PSEUDOHEMOPHILIA OR CHRONIC THROMBASTHENIA

THE CONDITIONS under discussion are those which have been called in the literature "pseudohemophilia or chronic hereditary thrombasthenia." These two dyscrasias were originally described nearly forty years ago, but it has only been in recent years that a clear differentiation has been drawn between the two entities and their characteristics more generally understood.

Glantzmann¹ in 1918 described a new diathesis occurring in both sexes characterized by bleeding of a purpuric type with hemorrhage from the mucous membranes and beneath the skin. In the original series the bleeding time, clotting time and platelet count were all reported to be normal. The clot retraction was delayed or absent. To this condition he gave the name "chronic hereditary hemorrhagic thrombasthenia" since he considered the platelets to be qualitatively defective. Subsequently cases considered to be in this group were reported by Van der Zande,² Rosenthal,³ Little and Ayres,⁴ Roskam⁵