Inherited thrombocytopenias: an approach to diagnosis and management

Int J Lab Hematol. 2013 Feb;35(1):14-25. doi: 10.1111/j.1751-553X.2012.01454.x. Epub 2012 Jul 30.

Abstract

Inherited thrombocytopenias vary in their presentation, associated features, and molecular etiologies. An accurate diagnosis is important to provide appropriate therapy as well as counseling for the individual and their family members. As the genetic basis of more disorders is understood, it will be possible to diagnose a greater fraction of patients as well as learn more about the process of megakaryopoiesis and platelet production.

Publication types

  • Review

MeSH terms

  • Blood Platelet Disorders / congenital*
  • Blood Platelet Disorders / diagnosis
  • Blood Platelet Disorders / physiopathology
  • Blood Platelet Disorders / therapy
  • Congenital Bone Marrow Failure Syndromes
  • Humans
  • Megakaryocytes / cytology
  • Megakaryocytes / metabolism
  • Megakaryocytes / pathology
  • Radius / abnormalities
  • Radius / physiopathology
  • Synostosis / diagnosis
  • Synostosis / genetics
  • Synostosis / physiopathology
  • Synostosis / therapy
  • Thrombocytopenia / congenital
  • Thrombocytopenia / diagnosis
  • Thrombocytopenia / etiology*
  • Thrombocytopenia / genetics
  • Thrombocytopenia / physiopathology
  • Thrombocytopenia / therapy
  • Thrombopoiesis
  • Ulna / abnormalities
  • Ulna / physiopathology
  • Upper Extremity Deformities, Congenital / diagnosis
  • Upper Extremity Deformities, Congenital / genetics
  • Upper Extremity Deformities, Congenital / physiopathology
  • Upper Extremity Deformities, Congenital / therapy
  • von Willebrand Disease, Type 2 / diagnosis
  • von Willebrand Disease, Type 2 / genetics
  • von Willebrand Disease, Type 2 / physiopathology
  • von Willebrand Disease, Type 2 / therapy

Supplementary concepts

  • Absent radii and thrombocytopenia
  • Congenital amegakaryocytic thrombocytopenia
  • Radioulnar Synostosis with Amegakaryocytic Thrombocytopenia