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 Immune thrombocytopenic purpura

Amihai Rottenstreich, Noa Israeli, Gabriel Levin, Misgav Rottenstreich, Uriel Elchalal, Yosef Kalish
OBJECTIVE: Gestational thrombocytopenia (GT) accounts for 75% of cases of thrombocytopenia in pregnancy. In most cases of GT, thrombocytopenia is mild (100-150 × 109 /L) and has no consequences for either the mother or the fetus. We aimed to investigate the characteristics, neonatal risk and recurrence rate of GT with a platelet count <100 × 109 /L. STUDY DESIGN: We reviewed the records of women who delivered during 2006-2016 at a large tertiary care university hospital, and who had platelet count <100 × 109 /L during pregnancy...
October 10, 2018: European Journal of Obstetrics, Gynecology, and Reproductive Biology
Isabella Menchetti, Yulia Lin, Christine Cserti-Gazdewich, Jenette Goldstein, Calvin Law, Alan Lazarus, Jeannie L Callum
BACKGROUND: Evans syndrome is a rare autoimmune disorder that is defined by the simultaneous or sequential presence of two or more cytopenias without an obvious underlying precipitating cause. Evans syndrome usually follows a chronic relapsing and remitting course and is quite rare, making it difficult to evaluate in clinical studies. CASE REPORT: A 66-year-old male patient with a 17-year history of Evans syndrome presented with fulminant autoimmune hemolytic anemia (AIHA)...
October 6, 2018: Transfusion
Sara Aljarad, Ahmad Alhamid, Ahmad Sankari Tarabishi, Ameen Suliman, Ziad Aljarad
Background: Idiopathic (immune) thrombocytopenic purpura (ITP) is an acquired disorder characterized by autoantibodies against platelet membrane antigens. Several studies found an association between Helicobacter Pylori infection and the incidence of ITP. So far, It is still unclear whether H. pylori eradication will increase platelet counts in adult ITP patients. We conduct this study to investigate platelet recovery in ITP patients after H. pylori eradication. Methods: This is a prospective study...
2018: BMC Hematology
Elien Roose, Gestur Vidarsson, Kadri Kangro, Onno J H M Verhagen, Ilaria Mancini, Linda Desender, Inge Pareyn, Nele Vandeputte, Aline Vandenbulcke, Chiara Vendramin, An-Sofie Schelpe, Jan Voorberg, Marie-Agnès Azerad, Laurent Gilardin, Marie Scully, Daan Dierickx, Hans Deckmyn, Simon F De Meyer, Flora Peyvandi, Karen Vanhoorelbeke
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by severe ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency, the presence of anti-ADAMTS13 autoantibodies and an open ADAMTS13 conformation with a cryptic epitope in the spacer domain exposed. A detailed knowledge of anti-ADAMTS13 autoantibodies will help identifying pathogenic antibodies and elucidating the cause of ADAMTS13 deficiency. We aimed at cloning anti-ADAMTS13 autoantibodies from iTTP patients to study their epitopes and inhibitory characteristics...
October 2018: Thrombosis and Haemostasis
Martin Schipperus, Georgia Kaiafa, Louise Taylor, Sally Wetten, Georg Kreuzbauer, Andy Boshier, Anouchka Seesaghur
INTRODUCTION: Romiplostim is a subcutaneously administered thrombopoietin-receptor agonist approved in the European Union for self-administration (or administration by a caregiver) in selected adult patients with chronic primary immune thrombocytopenia refractory to other treatments. To mitigate the risk of medication errors due to self-administration, the manufacturer has implemented additional risk minimisation measures (RMM) in the form of a Home Administration Training (HAT) pack to support the training of both healthcare professionals (HCPs) (guide and checklist for patient selection and training) and patients (a preparation mat, quick guide booklet, step-by-step guide, self-administration diary and DVD/video)...
September 19, 2018: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
Cyrielle Tomich, Sabrina Debruxelles, Yahsou Delmas, Sharmila Sagnier, Mathilde Poli, Stéphane Olindo, Pauline Renou, François Rouanet, Igor Sibon
INTRODUCTION: Immune thrombotic thrombocytopenic purpura (i-TTP), related to acquired ADAMTS-13 dysfunction, can lead to various neurological symptoms including ischemic stroke. To date the clinical, radiological, and biological characteristics of patients having a stroke as the inaugural manifestation of i-TTP are largely unknown. METHODS: Probable immune-TTP was defined by a low ADAMTS-13 activity associated with the presence of ADAMTS-13 inhibitors and/or favorable clinicobiological response under immunological treatments...
September 12, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Amihai Rottenstreich, Noa Israeli, Batia Roth, Uriel Elchalal, Hagai Amsalm, Nael Da'as, Misgav Rottenstreich, Yosef Kalish
OBJECTIVES: To characterize the risk factors associated with neonatal thrombocytopenia among pregnant women with immune thrombocytopenic purpura (ITP). METHODS: We reviewed the records of ITP patients who delivered during 2006-2016 at our medical center. RESULTS: Of 253 pregnancies, median maternal age at diagnosis was 29 [25-33] years, 222 (87.7%) had previously-diagnosed ITP and 31 (12.3%) were diagnosed with new-onset ITP during pregnancy...
September 13, 2018: Journal of Maternal-fetal & Neonatal Medicine
Lucy Neave, Marie Scully
Thrombotic microangiopathies (TMAs) are associated with microangiopathic hemolytic anemia and thrombocytopenia, resulting in microvascular thrombosis and end-organ damage. In pregnancy, this may be the result of pregnancy-related TMAs such as preeclampsia; hemolysis, elevated liver enzymes, and low platelets; or pregnancy-associated TMAs, specifically thrombotic thrombocytopenic purpura (TTP) or complement-mediated hemolytic uremic syndrome (CM HUS). TTP and CM HUS are rare disorders, and their diagnosis may be missed, no less because features at presentation may be misdiagnosed as a pregnancy-related TMA, such as hypertension, proteinuria, fetal growth restriction, or in utero fetal death...
October 2018: Transfusion Medicine Reviews
Keiko Matsuoka, Itaru Yanagihara, Yukiko Kawazu, Maho Sato, Masahiro Nakayama, Yukiko Nakura, Nao Kanagawa, Yukihiro Akeda, Futoshi Fujiwara, Kazutoshi Itoh, Hiroaki Kawata, Makoto Takeuchi
We report the first case of a teenage patient with chromosome 22q11.2 deletion syndrome who died of overwhelming postsplenectomy infection (OPSI) by Streptococcus pneumoniae despite appropriate prevention by pneumococcal vaccine. He had congenital heart disease and underwent several surgeries. Immunodeficiency had not been noticed clinically. Two years prior to death, splenectomy was performed for a drug-resistant idiopathic thrombocytopenic purpura and he was immunized with 23-valent pneumococcal polysaccharide vaccine (PPV23) 4 months after splenectomy...
August 29, 2018: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
Elizabeth M Staley, Wenjing Cao, Huy P Pham, Chong H Kim, Nicole K Kocher, Lucy Zheng, Radhika Gangaraju, Robin G Lorenz, Lance A Williams, Marisa B Marques, X Long Zheng
Immune-mediated thrombotic thrombocytopenic purpura is characterized by severe thrombocytopenia and microangiopathic hemolytic anemia. It is primarily caused by immunoglobin G type autoantibodies against ADAMTS13, a plasma metalloprotease that cleaves von Willebrand factor. However, reliable markers predictive of patient outcomes are yet to be identified. Seventy-three unique patients with a confirmed diagnosis of immune-mediated thrombotic thrombocytopenic purpura between April 2006 and December 2017 were enrolled from the Univeristy of Alabama at Birmingham Medical Center...
August 31, 2018: Haematologica
Shahana Gupta, Raja Kalayarasan, Sandip Chandrasekar, Senthil Gnanasekaran, Biju Pottakkat
Laparoscopic approach is considered as gold standard for splenectomy in patients with Immune Thrombocytopenic purpura (ITP). The evidence for safety and feasibility of laparoscopic splenectomy (LS) in patients with very severe thrombocytopenia (< 10,000 μL) is limited. A retrospective study of 32 ITP patients who underwent LS between July 2012 and November 2016. The ITP patients who had platelet counts < 10,000 μL (Group A, n = 15) and > 10,000 μL (Group B, n = 17) were compared with respect to operative time, blood loss, conversion rate, perioperative blood transfusion, the length of hospital stay and postoperative complications...
July 2018: Indian Journal of Hematology & Blood Transfusion
Danny Kanhai, René Mulder, Hans Kristian Ploos van Amstel, Roger Schutgens, Michael Lukens, Rienk Y J Tamminga
Congenital thrombocytopenia can easily be misdiagnosed as immune thrombocytopenic purpura, as is illustrated by this case of a woman and her two children. Doubts arose when steroid/IVIG therapy failed in the mother and the thrombocytopenia in the children persisted. By means of next-generation sequencing, two missense variants in cis in the ACTN1 gene of the affected family members were identified, both of unknown significance. We conclude, after further analysis of these mutations with, among others, in silico prediction tools, that the thrombocytopenia has a genetic cause, in particular the ACTN1 mutations, and is not immune mediated...
August 19, 2018: Pediatric Blood & Cancer
(no author information available yet)
The titer of live attenuated viral vaccines, such as MMR vaccines, varies between batches and over the shelf-life of a batch, with the highest titer expected at batch release. As higher titers may theoretically lead to increased reactogenicity, we compared the safety profile of an upper-range release titer MMR-RIT lot with commercial MMR II lots in a phase III, randomized, controlled study (NCT02184572). We vaccinated 1736 children with MMR-RIT (N = 1164) or MMR II (N = 572), both administered as first doses with varicella, hepatitis A, and pneumococcal conjugate vaccines at 12-15 months of age...
August 17, 2018: Human Vaccines & Immunotherapeutics
S Badagabettu, D M Nayak, A Kurien, V G Kamath, A Kamath, A George
INTRODUCTION: The awareness and knowledge on bleeding disorders is generally poor among the rural population. Accredited Social Health Activists (ASHAs) serve as the facilitators between the rural community and the health care system. Training of ASHAs in screening of rural population for early identification of bleeding disorders can enable prompt referral, timely detection and management of bleeding disorders. AIM: The aim of the study was to evaluate the effectiveness of an ASHA training programme for identification of suspected bleeding disorder cases...
September 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
Charlotte K Brierley, Sue Pavord
The autoimmune cytopenias are a group of disorders resulting primarily from autoantibody-mediated destruction of blood cells, with variable clinical sequelae depending on the severity and lineage affected. Disease presentation ranges from an asymptomatic finding on a routine full blood count to an acutely unwell patient suffering the clinical consequences of severe anaemia, neutropenia or thrombocytopenia. The cytopenia may be primary or secondary to underlying infectious, immune or malignant processes. Thrombotic thrombocytopenic purpura (TTP) is a distinct, rare but potentially life-threatening entity that classically but not invariably presents with a pentad of acute onset haemolytic anaemia, thrombocytopenia, neurological symptoms, renal impairment and fevers...
August 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
Neslihan Karakurt, İlker Uslu, Canan Albayrak, Leman Tomak, Elif Ozyazici, Davut Albayrak, Canan Aygun
: A major problem associated with immune thrombocytopenic purpura (ITP) in pregnancy is neonatal thrombocytopenia. We analyzed newborns born to mothers with ITP and examined predictive factors for thrombocytopenia. This retrospective study was performed in a single academic center from January 2007 to January 2018. Pregnant women with ITP and their babies are included. All neonates had a complete blood count and cranial ultrasound (USG) performed. Twenty seven neonates of 22 mothers were evaluated. A total of 23 (85%) of neonates were thrombocytopenic (<150 × 10/l) and in 20 (74%) platelet count was below 50 × 10/l...
September 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Aneet Singh, Bhagyashri Hungund, Lalit Kumar, Mallikarjun Pattanshetti
Bicytopenia is the reduction of any of the two cell lines of blood, i.e., erythrocytes, leukocytes or platelets. Many studies are done on pancytopenia but very few studies exist in the literature evaluating the spectrum of aetiologies of bicytopenia. To date, no study is available on bicytopenia in adults. We aimed to study the clinico-haematological profile of patients with bicytopenia and to investigate the different aetiologies of bicytopenia. Four hundred patients with bicytopenia admitted to KLE Dr. Prabhakar Kore Charitable Hospital and Medical Research Centre were selected using systematic random sampling and included in the study...
August 2018: Pathology
Meera Sridharan, Ronald S Go, Maria A V Willrich
Thrombotic microangiopathies (TMA) are a class of disorders characterized by microangiopathic hemolytic anemia, non-immune thrombocytopenia, and organ dysfunction. One type of TMA is atypical hemolytic uremic syndrome (aHUS) a disorder caused by hyper-activation of the alternative complement pathway due to over activation of C3 convertases and loss of complement regulatory mechanisms. The pathophysiological mechanism of aHUS involves increased continuous spontaneous hydrolysis of C3 to C3b which leads to tissue deposition of C3b, the membrane attack complex formation and subsequent tissue injury...
October 2018: Journal of Immunological Methods
Yuan-Yuan Lu, Na Guan, Qing-Hong Meng, Ming-Lei Li, Yun-Yun Liu, Ying Wang
OBJECTIVE: To explore the efficacy and safety of recombinant human thrombopoietin (rhTPO) combined with high-dose dexamethasone (DXM) in the treatment of children with refractory immune thrombocytopenic purpura (ITP). METHODS: Fifty-eight ITP children who had failed first-line therapy were randomly divided into two groups: DXM treatment (n=27) and rhTPO + DXM treatment (n=31). The DXM treatment group received two continuous cycles of DXM treatment; in each cycle, patients received high-dose DXM (0...
July 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
José Carlos Jaime-Pérez, Goel Treviño-Reyna, Patrizia Aguilar-Calderón, Olga G Cantú-Rodríguez, Luis Javier Marfil-Rivera, David Gómez-Almaguer
OBJECTIVES: To demonstrate the importance of regional efforts to register features and report frequency of hematology diseases in the context of incomplete national registries. METHODS: Frequencies and salient characteristics of hematologic diseases in Northeast Mexico were documented in a reference center at a tertiary care university hospital during the decade 2005-2015. Disease categories were grouped by age, sex and diagnosis. Age group distribution followed WHO guidelines in years as children (0-17), adults (18-64) and elders (+65)...
July 16, 2018: Hematology (Amsterdam, Netherlands)
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