منابع مشابه
Inherited thrombocytopenias.
Bleeding syndromes that arise through an inherited defect of platelet production constitute a heterogeneous group of rare platelet disorders of growing importance. Some, including the Bernard-Soulier syndrome (BSS) and Wiskott-Aldrich syndrome (WAS), associate a low circulating platelet count with a deficiency in a known functional protein (Table 1). In others, platelet dysfunction has not been...
متن کاملInherited thrombocytopenias.
Secondary causes of thrombocytopenia as immunologic thrombopenia purpura, or ITP, are far more common than inherited causes, which even as a group, are rare. Nevertheless, diagnosis is important and progress made in uncovering the molecular basis of these disorders has contributed greatly to our knowledge of these diseases. Inherited thrombocytopenias are a heterogeneous group of disorders. Dif...
متن کاملMolecular basis of inherited thrombocytopenias.
Inherited thrombocytopenias (IT) are a heterogeneous group of diseases caused by at least 20 different genes. At present, these genes account for approximately 50% of cases, suggesting that novel genes have yet to be identified for a comprehensive understanding of platelet biogenesis defects. This review provides an update of ITs focusing on the molecular basis and potential pathogenic mechanis...
متن کاملSurvival of Radiochromium-labeled Platelets in Thrombocytopenias.
V ARIOUS iSOTOPIC methods have been used to label platelets in an effort to clarify the pathogenesis of the thrombocytopenias.10’42’47 The suitability of radiochromium as a platelet label has been well established3 , ,44,4M, o The present study represents an attempt to correlate radiochromate platelet survival with the clinical picture in thrombocytopenic patients, as well as with the effects o...
متن کاملInherited thrombocytopenias: from genes to therapy.
BACKGROUND AND OBJECTIVES Inherited thrombocytopenias are a heterogeneous group of rare diseases characterized by a reduced number of blood platelets. Some of these diseases are exclusive to megakaryocytes and platelets, while in others the pathology extends to other cell types. Although the defective genes, coding for membrane glyoproteins, cytoskeleton components and intracellular signaling p...
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ژورنال
عنوان ژورنال: Terapevticheskii arkhiv
سال: 2018
ISSN: 2309-5342,0040-3660
DOI: 10.26442/terarkh20189074-13