Deficiency of ADAMTS13 in thrombotic thrombocytopenic purpura
International Journal of Hematology, 10/12/2009
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Tsai H–M – Mutations of the gene are detected in patients with the congenital form of thrombotic thrombocytopenic purpura. In the circulation, proteolysis of von Willebrand factor is critical in regulating vWF–platelet ... basis for a rational approach to improving the diagnosis and treatment of thrombotic thrombocytopenic purpura.
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Refractory chronic immune thrombocytopenic purpura in a child with acute lymphoblastic leukemia
International Journal of Hematology, 10/13/2009
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Horino S et al. – Immune thrombocytopenic purpura (ITP) has been associated with several hematologic malignancies such as Hodgkin and non–Hodgkin lymphomas and chronic lymphocytic leukemia ... lymphoblastic leukemia (ALL). This is the first child case of chronic ITP during chemotherapy for ALL and splenectomy was effective in this patient.
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Immune thrombocytopenia associated with Mycoplasma pneumoniae infection: a case report and review of literature
Blood Coagulation & Fibrinolysis, 10/07/2009
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immune thrombocytopenic purpura during an acute mycoplasma infection. Immune thrombocytopenic purpura should be considered early in patients with thrombocytopenia and mycoplasma infections, with the institution of usual therapy for immune thrombocytopenic purpura.
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Mutation of the H-bond acceptor S119 in the ADAMTS13 metalloprotease domain reduces secretion and substrate turnover in a patient with congenital thrombotic thrombocytopenic purpura
Blood, 10/02/2009
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Feys HB et al. – Hereditary thrombotic thrombocytopenic purpura is caused by mutations in ADAMTS13 resulting in defective processing of von Willebrand factor (VWF) which causes ... the functional and structural role of a recently identified ADAMTS13 metalloprotease domain mutation S119F was investigated. The S119–W262 H–bond is crucial for maximal turnover.
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Romiplostim in chronic immune thrombocytopenic purpura
Clinical Therapeutics, 10/23/2009
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Cersosimo RJ – Based on the findings from this review, romiplostim administration has been associated with a durable platelet response in these patients with refractory chronic ITP. Romiplostim has been found to be generally well ... clinical pharmacology,coagulation/bleeding dz,pharmacology/therapy,other drugs
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Celiac disease, Behçet, and idiopathic thrombocytopenic purpura in siblings of a patient with multiple sclerosis
Multiple Sclerosis, 10/26/2009
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Yamout B et al. – Multiple sclerosis (MS) is a demyelinating disease of uncertain etiology. Many genetic and environmental risk factors have been associated with this disease ... D deficiency. The authors report a 30–year–old woman with MS, the product of consanguineous marriage, and three siblings with three different autoimmune diseases: idiopathic thrombocytopenic purpura, celiac disease, and Behçet’s disease.
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Idiopathic thrombocytopenic purpura: Pathophysiology and management
International Journal of Hematology, 10/12/2009
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Ahn YS et al. – The management of ITP should include the search for and elimination of underlying causes and careful evaluation of hemostasis. Therapy is divided into definitive vs symptomatic measures. The former including ... lasting remission after therapy was stopped, while the later including glucocorticoids, gammaglobuin, antiD antibodies and others increases platelet counts but seldom sustains remission upon withdrawal.
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Medical treatments for idiopathic thrombocytopenic purpura during pregnancy
Cochrane Reviews, 10/27/2009
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Marti–Carvajal AJ et al. – Current evidence from one randomised controlled trial indicates that betamethasone does not reduce the risk of neonatal thrombocytopenia and neonatal bleeding in ITP during pregnancy ... clinical and pregnancy outcomes reported. The researchers used no treatment in the control group, which may have increased the risk of performance bias in the trial.
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High dose dexamethasone regulates interleukin-18 and interleukin-18 binding protein in idiopathic thrombocytopenic purpura
Haematologica, 10/07/2009
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Shan N et al. – In vitro, DXM had a significant effect on secretion of IL–18BP while diminishing IL–18 release from cultures of PBMCs. These results suggest ... ITP.
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Solar capillaritis as a cause of solar purpura
Clinical and Experimental Dermatology, 10/08/2009
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Waters A et al. – The authors report a 51–year–old woman who repeatedly developed an asymptomatic petechial eruption on her legs after strong sun exposure. Investigation found an action spectrum within the ultraviolet A waveband, and ... lesion found features of capillaritis.
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