PT  - JOURNAL ARTICLE
AU  - Khalil A. Aziz
TI  - An acquired form of Bernard Soulier syndrome associated with acute myeloid leukemia
DP  - 2005 Jul 01
TA  - Saudi Medical Journal
PG  - 1095--1098
VI  - 26
IP  - 7
4099  - http://smj.org.sa/content/26/7/1095.short
4100  - http://smj.org.sa/content/26/7/1095.full
SO  - Saudi Med J2005 Jul 01; 26
AB  - 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.