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primary immune thrombocytopenia

Cindy E Neunert
Immune thrombocytopenia resulting from antibody-mediated platelet destruction combined with impaired platelet production is a common cause of thrombocytopenia. The decision to treat newly diagnosed patients is based on several factors including ceasing hemorrhagic manifestations, increasing the platelet count, prevention of bleeding, and inducing remission. Current standard first-line therapy is a course of corticosteroids. Although this treatment paradigm increases the platelet count in the majority of patients, a high percentage relapse after discontinuation of corticosteroid therapy...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
Adeline Furudoï, Étienne Rivière, Estibaliz Lazaro, Enio Furudoï, Jean-François Viallard, Marie Parrens
Immune thrombocytopenia (ITP) is an acquired antibody-mediated disease, for which splenectomy remains a curative treatment. We analyzed histology and phenotypes of ITP-splenectomy specimens from 41 adult patients, with different previous ITP-specific treatments, including B-cell-depleting rituximab (RTX) or not, in an attempt to predict splenectomy success or failure on the basis of day 56 postoperative platelet counts. RTX-naive ITP-spleen samples, compared with those from a 20-patient control trauma cohort, contained the following nonspecific, ITP-evocative, white-pulp lesions: follicular helper T-cell (programmed death-1 and inducible T-cell COStimulator) expansion in reactive follicles (P=0...
December 1, 2017: American Journal of Surgical Pathology
Suzan O Mousa, Gamal T Soliman, Ahmed A-F Saedii, Emad N Hameed
Primary immune thrombocytopenia (ITP) is the most common cause of acquired thrombocytopenia in children. Anti-thyroid antibodies (aTA) have previously been found to be present in various autoimmune diseases. Our aim was to study the effect of aTA positivity (which are anti-thyroid peroxidase (aTPo) and/or anti-thyroglobulin (aTg)) on children with primary immune thrombocytopenia and their relation to treatment response. Sixty-one children with primary ITP were enrolled in the present study. They were further subdivided into: ND&P group (newly diagnosed and persistent) and chronic ITP group...
November 30, 2017: Pediatric Hematology and Oncology
Naruko Suzuki, Junji Hiraga, Yumi Hariyama, Yusuke Takagi, Haruhiko Ohashi, Yasuyuki Kishigami, Hidenori Oguchi, Yoshitoyo Kagami
Primary immune thrombocytopenia (ITP) is defined by a low platelet count secondary to antibody-mediated platelet destruction or reductions in platelet production. Although eltrombopag is a thrombopoietin receptor agonist that increases platelet production in refractory or relapsed ITP, the influence on pregnancy is limited. We present the case of a pregnant 25-year-old ITP patient referred to our hospital with a history of two induced abortions. After eradication of Helicobacter pylori and with oral prednisolone at 8 mg/day, platelet count remained below 10,000/µl...
November 29, 2017: International Journal of Hematology
Yuzuru Hosoda, Hiroshi Hagino, Norihiko Hino, Toru Motokura
Chemotherapy for lymphoma may be avoided in the presence of coincident cytopenia. In case of immune cytopenia secondary to lymphoma, treatment of cytopenia is the same for primary cases, however, chemotherapy for lymphoma may be effective at the cost of severe hematological toxicity. The present study reports a complex case of thrombocytopenia and direct antiglobulin test-negative hemolytic anemia, thus mimicking Evans syndrome, secondary to cluster of differentiation 5-positive B-cell lymphoma with massive splenomegaly, in a patient suffering from rheumatoid arthritis for two decades...
November 2017: Molecular and Clinical Oncology
Monia Ouederni, Monia Ben Khaled, Samia Rekaya, Ilhem Ben Fraj, Fethi Mellouli, Mohamed Bejaoui
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, HLH is an acquired syndrome. We report a case of a nine month-old-boy presented with hepatosplenomegaly, severe anemia, thrombocytopenia, hypertriglyceridemia and high hyperferritinemia. These clinical features of HLH prompted a wide range of infectious and auto-immune tests to be performed. After an extensive diagnostic workup, he was referred to the immune-hematologic unit for HLH suspicion with an unknown cause...
2017: Mediterranean Journal of Hematology and Infectious Diseases
Shixuan Wang, Yue Guan, Yunlong Wang, Huiyuan Li, Donglei Zhang, Mankai Ju, Yating Hao, Xuewen Song, Boyang Sun, Xueqing Dou, Renchi Yang
Phosphatase and tensin homolog (PTEN) is thought to mediate B cell activation by negatively regulating the phosphoinositide 3-kinase (PI3K) signaling pathway. This pathway is important for activation, growth, and proliferation. Although enhanced B cell receptor (BCR) signaling contributes to increased B cell activity in immune thrombocytopenia (ITP), the role of PTEN is unclear. In this study, we analyzed B cells of ITP patients using flow cytometry and found that all B cell subsets, excluding memory B cells, showed lower PTEN expression than cells from healthy controls (HCs)...
November 22, 2017: Molecular Immunology
Chul Hee Kim, Yoon Seok Choi, Ji Young Moon, Duck Yong Kim, So Yeon Lee, Hyo Jin Lee, Hwan Jung Yun, Samyong Kim, Deog Yeon Jo, Ik Chan Song
Background/Aims: Few studies have addressed whether there are differences in clinical efficacy between intravenous methylprednisolone (methyl-Pd) and intravenous immunoglobulin (IVIg) use. Methods: We retrospectively compared platelet responses and toxicities associated with these two treatments in adult patients with immune thrombocytopenia. Patients received intravenous methyl-Pd therapy followed by oral prednisolone (Pd) from 1993 to 2002 and IVIg together with oral Pd from 2003 to 2008...
November 27, 2017: Korean Journal of Internal Medicine
Jeff Hummel, Dorothee Bienzle, Annette Morrison, Michelle Cieplak, Kyle Stephenson, Josepha DeLay, J Paul Woods, Brian D Lichty, Byram W Bridle
Direct killing of malignant cells combined with induction of tumour-specific immune responses makes oncolytic vaccines attractive for cancer therapy. We previously developed a heterologous cancer immunization strategy that utilized a replication-defective adenovirus-vectored primary vaccine encoding a tumour antigen followed by boosting with a replication-competent Maraba virus expressing the same antigen. To assess the safety of oncolytic Maraba virus-based booster vaccines and inform the design of clinical trials, we conducted translational studies in cats, which have immune systems that are similar to people and spontaneously develop cancers of comparable types and etiologies...
November 16, 2017: Scientific Reports
Lina R Marins, Leonardo B Anizelli, Ana L Sarquis, Mariana D Romanowski
INTRODUCTION: Preeclampsia (PE) is the primary obstetrical cause in one to four perinatal deaths. Although the etiology and pathogenesis of preeclampsia is not fully known, a proinflammatory immune state prevails and can disrupt fetal hematopoiesis. Some of the effects on the newborn include neonatal thrombocytopenia, neutropenia, a reduction in T regulatory cells and an increased cytotoxic natural killer cell profile. METHOD: Electronic databases were searched, and defined criteria were applied to select articles for review...
November 7, 2017: Journal of Maternal-fetal & Neonatal Medicine
Lukas Löfling, Marie Linder, Charlotta Ekstrand, Honar Cherif, Helle Kieler, Shahram Bahmanyar
Patients with primary chronic immune thrombocytopenia (cITP) have been reported to use more anti-infective medications, even before diagnosis of immune thrombocytopenia (ITP). The more common use of anti-infective medications may be due to general health problems, requiring medication in the early stages of cITP and before the diagnosis is set, rather than infections preceding the disease. Accordingly, cITP may not only be associated with use of anti-infective medications but also with medications for more general symptoms...
October 23, 2017: Thrombosis Research
Mustafa Yilmaz, Semiha Ayhan
Although changes in the number and function of regulatory T lymphocytes have been reported in primary immune thrombocytopenia (ITP), no study has investigated whether quantification of these cell types in peripheral blood could be used as early predictive marker of treatment outcome. And, it is not clear whether any change occurs in peripheral blood memory B lymphocyte levels in ITP. Hence, the aim of this study was to investigate the percentage of regulatory T lymphocytes and memory B lymphocytes in peripheral blood of ITP patients compared to controls, and also examine whether these levels have any significant predictive value for therapy outcome...
December 2017: Indian Journal of Hematology & Blood Transfusion
Mari Tanaka, Hiraku Tsujimoto, Kojiro Yamamoto, Saeko Shimoda, Kazumasa Oka, Hiroya Takeoka
RATIONALE: TAFRO syndrome is a systemic inflammatory disease characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, MyeloFibrosis, Renal dysfunction, and Organomegaly. Progressive renal insufficiency is a predominant symptom; however, the mechanism of acute kidney injury (AKI) remains unclear, probably because severe thrombocytopenia prevents kidney biopsy. We report a rare case of TAFRO syndrome with histologically confirmed renal involvement. PATIENTS CONCERNS: A 70-year-old man developed fever, anasarca, AKI, thrombocytopenia, and hepatosplenomegaly...
October 2017: Medicine (Baltimore)
Silvia Cantoni, Monica Carpenedo, Maria Gabriella Mazzucconi, Valerio De Stefano, Valentina Carrai, Marco Ruggeri, Giorgina Specchia, Nicola Vianelli, Fabrizio Pane, Ugo Consoli, Andrea Artoni, Francesco Zaja, Mariella D'Adda, Andrea Visentin, Felicetto Ferrara, Wilma Barcellini, Domenica Caramazza, Erminia Baldacci, Elena Rossi, Alessandra Ricco, Angela Ciminello, Francesco Rodeghiero, Michele Nichelatti, Roberto Cairoli
No abstract text is available yet for this article.
October 6, 2017: American Journal of Hematology
Yasuyuki Arai, Tomoyasu Jo, Hiroyuki Matsui, Tadakazu Kondo, Akifumi Takaori-Kondo
Corticosteroids such as prednisolone and dexamethasone have been established as up-front therapy for the treatment of newly diagnosed immune thrombocytopenia. Recent studies have indicated that other treatments such rituximab or thrombopoietin receptor agonist can also be effective choices. We performed a systematic review and network meta-analysis to establish a clinically meaningful hierarchy of the efficacy and safety of treatment for newly diagnosed primary immune thrombocytopenia in adults. Randomized controlled trials evaluating medical treatments for newly diagnosed immune thrombocytopenia were included...
September 29, 2017: Haematologica
Adrian Newland, Eun-Ju Lee, Vickie McDonald, James B Bussel
Immune thrombocytopenia (ITP) is an acquired autoimmune disorder characterized by phagocytosis and destruction of autoantibody-coated platelets via spleen tyrosine kinase (Syk)-mediated signal transduction in macrophages. Effectiveness of existing therapies varies, and even leading treatments (e.g., IVIg, splenectomy, rituximab, thrombopoietic agents) do not provide optimal management for a substantial number of patients with chronic ITP. Fostamatinib disodium is an orally-bioavailable investigational agent being developed for treatment of primary persistent/chronic adult ITP...
October 2, 2017: Immunotherapy
Sif Gudbrandsdottir, Marie Brimnes, Tania Køllgaard, Hans C Hasselbalch, Claus H Nielsen
OBJECTIVE: To investigate the cytokine production and surface marker composition of B cells in adult patients with newly diagnosed primary immune thrombocytopenia (ITP) before and 12 months after treatment with rituximab + dexamethasone (RTX+DXM) or dexamethasone (DXM). METHODS: Peripheral blood mononuclear cells were isolated from nine patients treated with RTX+DXM, seven patients treated with DXM, and seven healthy donors. Expression of the cell-surface markers CD5, CD27, CD25, and CD19, and intracellular content of IL-6 and IL-10 were measured by flow cytometry...
September 27, 2017: European Journal of Haematology
Marica Pavkovic, Aleksandar Petlichkovski, Oliver Karanfilski, Lidija Cevreska, Aleksandar Stojanovic
INTRODUCTION: Immune thrombocytopenia (ITP) is an autoimmune blood disease of unknown etiology. The aim of our study was to investigate a possible role of FCGR2A and FCGR3A polymorphisms in the development of primary ITP. METHODS: We analyzed 125 adult patients with ITP and 120 healthy controls. Genotyping was performed by using PCR-RFLP methods. RESULTS: Our results showed significantly higher frequency of high-affinity FCGR3A-158V allele in patients with ITP compared with control subjects (47...
September 23, 2017: Hematology (Amsterdam, Netherlands)
Fei-Er Feng, Ru Feng, Min Wang, Jia-Min Zhang, Hao Jiang, Qian Jiang, Jin Lu, Hui Liu, Jun Peng, Ming Hou, Jian-Liang Shen, Jing-Wen Wang, Lan-Ping Xu, Kai-Yan Liu, Xiao-Jun Huang, Xiao-Hui Zhang
BACKGROUND: Primary immune thrombocytopenia is a severe bleeding disorder. About 50-85% of patients achieve initial remission from first-line therapies, but optimal second-line treatment remains a challenge. All-trans retinoic acid (ATRA) has an immunomodulatory effect on haemopoiesis, making it a possible treatment option. We aimed to evaluate the efficacy and safety of ATRA plus danazol versus danazol in non-splenectomised patients with corticosteroid-resistant or relapsed primary immune thrombocytopenia...
October 2017: Lancet Haematology
Simon Panzer, Ingrid Pabinger
No abstract text is available yet for this article.
October 2017: Lancet Haematology
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