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Immune thrombocytopenia purpura

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https://www.readbyqxmd.com/read/29673280/safety-and-efficacy-of-thrombopoeitin-mimetics-for-refractory-immune-thrombocytopenia-purpura-in-patients-with-systemic-lupus-erythematosus-or-antiphospholipid-syndrome-a-case-series
#1
A Lusa, A Carlson
Background While thrombopoeitin (TPO) agonists that act to simulate platelet production have been approved for use in steroid-refractory chronic immune thrombocytopenia purpura (ITP), there are few data on the safety and efficacy of these medications in patients with concurrent systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS). Given that these agents can increase all hematopoietic cell lineages, it is unclear if there is an increased risk for exacerbation of the underlying lymphocyte-driven autoimmune disease in this population...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29668541/cutaneous-hemorrhage-types-as-supportive-factors-for-predicting-chronic-immune-thrombocytopenia-in-children
#2
Sarit Kalfon, Haitham Hamadeh, Yaakov Schachter, Nechama Sharon
Our objective was to assess risk factors for developing chronic immune thrombocytopenia (ITP) in children. The charts of all consecutive children diagnosed with ITP between 2000 and 2015 at a single center were retrospectively reviewed, and clinical characteristics at initial presentation were analyzed. Sixty-two children were included in the study (mean age, 6.15 y); 44 (71%) were found to have acute ITP, and 18 (29%) developed chronic ITP (permanent or relapsing thrombocytopenia >12 mo). In a univariate analysis, cutaneous hemorrhages were observed significantly more in acute patients (90...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29623717/-idiopathic-thrombocytopenia-refractery-to-therapy-of-cyclosporine-a-in-clinical-practice-case-report
#3
Lucia Schwarzová, Zdenko Pirník, Natália Štecová
Primary immune thrombocytopenia (idiopathic thrombocytopenic purpura; ITP) is an autoimmune disorder characterized by isolated thrombocytopenia without abnormalities in the erythroid and myeloid/lymphoid lineages. The incidence of ITP in adults is estimated at approximately 1.6-3.9 per 100 000 persons/year. The aim of this study was to analyze a case report of a patient with diagnosed immune thrombocytopenia (D69.3) resistant to cyclosporine A therapy as well as an evaluation of factors that could contribute to an inadequate response to cyclosporine A...
2018: Ceská a Slovenská Farmacie
https://www.readbyqxmd.com/read/29620681/-immune-thrombocytopenia-as-key-feature-of-a-novel-ada2-deficiency-variant-implication-on-differential-diagnostics-of-itp-in-children
#4
Mikael Sundin, Per Marits, Stefan Nierkens, Antonios G A Kolios, Jakob Nilsson
Thrombocytopenia presenting during early childhood is most commonly diagnosed as immune/idiopathic thrombocytopenic purpura (ITP), where the antibody-mediated destruction of thrombocytes is often transient. If treatment is indicated, the majority of patients respond to immune-modulation by intravenous immunoglobulin G infusion or systemic corticosteroids. Differential diagnoses to childhood ITP includes thrombocytopenia due to infections, drugs, rheumatologic conditions, immune dysregulation, and inherited bone marrow failures, for example, congenital amegakaryocytic thrombocytopenia...
April 3, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29618690/-neutrophil-recovery-by-eltrombopag-treatment-in-a-patient-with-adult-onset-autoimmune-neutropenia-and-immune-thrombocytopenia
#5
Masahiro Tokunaga, Masahide Yamada, Atsushi Kondo, Mai Mishima, Shinichi Yoshikawa, Shinya Inoue, Takako Morita, Nobuhiko Tominaga
Adult-onset autoimmune neutropenia (AIN) is rarely self-limiting, unlike infant-onset AIN. Although several therapeutic agents have been reported, including corticosteroids, more effective treatment options may exist. Here, we describe neutrophil recovery by eltrombopag in a 52-year-old male AIN patient with immune thrombocytopenia (ITP) who was referred to our hospital with severe neutropenia. Within a year of referral, he developed moderate thrombocytopenia. He was diagnosed with AIN and concurrent ITP, based on the detection of antineutrophil antibodies and bone marrow aspiration, respectively...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29607959/successful-treatment-of-aggressive-mature-b-cell-lymphoma-mimicking-immune-thrombocytopenic-purpura
#6
Koya Ono, Yasushi Onishi, Masahiro Kobayashi, Satoshi Ichikawa, Shunsuke Hatta, Shotaro Watanabe, Yoko Okitsu, Noriko Fukuhara, Ryo Ichinohasama, Hideo Harigae
A 55-year-old woman suffered from hemorrhagic tendency. She had severe thrombocytopenia without any hematological or coagulatory abnormalities, and a bone marrow examination revealed an increased number of megakaryocytes without any abnormal cells or blasts. No lymphadenopathy or hepatosplenomegaly was observed on computed tomography. She was initially diagnosed with immune thrombocytopenic purpura (ITP). None of the treatments administered for ITP produced a response. However, abnormal cells were eventually found during the third bone marrow examination...
March 30, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29554892/immune-thrombocytopenic-purpura-presenting-in-a-patient-after-renal-transplant-for-diabetic-nephropathy
#7
Raja Muhammad Rashid, Zahid Nabi, Ahmad Zaki Ansari, Quratul-Ain Qaiser
BACKGROUND: Immune thrombocytopenic purpura (ITP) is primarily characterized by immune-mediated destruction of platelets in circulation. Major treatment options range from careful observation, steroids, immunosuppressive medications, immunoglobulins to splenectomy. Interestingly and rarely, ITP has also been reported after solid organ transplantation in patients receiving immunosuppressive medications. While the incidence of new onset ITP after solid organ transplant is comparatively well documented, new onset ITP after renal transplant has only been reported in two patients...
March 20, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29512619/a-practical-and-case-based-approach-to-thrombocytopenia-in-cardiology-practice
#8
Erman Öztürk, Ferit Onur Mutluer
In cardiology practice, anticoagulation and antiplatelet therapies are essential for most patients. As of yet, there is no high quality evidence regarding these treatments in thrombocytopenic patients, which continues to be an issue. Thrombocytopenia is defined as a platelet count of <150x109/L and is classified as severe when the platelet count is <50x109/L. Pseudothrombocytopenia, drug-induced thrombocytopenia, immune thrombocytopenia, heparin-induced thrombocytopenia, and thrombotic thrombocytopenic purpura are some of the main causes of thrombocytopenia...
March 2018: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/29487060/eltrombopag-for-use-in-children-with-immune-thrombocytopenia
#9
REVIEW
Taylor Olmsted Kim, Jenny Despotovic, Michele P Lambert
Eltrombopag is currently the only US Food and Drug Administration-approved thrombopoietin receptor agonist for the treatment of chronic immune thrombocytopenia (ITP) in children. This oral, once-per-day therapy has shown favorable efficacy and adverse effect profiles in children. Two multicenter, double-blind, placebo controlled clinical trials (PETIT [Efficacy and Safety Study of Eltrombopag in Pediatric Patients With Thrombocytopenia From Chronic Idiopathic Thrombocytopenic Purpura (ITP)] and PETIT2 [Study of a New Medication for Childhood Chronic Immune Thrombocytopenia (ITP), a Blood Disorder of Low Platelet Counts That Can Lead to Bruising Easily, Bleeding Gums, and/or Bleeding Inside the Body]) demonstrated efficacy in raising platelet counts, reducing bleeding, and reducing the need for concomitant ITP therapies with relatively few adverse effects...
February 27, 2018: Blood Advances
https://www.readbyqxmd.com/read/29464836/the-presentation-and-management-of-platelet-disorders-in-pregnancy
#10
Bernardus G Goldman, Mark P Hehir, Sahr Yambasu, Edward M O'Donnell
Thrombocytopenia, defined as a platelet count less than 150 000 per microlitre, occurs in 7%-12% of all pregnancies. Apart from anaemia, it is the most common haematological disorder in pregnancy. Despite its frequent occurrence, thrombocytopenia often leads to difficulties of diagnosis and management in pregnancy. Typically, a pregnant woman will have platelet counts of 150 000 to 450 000 per microlitre and platelet counts may be slightly lower than those of healthy, non-pregnant controls. Approximately, 8% of pregnant women will develop mild thrombocytopenia (100 000-150 000 per microlitre) and while 65% of these women will have no underlying pathology, all pregnant women with platelet counts of less than 100 000 per microlitre should undergo further clinical and laboratory assessment...
February 21, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29430236/idiopathic-thrombocytopenia-purpura-masking-hodgkin-disease-a-paraneoplastic-syndrome-or-simply-a-mere-association
#11
Nicholas Poponea, Mohanad Suede, Mohammad Muhsin Chisti
We report a 74-year-old female who presented to the emergency department complaining of bruising and stroke-like symptoms. She underwent a negative stroke work-up but was found to have profoundly low platelets and splenomegaly on examination. An abdominal CT scan was ordered, showing pelvic lymphadenopathy. Lymphoma was suspected. However, subsequent bone marrow and lymph node biopsies showed no evidence of this. She was treated for immune thrombocytopenia purpura (ITP) to no avail while a lymphoma work-up continued...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29399327/recent-advances-in-understanding-and-management-of-acquired-thrombocytopenia
#12
REVIEW
Srikanth Nagalla, Ravindra Sarode
There are numerous congenital and acquired causes of thrombocytopenia. Thrombocytopenia could be a result of decreased bone marrow production, increased consumption, increased destruction, splenic sequestration or a combination of these causes. In this review, we have focused on some of the serious acquired causes of thrombocytopenia. There have been some significant advances in our understanding of the pathophysiology, diagnostic testing, and treatment of immune thrombocytopenia, heparin-induced thrombocytopenia, thrombotic thrombocytopenic purpura, and atypical hemolytic uremic syndrome over the past five years...
2018: F1000Research
https://www.readbyqxmd.com/read/29397857/-research-progress-on-the-establishment-of-animal-model-with-immune-thrombocytopenic-purpura-review
#13
Hao-Lan Wang, Bao-Shan Liu
Immune thrombocytopenic purpura (ITP) is a chronic and recurrent autoimmune disease, which seriously affects the life quality of patients. At present, a series of new advances have been made in the pathogenesis of ITP, particularly, in the abnormal cellular immunity. However, in the medical studiess generally research of ITP cellular immunity was limited. Therefore, it is urgent to establish an ideal ITP model for the study of ITP pathogenesis, so as to contribute to promote the ITP new treatment progeamme...
February 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29357939/inherited-thrombotic-thrombocytopenic-purpura-mimicking-immune-thrombocytopenic-purpura-during-pregnancy-a-case-report
#14
Valter Romão de Souza, Ana Beatriz Cavalcante de Oliveira, Ana Maria Vanderlei, Amanda Queiroz da Mota Silveira Aroucha, Bruna Pontes Duarte, Aureli Nunes Machado, Lívia Netto Chaer, Cláudia Wanderley de Barros Correia, Maria da Conceição de Barros Correia, Manuela Freire Hazin Costa
BACKGROUND: Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. The deficiency in ADAMTS13 metalloprotease, which cleaves the von Willebrand factor, may be congenital or acquired. The congenital form is caused by inherited mutations in the ADAMTS13 gene. The diagnosis is challenging due to the nonspecific signs and symptoms, as well as the rarity of the disease...
January 22, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29357781/dynamic-dosing-of-romiplostim-in-patients-with-immune-thrombocytopenia-purpura-two-case-reports
#15
Jeffrey A Gilreath, Mei Wei, Shilpa Paul, Charles J Parker, David D Stenehjem, George M Rodgers
Romiplostim is a thrombopoietin receptor agonist approved for the treatment of immune thrombocytopenia purpura. When following FDA-approved romiplostim prescribing recommendations to withhold treatment for platelet counts above 400k/µL, some patients exhibit a precipitous decline in their platelet count potentially causing patient harm. We present two cases where stable platelet counts were achieved only through persistent weekly dosing of romiplostim despite platelet counts above 400k/µL on the day of administration...
January 1, 2018: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/29356300/pathophysiology-of-thrombotic-thrombocytopenic-purpura-and-hemolytic-uremic-syndrome
#16
J A Kremer Hovinga, S R Heeb, M Skowronska, M Schaller
Thrombotic microangiopathies are rare disorders characterized by the concomitant occurrence of severe thrombocytopenia, microangiopathic hemolytic anemia, and a variable degree of ischemic end-organ damage. The latter particularly affects the brain, the heart, and the kidneys. The primary forms, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), although their clinical presentations often overlap, have distinctive pathophysiologies. TTP is the consequence of a severe ADAMTS-13 deficiency, either immune-mediated as a result of circulating autoantibodies, or caused by mutations in ADAMTS-13...
April 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29348920/wiskott-aldrich-syndrome-that-was-initially-diagnosed-as-immune-thrombocytopenic-purpura-secondary-to-a-cytomegalovirus-infection
#17
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
#18
REVIEW
M Levi, M Scully, M Singer
The interaction between platelets and the vessel wall is mediated by various receptors and adhesive proteins, of which von Willebrand factor (VWF) is the most prominent. The multimeric size of VWF is an important determinant of a more intense platelet-vessel wall interaction, and is regulated by the VWF-cleaving protease ADAMTS-13. A deficiency in ADAMTS-13 leads to higher concentrations of ultralarge VWF multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura, a thrombotic microangiopathy characterized by thrombocytopenia, non-immune hemolysis, and organ dysfunction...
April 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
#19
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...
March 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
#20
LETTER
M Fouchard, B Pan Petesch, C Abasq-Thomas, A Saraux, L Misery, E Brenaut
No abstract text is available yet for this article.
January 5, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
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