J Korean Pediatr Soc.  1981 Jan;24(1):88-93.

A case of Thrombasthenia

• Cho SS
• Kim KT
• Ahn DH
• Sohn KC

Abstract

A 6 year 6month-old boy was admitted with complains of severe spontaneou epistaxis and easy brusability. He was one of five siblings, and two of them died of recurred severe epistaxis at their age of six years. There was no history of bleeding tendency to his antecedents and parents, whose marriage was unrelated. Platelet count and partial thromboplastin time were noraml with prolonged bleeding time, but clot retraction was poor. Platelet adhesion(Salzman method) was decreased and platelet aggregation test showed flat curve with no response to epinephrine and ADP. These tests of other were normal. Diagnosis of thrombasthenia was made with the above history and laboratory results.

Keyword

Thromabasthenia; Clot retraction test; Platelet aggregation test

MeSH Terms

Adenosine Diphosphate
Bleeding Time
Blood Platelets
Clot Retraction
Diagnosis
Epinephrine
Epistaxis
Hemorrhage
Humans
Male
Marriage
Parents
Partial Thromboplastin Time
Platelet Aggregation
Platelet Count
Siblings
Thrombasthenia*
Adenosine Diphosphate
Epinephrine