Congenital and acquired platelet disorders: current dilemmas and treatment strategies.
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.
White
Gilbert C
GC
Blood Research Institute, Milwaukee, WI 53226, USA. gcwhite@bcsew.edu
D001327:Autoimmune Diseases / Q000209:etiology / Q000378:metabolism; D001606:Bernard-Soulier Syndrome / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D001792:Blood Platelets / Q000378:metabolism / Q000473:pathology; D005167:Factor VII / Q000627:therapeutic use*; D015942:Factor VIIa; D006487:Hemostasis; D006801:Humans; D017713:Platelet Transfusion / Q000009:adverse effects; D011994:Recombinant Proteins / Q000627:therapeutic use; D015398:Signal Transduction; D013915:Thrombasthenia / Q000188:drug therapy* / Q000378:metabolism
D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa