Faculty Collaboration Database

Congenital and acquired platelet disorders: current dilemmas and treatment strategies. Semin Hematol 2006 Jan;43(1 Suppl 1):S37-41

Date

01/24/2006

Pubmed ID

16427384

DOI

10.1053/j.seminhematol.2005.11.011

Scopus ID

2-s2.0-31444437760 (requires institutional sign-in at Scopus site)   25 Citations

Abstract

Platelets are important for primary hemostasis. When a blood vessel is damaged, platelets adhere to exposed subendothelial connective tissue and form a hemostatic plug. Formation of the plug is contingent upon a series of processes, with adhesion, activation, and aggregation all being involved. Patients with quantitative platelet disorders have reduced numbers of platelets. Patients with qualitative disorders have platelets that exhibit abnormal functioning. Defects that impair function can affect platelet receptors, secretory responses, or intracellular signaling pathways. Examples of qualitative platelet disorders include Glanzmann's thrombasthenia (GT) and Bernard-Soulier syndrome (BSS). The treatment of platelet disorders is primarily with platelet concentrates. However, in patients with abnormalities of their platelet surface receptors, platelet transfusion may provoke an immune response. Recombinant factor VIIa (rFVIIa) may provide hemostatically effective therapy in such patients.

Author List

White GC 2nd

Author

Gilbert C. White MD Professor in the Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Autoimmune Diseases
Bernard-Soulier Syndrome
Blood Platelets
Factor VII
Factor VIIa
Hemostasis
Humans
Platelet Transfusion
Recombinant Proteins
Signal Transduction
Thrombasthenia