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Idiopathic thrombocytopenia

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https://www.readbyqxmd.com/read/29157612/tafro-syndrome
#1
REVIEW
Takuro Igawa, Yasuharu Sato
TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD...
February 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29138381/-a-case-of-lung-cancer-associated-with-drug-induced-immune-thrombocytopenia
#2
Mayuka Yamane, Toshihito Otani, Yojiro Onari
A Stage IV lung adenocarcinoma was diagnosed in the left upper lobe of an 81-year-old man 2.5 years ago. Following another form of chemotherapy, he then received docetaxel as fourth-line therapy. After 21 days of therapy, although his white blood cell count recovered, his platelet count decreased to 20,000/mL and continued to decrease. Subsequently, he was closely monitored without therapy, and eventually, his platelet count returned within the normal range after 112 days. Blood biochemistry and bone marrow paracentesis findings suggested the presence of paraneoplastic syndrome, idiopathic thrombocytopenic purpura, and drug-induced immune thrombocytopenia...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29123569/diagnostic-dilemma-severe-thrombotic-microangiopathy-in-pregnancy
#3
Sarah Birkhoelzer, Alexandra Belcher, Helen Peet
A diagnostic dilemma occurred when thrombotic microangiopathy developed during pregnancy. The diagnostic criteria of thrombotic microangiopathy include thrombocytopenia (platelets <100) and microangiopathic haemolytic anaemia (including thrombotic thrombocytopenic purpura and haemolytic-uraemic syndrome). An urgent interdisciplinary approach is required to treat thrombotic microangiopathy in pregnancy to differentiate between thrombotic microangiopathy and HELLP syndrome (haemolysis, elevated liver enzymes, low platelets)...
November 2017: J Intensive Care Soc
https://www.readbyqxmd.com/read/29066639/idiopathic-catastrophic-thrombosis-with-happy-ending
#4
Julia Heid, Andreas Greinacher, Hugo A Katus, Oliver J Müller
A 59-year-old male patient suffered three life-threatening instent thromboses after an initial resuscitation due to an ST-segment elevation myocardial infarction of the anterior cardiac wall. With a high-risk profile for heparin-induced thrombocytopenia (HIT), he was placed on argatroban after the second reinfarction. Under this apparently appropriate treatment, a third reinfarction occurred, and the patient had to undergo high-risk cardiac bypass surgery. Later on, a deep vein thrombosis and an intracardiac thrombus formed...
October 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29050117/-pediatric-idiopathic-hypereosinophilic-syndrome-with-pulmonary-embolism-a-case-report-and-review-of-literature
#5
N Dong, X Y Dong
Objective: To explore clinical features of idiopathic hypereosinophilic syndrome combined with pulmonary embolism. Method: A retrospective analysis of a patient with idiopathic hypereosinophilic syndrome and pulmonary embolism diagnosed and treated in the Respiratory Department of Shanghai Children's Hospital in September 2016 was performed. A literature search was performed with"Eosinophils increased, thrombosis"as the Chinese keywords in Wanfang database and"idiopathic hypereosinophilic syndrome, deep vein thrombosis"as the English key words in PubMed database...
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29021840/vaccination-and-autoimmune-diseases-is-prevention-of-adverse-health-effects-on-the-horizon
#6
REVIEW
Maria Vadalà, Dimitri Poddighe, Carmen Laurino, Beniamino Palmieri
Autoimmune diseases, including multiple sclerosis and type 1 diabetes mellitus, affect about 5% of the worldwide population. In the last decade, reports have accumulated on various autoimmune disorders, such as idiopathic thrombocytopenia purpura, myopericarditis, primary ovarian failure, and systemic lupus erythematosus (SLE), following vaccination. In this review, we discuss the possible underlying mechanisms of autoimmune reactions following vaccinations and review cases of autoimmune diseases that have been correlated with vaccination...
September 2017: EPMA Journal
https://www.readbyqxmd.com/read/28860441/-a-case-of-merkel-cell-carcinoma-complicated-with-severe-thrombocytopenia-treated-with-carboplatin-etoposide-regimen-after-surgery
#7
Yuko Saito, Takayoshi Kiba, Toru Takahashi, Hiroyuki Hirakawa, Miho Saito, Chiaki Otomo, Miho Sato, Yoshiteru Watanabe, Masanobu Sakaguchi, Risako Kadota, Naoya Noguchi, Takahiro Suzuki, Toshikazu Awataguchi, Fumi Shoji, Nobuo Ota
Thrombocytopenia is often caused by myelosuppression during chemotherapy. However, when platelet transfusions are required, pathological conditions such as idiopathic thrombocytopenic purpura(ITP)and thrombotic thrombocytopenic purpura( TTP)also occur. We report a case of Merkel cell carcinoma complicated with severe thrombocytopenia treated with carboplatin/etoposide regimen after surgery. The patient's platelet count did not increase in spite of platelet transfusions. However, the platelet count increased after steroid treatment was chosen under the diagnosis of ITP...
August 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28860338/hif1a-is-a-critical-downstream-mediator-for-hemophagocytic-lymphohistiocytosis
#8
Rui Huang, Yoshihiro Hayashi, Xiaomei Yan, Jiachen Bu, Jieyu Wang, Yue Zhang, Yile Zhou, Yuting Tang, Lingyun Wu, Zefeng Xu, Xin Liu, Qianfei Wang, Jianfeng Zhou, Zhijian Xiao, James P Bridges, Rebecca A Marsh, Kejian Zhang, Michael B Jordan, Yuhua Li, Gang Huang
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by overwhelming immune activation. A steroid and chemotherapy-based regimen remains as the first-line of therapy but it has substantial morbidity. Thus, novel, less toxic therapy for HLH is urgently needed. Although differences exist between familial HLH (FHL) and secondary HLH (sHLH), they have many common features. Using bioinformatic analysis with FHL and systemic juvenile idiopathic arthritis, which is associated with sHLH, we identified a common hypoxia-inducible factor 1A (HIF1A) signature...
November 2017: Haematologica
https://www.readbyqxmd.com/read/28791286/autoimmune-thrombotic-thrombocytopenic-purpura-two-rare-cases-associated-with-juvenile-idiopathic-arthritis-and-multiple-sclerosis
#9
Despoina Dimopoulou, Athina Dimosiari, Eudokia Mandala, Theodoros Dimitroulas, Alaxandros Garyfallos
Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28723784/thrombocytopenia-in-pregnancy-with-different-diagnoses-differential-clinical-features-treatments-and-outcomes
#10
MULTICENTER STUDY
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
#11
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
#12
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
#13
REVIEW
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...
August 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28654425/association-between-gene-polymorphisms-and-clinical-features-in-idiopathic-thrombocytopenic-purpura-patients
#14
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
#15
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...
December 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28596653/neonatal-outcomes-of-pregnancy-with-immune-thrombocytopenia
#16
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
#17
REVIEW
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...
July 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
#18
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
#19
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
#20
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
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