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Autoimmune Thrombocytopenia

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https://www.readbyqxmd.com/read/30105755/splenectomy-in-patients-with-chronic-myelomonocytic-leukemia-indications-histopathological-findings-and-clinical-outcomes-in-a-single-institutional-series-of-39-patients
#1
Prateek Pophali, Pedro Horna, Terra L Lasho, Christy M Finke, Rhett P Ketterling, Naseema Gangat, David Nagorney, Ayalew Tefferi, Mrinal M Patnaik
In a 28-year period, 39 (7%) patients with chronic myelomonocytic leukemia (CMML) (median age 66 years, 64% male) underwent a splenectomy at our institution. Primary indications for splenectomy were refractory thrombocytopenia (36%), progressive spleen related symptoms (33%), emergent splenectomy for splenic rupture (21%), refractory anemia (8%) and prior to allogenic stem cell transplant (3%). Eleven (28%) patients had anemia at the time of splenectomy, of which 3 (27%) were autoimmune. The median time to splenectomy from CMML diagnosis was 6 months (0-40); peri-operative morbidity and mortality rates were 43% and 13%, while the median post-splenectomy survival was 25 months (11-38)...
August 13, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/30103822/extensive-abdominal-wall-ulceration-as-a-late-manifestation-of-antiphospholipid-syndrome-a-case-report
#2
Yogesh Sharma, Karen Humphreys, Campbell Thompson
BACKGROUND: Antiphospholipid syndrome is an autoimmune disorder characterized by the presence of antiphospholipid antibodies and commonly presents with vascular thromboembolic phenomena, thrombocytopenia, and obstetric complications. Antiphospholipid syndrome can be classified as either primary or secondary to other connective tissue diseases. Dermatologic manifestations are common; however, non-vasculitic skin ulceration is an uncommon manifestation of antiphospholipid syndrome with limited treatment options...
August 14, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/30083170/follicular-helper-t-cells-in-digeorge-syndrome
#3
Adam Klocperk, Zuzana Paračková, Markéta Bloomfield, Michal Rataj, Jan Pokorný, Susanne Unger, Klaus Warnatz, Anna Šedivá
DiGeorge syndrome is an immunodeficiency characterized by thymic dysplasia resulting in T cell lymphopenia. Most patients suffer from increased susceptibility to infections and heightened prevalence of autoimmune disorders, such as autoimmune thrombocytopenia. B cells in DiGeorge syndrome show impaired maturation, with low switched-memory B cells and a wide spectrum of antibody deficiencies or dysgammaglobulinemia, presumably due to impaired germinal center responses. We set out to evaluate circulating follicular helper T cells (cTFHs) in DiGeorge syndrome, as markers of T-B interaction in the germinal centers in a cohort of 17 patients with partial DiGeorge and 21 healthy controls of similar age...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/30080751/weekly-rituximab-followed-by-monthly-rituximab-treatment-for-autoimmune-disease-associated-with-ras-associated-autoimmune-leukoproliferative-disease
#4
Hidemi Toyoda, Takao Deguchi, Shotaro Iwamoto, Kentaro Kihira, Hiroki Hori, Yoshihiro Komada, Masahiro Hirayama
Recently, a new disease of lymphocyte homeostasis caused by somatic mosaicism for the RAS mutation has been discovered (known as RALD, RAS-associated leukoproliferative disorder). Since few cases have been reported in literature, the prognosis and standard treatment for autoimmune diseases associated with RALD remain poorly understood. Standard rituximab therapy (375 mg/m for 4 wk) is effective in patients with autoimmune diseases, but early recurrences are common. We highlight the potential for monthly administration of rituximab in a patient with autoimmune thrombocytopenia and hemolytic anemia associated with RALD...
August 4, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/30078793/-reference-guide-for-adult-chronic-neutropenia
#5
Yasuhisa Yokoyama, Akiyoshi Takami, Yasuo Mori, Shinya Sato, Takahiro Suzuki, Masahiko Gosho, Naoshi Obara, Yasushi Miyazaki, Koichi Akashi, Shigeru Chiba
Chronic neutropenia is a rare hematological entity characterized by isolated neutropenia without anemia or thrombocytopenia. The diagnostic criteria and classification of chronic neutropenia have been more elusive in adult cases than pediatric cases. We herein propose the Reference Guide for Adult Chronic Neutropenia. In this guide, diagnosis of chronic neutropenia is based on recurrent or continuous neutropenia defined by absolute neutrophil count (ANC) less than 1,500/µl for over three or more months. Severity of chronic neutropenia is defined as follows: severe, ANC <500/µl; moderate, ANC between 500 and 1,000/µl; mild, ANC between 1,000 and 1,500/µl...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/30072997/is-it-safe-to-switch-from-intravenous-immunoglobulin-to-subcutaneous-immunoglobulin-in-patients-with-common-variable-immunodeficiency-and-autoimmune-thrombocytopenia
#6
Philipp Scheuerlein, Larissa Pietsch, Nadezhda Camacho-Ordonez, Veronika Reiser, Smita Patel, Siobhan O Burns, Klaus Warnatz, Bodo Grimbacher
Background: A significant amount of common variable immunodeficiency (CVID) patients manifest with autoimmunity. Particularly, autoimmune thrombocytopenia (AITP) is commonly seen. Intravenous immunoglobulins (IVIG) are an established treatment option for both, CVID and AITP. Nonetheless, due to fewer systemic side effects, immunoglobulins are increasingly applied subcutaneously (SCIG). Objective: To compare the efficacy and safety of IVIG and SCIG treatment in patients with both CVID and clinical relevant thrombocytopenia in the prevention of AITP bouts...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/30072561/autoimmune-cytopenias-and-thrombotic-thrombocytopenic-purpura
#7
Charlotte K Brierley, Sue Pavord
The autoimmune cytopenias are a group of disorders resulting primarily from autoantibody-mediated destruction of blood cells, with variable clinical sequelae depending on the severity and lineage affected. Disease presentation ranges from an asymptomatic finding on a routine full blood count to an acutely unwell patient suffering the clinical consequences of severe anaemia, neutropenia or thrombocytopenia. The cytopenia may be primary or secondary to underlying infectious, immune or malignant processes. Thrombotic thrombocytopenic purpura (TTP) is a distinct, rare but potentially life-threatening entity that classically but not invariably presents with a pentad of acute onset haemolytic anaemia, thrombocytopenia, neurological symptoms, renal impairment and fevers...
August 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/30060721/mortality-and-prognostic-factors-in-chinese-patients-with-systemic-lupus-erythematosus
#8
L Mu, Y Hao, Y Fan, H Huang, X Yang, A Xie, X Zhang, L Ji, Y Geng, Z Zhang
Objectives To investigate the mortality and causes of death in Chinese patients with systemic lupus erythematosus. Methods We collected the clinical data of all consecutive adult systemic lupus erythematosus patients at the Rheumatology department of Peking University First Hospital between January 2007 and December 2015. The primary causes of death were identified, the standardized mortality ratio and years of life lost were calculated, and the survival and variables associated with mortality were determined by Kaplan-Meier and Cox regression analysis respectively...
July 31, 2018: Lupus
https://www.readbyqxmd.com/read/30055825/altered-distribution-and-function-of-splenic-innate-lymphoid-cells-in-adult-chronic-immune-thrombocytopenia
#9
Sylvain Audia, Thomas Moulinet, Marion Ciudad-Bonté, Maxime Samson, Olivier Facy, Pablo Ortega-Deballon, Philippe Saas, Bernard Bonnotte
Innate lymphoid cells (ILCs) have been characterized as innate immune cells capable to modulate the immune response in the mucosae. Human ILCs have been rarely described in secondary lymphoid organs except in tonsils. Moreover, their function and phenotype in human secondary lymphoid organs during autoimmune diseases have never been studied. We took advantage of splenectomy as a treatment of immune thrombocytopenia (ITP) to describe and compare splenic ILC from 18 ITP patients to 11 controls. We first confirmed that ILC3 represented the most abundant ILC subset in human non-inflamed spleens, accounting for 90% of total ILC, and that they were mostly constituted of NKp44- cells...
July 23, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/30046699/anti-glycoprotein-vi-mediated-immune-thrombocytopenia-an-under-recognized-and-significant-entity
#10
David J Rabbolini, Elizabeth E Gardiner, Marie-Christine Morel-Kopp, Scott Dunkley, Anila Jahangiri, Christine S-M Lee, William S Stevenson, Christopher M Ward
<AbstractText>Idiopathic immune thrombocytopenia (ITP) is an autoimmune disorder characterized by relapsing/ remitting thrombocytopenia. Bleeding complications are infrequent with platelet counts above 30×109 /L, and this level is commonly used as a threshold for treatment. The question of another/ co-existent diagnosis or an alternate mechanism of platelet destruction arises when bleeding is experienced with platelet counts above this threshold. We report a case of anti-GPVI mediated ITP that was diagnosed following investigations performed to address this key clinical question...
October 2017: Research and Practice in Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/30021206/abnormal-expression-of-long-noncoding-rnas-in-primary-immune-thrombocytopenia-a-microarray-related-study
#11
Tengda Li, Mingli Gu, Peng Liu, Yun Liu, Jie Guo, Weiwei Zhang, Anmei Deng, Cheng Qian
BACKGROUND/AIMS: Long noncoding RNAs (lncRNAs) are important regulators of biological processes and they contribute to the pathological developments of various diseases, including autoimmune diseases. To gain the further understanding, we estimate the expression of lncRNAs in primary immune thrombocytopenia (ITP). METHODS: In this study, microarray studies were performed to characterize expression profiles of various lncRNAs and mRNAs in blood samples collected from ITP patients...
July 18, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/30015642/pathophysiology-of-immune-thrombocytopenia
#12
June Li, Jade A Sullivan, Heyu Ni
PURPOSE OF REVIEW: Immune thrombocytopenia (ITP) is a common autoimmune bleeding disorder with as of yet, no established clinical prognostic or diagnostic biomarkers. Patients frequently experience a markedly decreased quality of life and may be at risk for severe/fatal haemorrhage. Here, we address discoveries in the pathogenesis of ITP, and novel therapeutic strategies in mouse models and human patients. Consolidation of these findings should be important in providing insight to establish future prognostic protocols as well as cutting-edge therapeutics to target refractory ITP...
September 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/30008456/risk-factors-for-future-scleroderma-renal-crisis-at-systemic-sclerosis-diagnosis
#13
Sarah M Gordon, Rodger S Stitt, Robert Nee, Wayne T Bailey, Dustin J Little, Kendral R Knight, James B Hughes, Jess D Edison, Stephen W Olson
OBJECTIVE: Systemic sclerosis (SSc) is a disease of autoimmunity, fibrosis, and vasculopathy. Scleroderma renal crisis (SRC) is one of the most severe complications. Corticosteroid exposure, presence of anti-RNA polymerase III antibodies (ARA), skin thickness, and significant tendon friction rubs are among the known risk factors at SSc diagnosis for developing future SRC. Identification of additional clinical characteristics and laboratory findings could expand and improve the risk profile for future SRC at SSc diagnosis...
July 15, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29998369/decreased-serum-thrombospondin-1-and-elevation-of-its-autoantibody-are-associated-with-multiple-exacerbated-clinical-manifestations-in-systemic-lupus-erythematosus
#14
Yang Meng, Meng Zhang, Xiaozhen Zhao, Yongjing Cheng, Rulin Jia, Yan Wang, Xiaolin Sun
The pathological effects of thrombospondin-1 (TSP-1) have been studied in many preclinical tumor models and rheumatoid arthritis. However, the role of TSP-1 and anti-thrombospondin-1 autoantibodies (ATSA) in systemic lupus erythematosus (SLE) has not been specifically defined. In this study, we investigated the clinical relevance and functional significance of TSP-1 and ATSA in SLE patients. Serum levels of TSP-1 and ATSA were measured by ELISA in 138 adult SLE patients and 60 healthy controls. Blood cell counts, rheumatoid factor (RF), immunoglobulins, erythrocyte sedimentation rate (ESR), complements, and SLE-related autoantibodies were measured by standard laboratory techniques...
July 11, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29985728/associations-of-circulating-cell-free-microrna-with-vasculopathy-and-vascular-events-in-systemic-lupus-erythematosus-patients
#15
S D Kay, A L Carlsen, A Voss, M Burton, Acp Diederichsen, M K Poulsen, Nhh Heegaard
OBJECTIVE: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a high risk of atherosclerosis and cardiovascular disease (CVD). MicroRNAs (miRNAs) are small non-coding RNAs that modulate protein translation, and dysregulation is seen in autoimmunity, atherosclerosis, and CVD. We investigate associations between circulating miRNAs and markers of atherosclerosis in SLE patients. METHOD: A group (n = 121) of well-characterized SLE patients were screened for atherosclerosis by cardiac computed tomography and carotid ultrasound...
July 9, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29981476/severe-immune-thrombocytopenia-in-pregnancy-treated-with-eltrombopag-a-case-report
#16
Iolanda João Mora Cruz Freitas Ferreira, Filipa Sousa, Elsa Marques Vasco, Ana Luísa Fialho de Amaral Areia, José Paulo Achando Silva Moura
Immune thrombocytopenia (ITP) during pregnancy is an acquired autoimmune disease present in 1-2 of every 1000 pregnancies. Thrombopoietin (TPO)-mimetic drugs, such as eltrombopag, have been successfully used for treatment of ITP during pregnancy, but studies regarding its safety during gestation are lacking. A 33-year-old nulliparous woman with a history of chronic ITP, presented at the emergency department with petechiae, epistaxis, bruises, conjunctival effusions and a platelet count of 3×109 /L at 25 weeks gestation...
July 4, 2018: Journal of gynecology obstetrics and human reproduction
https://www.readbyqxmd.com/read/29979258/autoimmune-haemolytic-anaemia-and-autoimmune-thrombocytopenia-in-childhood-onset-systemic-lupus-erythematosus-updates-on-pathogenesis-and-treatment
#17
Jessica Neely, Emily von Scheven
PURPOSE OF REVIEW: Autoimmune haemolytic anaemia (AIHA) and autoimmune thrombocytopenia are common complications of childhood-onset lupus, which may be life-threatening. A greater understanding of the pathogenesis of these haematologic manifestations will enhance our understanding of the biology of systemic lupus erythematosus (SLE) and inform the identification of novel treatments. RECENT FINDINGS: The mechanisms underlying AIHA and autoimmune thrombocytopenia are incompletely understood and likely multifactorial...
September 2018: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/29978544/risk-factors-for-bleeding-including-platelet-count-threshold-in-newly-diagnosed-immune-thrombocytopenia-adults
#18
Marie-Léa Piel-Julian, Matthieu Mahévas, Johanne Germain, Laetitia Languille, Thibault Comont, Maryse Lapeyre-Mestre, Bernard Payrastre, Odile Beyne-Rauzy, Marc Michel, Bertrand Godeau, Daniel Adoue, Guillaume Moulis
BACKGROUND: The aim of this cross-sectional study was to assess risk factors of bleeding in ITP adults, including the determination of platelet count thresholds. METHODS: We selected all newly diagnosed ITP adults included in the CARMEN register and at the French referral center for autoimmune cytopenias. Frequencies of any bleeding, mucosal bleeding and severe bleeding (gastro-intestinal, intracranial or macroscopic hematuria) at ITP onset were assessed. Platelet count thresholds were assessed using receiver-operating characteristics curves...
July 6, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29950233/-changes-of-il-21-and-its-mediated-jak-stat-signaling-pathway-in-patients-with-immune-thrombocytopenia
#19
Qian Zhang, Hai Bai, Xiao-Hui Yu, Bing Wu, Yao-Zhu Pan, Cun-Bang Wang, Li-Ping Zhao, Wen-Bo Li, Feng Xu, Jun Zhang
OBJECTIVE: To explore the correlation between JAK/STAT signaling pathways and pathogenesis of immune thrombocytopenia(ITP). METHODS: Twenty-six newly-diagnosed ITP patients was included in this study. They all meet the clinical and hematological criteria for the diagnosis of ITP, and patients with coronary heart disease, severe refractory hypertension, diabetes or with severe liver or kidney function incompetence were ruled out. 24 healthy control without autoimmune diseases, viral infectious diseases and with normal liver and kidney functions were also included...
June 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29945940/the-remarkable-diversity-of-thrombotic-thrombocytopenic-purpura-a-perspective
#20
REVIEW
James N George
Understanding the autoimmune etiology of acquired thrombotic thrombocytopenic purpura (TTP) has provided precision for the diagnosis and a rationale for immunosuppressive treatment. These advances have also allowed recognition of the remarkable clinical diversities of patients' initial presentations and their long-term outcomes. These diversities are illustrated by the stories of patients from the Oklahoma TTP Registry. The initial presentation of TTP may be the discovery of unexpected severe thrombocytopenia in a patient with minimal or no symptoms...
June 26, 2018: Blood Advances
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