Haemorrhagic Thrombocytopathy Associated with Dilatation of the Platelet—Membrane Complex

David Green*, Chung‐hsin ‐h Ts'ao, Isaac Cohen, Ennio C. Rossi

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

13 Scopus citations

Abstract

We evaluated eight patients from four families because of a history of excessive bleeding. Most patients had prolonged bleeding times, absent secondary wave of platelet aggregation in response to epinephrine, collagen and adenosine diphosphate (ADP), and defective 14C‐serotonin release and platelet factor 3 availability. These findings are characteristic of a platelet release defect. Electron‐microscopic examination of the platelets of seven patients revealed a common abnormality. From 30% to 70% of the platelets in any given sample exhibited a prominent membrane complex and dilated, tortuous surface‐connected canalicular system (‘swiss‐cheese’ platelet). In two patients there was coincident storage‐pool disease, but the remainder had adequate dense bodies and a normal ratio of ATP to ADP. SDS‐polyacrylamide gel electrophoresis of platelet proteins and glycoproteins showed no abnormalities. The patency of the canalicular system was demonstrated in one patient by the observation that dense bodies appeared in the cannaliculi and outside the platelets following collageninduced aggregation of polylysine‐treated platelets. Since platelet‐aggregation responses to the calcium ionophore A23187 were normal, we conclude that the defective platelet function in these patients may be due to impaired calcium mobilization from the morphologically abnormal membrane complex.

Original languageEnglish (US)
Pages (from-to)595-600
Number of pages6
JournalBritish Journal of Haematology
Volume48
Issue number4
DOIs
StatePublished - Aug 1981

ASJC Scopus subject areas

  • Hematology

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