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Idiopathic thrombocytopenia

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https://www.readbyqxmd.com/read/28723784/thrombocytopenia-in-pregnancy-with-different-diagnoses-differential-clinical-features-treatments-and-outcomes
#1
Xiaoyue Wang, Yan Xu, Wenxiang Luo, Hui Feng, Yizhou Luo, Yanli Wang, Hui Liao
To investigate the clinical features and perinatal treatment of thrombocytopenia induced by different causes during pregnancy.Clinical data from 195 pregnant women with thrombocytopenia attending 2 tertiary hospitals from January 2014 to October 2016 were retrospectively studied. The obtained data were analyzed with SPSS 19.0 software.There were 117 (60.0%), 55 (28.2%), and 23 cases (11.8%) of pregnancy-associated thrombocytopenia (PAT), idiopathic thrombocytopenia (ITP), and hypertensive disorder in pregnancy (PIH), respectively...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28717524/anasarca-fever-thrombocytopenia-organomegaly-and-multiorgan-failure-in-a-24-year-old-pregnant-woman
#2
Guillaume Morel, Joy Mootien, Philippe Guiot, Khaldoun Kuteifan
TAFRO syndrome is a distinct idiopathic multicentric Castleman disease characterized by the association of thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. We report the first case occurring in a Caucasian pregnant woman. At 34 weeks of gestation, our patient presented with all clinical and biological symptoms compatible with a TAFRO syndrome. Tough quick cesarean section was performed as symptoms got worse with onset of multiorgan failure requiring mechanical ventilation for acute respiratory distress, continuous renal replacement, and vasopressors...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28698781/idiopathic-pulmonary-embolism-in-a-case-of-severe-family-ankrd26-thrombocytopenia
#3
Jerome Guison, Gilles Blaison, Oana Stoica, Remy Hurstel, Marie Favier, Remy Favier
Venous thrombosis affecting thrombocytopenic patients is challenging. We report the case of a woman affected by deep vein thrombosis and pulmonary embolism in a thrombocytopenic context leading to the discovery of a heterozygous mutation in the gene encoding ankyrin repeat domain 26 (ANKRD26) associated with a heterozygous factor V (FV) Leiden mutation. This woman was diagnosed with lower-limb deep vein thrombosis complicated by pulmonary embolism. Severe thrombocytopenia was observed. The genetic study evidenced a heterozygous FV Leiden mutation...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28654461/natural-history-pathogenesis-and-treatment-of-evans-syndrome-in-children
#4
Elpis Mantadakis, Evangelia Farmaki
Primary Evans syndrome (ES) is defined by the concurrent or sequential occurrence of immune thrombocytopenia and autoimmune hemolytic anemia in the absence of an underlying etiology. The syndrome is characterized by a chronic, relapsing, and potentially fatal course requiring long-term immunosuppressive therapy. Treatment of ES is hardly evidence-based. Corticosteroids are the mainstay of therapy. Rituximab has emerged as the most widely used second-line treatment, as it can safely achieve high response rates and postpone splenectomy...
June 26, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28654425/association-between-gene-polymorphisms-and-clinical-features-in-idiopathic-thrombocytopenic-purpura-patients
#5
Hadi Rezaeeyan, Kaveh Jaseb, Arash Alghasi, Ali Amin Asnafi, Najmaldin Saki
: Immune thrombocytopenic purpura (ITP) is an autoimmune disease in which increased platelet destruction and thrombocytopenia are diagnostic features. In fact, the exact pathogenesis of this disease is still unknown, but genetic changes can be a potential factor in the development of ITP. In this study, the relationship between polymorphisms with platelet destruction has been studied, which leads to decreased platelet count. Relevant literature was identified by a PubMed search (2000-2016) of English language papers using the terms 'ITP', 'polymorphism,' and 'immune system'...
June 24, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28627134/u-s-food-and-drug-administration-approval-summary-eltrombopag-for-the-treatment-of-pediatric-patients-with-chronic-immune-idiopathic-thrombocytopenia
#6
Lori A Ehrlich, Virginia E Kwitkowski, Gregory Reaman, Chia-Wen Ko, Lei Nie, Richard Pazdur, Ann T Farrell
The U.S. Food and Drug Administration (FDA) approved eltrombopag for pediatric patients with chronic immune (idiopathic) thrombocytopenia (ITP) ages ≥6 on June 11, 2015, and ages ≥1 on August 24, 2015. Approval was based on the FDA review of two randomized trials that included 159 pediatric patients with chronic ITP who had an insufficient response to corticosteroids, immunoglobulins, or splenectomy. This manuscript describes the basis for approval of these applications. The FDA concluded that eltrombopag has shown efficacy and a favorable benefit to risk profile for pediatric patients with chronic ITP...
June 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28596653/neonatal-outcomes-of-pregnancy-with-immune-thrombocytopenia
#7
Nuriye Aslı Melekoğlu, Ali Bay, Elif H Aktekin, Mehmet Yilmaz, Ercan Sivasli
Neonates born to mothers with immune thrombocytopenia (ITP) have an increased risk for neonatal thrombocytopenia and hemorrhagic complications. The aim of this study was to determine the maternal and neonatal outcomes of pregnancies with ITP and also to identify risk factors that predicts neonatal thrombocytopenia. We performed a retrospective analysis of 40 pregnancies with ITP and their 40 neonates. Among the 40 neonates, thrombocytopenia (platelet count of less than 150 × 10(9)/L) was detected in 15 neonates (37...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28586251/the-relationship-between-idiopathic-cytopenias-dysplasias-of-uncertain-significance-icus-idus-and-autoimmunity
#8
Wilma Barcellini
This review examines the several lines of evidence that support the relationship between myelodysplasia and autoimmunity, i.e. their epidemiologic association, the existence of common immune-mediated physiopathologic mechanisms, and the response to similar immunosuppressive therapies. The same relationship is reviewed here considering idiopathic cytopenia of uncertain significance (ICUS) and idiopathic dysplasia of uncertain significance (IDUS), two recently recognized provisional conditions characterized by isolated/unexplained cytopenia and/or dysplasia in <10% bone marrow cells...
June 15, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28566594/the-effective-treatment-with-cyclosporine-of-a-ulcerative-colitis-patient-with-concurrent-idiopathic-thrombocytopenic-purpura-who-subsequently-developed-spontaneous-pneumomediastinum
#9
Tsutomu Iwasa, Kazuhiko Nakamura, Eikichi Ihara, Akira Aso, Tetsuhide Ito
Although extraintestinal manifestations of inflammatory bowel diseases are not uncommon, few reports have described concurrent idiopathic thrombocytopenic purpura (ITP). Spontaneous pneumomediastinum is also a rare complication of ulcerative colitis (UC). This report describes the case of a 14-year-old boy who experienced recurrent ulcerative colitis 3 months after temporary improvement following treatment with prednisolone (20 mg/day) and granulocyte/monocyte adsorption apheresis. His platelet counts decreased, suggesting ITP...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28496360/epidural-anesthesia-for-labor-and-delivery-in-a-patient-with-may-hegglin-anomaly-a-case-report
#10
Annas Muhammad Muzannar, Mohammed Al Harbi, Raza Rathore, Nasser Tawfeeq, Freddie Wambi, Nasir Mahmood, Sonia Albrechtova, Vassilios Dimitriou
We report a case of May-Hegglin anomaly (MHA) in a woman who had a successful labor and delivery under epidural anesthesia. MHA is an inherited thrombocytopenia easily misdiagnosed as idiopathic (immune) thrombocytopenic purpura (ITP). Early and appropriate diagnosis of MHA during pregnancy is essential for optimal maternal and neonatal delivery outcome. Additionally, it can avoid unnecessary diagnostic studies, such as bone marrow aspiration and biopsy, and even harmful therapies with corticosteroids, immunosuppressive agents, and splenectomy...
2017: Local and Regional Anesthesia
https://www.readbyqxmd.com/read/28451010/-acquired-amegacaryocytic-thrombocytopenic-purpura-hiding-acute-myeloid-leukemia
#11
Hicham Eddou, Ali Zinebi, Abdelaziz Khalloufi, Mohammed Sina, Mehdi Mahtat, Kamal Doghmi, Mohammed Mikdame, Mohammed Karim Moudden, Mohammed El Baaj
Acquired amegakaryocytic thrombocytopenic purpura is a very rare condition characterized by severe thrombocytopenia linked to the reduction or disappearance of megakaryocytes in the bone marrow. It may be primary idiopathic or secondary to many pathological conditions including hematologic disorders. We report the case of a 24-year-old patient admitted for haemorrhagic syndrome caused by immunological thrombocytopenic purpura. The diagnosis was acquired amegakaryocytosis after the failure of corticotherapy and the performance of myelography...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28450690/a-case-of-chronic-sarcoid-myopathy-with-basedow-s-disease-and-sjogren-s-syndrome-a-case-series-of-sarcoid-myopathy
#12
Takashi Isobe, Madoka Mori-Yoshimura, Yasushi Oya, Yuko Saito, Miho Murata, Ichizo Nishino, Yuji Takahashi
We report a 62-year-old woman with a history of Basedow's disease and Sjogren's syndrome who presented with slowly progressive limb muscle weakness over the course of ten years. On physical examination, she had dry eye and mouth, but was otherwise normal. Neurological examination revealed symmetrical proximal dominant muscle weakness. Polymyositis was suspected at initial diagnosis due to her clinical course, physical examination, and autoimmune disease. However, the final diagnosis based on a muscle biopsy was the chronic myopathic type of sarcoid myopathy...
April 27, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28413179/cyclic-thrombocytopenia-synchronizing-with-the-menstrual-cycle-showing-periodic-phases-of-thrombocytopenia-and-rebound-thrombocytosis
#13
Taro Kurihara, Masahiko Sumi, Hiroko Kaiume, Wataru Takeda, Takehiko Kirihara, Keijiro Sato, Toshimitsu Ueki, Yuki Hiroshima, Hikaru Kobayashi
A 37-year-old woman was admitted to our hospital for purpura involving the extremities and thrombocytopenia. Prednisolone (PSL) was administered based on a diagnosis of idiopathic thrombocytopenic purpura (ITP), but was not effective for maintaining her platelet count within the normal range, which showed cyclic fluctuation corresponding to the menstrual cycle. Therefore, we discontinued PSL, and cyclic thrombocytopenia (CTP) was diagnosed. CTP is a rare disease which is usually treated as ITP but with no response...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28395431/-nix-mediated-mitochondrial-autophagy-in-pathogenesis-of-myelodysplastic-syndrome-anemia
#14
L Y Yang, N B Cui, H Q Wang, R Fu, W Qu, E B Ruan, X M Wang, G J Wang, Y H Wu, H Liu, J Song, J Guan, L M Xing, L J Li, H J Jiang, H Liu, Y H Wang, C Y Liu, W Zhang, Z H Shao
Objective: To investigate the change of NIX level of bone marrow nucleated red blood cells in anemia patients with myelodysplastic syndromes (MDS), to explore the significance of NIX-mediated mitochondrial autophagy in the pathogenesis of MDS anemia. Methods: A total of 54 patients with MDS diagnosed in the Department of Hematology of General Hospital, Tianjin Medical University from July 2015 to July 2016 were enrolled into the MDS group, 33 cases of immune thrombocytopenia or idiopathic leukopenia as controls...
April 11, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28383422/acquired-idiopathic-thrombotic-thrombocytopenic-purpura-successfully-treated-with-intravenous-immunoglobulin-and-glucocorticoid-a-case-report
#15
Hiro Nakao, Akira Ishiguro, Nahoko Ikoma, Kentaro Nishi, Chemin Su, Hisaya Nakadate, Mitsuru Kubota, Masaki Hayakawa, Masanori Matsumoto
RATIONALE: Plasma exchange is the principal treatment for acquired thrombotic thrombocytopenic purpura (TTP) but is invasive and may have adverse effects. Reports of immunoglobulin therapy for acquired TTP without plasma exchange are rare. PATIENT CONCERNS: A 14-year-old girl was admitted because of hemolytic anemia and thrombocytopenia. DIAGNOSIS: Acquired TTP was diagnosed based on low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13) activity and a high ADAMTS13 inhibitor level...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28255140/idiopathic-thrombocytopenic-purpura-in-childhood-langerhans-cell-histiocytosis-in-adulthood-more-than-a-chance-association
#16
Y C Kuan, M N Nurain
Described herein, a case of Langerhans cell histiocytosis (LCH) in an adult with Idiopathic Thrombocytopenic Purpura (ITP) diagnosed at age ten. She presented with cranial diabetes insipidus, later developed hypogonadotrophic hypogonadism and multiple cervical lympadenopathy from which histopathology of excisional biopsy confirmed LCH. Magnetic resonance imaging showed thickened pituitary stalk. Association of ITP and LCH is unknown but the question of LCH presenting as isolated thrombocytopenia in childhood only to be discovered in adulthood when there was pituitary and bone involvement remains...
February 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28239462/thrombocytopenia-in-patients-with-melanoma-receiving-immune-checkpoint-inhibitor-therapy
#17
Eileen Shiuan, Kathryn E Beckermann, Alpaslan Ozgun, Ciara Kelly, Meredith McKean, Jennifer McQuade, Mary Ann Thompson, Igor Puzanov, John P Greer, Suthee Rapisuwon, Michael Postow, Michael A Davies, Zeynep Eroglu, Douglas Johnson
BACKGROUND: Immune checkpoint inhibitors, including antibodies against programmed death 1 (PD-1) and cytotoxic T-lymphocyte antigen 4 (CTLA-4), are being used with increasing frequency for the treatment of cancer. Immune-related adverse events (irAEs) including colitis, dermatitis, and pneumonitis are well described, but less frequent events are now emerging with larger numbers of patients treated. Herein we describe the incidence and spectrum of thrombocytopenia following immune checkpoint inhibitor therapy and two severe cases of idiopathic thrombocytopenic purpura (ITP)...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28205564/elevated-serum-interferon-%C3%AE-induced-protein-10-kda-is-associated-with-tafro-syndrome
#18
Noriko Iwaki, Yuka Gion, Eisei Kondo, Mitsuhiro Kawano, Taro Masunari, Hiroshi Moro, Koji Nikkuni, Kazue Takai, Masao Hagihara, Yuko Hashimoto, Kenji Yokota, Masataka Okamoto, Shinji Nakao, Tadashi Yoshino, Yasuharu Sato
Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder. It is characterized by inflammatory symptoms, and interleukin (IL)-6 contributes to the disease pathogenesis. Human herpesvirus 8 (HHV-8) often drives hypercytokinemia in MCD, although the etiology of HHV-8-negative MCD is idiopathic (iMCD). A distinct subtype of iMCD that shares a constellation of clinical features including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been reported as TAFRO-iMCD, however the differences in cytokine profiles between TAFRO-iMCD and iMCD have not been established...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28194178/role-of-helicobacter-pylori-eradication-therapy-on-platelet-recovery-in-chronic-immune-thrombocytopenic-purpura
#19
Khan Sheema, Ujjan Ikramdin, Naz Arshi, Naz Farah, Sheikh Imran
Background. Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder in which the immune system destroys native platelets. In this condition an autoantibody is generated against a platelet antigen. ITP affects women more often than men and is more common in children than adults. Objective. To assess the effect of Helicobacter pylori eradication therapy (HPET) on platelet count in Helicobacter pylori associated chronic immune thrombocytopenic purpura (chronic ITP) in adult. Materials and Methods. It is an interventional prospective study conducted at Liaquat University of Medical and Health Sciences, Jamshoro, from 2014 to 2015...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28176474/monoclonal-gammopathy-of-renal-significance-triggering-atypical-haemolytic-uraemic-syndrome
#20
REVIEW
Usman Mahmood, Nicole Isbel, Peter Mollee, Andrew Mallett, Sridevi Govindarajulu, Ross Francis
Haemolytic uraemic syndrome is a rare condition with an overall incidence of one to two cases in a population of 100 000 and approximately 10% of these cases are classified as atypical. Atypical haemolytic uraemic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and acute kidney injury. aHUS can be genetic, acquired or idiopathic (negative genetic screening and no environmental triggers). We describe a case of aHUS triggered by monoclonal gammopathy of renal significance (MGRS) successfully treated with plasmapheresis and a bortezomib-based chemotherapy regimen, resulting in marked improvement in renal function and other markers of haemolysis...
February 2017: Nephrology
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