Abstract |
Patient B.G. is a 29-yr-old female with a lifelong bleeding disorder characterized clinically by a highly increased bleeding time, menorrhagias, long-lasting bleeding after cuts and tooth extractions and large post-traumatic haematomas. Her coagulation tests were within normal range, platelet count was 140,000-160,000 per microliters, but platelet function was impaired as demonstrated by the absence of collagen-induced aggregation, although no abnormalities were detected in aggregation response to ADP and ristocetin. Morphologically her platelets were characterized by gigantic size-average profile area was about 2.5 times higher than that of control donors, and severe deficiency of alpha-granules-only 16% of their number in control donors. These features taken together indicated the diagnosis of grey platelet syndrome. As has been shown by quantitative immunoblotting, patient's platelets contained small amounts of alpha-granule membrane protein P-selectin-about 15% of that in control donors. The content of plasma membrane glycoproteins IIb-IIIa and Ib was not reduced, suggesting the specific deficiency of alpha-granule membrane protein. Thus, B.G. is the second patient described in the literature (see also Lages et al, J Clin Invest 1991: 87: 919-929) with combined deficiency of alpha-granules and P-selectin.
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Authors | A V Mazurov, D V Vinogradov, S G Khaspekova, A V Krushinsky, L V Gerdeva, S A Vasiliev |
Journal | European journal of haematology
(Eur J Haematol)
Vol. 57
Issue 1
Pg. 38-41
(Jul 1996)
ISSN: 0902-4441 [Print] England |
PMID | 8698129
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- P-Selectin
- Platelet Glycoprotein GPIIb-IIIa Complex
- Platelet Membrane Glycoproteins
- Ristocetin
- Adenosine Diphosphate
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Topics |
- Adenosine Diphosphate
(pharmacology)
- Adult
- Bleeding Time
- Blood Platelet Disorders
(blood, pathology)
- Blood Platelets
(chemistry, ultrastructure)
- Cytoplasmic Granules
(ultrastructure)
- Female
- Humans
- Microscopy, Electron
- P-Selectin
(analysis)
- Platelet Aggregation
(drug effects)
- Platelet Glycoprotein GPIIb-IIIa Complex
(analysis)
- Platelet Membrane Glycoproteins
(deficiency)
- Ristocetin
(pharmacology)
- Syndrome
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