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Immune thrombocytopenia

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https://www.readbyqxmd.com/read/28641574/intermittent-low-platelet-counts-hampering-diagnosis-of-x-linked-thrombocytopenia-in-children-report-of-two-unrelated-cases-and-a-novel-mutation-in-the-gene-coding-for-the-wiskott-aldrich-syndrome-protein
#1
Samuel Souza Medina, Lúcia Helena Siqueira, Marina Pereira Colella, Gabriela Goes Yamaguti-Hayakawa, Bruno Kosa Lino Duarte, Maria Marluce Dos Santos Vilela, Margareth Castro Ozelo
BACKGROUND: Thrombocytopenia can occur in different circumstances during childhood and although immune thrombocytopenia is its most frequent cause, it is important to consider other conditions, especially when there is a persistent or recurrent low platelet count. We report two cases of intermittent thrombocytopenia, previously misdiagnosed as immune thrombocytopenia. CASES PRESENTATION: Both cases described were boys who presented with an intermittent pattern of thrombocytopenia, with a persistently low mean platelet volume...
June 22, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28637667/thrombocytopenia-in-pregnancy
#2
Douglas B Cines, Lisa D Levine
Thrombocytopenia develops in 5-10% of women during pregnancy or in the immediate post-partum period. A low platelet count is often an incidental feature, but it can also provide a biomarker of a coexisting systemic or gestational disorder and a potential reason for a maternal intervention or treatment that might pose harm to the fetus. This chapter reflects our approach to these issues with an emphasis on advances in understanding and managing the more common causes of thrombocytopenia in pregnancy made over the past 5-10 years...
June 21, 2017: Blood
https://www.readbyqxmd.com/read/28630121/a-novel-recombinant-human-thrombopoietin-therapy-for-the-management-of-immune-thrombocytopenia-in-pregnancy
#3
Zhangyuan Kong, Ping Qin, Shan Xiao, Hai Zhou, Hong Li, Renchi Yang, Xiaofan Liu, Jianmin Luo, Zhichun Li, Guochao Ji, Zhongguang Cui, Yusheng Bai, Yuxia Wu, Linlin Shao, Jun Peng, Jun Ma, Ming Hou
The aim of this study was to determine the safety and efficacy of recombinant human thrombopoietin (rhTPO) for the management of immune thrombocytopenia (ITP) during pregnancy. Pregnant ITP patients were enrolled in the study if they had a platelet count of < 30 × 10(9)/L, were experiencing bleeding manifestations, had failed to respond to corticosteroids and/or intravenous immunoglobulin (IVIG), and had developed refractoriness to platelet transfusion. Thirty-one patients received rhTPO at an initial dose of 300U/kg once daily for 14 days...
June 19, 2017: Blood
https://www.readbyqxmd.com/read/28629237/improved-pregnancy-outcome-following-earlier-splenectomy-in-women-with-immune-thrombocytopenia-a-5-year-observational-study
#4
Mohamed Rezk, Alaa Masood, Ragab Dawood, Mahmoud Emara, Hanan El-Sayed
OBJECTIVE: to assess prospectively the maternal and fetal outcome in women with immune thrombocytopenic purpura (ITP) who undergone earlier splenectomy compared to women on medical therapy. METHODS: a 5-year observational study included pregnant women in the first trimester previously diagnosed with primary ITP with 74 patients underwent splenectomy before pregnancy and 86 patients on medical therapy. Patients were followed throughout pregnancy and labor to record their obstetric outcome...
June 19, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28628633/pro-inflammatory-immune-responses-are-associated-with-clinical-signs-and-symptoms-of-human-anaplasmosis
#5
Anna M Schotthoefer, Steven J Schrodi, Jennifer K Meece, Thomas R Fritsche, Sanjay K Shukla
Human anaplasmosis (HA) is an emerging tick-borne disease that may present as a mild flu-like illness or a life threatening, sepsis-like condition. Although disease severity is hypothesized to relate to immunopathology and immune dysfunction in humans, studies to directly measure immune responses in infected humans have been very limited. We quantified cytokines in 80 confirmed HA patients using a multiplex chemiluminescence immunoassay system and compared similarly measured responses in 1000 control subjects...
2017: PloS One
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/28611471/evaluation-of-the-immature-platelet-fraction-contribute-to-the-differential-diagnosis-of-hereditary-immune-and-other-acquired-thrombocytopenias
#7
F L B Ferreira, M P Colella, S S Medina, C Costa-Lima, M M L Fiusa, L N G Costa, F A Orsi, J M Annichino-Bizzacchi, K Y Fertrin, M F P Gilberti, M C Ozelo, E V De Paula
The differential diagnosis of immune (ITP) and hereditary macrothrombocytopenia (HM) is key to patient management. The immature platelet fraction (IPF) represents the subset of circulating platelets with higher RNA content, and has been shown to distinguish hypo- from hyperproliferative thrombocytopenias. Here we evaluated the diagnostic accuracy of IPF in the differential diagnosis between HM and other thrombocytopenias in a population of patients with post-chemotherapy thrombocytopenia (n = 56), bone marrow failure (n = 22), ITP (n = 105) and HM (n = 27)...
June 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28601896/influence-of-tnf-and-il6-gene-polymorphisms-on-the-severity-of-cytopenias-in-argentine-patients-with-myelodysplastic-syndromes
#8
Yesica Bestach, Virginia Palau Nagore, María G Flores, Jacqueline González, Jorge Arbelbide, Nora Watman, Yamila Sieza, Irene Larripa, Carolina Belli
Myelodysplastic syndromes (MDS) represent a heterogeneous group of hematologic disorders characterized by cytopenia(s) and predisposition to leukemic progression. An immune dysregulation and an aberrant bone marrow microenvironment seem to be key elements in the physiopathological process of MDS. In order to evaluate a possible association between susceptibility and clinic-pathologic features, we genotyped 153 MDS patients for functional cytokine polymorphisms: TNF (-308 G/A), IFNG (+874 A/T and +875 CAn), IL6 (-174 G/C), and TGFB1 (+869 C/T and +915 G/C)...
June 10, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28601030/aggressive-gastrointestinal-food-allergy-in-neonates-and-its-possible-relationship-to-necrotizing-enterocolitis
#9
Kazuto Suda, Toshihiro Yanai, Miki Toma, Tsubasa Aiyoshi, Takato Sasaki, Toshihiro Muraji
INTRODUCTION: The incidence of gastrointestinal food allergy (FA) in neonates is increasing. Despite this, cases of patients with gastrointestinal FA who develop necrotizing enterocolitis (NEC) requiring laparotomy are extremely rare. PRESENTATION OF CASE: We describe two cases that presented with bloody stool with a probable diagnosis of FA as eosinophils were positive in the stool at onset. Both cases failed conservative treatment. Jejunostomy and ileostomy were performed in both cases due to secondary NEC with underlying acute FA...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28596654/is-there-a-relationship-between-cxcr4-gene-expression-and-prognosis-of-immune-thrombocytopenia-in-children
#10
Sajedeh Saeidi, Javad Mohammadi-Asl, Mohammad Ali Jalali Far, Ali Amin Asnafi, Firouzeh Dehuri, Yousef Tavakolifar, Najmaldin Saki
Immune thrombocytopenia (ITP) is a common autoimmune disorder characterized by decreased platelet count (thrombocytopenia) and bleeding symptoms due to production of autoantibodies against platelets. Chemokines are molecules inducing chemotaxis and play an important role in megakaryopoiesis, including CXCR4 chemokine receptor. CXCR4 is expressed on cells of megakaryocytic series, especially platelets, and triggers several mechanisms in these cells. The purpose of this study was to evaluate the pattern of CXCR4 gene changes upon diagnosis and after treatment and its comparison with laboratory findings in peripheral blood samples from newly diagnosed ITP patients...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28596653/neonatal-outcomes-of-pregnancy-with-immune-thrombocytopenia
#11
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/28592772/current-topics-in-primary-immune-thrombocytopenia
#12
Yoshiaki Tomiyama
Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by early platelet destruction due to antiplatelet autoantibodies. Recently, significant progress has been made regarding the pathophysiology of ITP. Nonetheless, the diagnosis of ITP in daily clinical practice is still based on differential diagnosis because of the lack of laboratory tests that can diagnose autoimmunity in ITP. In this chapter, the recent topics regarding the diagnosis and management of ITP have been reviewed.
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28592755/acute-myeloid-leukemia-developing-secondary-immune-thrombocytopenia-after-umbilical-cord-blood-transplantation
#13
Rena Matsumoto, Kazuhiro Ito, Naoko Hosono, Yasufumi Matsuda, Katsunori Tai, Ippei Sakamaki, Goh Aoki, Hirohito Yamazaki, Shinji Nakao, Takahiro Yamauchi
A 64-year-old man was diagnosed with acute myeloid leukemia M2 (FLT3-ITD-positive). After induction chemotherapy and four courses of consolidation therapy, he underwent umbilical cord blood transplantation (CBT) in his first remission. He developed acute graft-versus-host disease (skin stage 2) after successful engraftment. On post-transplantation day 147, he was admitted to the hospital suffering from pneumonia. During the treatment, drastic thrombocytopenia was observed on day 251. Both platelet-associated immunoglobulin G and platelet antibody producing B cells were detected, and he was diagnosed with immune thrombocytopenia (ITP)...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28586251/the-relationship-between-idiopathic-cytopenias-dysplasias-of-uncertain-significance-icus-idus-and-autoimmunity
#14
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/28578979/hiv-associated-kidney-diseases-clarifying-concordance-between-renal-failure-in-hiv-infection-and-histopathologic-manifestations-at-kidney-biopsy
#15
REVIEW
Carla L Ellis
Patients with HIV infection have a wide spectrum of renal diseases. Some are known to be the direct effect of the viral infection while others are renal diseases that also occur in uninfected populations. HIV associated nephropathy (HIVAN) is considered to be a subtype of primary focal and segmental glomerulosclerosis that is distinct in HIV infected patients. It is more frequent in the African-American population and associated with mutations of the apolipoprotein L1 (APOL1) gene. HIV associated immune complex kidney disease (HIVICD) encompasses a spectrum of HIV associated renal diseases characterized by the presence of immune complex deposition within glomeruli...
May 5, 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28577662/human-platelet-antigens-and-primary-immune-thrombocytopenia
#16
Vagner Castro
No abstract text is available yet for this article.
April 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28577648/molecular-typing-of-human-platelet-antigens-in-immune-thrombocytopenia-patients-in-northern-brazil
#17
Julia Cavalcante do Carmo, Prissyla de Souza Klippel, Sabrine da Costa Cordeiro, Ângela Maria Dos Santos Fernandes, Raquel Medeiros Pinto, Simone Schneider Weber, Cleiton Fantin
BACKGROUND: Immune thrombocytopenia is an immune disease characterized by thrombocytopenia and bleeding due to platelet antibodies against platelet membrane glycoproteins. Human platelet antigens are derived from polymorphisms of these glycoproteins. The aim of this study was to investigate human platelet antigen frequencies in immune thrombocytopenia patients from the state of Amazonas, Brazil and investigate the potential association between specific antigens and risk for immune thrombocytopenia...
April 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28573819/use-of-eltrombopag-for-secondary-immune-thrombocytopenia-in-clinical-practice
#18
Tomás J González-López, María T Alvarez-Román, Cristina Pascual, Blanca Sánchez-González, Fernando Fernández-Fuentes, Gloria Pérez-Rus, José A Hernández-Rivas, Silvia Bernat, José M Bastida, María P Martínez-Badas, Violeta Martínez-Robles, Inmaculada Soto, Pavel Olivera, Estefanía Bolaños, Rafael Alonso, Laura Entrena, Marta Gómez-Nuñez, Arancha Alonso, María Yera Cobo, Isabel Caparrós, María Tenorio, Esther Arrieta-Cerdán, Elsa Lopez-Ansoar, Javier García-Frade, José R González-Porras
Eltrombopag is a second-line treatment in primary immune thrombocytopenia (ITP). However, its role in secondary ITP is unknown. We evaluated the efficacy and safety of eltrombopag in secondary ITP in daily clinical practice. Eighty-seven secondary ITP patients (46 with ITP secondary to autoimmune syndromes, 23 with ITP secondary to a neoplastic disease subtype: lymphoproliferative disorders [LPDs] and 18 with ITP secondary to viral infections) who had been treated with eltrombopag were retrospectively evaluated...
June 1, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28567196/cytopenias-among-art-naive-patients-with-advanced-hiv-disease-on-enrolment-to-care-and-treatment-services-at-a-tertiary-hospital-in-tanzania-a-cross-sectional-study
#19
Daniel W Gunda, Kahamba G Godfrey, Semvua B Kilonzo, Bonaventura C Mpondo
BACKGROUND: HIV/AIDS causes high morbidity and mortality through both immunosuppression and complications not directly related to immunosuppression. Haematological abnormalities, including various cytopenias, occur commonly in HIV through immune and non-immune pathways. Though these complications could potentially cause serious clinical implications, published literature on the magnitude of this problem and its associated factors in Tanzania is scarce. This study aimed at determining the prevalence and risk factors of HIV-associated cytopenias among ART-naive patients enrolling for care and treatment services at Bugando Care and Treatment Centre (CTC) in Mwanza, Tanzania...
March 2017: Malawi Medical Journal: the Journal of Medical Association of Malawi
https://www.readbyqxmd.com/read/28565834/intravenous-infusion-umbilical-cord-derived-mesenchymal-stem-cell-in-primary-immune-thrombocytopenia-a-two-year-follow-up
#20
Xiaohua Wang, Xiaoguang Yin, Wei Sun, Jin Bai, Yawen Shen, Qiang Ao, Yongquan Gu, Ying Liu
Four patients with chronic refractory immune thrombocytopenic purpura (ITP) received human umbilical cord-derived mesenchymal stem cells (hUC-MSCs). The hUC-MSC dose was 5×10(7) to 1×10(8). Complete remission (CR) was achieved in three patients in 12 months and one patient in 24 months. Three patients received the second hUC-MSC transplantation with the same dose. The median time between hUC-MSC transplantation and response was 12.5 days (range, 7-16). There were no severe adverse events during and post hUC-MSC transplantation...
May 2017: Experimental and Therapeutic Medicine
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