Inherited platelet disorders: A clinical approach to diagnosis and management

Kelly Cox, Victoria Price, Walter HA Kahr

Producción científica: Contribución a una revistaArtículo de revisiónrevisión exhaustiva

27 Citas (Scopus)

Resumen

Inherited platelet disorders encompass a heterogeneous group of bleeding disorders where a variety of molecular defects can affect platelet number, function or both. The defects involve deficiencies or dysfunction of platelet receptors, signaling pathways, cytoskeletal proteins, granule contents and abnormalities in procoagulant activity. These disorders can be difficult to distinguish clinically as they present with the common symptom of mucocutaneous bleeding. Inherited thrombocytopenia needs to be considered in all patients suspected of having primary immune thrombocytopenia, where platelets may also have functional defects. After a careful history and physical examination, initial investigations include a complete blood count with a peripheral smear, followed by appropriate specific investigations that often require specialized referral centers. This article is a summary of the current data on clinical presentation, pathogenesis, diagnosis and management of inherited platelet disorders.

Idioma originalEnglish
Páginas (desde-hasta)455-472
Número de páginas18
PublicaciónExpert Review of Hematology
Volumen4
N.º4
DOI
EstadoPublished - ago. 2011

ASJC Scopus Subject Areas

  • Hematology

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