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Saudi Med J. 2005 Jul;26(7):1095-8.

An acquired form of Bernard Soulier syndrome associated with acute myeloid leukemia.

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1
Regional Department of Immunology, Birmingham Heartlands Hospital, Bordesley Green East, Birmingham B9 5SS, United Kingdom. khalilazizh@yahoo.co.uk

Abstract

OBJECTIVE:

To investigate glycoprotein-1b (GP-1b) expression on platelets from patients with acute myeloid leukemia (AML).

METHODS:

Purified platelets, obtained from AML-patients and normal control subjects, were examined for surface membrane GP1b-expression by flow cytometry and GP1b-mediated aggregation responses by aggregometry. The level of elastase in plasma from patients and controls was measured by enzymed-linked immunosorbent assay. The whole of this work was carried out at the University of Liverpool, Liverpool, United Kingdom during the period of 1994-2001.

RESULTS:

Platelets from the majority of AML-patients showed reduced GP1b-expression and reduced GP1b-mediated aggregation responses. Reduction in platelet GP1b-expression was associated with increased plasma elastase levels.

CONCLUSION:

The present study suggests that elastase, released from leukemic blasts, degrades platelet GP1b, resulting in dysfunctional circulating platelets in AML-patients. These results could explain the bleeding disorders observed in these patients.

PMID:
16047060
[Indexed for MEDLINE]
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