Inherited thrombocytopenias: Molecular mechanisms

被引：25

作者：

Balduini, CL

Savoia, A

机构：

[1] Univ Pavia, Policlin San Matteo, Clin Med 3, IRCCS, I-27100 Pavia, Italy

[2] TIGEM, Naples, Italy

来源：

SEMINARS IN THROMBOSIS AND HEMOSTASIS | 2004年 / 30卷 / 05期

关键词：

platelets; megakaryocytes; inherited thrombocytopenias; bleeding; pathogenesis;

D O I：

10.1055/s-2004-835672

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Each megakaryocyte forms 10(3) platelets and 10(11) platelets are replenished daily. The unique and amazing mechanisms that allow megakaryocytes to become giant and polyploid and to release such a large number of platelets are still poorly understood. The study of inherited thrombocytopenias offers the possibility to gain new information on these processes because several different forms, deriving from defective megakaryocytic commitment, differentiation, maturation, or platelet formation, have been identified. Moreover, in the presence of some genetic defects, megakaryocytes produce platelets with a shortened life span. In this review, we summarize what we have learned about inherited thrombocytopenias in the last few years.

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收藏

页码：513 / 523

页数：11

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