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Investigations in a Family with Thrombasthenia of Moderately Severe Type with 16 Affected Members
Author(s) -
Cronberg Stig,
Nilsson Inga Marie
Publication year - 1968
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1968.tb01713.x
Subject(s) - thrombasthenia , platelet , bleeding time , medicine , coagulation , clot retraction , hemorrhagic diathesis , blood platelet disorders , platelet aggregation , gastroenterology , pathology
A large family with a congenital bleeding disorder in 16 of its members was investigated. The disease was dominantly inherited, and the symptoms were ready bruisability, tendency to nose bleeding and bleeding after tooth extraction and operation. The Ivy bleeding time was always, and the Duke bleeding time occasionally, prolonged. The platelet adhesiveness as tested in Hellem's whole blood method was decreased and mostly also in Hellem's plasma‐ADP method and Salzman's method. Spontaneous aggregation in platelet‐rich citrated plasma was absent even when the plasma was placed on a glass slide. After suspension in 3.8 per cent citrate solution the platelets showed an abnormal swelling. The prothrombin consumption test was occasionally pathologic. Factor VIII and other coagulation factors were normal. Moderately severe thrombasthenia is suggested as an appropriate name for the disease.