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Immune thrombocytopenic purpura

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https://www.readbyqxmd.com/read/29348920/wiskott-aldrich-syndrome-that-was-initially-diagnosed-as-immune-thrombocytopenic-purpura-secondary-to-a-cytomegalovirus-infection
#1
Ryota Kaneko, Shohei Yamamoto, Naoko Okamoto, Kosuke Akiyama, Ryosuke Matsuno, Daisuke Toyama, Akihiro Hoshino, Kohsuke Imai, Keiichi Isoyama
Wiskott-Aldrich syndrome is a rare X-linked recessive disease resulting from variations in the WAS gene. Wiskott-Aldrich syndrome is sometimes difficult to differentiate from immune thrombocytopenic purpura. A 2-month-old boy was admitted to our hospital for purpura and thrombocytopenia. His mean platelet volume was reported to be normal. Treatment with intravenous immunoglobulins failed to improve the patient's platelet count. Subsequently, an acute cytomegalovirus infection was confirmed by serological testing and antigenemia...
2018: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29337416/the-role-of-adamts-13-in-the-coagulopathy-of-sepsis
#2
REVIEW
Marcel Levi, Marie Scully, Mervyn Singer
The interaction between platelets and the vessel wall is mediated by various receptors and adhesive proteins, of which von Willebrand factor is most prominent. The multimeric size of von Willebrand factor is an important determinant of a more intense platelet-vessel wall interaction. and is regulated by the von Willebrand factor cleaving protease, ADAMTS-13. A deficiency in ADAMTS-13 leads to higher concentrations of ultra-large von Willebrand factor multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura (TTP), a thrombotic microangiopathy characterized by thrombocytopenia, non-immune haemolysis, and organ dysfunction...
January 16, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29324461/efficacy-of-treatment-immune-thrombocytopenic-purpura-in-pregnancy-with-corticosteroids-and-intravenous-immunoglobulin-a-prospective-follow-up-of-suggested-practice
#3
Gisela Wegnelius, Katarina Bremme, Pelle G Lindqvist
: The current study is performed to assess a routine for treatment of immune thrombocytopenic purpura in pregnancy. A prospective programme for monitoring and treatment with intravenous immunoglobulin or cortisone in pregnancies with immune thrombocytopenic purpura was suggested to all delivery units in Sweden. Treatment should be avoided if platelet counts were more than 20 × 10/l during pregnancy with no bleeding complications and with a target of 100 × 10/l at delivery. Descriptive statistics and logistic regression analysis were used...
January 10, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29314277/erasmus-syndrome-associated-with-an-immune-thrombocytopenic-purpura
#4
Maxime Fouchard, Brigitte Pan Petesch, Claire Abasq-Thomas, Alain Saraux, Laurent Misery, Emilie Brenaut
We report the observation of a 57-year-old patient followed for an Erasmus syndrome (a systemic sclerosis developing secondary to silica exposure) (1) diagnosed in 2002 after being exposed as a dental technician, for whom a severe thrombocytopenia was discovered. He suffered from a pulmonary fibrosis, polyarthralgia, esophageal dysphagia and cutaneous involvement such as finger ulcerations and sclerodactylia. The treatment was made of nifedipine 30mg/day, esomeprazole 20mg/day and bosentan 25 mg twice a day...
January 5, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29308775/a-case-of-immune-thrombocytopenic-purpura-with-prolonged-aptt-time-a-clotter-hidden-in-a-bleeder
#5
M H Lee, P J Khoo, L T Gew, C F Ng
We report the case of a 23-year-old woman who presented with prolonged menstruation and multiple bruises on the limbs and trunk. Investigations revealed severe thrombocytopenia and deranged coagulation profile with markedly prolonged activated partial thromboplastin time (aPTT). Lupus anticoagulant, anti-cardiolipin antibody and anti-beta-2-glycoprotein 1 antibody were positive. She was diagnosed with Immune Thrombocytopenic Purpura (ITP) with positive antiphospholipid antibody serology and given a course of intravenous methylprednisolone and tapering doses of oral prednisolone...
December 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/29307971/cerebral-venous-thrombosis-after-intravenous-immunoglobulin-therapy-in-immune-thrombocytopenic-purpura
#6
Joe James, P V Shiji, Chandni Radhakrishnan
A common misconception is that immune thrombocytopenic purpura (ITP) causes only bleeding diathesis. From this case vignette of a young male with ITP who had cerebral venous thrombosis, we highlight the importance of considering venous thrombosis in such patients when they present with focal cerebral signs.
December 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/29297955/predictive-factors-for-response-to-ivig-in-children-with-itp
#7
Yukiko Higashide, Tsukasa Hori, Yuko Yoto, Hiroyuki Kabutoya, Saho Honjo, Yoshiyuki Sakai, Masanori Nojima, Minami Yoda, Masaki Yamamoto, Hiroyuki Tsutsumi
BACKGROUND: Immune thrombocytopenic purpura (ITP) is commonly treated with intravenous immunoglobulin (IVIG). METHODS: We retrospectively evaluated whether pretreatment clinical and laboratory findings could predict the short- and long-term response to IVIG. RESULTS: Short-term response was estimated by platelet counts 2 weeks after IVIG, and long-term response was assessed by thrombocytopenia-free survival (TFS). TFS was defined as the probability of survival without treatment failure after initial IVIG, such as relapse, requiring additional therapeutic interventions, or progressing to chronic ITP...
January 3, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29296878/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#8
REVIEW
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...
November 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296854/cyclosporine-or-steroids-as-an-adjunct-to-plasma-exchange-in-the-treatment-of-immune-mediated-thrombotic-thrombocytopenic-purpura
#9
Spero R Cataland, Peter J Kourlas, Shangbin Yang, Susan Geyer, Leslie Witkoff, Haiwa Wu, Camila Masias, James N George, Haifeng M Wu
Although steroids are routinely used as an adjunct to plasma exchange (PEX) therapy in the treatment of immune-mediated thrombotic thrombocytopenic purpura (iTTP), limited data regarding their efficacy or effect on ADAMTS13 biomarkers are available. We report the results of a prospective, randomized study that compared the effectiveness of prednisone or cyclosporine (CSA) as adjuncts to PEX in the treatment of iTTP. A total of 26 of the planned 72 subjects were enrolled and treated from November 2007 until February 2014 before the study was halted after a planned interim analysis...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296757/rituximab-prophylaxis-to-prevent-thrombotic-thrombocytopenic-purpura-relapse-outcome-and-evaluation-of-dosing-regimens
#10
John-Paul Westwood, Mari Thomas, Ferras Alwan, Vickie McDonald, Sylvia Benjamin, William A Lester, Gillian C Lowe, Tina Dutt, Quentin A Hill, Marie Scully
Acute antibody-mediated thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy with high morbidity and mortality. Rituximab is highly effective as prophylaxis in patients at risk of acute TTP relapse, but the ideal dosing regimen is unknown. A multicenter retrospective cohort study evaluated outcomes of patients given rituximab prophylaxis to prevent TTP relapse. Rituximab was given in 76 episodes to 45 patients (34 women and 11 men). Four once-per-week infusions of standard- (375 mg/m2 [24 episodes]), reduced- (200 mg [19 episodes]), and intermediate- (500 mg [17 episodes]) dose rituximab were given; in the remaining 16 episodes, patients received 100 to 1000 mg rituximab in 1 to 5 doses...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29287794/effect-of-glucocorticoid-treatment-on-baff-and-april-expression-in-patients-with-immune-thrombocytopenia-itp
#11
Julian Kamhieh-Milz, Nuha Ghosoun, Viktor Sterzer, Abdulgabar Salama
Immune thrombocytopenic purpura (ITP) is an idiopathic bleeding disorder. B cell activating factor (BAFF) and 'A proliferation-inducing ligand' (APRIL) have regulatory effects on B and T cells and may represent relevant factors in the pathogenesis of ITP. Serum levels and gene expression were investigated in ITP patients. Both BAFF and APRIL serum levels were significantly elevated in active ITP. However, gene expression analysis revealed both factors to have a tendency toward downregulation. Glucocorticoid treatment significantly reduced BAFF but not APRIL serum levels, which may be mediated by differences in transcription factor binding sites...
December 26, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29279696/immune-thrombocytopenia-as-a-consequence-of-rocky-mountain-spotted-fever
#12
Cherisse Baldeo, Karan Seegobin, Lara Zuberi
Primary immune thrombocytopenia (ITP) - also called idiopathic thrombocytopenic purpura or immune thrombocytopenic purpura - is an acquired thrombocytopenia caused by autoantibodies against platelet antigens. It is one of the more common causes of thrombocytopenia in otherwise asymptomatic adults. Rocky Mountain spotted fever (RMSF) is a potentially lethal, but curable, tick-borne disease. We present a case of ITP that was triggered by RMSF.
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29276711/acute-kidney-injury-immune-thrombocytopenic-purpura-and-the-infection-that-binds-them-together-disseminated-histoplasmosis
#13
Pooja Sethi, Jennifer Treece, Chidinma Onweni, Vandana Pai, Sowminya Arikapudi, Lakshmi Kallur, Varun Kohli, Jonathan Moorman
Untreated human immunodeficiency virus (HIV) can be complicated by opportunistic infections, including disseminated histoplasmosis (DH). Although endemic to portions of the United States and usually benign, DH can rarely act as an opportunistic infection in immunocompromised patients presenting with uncommon complications such as acute kidney injury and idiopathic thrombocytopenic purpura. We report a rare presentation of DH presenting with acute kidney injury and immune thrombocytopenic purpura in an immunocompromised patient with HIV...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29260065/purtscher-s-retinopathy-as-the-presenting-manifestation-of-immune-thrombocytopenic-purpura
#14
Caroline C Awh, Bobeck S Modjtahedi, Dean Eliott
Purpose: To report the case of a 52-year-old man with Purtscher's retinopathy as the presenting manifestation of immune thrombocytopenic purpura (ITP). Observations: Treatment with corticosteroids led to the resolution of hematologic findings within 1 week, and normal visual acuity was achieved after 2 months with no additional treatment. Conclusions and importance: This is the first reported association between Purtscher's retinopathy and ITP...
June 2017: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29222284/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#15
REVIEW
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
https://www.readbyqxmd.com/read/29222249/thrombocytopenia-in-pregnancy
#16
REVIEW
Douglas B Cines, Lisa D Levine
Thrombocytopenia develops in 5% to 10% of women during pregnancy or in the immediate postpartum period. A low platelet count is often an incidental feature, but it might 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 made over the past 5 to 10 years in understanding and managing the more common causes of thrombocytopenia in pregnancy...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29193103/intravenous-immunoglobulin-induced-acute-thrombocytopenia
#17
Anna Gurevich-Shapiro, Lilach Bonstein, Galia Spectre, Nardeen Atweh, Tsipora Gruenewal, Michael Shapiro, Boaz Tadmor, Pia Raanani
BACKGROUND: Intravenous immunoglobulin (IVIG) has known efficacy in various hematologic conditions, including immune thrombocytopenic purpura. STUDY DESIGN AND METHODS: We present the clinical course of a patient with splenic marginal zone lymphoma, who developed acute thrombocytopenia on three consecutive episodes, with nadir counts of 27 × 109 , 50 × 109 , and 9 × 109 /L, upon administration of Intratect IVIG for hypogammaglobulinemia. An immunofluorescence test applying flow cytometry and monoclonal antibody immobilization of platelet antigens (MAIPA) assay were used to evaluate the reaction between IgG present in the IVIG preparations and the patient's or healthy donors' platelets (PLTs)...
November 29, 2017: Transfusion
https://www.readbyqxmd.com/read/29138381/-a-case-of-lung-cancer-associated-with-drug-induced-immune-thrombocytopenia
#18
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/29120812/imaging-findings-in-systemic-childhood-diseases-presenting-with-dermatologic-manifestations
#19
REVIEW
Adam Z Fink, Julia K Gittler, Radhika N Nakrani, Jonathan Alis, Einat Blumfield, Terry L Levin
PURPOSE: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. METHODS: We review the imaging findings in childhood diseases associated with dermatologic manifestations. FINDINGS: Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura)...
October 31, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/29120578/role-of-cannabinoid-cb2-receptor-gene-cnr2-polymorphism-in-children-with-immune-thrombocytopenic-purpura-in-beni-suef-governorate-in-egypt
#20
Dina A Ezzat, Amira A Hammam, Waleed M El-Malah, Rasha A Khattab, Eman M Mangoud
The cannabinoid system is involved in the immune regulation by modulation of Th cells type 1 and 2. It is composed of the CB2 receptor which is expressed at 10 to 100 folds greater levels on immune cells than the CB1 receptors. The CB2 is encoded by the cannabinoid CB receptor gene (CNR2) gene. This study aims to investigate the polymorphism in CNR2 gene variation rs 35761398 (Q63R) in Egyptian children with immune thrombocytopenic purpura and to investigate the relation between this gene polymorphism and either the susceptibility to or the chronicity of the disease...
January 2017: Egyptian Journal of Immunology
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