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 Immune thrombocytopenic purpura

Feng-Xia Zhan, Juan Li, Min Fang, Juan Ding, Qian Wang
BACKGROUND The disequilibrium of T helper (Th) cells play an important role in the occurrence and development of immune thrombocytopenic purpura (ITP). Th22 cells, as a newly discovered subset of T lymphocytes, plays an important role in autoimmune disorders and inflammatory diseases. MATERIAL AND METHODS This study explored the role of different lymphocyte subsets in chronic ITP. To explore the value of Th22 cells in the diagnosis of ITP, the numbers of Th1, Th17, and Th22 cells were detected by a 4-color flow cytometric in 32 chronic ITP patients and 30 healthy controls...
December 4, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
Kathryn Dane, Shruti Chaturvedi
The advent of plasma exchange has dramatically changed the prognosis of acute thrombotic thrombocytopenic purpura (TTP). Recent insights into TTP pathogenesis have led to the development of novel therapies targeting pathogenic anti-ADAMTS13 antibody production, von Willebrand factor (VWF)-platelet interactions, and ADAMTS13 replacement. Retrospective and prospective studies have established the efficacy of rituximab as an adjunct to plasma exchange for patients with acute TTP, either upfront or for refractory disease...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
Tetsuo Nakayama
Vaccine adverse events and controversial safety issues have occurred in recent decades in Japan: aseptic meningitis following the measles-mumps-rubella combined vaccine (MMR), anaphylaxis after immunization with live virus vaccines and inactivated split influenza vaccine, an increased incidence of febrile illness following the simultaneous administration of inactivated vaccines, and chronic pain with neurological illness after immunization with the human papilloma virus vaccine (HPV). Vaccine adverse events are a matter of concern for the public as well as general practitioners; some are within the range of assumptions that adverse reactions after live attenuated vaccines are related to the nature of their parental wild-type viruses...
November 29, 2018: Vaccine
Shashikant Apte, Jordi Navarro-Puerto, Sharat Damodar, Vijay Ramanan, Joseph John, Giraldo Kato, Cecil Ross, Chirag Shah, Marcela Torres, Chieh-Lin 'Kathy' Fu, Karen Rucker, Paul Pinciaro, Gladis Barrera, Maria Esperança Aragonés, Jaume Ayguasanosa
AIM: To evaluate the safety and efficacy of 10% intravenous immunoglobulin (IVIG; Flebogamma®  10% DIF) in individuals with chronic immune thrombocytopenic purpura (ITP). PATIENTS & METHODS: Patients aged 3-70 years, diagnosed with chronic ITP, received 1 g/kg IVIG over two consecutive days. RESULTS:  64 evaluable patients (51 adults, 13 children) with chronic ITP received IVIG. The primary efficacy end point (increased platelet counts from ≤20 × 109 /l to ≥50 × 109 /l by day 8) was achieved by 81...
November 30, 2018: Immunotherapy
Hadi Goubran, Caroline Hart, Ibraheem Othman, Jerard Seghatchian
Although Immune thrombocytopenic purpura is a common disorder that family physicians, internists and hematologists face in their everyday practice, its diagnosis rests only on "exclusion" and its therapy is based on algorithms where "trial and error" is the rule. Flow cytometry, if simplified and standardized, could provide a quicker and better diagnostic accuracy. Studies of the lymphocyte subset using flow cytometry and more elaborate immune studies are paving the way for a better understanding of the disease and in identification of prognostic markers...
October 30, 2018: Transfusion and Apheresis Science
Tülin Güngör, Özlem Arman Bilir, Vildan Koşan Çulha, Ali Güngör, Abdurrahman Kara, Fatih Mehmet Azık, Hüsniye Neşe Yaralı
OBJECTIVE: Immune thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia children. The aim of this retrospective study is to describe presenting features and clinical characteristics of ITP and evaluate clinical course, treatment modalities, and complications and determine the effects of preceding infection history, age, gender, treatment modality, and admission platelet count on chronicity. METHOD: Two hundred and eleven patients who were diagnosed ITP and followed-up in Department of Pediatric Hematology, Ankara Children Hematology Oncology Education and Research Hospital between January 2008 and September 2012 were included...
November 3, 2018: Pediatrics and Neonatology
Maite Hurtado Uriarte, Carolina Larrarte, Laura Bravo Rey
We had the challenged to treat a 40-year-old female with Systemic Scleroderma who was showing unspecific symptoms. During her time at the hospital she rapidly develops renal dysfunction, associated with hypertension. She required renal replacement therapy initiation and we observed a decline in hemoglobin and platelets numbers. We confirm a microangiopathic hemolytic anemia and rule out other immune diseases or thrombotic thrombocytopenic purpura. Systemic Sclerosis is a chronic immune disorder of unknown origin that it is not completely understood...
2018: Case Reports in Nephrology
Svetlana Vakkilainen, Riikka Mäkitie, Paula Klemetti, Helena Valta, Mervi Taskinen, Eystein Sverre Husebye, Outi Mäkitie
Background: Mutations in RMRP , encoding a non-coding RNA molecule, underlie cartilage-hair hypoplasia (CHH), a syndromic immunodeficiency with multiple pathogenetic mechanisms and variable phenotype. Allergy and asthma have been reported in the CHH population and some patients suffer from autoimmune (AI) diseases. Objective: We explored AI and allergic manifestations in a large cohort of Finnish patients with CHH and correlated clinical features with laboratory parameters and autoantibodies. Methods: We collected clinical and laboratory data from patient interviews and hospital records...
2018: Frontiers in Immunology
Kumpol Aiempanakit, Benjawan Apinantriyo
RATIONALE: Thrombotic thrombocytopenic purpura (TTP) and hemophagocytic lymphohistiocytosis (HLH) are rare hematologic conditions and have high mortality. Both TTP and HLH result from deregulation of the immune system. There are no published reports of coexisting TTP and HLH in elderly patients. PATIENT CONCERNS: A 67-year-old Asian male presented with altered consciousness and fever for 2 days. Physical examination revealed markedly pale, mild icterus with petechiae and purpura...
November 2018: Medicine (Baltimore)
Kam Chau Yung, Ze Wen Zhang, Wen Jun Yu, Jin Feng Qiu, Cheng Wei Xu, Chun Ling He, Xian Ru Xu, Jun Yin
To observe the differences in proteins between adult patients with chronic immune thrombocytopenic purpura (ITP) and healthy adults. 30 patients with chronic ITP and 30 healthy controls were enrolled into the study. The platelet total protein was extracted from peripheral venous blood of 10 chronic ITP patients and 10 healthy controls respectively, and subjected to two-dimensional electrophoresis (2-DE) to find the differential protein spot between chronic ITP patients and healthy controls, then the differential protein spots were identified by mass spectrometry...
October 2018: Indian Journal of Hematology & Blood Transfusion
Göksel Leblebisatan, Yurdanur Kilinc, Metin Cil, İlgen Sasmaz, Ayse Ozkan
Objective Child patients of chronic thrombocytopenic purpura with severe and resistant thrombocytopenia were evaluated to observe whether their clinical or laboratory states improve by one of the thrombomimetic therapeutic agent called Eltrombopag as in adults in a single center of different country from previous studies. Materials and Methods Nineteen patients with chronic immune thrombocytopenia were treated with Eltrombopag to dose in international guidelines. Results Approximately half (11/19:58%) of the patients benefitted from the treatment with Eltrombopag either by an increase of platelet levels at safe levels with a decrease in the frequency of bleedings which needed rescue treatment...
October 2018: Indian Journal of Hematology & Blood Transfusion
Mohammad S Abdelgawwad, Wenjing Cao, Liang Zheng, Nicole K Kocher, Lance A Williams, X Long Zheng
Objective- ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats-13) cleaves VWF (von Willebrand factor). This process is essential for hemostasis. Severe deficiency of plasma ADAMTS13 activity, most commonly resulting from autoantibodies against ADAMTS13, causes thrombotic thrombocytopenic purpura. Therapeutic plasma exchange is the standard of care to date, which removes autoantibodies and replenishes ADAMTS13. However, such a therapy is often ineffective to raise plasma ADAMTS13 activity, and in-hospital mortality rate remains as high as 20%...
November 2018: Arteriosclerosis, Thrombosis, and Vascular Biology
Ewelina Grywalska, Dorota Siwicka-Gieroba, Michał Mielnik, Martyna Podgajna, Krzysztof Gosik, Wojciech Dąbrowski, Jacek Roliński
Splenectomy is a surgery indicated in case of splenic rupture after injury, when there are tumors in the spleen, or as a treatment for certain diseases, such as idiopathic thrombocytopenic purpura and spherocytosis. The aims of the study were to assess the immunological response to the Haemophilus influenzae type b (Hib) vaccine and the post-vaccination changes in lymphocyte subsets and cell activation markers in splenectomized patients and healthy volunteers. Blood samples were collected from 25 patients that had undergone splenectomy and from 15 healthy, non-splenectomized volunteers...
October 23, 2018: Human Vaccines & Immunotherapeutics
José Carlos Jaime-Pérez, Patrizia Elva Aguilar-Calderón, Lorena Salazar-Cavazos, David Gómez-Almaguer
Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%-73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death...
2018: Journal of Blood Medicine
Huma Zafar, Saadia Anwar, Mahwish Faizan, Shazia Riaz
Objective: The study aimed to demonstrate the pattern of clinical presentations and outcome of acute Immune Thrombocytopenia (ITP) in our Centre. Methods: A descriptive, observational study was conducted by collecting and analysing the data of 103 patients of acute ITP, ageing between 1-14 years, at The Children's Hospital, Lahore from January 2016 to December 2016. We collected the data regarding age, sex, clinical presentations, history of preceding viral infections, vaccination history, laboratory values, different treatment options used, and response to the treatment concerning complete response, partial response and poor responders...
September 2018: Pakistan Journal of Medical Sciences Quarterly
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
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