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Idiopathic thrombocytopenia

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https://www.readbyqxmd.com/read/29766467/thrombotic-microangiopathy-on-kidney-biopsy-in-a-patient-with-tafro-syndrome
#1
Toshikazu Ozeki, Masashi Tsuji, Junichiro Yamamoto, Chisato Shigematsu, Shoichi Maruyama
TAFRO syndrome represents a characteristic constellation of symptoms comprising Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, and Organomegaly, and is considered to be a clinicopathologic variant of idiopathic multicentric Castleman disease. A 51-year-old woman was admitted to the hospital complaining of abdominal distension. Findings on physical examination were indicative of anasarca. Computed tomography revealed mild splenomegaly, pericardial effusion, pleural effusion, ascites, and paraaortic lymphadenopathy...
May 15, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29758336/grand-rounds-hepatic-manifestations-of-telomere-biology-disorders
#2
REVIEW
Mrinal M Patnaik, Patrick S Kamath, Douglas A Simonetto
Clinical Case A 51 year old Caucasian male was referred for evaluation of variceal bleeding. Laboratory tests were remarkable for mild thrombocytopenia and moderate alkaline phosphatase elevation. Synthetic liver function was well preserved. Abdominal computed tomography scan revealed moderate splenomegaly, gastric varices, and normal hepatic contour. A transjugular liver biopsy was performed revealing findings of nodular regenerative hyperplasia (NRH) with no significant fibrosis or necroinflammatory activity...
May 11, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29719658/occurrence-and-potential-causative-factors-of-immune-mediated-hemolytic-%C3%A2-anemia-in-cattle-and-river-buffaloes
#3
Seyedeh Missagh Jalali, Masood Ghorbanpour, Mohammad Razi Jalali, Aria Rasooli, Pegah Safaie, Farideh Norvej, Imaneh Delavari
The main objectives of this study were to determine the occurrence and potential causative factors of Immune-mediated hemolytic anemia (IMHA) in native cattle and water buffaloes from southwest of Iran. Fifty-three anemic animals (37 cattle and 16 buffaloes) were studied. A full clinical history and physical examinations were undertaken for all animals. Four clinically healthy cattle and four healthy buffaloes were also used as control animals. Blood samples were subjected to a complete blood count, Coombs' test, erythrocyte osmotic fragility test and serum biochemical analysis...
2018: Veterinary Research Forum
https://www.readbyqxmd.com/read/29702119/glomeruloid-hemangioma-associated-with-tafro-syndrome
#4
Aya Shinozaki-Ushiku, Takaaki Higashihara, Masako Ikemura, Junichiro Sato, Masaomi Nangaku, Tetsuo Ushiku, Masashi Fukayama
Glomeruloid hemangioma is a rare cutaneous lesion which has been considered as a specific cutaneous marker of POEMS syndrome. Herein, we present the first case of glomeruloid hemangioma associated with TAFRO syndrome, a unique variant of idiopathic multicentric Castleman's disease (iMCD). The patient is a 74-year-old female presented with fever, cervical lymphadenopathy, thrombocytopenia, bilateral pleural effusions and ascites. Biopsy of the lymph node revealed MCD-like histology and bone marrow biopsy showed mild reticulin fibrosis, consistent with TAFRO syndrome...
April 24, 2018: Human Pathology
https://www.readbyqxmd.com/read/29701391/diagnostic-challenges-in-chronic-constrictive-pericarditis
#5
Ana Braga, Afonso Oliveira, Kevin Domingues, Maria João Andrade, Miguel Abecacis, José Pedro Neves
INTRODUCTION: Chronic constrictive pericarditis (CCP) is a disease that has multiple possible causes and is associated with variable clinical findings, depending on its severity. It develops insidiously, and in many cases, particularly in developed countries, no antecedent diagnosis can be found. These cases are termed idiopathic. Tuberculosis is the leading cause of constrictive pericarditis in developing nations but represents only a small minority in developed countries. METHODS: Here the authors describe two different case reports where tuberculosis was the probable cause of CCP...
July 2017: Revista Portuguesa de Cirurgia Cardio-torácica e Vascular
https://www.readbyqxmd.com/read/29684500/the-clinicopathological-comparison-among-nodal-cases-of-idiopathic-multicentric-castleman-disease-with-and-without-tafro-syndrome
#6
Nozomu Kurose, Chizuru Futatsuya, Ken-Ichi Mizutani, Motona Kumagai, Akihiro Shioya, Xin Guo, Akane Aikawa, Satoko Nakada, Shino Fujimoto, Hiroshi Kawabata, Yasufumi Masaki, Kazue Takai, Sadao Aoki, Masaru Kojima, Shigeo Nakamura, Sohsuke Yamada
Multicentric Castleman disease (MCD) is a systemic inflammatory disease potentially caused by an increase in the serum interleukin-6 (IL-6) level. Idiopathic MCD (iMCD) is histopathologically classified into three types: plasmacytic (PC), mixed, and hypervascular (hyperV) types. Recently, a unique clinical phenotype with a poor prognosis overlap with iMCD, thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, and organomegaly (TAFRO syndrome), has been reported from Japan, but its detailed clinicopathological features remain unclear...
April 20, 2018: Human Pathology
https://www.readbyqxmd.com/read/29683873/eltrombopag-induced-acute-liver-failure-in-a-pediatric-patient-a-pharmacokinetic-and-pharmacogenetic-analysis
#7
M Marano, J Serafinelli, S Cairoli, D Martinelli, M Pisani, G Palumbo, M G Cefalo, C Cecchetti, M Di Nardo, F S Falvella, B M Goffredo
Eltrombopag is an oral thrombopoietin-receptor-agonist (TPO-RA) approved for the treatment of patients with chronic idiopathic thrombocytopenic purpura (ITP), who are more than one year old, and show poor response to first-line therapy. ITP is a hematological disorder characterized by isolated thrombocytopenia in the absence of secondary causes or disorders. Eltrombopag is generally well tolerated in the pediatric population, therefore therapeutic drug monitoring (TDM) is not usually performed in clinical practice...
April 20, 2018: Therapeutic Drug Monitoring
https://www.readbyqxmd.com/read/29675946/plasma-proteomics-identifies-a-chemokine-storm-in-idiopathic-multicentric-castleman-disease
#8
Sheila K Pierson, Aaron J Stonestrom, Dustin Shilling, Jason Ruth, Christopher S Nabel, Amrit Singh, Yue Ren, Katie Stone, Hongzhe Li, Frits van Rhee, David C Fajgenbaum
Human Herpesvirus-8 (HHV-8)-negative/idiopathic multicentric Castleman disease (iMCD) is a poorly understood disease involving polyclonal lymphoproliferation with dysmorphic germinal centers, constitutional symptoms, and multi-organ failure. Patients can experience thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly, and normal immunoglobulin levels, - iMCD-TAFRO. Others experience thrombocytosis, milder effusions, and hypergammaglobulinemia, -iMCD-Not Otherwise Specified (iMCD-NOS)...
April 20, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29658768/idiopathic-portal-hypertension-with-regard-to-thiopurine-treatment
#9
Salvador Machlab, Mireia Miquel, Mercedes Vergara
Idiopathic portal hypertension (IPH) is an infrequent adverse reaction to the use of thiopurines that tends to be overlooked. Herein, we present a patient with ileocolic Crohn's disease treated with azathioprine who presented ascites, esophageal varices and splenomegaly without any signs of liver cirrhosis. A portal hemodynamics study revealed a normal portosystemic gradient compatible with presinusoidal portal hypertension. Finally, IPH was diagnosed after a liver biopsy. IPH secondary to thiopurines is due to a 6-thioguanine nucleotide (6-TGN)-dependent reaction and occurs predominantly between three months and three years after the start of treatment...
April 16, 2018: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29623717/-idiopathic-thrombocytopenia-refractery-to-therapy-of-cyclosporine-a-in-clinical-practice-case-report
#10
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
#11
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/29607477/terson-syndrome-in-a-patient-with-idiopathic-thrombocytopenia
#12
O M Uhumwangho, V B Osaguona
The authors report a case of Terson syndrome in an adult with immune thrombocytopenia. Terson syndrome refers to an intraocular hemorrhage secondary to an intracranial hemorrhage or an acute rise in intracranial pressure. It is a rare but life-threatening complication and may be complicated with blindness in those who survive.
January 2018: West African Journal of Medicine
https://www.readbyqxmd.com/read/29580123/ethnic-and-racial-difference-in-helicobacter-pylori-infection-in-patients-with-immune-thrombocytopenia-treated-at-a-major-urban-medical-center
#13
Caitlin M O'Neill, Ilene C Weitz, Casey O'Connell, Howard A Liebman
Immune thrombocytopenia (ITP) is an autoimmune disorder with a complex immunopathology and pathogenesis characterized by thrombocytopenia and bleeding manifestations. The disorder is separated into primary (idiopathic) ITP and secondary ITP, when associated with other immune or lymphoproliferative disorders and certain chronic infections. Helicobacter pylori (H. pylori) is a recognized bacterial cause of ITP. In regions with high prevalence of infection, bacterial eradication has resulted in improvement in platelet count...
March 26, 2018: Platelets
https://www.readbyqxmd.com/read/29496669/next-generation-sequencing-of-idiopathic-multicentric-and-unicentric-castleman-disease-and-follicular-dendritic-cell-sarcomas
#14
Alexandra Nagy, Aparna Bhaduri, Nahid Shahmarvand, Jahanbanoo Shahryari, James L Zehnder, Roger A Warnke, Tariq Mughal, Siraj Ali, Robert S Ohgami
Castleman disease (CD) is a rare lymphoproliferative disorder subclassified as unicentric CD (UCD) or multicentric CD (MCD) based on clinical features and the distribution of enlarged lymph nodes with characteristic histopathology. MCD can be further subtyped based on human herpes virus 8 (HHV8) infection into HHV8-associated MCD, HHV8- /idiopathic MCD (iMCD), and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin change (POEMS)-associated MCD. In a subset of cases of UCD, an associated follicular dendritic cell sarcoma (FDCS) may be seen...
March 13, 2018: Blood Advances
https://www.readbyqxmd.com/read/29491289/concurrent-autoimmune-neutropenia-and-idiopathic-thrombocytopenic-purpura-associated-with-igg4-related-diease
#15
Yayoi Shimazu, Tatsuki Uchiyama, Chisaki Mizumoto, Tomoharu Takeoka, Masaaki Tsuji, Kenjiro Tomo, Koji Takaori, Naoki Sakai, Tomoko Okuno, Tatsuharu Ohno
IgG4-related disease (IgG4RD) is a multi-organ disorder characterized by an elevated serum IgG4 level and IgG4-positive plasma cell infiltration of the affected organs, accompanied by tissue fibrosis and sclerosis. Although it can affect any organ, to our knowledge, no cases involving concurrent autoimmune neutropenia and thrombocytopenia have been reported. A 62-year-old man visited our hospital and was diagnosed with IgG4RD accompanied by interstitial pneumonitis, lymphadenopathy, and interstitial nephritis...
February 28, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29487060/eltrombopag-for-use-in-children-with-immune-thrombocytopenia
#16
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/29484012/disseminated-porokeratosis-with-idiopathic-thrombocytopenia-case-report-and-literature-review-of-porokeratosis-and-related-disorders
#17
Anastasiya Atanasova Chokoeva, Uwe Wollina, Torello Lotti, Georgi Konstantinov Maximov, Ilia Lozev, Georgi Tchernev
BACKGROUND: Porokeratosis is characterised by one or more atrophic patches surrounded by a distinctive peripheral keratotic ridge, typically found on sun-exposed areas, with several clinical variants and typical histological findings. Despite ultraviolet radiation, varies antibody - related autoimmune disease treated with systemic steroids and other immunosuppressive conditions such as chronic liver disease, HIV and organ transplantations have been implicated in its etiopathology. CASE REPORT: We present a case of porokeratosis, associated with idiopathic thrombocytopenia in 74 - year old, otherwise healthy male patient, as we discuss the previously reported associated disorders...
January 25, 2018: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29430236/idiopathic-thrombocytopenia-purpura-masking-hodgkin-disease-a-paraneoplastic-syndrome-or-simply-a-mere-association
#18
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/29345096/psychiatric-sequelae-of-corticosteroid-use-in-hematology-in-australia-a-qualitative-study
#19
Dianne Clifton, Margaret Ross, Clare O'Callaghan
Despite widespread steroid usage for treating hematological conditions, minimal attention focuses on associated psychiatric side-effects. In the present study, we examined hematology patients' experiences of high-dose steroid treatment. This was undertaken by the use of a qualitative, descriptive design, which included convenience sampling and the inductive, cyclic, and constant comparative thematic analysis of interview transcripts. Eighteen patients participated, who were diagnosed with lymphoma, myeloma, leukemia, or idiopathic thrombocytopenia purpura...
January 18, 2018: Nursing & Health Sciences
https://www.readbyqxmd.com/read/29344089/bone-marrow-failure-may-be-caused-by-chromosome-anomalies-exerting-effects-on-runx1t1-gene
#20
R Valli, L Vinti, A Frattini, M Fabbri, G Montalbano, C Olivieri, A Minelli, F Locatelli, F Pasquali, E Maserati
Background: The majority of the cases of bone marrow failure syndromes/aplastic anaemias (BMFS/AA) are non-hereditary and considered idiopathic (80-85%). The peripheral blood picture is variable, with anaemia, neutropenia and/or thrombocytopenia, and the patients with idiopathic BMFS/AA may have a risk of transformation into a myelodysplastic syndrome (MDS) and/or an acute myeloid leukaemia (AML), as ascertained for all inherited BMFS. We already reported four patients with different forms of BMFS/AA with chromosome anomalies as primary etiologic event: the chromosome changes exerted an effect on specific genes, namely RUNX1, MPL, and FLI1, leading to the disease...
2018: Molecular Cytogenetics
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