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Idiopathic thrombocytopenia

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https://www.readbyqxmd.com/read/27928110/stable-liver-function-during-long-term-administration-of-eltrombopag-a-thrombopoietin-receptor-agonist-in-patients-with-chronic-liver-disease
#1
Tomohiro Kurokawa, Soichiro Murata, Nobuhiro Ohkohchi
Liver cirrhosis is the end stage of chronic liver disease, but no definitive pharmacological treatment is currently available. It has been reported that thrombopoietin (TPO) promotes liver regeneration and improves liver cirrhosis by increasing platelet count. We have shown the direct effect of platelet transfusion on the improvement of liver function in patients with chronic liver disease. However, platelet transfusion often causes adverse events, such as platelet transfusion refractoriness and pruritus. Therefore, we conducted an exploratory clinical trial and administered eltrombopag, an orally bioavailable, small-molecule, non-peptide TPO receptor agonist that has been approved for the treatment of chronic idiopathic thrombocytopenic purpura...
2016: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/27927231/identification-of-a-potent-small-molecule-capable-of-regulating-polyploidization-megakaryocyte-maturation-and-platelet-production
#2
Nick Huang, Mabel Lou, Hua Liu, Cecilia Avila, Yupo Ma
BACKGROUND: Megakaryocytic cell maturation involves polyploidization, and megakaryocyte (MK) ploidy correlates with their maturation and platelet production. Retardation of MK maturation is closely associated with poor MK engraftment after cord blood transplantation and neonatal thrombocytopenia. Despite the high prevalence of thrombocytopenia in a range of setting that affect infants to adults, there are still very limited modalities of treatment. METHODS: Human CD34(+) cells were isolated from cord blood or bone marrow samples acquired from consenting patients...
December 8, 2016: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#3
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27902998/autoimmune-cytopenias-diagnosis-management
#4
Christian P Nixon, Joseph D Sweeney
The autoimmune cytopenias are a related group of disorders in which differentiated hematopoietic cells are destroyed by the immune system. Single lineage disease is characterized by the production of autoantibodies against red cells (autoimmune hemolytic anemia [AIHA]), platelets (autoimmune thrombocytopenia [ITP]) and neutrophils (autoimmune neutropenia [AIN]) whereas multilineage disease may include various combinations of these conditions. Central to the genesis of this disease is the breakdown of central and/or peripheral tolerance, and the subsequent production of autoantibodies by both tissue and circulating self-reactive B lymphocytes with support from T helper lymphocytes...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27900999/evaluating-anti-smd1-amino-acid-83-119-peptide-reactivity-in-children-with-systemic-lupus-erythematosus-and-other-immunological-diseases
#5
Hai-Ou Yang, Xiao-Qing Zhang, Qi-Hua Fu
BACKGROUND: SmD1-amino-acid 83-119 peptide (SmD183-119) is the major epitope of Smith (Sm) antigen, which is specific for adult systemic lupus erythematosus (SLE). The anti-SmD183-119 antibody has exhibited higher sensitivity and specificity than anti-Sm antibody in diagnosing adult SLE. However, the utility of anti-SmD183-119antibodies remains unclear in children with SLE (cSLE). This study aimed to assess the characteristics of anti-SmD183-119antibody in the diagnosis of cSLE. METHODS: Samples from 242 children with different rheumatological and immunological disorders, including autoimmune diseases (SLE [n = 46] and ankylosing spondylitis [AS, n = 11]), nonautoimmune diseases (Henoch-Schonlein purpura [HSP, n = 60], idiopathic thrombocytopenia purpura [n = 27], hematuria [n = 59], and arthralgia [n = 39]) were collected from Shanghai Children's Medical Center from March 6, 2012 to February 27, 2014...
2016: Chinese Medical Journal
https://www.readbyqxmd.com/read/27898556/treatment-options-in-immune-thrombocytopenia
#6
Laura Dubis, Miranda Collins
Immune thrombocytopenia (ITP) is an autoimmune condition that can cause life-threatening bleeding. ITP may develop idiopathically, after an infection or administration of a medication, or as a comorbid condition. Treatment depends on whether life-threatening, active bleeding is occurring. Although corticosteroids remain a standard of care, new medications offer patients an alternative to splenectomy when other pharmacologic treatments fail.
December 2016: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/27876239/case-reports-of-idiopathic-thrombocytopenia-unresponsive-to-first-line-therapies-treated-with-traditional-herbal-medicines-based-on-syndrome-differentiation
#7
Juno Yang, Beom-Joon Lee, Jun-Hwan Lee
The objective of our study is to present two cases showing the effects of traditional Korean herbal medicines based on traditional Korean medicine (TKM) for the treatment of immune thrombocytopenic purpura (ITP). One patient showed no response to treatment with steroids and an immunosuppressive agent. Moreover, liver toxicity and side effects of steroids were evident. However, after he ceased conventional treatment and started to take an herbal medicine, his liver function normalized and the steroid side effects resolved...
October 21, 2016: Explore: the Journal of Science and Healing
https://www.readbyqxmd.com/read/27861740/itp-from-idiopathic-purpura-to-immune-thrombocytopenia-and-back
#8
EDITORIAL
Jesper Stentoft
No abstract text is available yet for this article.
November 11, 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27819553/pt-vwd-posing-diagnostic-and-therapeutic-challenges-small-case-series
#9
Analía Sánchez-Luceros, Adriana I Woods, Emilse Bermejo, Shilpa Shukla, Suchitra Acharya, Michelle Lavin, Natalia Rydz, Maha Othman
Despite the increased worldwide awareness, over the last decade, of the platelet-type von Willebrand Disease (PT-VWD), many uncertainties remain around this rare platelet bleeding disorder. This report aims to correctly identify and study the phenotype of new patients and highlights the diagnostic and therapeutic challenges this disease remains to pose. We describe four PT-VWD cases confirmed by genetic analysis in which either the diagnosis and/or the treatment posed challenge. We provide the details of the clinical presentation, laboratory analysis, and the treatment and the responses in each case...
November 7, 2016: Platelets
https://www.readbyqxmd.com/read/27696756/anti-il6-r-tocilizumab-for-severe-juvenile-idiopathic-arthritis-associated-uveitis-refractory-to-anti-tnf-therapy-a-multicenter-study-of-25-patients
#10
Vanesa Calvo-Río, Montserrat Santos-Gómez, Inmaculada Calvo, Ma Isabel González-Fernández, Berta López Montesinos, Marina Mesquida, Alfredo Adán, María Victoria Hernández, Olga Maíz, Antonio Atanes, Beatriz Bravo, Consuelo Modesto, Gisela Díaz-Cordovés, Natalia Palmou-Fontana, Javier Loricera, M C González-Vela, Rosalía Demetrio-Pablo, J L Hernández, Miguel A González-Gay, Ricardo Blanco
OBJECTIVE: To assess the efficacy of Tocilizumab (TCZ) for Juvenile Idiopathic Arthritis (JIA)-associated uveitis. METHODS: Multicenter study of patients with JIA-associated uveitis refractory to conventional immunosuppressive drugs and anti-TNFα agents. RESULTS: We assessed 25 (21 women) patients (47 affected eyes); mean age 18.5±8.3 years. Uveitis was bilateral in 22. Cystoid macular edema (CME) was present in 9 patients. Ocular sequelae found at TCZ onset included: cataracts (13), glaucoma (7), synechiae (10), band keratopathy (12), maculopathy (9), and amblyopia (5)...
October 1, 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/27663147/sweet-syndrome-long-term-follow-up-of-138-patients
#11
J Marcoval, C Martín-Callizo, F Valentí-Medina, M Bonfill-Ortí, L Martínez-Molina
BACKGROUND: Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy-associated SS have been published in recent years. AIM: To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long-term follow-up of patients with SS. METHODS: Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed...
October 2016: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/27651973/primary-splenic-angiosarcoma-presenting-as-idiopathic-thrombocytopenic-purpura-a-case-report-and-review-of-the-literature
#12
S Christopher N Frontario, Anna Goldenberg-Sandau, Darshan Roy, Roy Sandau
Angiosarcoma of the spleen is a rare malignancy that arises from vascular endothelial origin. This neoplasm is highly malignant and diagnosis is often delayed due to the vague presentation of clinical symptoms. A case report and concise review of the current diagnostic criteria and surgical treatment are provided to aid in the detection and treatment of this malignancy. We present a case of a 56-year-old female who presented with massive splenomegaly secondary to angiosarcoma of the spleen. The patient suffered from longstanding symptomatic anemia and thrombocytopenia...
2016: Case Reports in Surgery
https://www.readbyqxmd.com/read/27622772/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome
#13
D C Moreira, C J Ng, R Quinones, X Liang, D W Chung, J Di Paola
: Essentials Idiopathic systemic capillary leak syndrome (SCLS) is characterized by episodes of vascular leakage. We present the case of a patient with SCLS who developed microangiopathic hemolytic anemia (MAHA). We propose that this anemia is the result of ADAMTS-13 loss in the third-space fluid. This suggests that MAHA can occur in patients with significant extravasation of proteins. SUMMARY: Idiopathic systemic capillary leak syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema...
September 13, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27597907/simultaneous-manifestation-of-chronic-myelomonocytic-leukemia-and-multiple-myeloma-during-treatment-by-prednisolone-and-eltrombopag-for-immune-mediated-thrombocytopenic-purpura
#14
Masao Hagihara, Morihiro Inoue, Kenichiro Kodama, Tomoyuki Uchida, Jian Hua
An 80-year-old man was admitted to our hospital because of severe thrombocytopenia. He was diagnosed with idiopathic thrombocytopenia, and prednisolone together with eltrombopag was started, leading to significant improvement of platelet counts. Four years later, there was a prominent increase of peripheral blood monocytes, which was accompanied by recurrence of thrombocytopenia. Bone marrow aspirates and serum electrophoresis revealed coexistence of chronic myelomonocytic leukemia (CMML) and multiple myeloma (MM)...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27514869/idiopathic-thrombotic-thrombocytopenic-purpura-strongest-risk-factor-for-relapse-from-remission-is-having-had-a-relapse
#15
Jennifer Mai Falk, Inge Scharrer
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare, episodic clinical syndrome involving the production of thrombi in the microvasculature accompanied by thrombocytopenia and symptoms of organ ischemia. Idiopathic TTP develops when a patient produces antibodies that react with the protease ADAMTS13. The course after an episode is unpredictable; patients may relapse frequently or never. There is no laboratory value that can reliably predict potential relapse. STUDY DESIGN AND METHODS: To assess diagnostic and predictive values for risk of relapse, plasma samples from 27 patients with idiopathic TTP in remission were analyzed for anti-ADAMTS13 immunoglobulin (Ig)G, ADAMTS13 activity, and ADAMTS13 inhibitor titer...
August 11, 2016: Transfusion
https://www.readbyqxmd.com/read/27498733/primary-immune-thrombocytopenia-accompanied-by-pituitary-apoplexy
#16
Takahiro Tsuji, Hiromi Mochinaga, Hiroshi Yamasaki, Hiroyuki Tsuda
An 83-year-old woman was admitted to our hospital with a severe headache and purpura. She had previously been diagnosed with idiopathic thrombocytopenia purpura (ITP) and achieved complete remission with steroid therapy. Steroid therapy had been completed one week prior to the current admission. The recurrence of severe thrombocytopenia (<1.0×10(4) platelets/μl) was detected and a CT scan revealed pituitary hemorrhage without pituitary adenoma. She received steroid therapy combined with intravenous immunoglobulin, which resulted in the amelioration of ITP and improvements in the pituitary hemorrhage...
July 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27498731/successful-rituximab-treatment-in-an-elderly-patient-with-recurrent-thrombotic-thrombocytopenic-purpura
#17
Etsuko Matsubara, Jun Yamanouchi, Takaaki Hato, Kazuto Takeuchi, Toshiyuki Niiya, Masaki Yasukawa
An 81-year-old man presenting with fever, neurological symptoms, thrombocytopenia, and hemolytic anemia was diagnosed with acquired idiopathic thrombotic thrombocytopenic purpura (TTP). His disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) activity was <1% and the ADAMTS13 inhibitor titer was 3.2 BU/ml. He received plasma exchange and steroid administration until remission was achieved. Seven months later, he suffered from paralysis of the right hand, hemolytic anemia, and thrombocytopenia...
July 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27407277/macrophage-activation-syndrome-in-a-patient-with-systemic-onset-of-the-juvenile-idiopathic-arthritis
#18
Deepak Jain, Hari K Aggarwal, Avinash Rao, Anshul Mittal, Promil Jain
Systemic onset juvenile idiopathic arthritis (sJIA) is defined as arthritis affecting one or more joint usually in the juvenile age group (< 16 years of age) with or preceded by fever of at least 2 weeks duration that is documented to be daily ("quotidian") for at least 3 days which may be associated with evanescent (non-fixed) erythematous rash or generalized lymph node enlargement or hepatomegaly/splenomegaly/both or serositis. Macrophage activation syndrome (MAS) is a life-threatening complication of sJIA marked by sudden onset of non-remitting high fever, profound depression in all three blood cell lines (i...
2016: Reumatologia
https://www.readbyqxmd.com/read/27343486/unexpected-platelets-elevation-in-a-patient-with-idiopathic-thrombocytopenia-treated-with-oseltamivir-for-influenza-infection
#19
Pierre Bigot, Marine Auffret, Sophie Gautier, Marie Weinborn, Nicolas-Kader Ettahar, Patrick Coupé
Oseltamivir is a neuraminidase inhibitor approved for the prevention and treatment of influenza. Few haematological side effects have been reported with oseltamivir. We report herein the case of an unexpected platelet increase in a 46-year-old woman with idiopathic thrombocytopenia (ITP) treated with oseltamivir for influenza. The mechanism may involve the neuraminidase inhibition which decrease platelet surface sialic acid content and reduce their removal by the reticuloendothelial system. Oseltamivir may be responsible for platelet increase especially in patients with ITP...
October 2016: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/27282765/platelets-are-versatile-cells-new-discoveries-in-hemostasis-thrombosis-immune-responses-tumor-metastasis-and-beyond
#20
Xiaohong Ruby Xu, Dan Zhang, Brigitta Elaine Oswald, Naadiya Carrim, Xiaozhong Wang, Yan Hou, Qing Zhang, Christopher Lavalle, Thomas McKeown, Alexandra H Marshall, Heyu Ni
Platelets are small anucleate blood cells generated from megakaryocytes in the bone marrow and cleared in the reticuloendothelial system. At the site of vascular injury, platelet adhesion, activation and aggregation constitute the first wave of hemostasis. Blood coagulation, which is initiated by the intrinsic or extrinsic coagulation cascades, is the second wave of hemostasis. Activated platelets can also provide negatively-charged surfaces that harbor coagulation factors and markedly potentiate cell-based thrombin generation...
December 2016: Critical Reviews in Clinical Laboratory Sciences
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