Hereditary thrombocytopathies with abnormal release reaction (author's transl).

Abstract

The authors have examined 8 cases with Hermansky-Pudlak syndrome in whom besides the usual abnormalities of abnormal aggregation with collagen, absence of second wave of aggregation, reduction of the 5-HT uptake, presence of 5-HIAA in the platelets, two new abnormalities are described: the presence of a large amount of an unidentified metabolite after 5-HT incorporation which differs from 5-HT and 5 hydroxytryptophol and an abnormal incorporation of labelled glycerol in the triglycerides. Correlation between abnormal lipid metabolism and defective 5-HT incorporation is discussed.

• Authors: F Hermansky, P Cieslar
• Journal: Nouvelle revue francaise d'hematologie (Nouv Rev Fr Hematol (1978)) Vol. 16 Issue 3 Pg. 413-20 ( 1976) Germany
• Vernacular Title: Thrombopathies héréditaires par trouble de libération
• PMID: 1012917 (Publication Type: English Abstract, Journal Article)

Chemical References

• Triglycerides
• Ristocetin
• Serotonin
• Adenosine Diphosphate
• Collagen
• Glycerol
• Epinephrine

Topics

• Adenosine Diphosphate (pharmacology)
• Albinism (genetics)
• Bone Marrow (metabolism)
• Bone Marrow Cells
• Clot Retraction
• Collagen (pharmacology)
• Cytoplasmic Granules
• Epinephrine (pharmacology)
• Glycerol (metabolism)
• Hemorrhagic Disorders (genetics)
• Humans
• Macrophages (metabolism)
• Platelet Aggregation
• Ristocetin (pharmacology)
• Serotonin (metabolism)
• Syndrome
• Triglycerides (metabolism)