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Immune thrombocytopenia 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/29297955/predictive-factors-for-response-to-ivig-in-children-with-itp
#5
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
#6
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/29287794/effect-of-glucocorticoid-treatment-on-baff-and-april-expression-in-patients-with-immune-thrombocytopenia-itp
#7
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
#8
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/29234214/clinical-practice-updates-in-the-management-of-immune-thrombocytopenia
#9
Ayesha M Khan, Halina Mydra, Ana Nevarez
Immune thrombocytopenia (ITP), previously called idiopathic thrombocytopenia purpura, is an autoimmune disorder characterized by a severe reduction in peripheral blood platelet count. In healthy individuals, normal platelet count ranges from 150-450 × 109/L, while in thrombocytopenia counts fall to less than 100 × 109/L.1 In adults, the incidence of ITP is approximately two to four per 100,000.2,3 Bleeding risks, specifically hemorrhage and intracranial hemorrhage, represent the most serious complications for patients with ITP...
December 2017: P & T: a Peer-reviewed Journal for Formulary Management
https://www.readbyqxmd.com/read/29222284/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#10
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
#11
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
#12
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
#13
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/29119304/is-thrombocytosis-always-an-indicator-of-autosplenectomy-in-patients-with-systemic-lupus-erythematosus
#14
Döndü Üsküdar Cansu, Hava Üsküdar Teke, Ahmet Musmul, Cengiz Korkmaz
In systemic lupus erythematosus (SLE), the most commonly encountered finding related to platelets is thrombocytopenia whereas thrombocytosis is rarely reported. Our aim here was to reveal the type and the frequency of thrombocytosis in SLE patients along with its causes. Data of patients were evaluated retrospectively. Patients who had a platelet count of > 450,000/mm3 (> 450 × 109/L) in at least two subsequent counts and lasting more than 6 months during the follow-up were considered to have "persistent thrombocytosis"...
November 8, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29114398/multi-system-manifestations-of-mycoplasma-pneumoniae-infection-in-a-young-patient
#15
Ibrahim Al Busaidi, Mohammed Al-Amin, Shadin Ibrahim, Abdullah Balkhair, Zied Gaifer
Introduction.Mycoplasma pneumoniae is a small cell-wall-lacking bacterium that belongs to the mycoplasma (Mollicutes) prokaryote micro-organisms. It is a common cause of both upper and lower respiratory tract infections in all age groups. Respiratory illness is the most common manifestation of M. pneumoniae infection; however, extrapulmonary involvement may be present or predominant. The skin, mucus membranes, central nervous system, cardiovascular system, haematopoietic system, kidneys and musculoskeletal system are the most commonly involved extrapulmonary sites...
September 2017: JMM Case Reports
https://www.readbyqxmd.com/read/29095327/cerebral-venous-sinus-thrombosis-due-to-low-molecular-weight-heparin-induced-thrombocytopenia
#16
Ezequiel Gleichgerrcht, Ming Y Lim, Tanya N Turan
INTRODUCTION: Heparin-induced thrombocytopenia (HIT) is an immune-mediated complication of heparin exposure. A limited number of studies have reported cerebral venous sinus thrombosis (CVST) as the presenting thrombotic event induced by HIT, only one of which occurred with exposure to low-molecular-weight heparin (LMWH), with death as outcome. Here, we present a unique case of LMWH-induced HIT leading to CVST but resulting in good clinical outcome. CASE REPORT: A 52-year-old woman received subcutaneous LMWH for deep vein thrombosis prophylaxis while in rehabilitation following kyphoplasty for spinal fracture related to recent trauma...
November 2017: Neurologist
https://www.readbyqxmd.com/read/29078927/acquired-bleeding-disorders
#17
REVIEW
Alisheba Hurwitz, Richard Massone, Bernard L Lopez
Emergency medicine practitioners treat bleeding patients on a regular basis. Disorders of hemostasis are an additional challenge in these patients but can be assessed and managed in a systematic fashion. Of particular importance to the emergency clinician are the iatrogenic causes of abnormal hemostasis. Other acquired causes of abnormal hemostasis include renal disease, immune thrombocytopenia, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, acquired coagulation factor inhibitors, acute traumatic coagulopathy, liver disease, and disseminated intravascular coagulopathy...
December 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29078925/thrombotic-microangiopathies-ttp-hus-hellp
#18
REVIEW
Shane Kappler, Sarah Ronan-Bentle, Autumn Graham
Thrombocytopenia, strictly defined as a platelet count less than 150,000, is common in the emergency department. Recognition, diagnostic investigation, and proper disposition of a thrombocytopenic patient are imperative. One group of disorders leading to thrombocytopenia is the thrombotic microangiopathies, hallmarked by platelet destruction. These thrombotic microangiopathies include thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS) and hemolysis, elevated liver enzyme levels, low platelet count (HELLP), which should be distinguished from similar disease processes such as immune thrombocytopenia (ITP), disseminated intravascular coagulation (DIC) and heparin induced thrombocytopenia (HIT)...
December 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29069007/successful-eltrombopag-treatment-of-severe-refractory-thrombocytopenia-in-chronic-myelomonocytic-leukemia-two-cases-reports-a-care-compliant-article
#19
Yayue Gao, Ming Gong, Chunxia Zhang, Xudong Kong, Yigai Ma
RATIONALE: Thrombocytopenia in chronic myelomonocytic leukemia (CMML) is usually attributed to impaired marrow production resulting from cytotoxic drug use or CMML itself ("CMML-induced thrombocytopenia"). In very rare cases, immune thrombocytopenia (ITP) can be a complication of CMML ("CMML-associated ITP"). However, treatment of severe thrombocytopenia in patients with CMML is still a challenge. PATIENT CONCERNS: Case 1 was a 61-year-old female patient admitted to our hospital because of skin petechiae and purpura for 6 days...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29020887/the-potential-association-of-tumor-necrosis-factor-%C3%AE-eta-252-g-a-cytokine-gene-polymorphism-with-immune-thrombocytopenic-purpura-among-egyptian-children
#20
Dalia Saber Morgan, Rasha Abdel-RaoufAbdel-Aziz Afifi, Shereen Mohamed El-Hoseiny, Dalia Gamil Amin, Sara Yahia Gaber Ibrahim
OBJECTIVES: The main objective of this study was to study tumor necrosis factor beta (TNFB) + 252G/A gene polymorphism, known to be related to autoimmunity, in immune thrombocytopenia (ITP) patients. We also aimed to investigate the association between TNFB + 252G/A polymorphism and susceptibility to develop persistent/chronic ITP. METHODS: One hundred pediatric ITP patients, as well as 50 age- and sex-matched healthy Egyptian subjects, were included. Genotyping of TNF-β gene (G252A) was done using the PCR-RFLP method...
October 12, 2017: Hematology (Amsterdam, Netherlands)
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