MMF), an adjunct immunosuppressive, and short-term corticosteroids for the treatment of persistent or chronic autoimmune cytopenias in children...
September 12, 2016: Pediatric Blood & Cancer
Uroosa Ibrahim, Muhammad N Siddique, Gautam Valecha, Masoud Asgari, Edhan Isaac, Meekoo Dhar
A 46‑year‑old obese male with a medical history of thalassemia minor presented to the emergency room with complaints of severe fatigue and jaundice worsening over two weeks. On further evaluation, the patient was found to have significant hyperbilirubinemia and transaminitis. The hospital course was further complicated by pancytopenia requiring multiple transfusions, worsening hyperbilirubinemia, severe hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia. He was also found to have splenomegaly and evidence of hemophagocytosis on bone marrow biopsy...
2016: Curēus
Venkateswara K Kollipara, Patrick L Brine, David Gemmel, Sisham Ingnam
Pernicious anemia is an autoimmune disease with a variety of clinical presentations. We describe a case of pernicious anemia presenting with pancytopenia with hemolytic features. Further workup revealed very low vitamin B12 levels and elevated methylmalonic acid. It is important for a general internist to identify pernicious anemia as one of the cause of pancytopenia and hemolytic anemia to avoid extensive workup. Pernicious anemia can present strictly with hematological abnormalities without neurological problems or vice versa as in our case...
2016: Journal of Community Hospital Internal Medicine Perspectives
Maria Atta, Eimear T Brannigan, Barbara J Bain
No abstract text is available yet for this article.
September 6, 2016: American Journal of Hematology
Shingo Noguchi, Kazuhiro Yatera, Mitsutaka Jinbo, Sohsuke Yamada, Ikuko Shimabukuro, Kei Yamasaki, Takashi Kido, Hiroshi Ishimoto, Chiharu Yoshii, Hiroshi Mukae
We herein report a case of IgG4-related lung disease (IgG4-RLD) associated with autoimmune hemolytic anemia (AIHA). A 73-year-old Japanese female visited our hospital for an examination following an abnormal chest X-ray in 1999. She was diagnosed with bronchiolitis and AIHA, and treatment with prednisolone was started. After seven years, she visited our department due to a cough. Chest computed tomography (CT) demonstrated focal consolidation with ground-glass attenuations and thickened bronchial walls in the bilateral lungs...
2016: Internal Medicine
Pandiarajan Vignesh, Deepti Suri, Amit Rawat, Yu Lung Lau, Anmol Bhatia, Ashim Das, Anirudh Srinivasan, Sivashanmugam Dhandapani
Patients with Wiskott-Aldrich syndrome (WAS) are predisposed to malignancy and autoimmunity in addition to infections. We report a male child with WAS, who had presented with recurrent pneumonia, eczema, thrombocytopenia, autoimmune hemolytic anemia, and vasculitic skin lesions. Genetic analysis revealed a classical genotype WAS 155C>T; R41X. At 2 years of follow-up, he developed persistent headache and progressive hepatomegaly. Brain imaging showed a mass in the right frontal region, which on histopathology was shown to be high-grade non-Hodgkin lymphoma...
August 27, 2016: Pediatric Blood & Cancer
Atsuhito Uneda, Kenta Suzuki, Shuichi Okubo, Koji Hirashita, Masatoshi Yunoki, Kimihiro Yoshino
BACKGROUND: Nocardia infection of the central nervous system leading to brain abscess is a rare condition but has a high mortality rate. Among the species of Nocardia, only three cases of brain abscess due to Nocardia asiatica infection have been reported. CASE DESCRIPTION: A 65-year-old man with a history of autoimmune hemolytic anemia treated with prednisolone presented to our hospital because of occipital headache. Brain magnetic resonance imaging showed bilateral occipital lesions...
2016: Surgical Neurology International
Paola Ester López-Díaz, María Del Rocío Ruiz-Olivera, Luis Alberto Hernández-Osorio, Jaime Vargas-Arzola, Xareni Valle-Jiménez, Sergio Roberto Aguilar-Ruiz, Honorio Torres-Aguilar
Irregular antibodies are produced by alloimmunization because of pregnancies or blood transfusions. They are called "irregular" due to target erythrocyte antigens from "rare blood systems," those different from the ABO system. Irregular antibodies have been widely investigated in immunohematology since their presence in blood donors may lead to difficulties in blood typing and in blood cross-matching, or to induce hemolytic transfusion reactions. Nevertheless, their incidence and participation in the physiopathology of autoimmune diseases have not been thoroughly studied...
August 25, 2016: Immunologic Research
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