Impaired platelet function and peripartum bleeding in women with Gaucher disease

Michal J. Simchen; Rotem Oz; Boris Shenkman; Ari Zimran; Deborah Elstein; Gili Kenet

doi:10.1160/TH10-07-0503

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2011; 105(03): 509-514
DOI: 10.1160/TH10-07-0503

Platelets and Blood Cells

Schattauer GmbH

Impaired platelet function and peripartum bleeding in women with Gaucher disease

Michal J. Simchen

¹Department of Obstetrics and Gynecology, Sheba Medical Center, Tel Hashomer, Israel

,

Rotem Oz

¹Department of Obstetrics and Gynecology, Sheba Medical Center, Tel Hashomer, Israel

,

Boris Shenkman

²Department of Thrombosis, Sheba Medical Center, Tel Hashomer, Israel

,

Ari Zimran

³Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel

,

Deborah Elstein

²Department of Thrombosis, Sheba Medical Center, Tel Hashomer, Israel

,

Gili Kenet

³Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel

› Author Affiliations

Abstract

Summary

The risk of bleeding during delivery may be increased in women with Gaucher disease. We aimed to evaluate potential predictors for peripartum haemorrhage (PPH) during childbirth in these patients, while focusing upon coagulation tests and platelet function assays. Women with type 1 Gaucher disease who gave birth at Sheba Medical Center between 1999–2009 comprised the study cohort. Data collected included disease history, enzyme treatment, platelet counts, delivery and pregnancy outcome. PPH was defined as excessive bleeding during or immediately following delivery. Coagulation studies and platelet function tests, including aggregometry and cone and platelet (CPA) analyses, were performed on all women. We compared women with PPH (bleeders) and non-bleeders. Furthermore, women with abnormal CPA platelet function tests were compared with those with normal CPA platelet function with regards to the risk for PPH in at least one pregnancy. Forty-five pregnancies of 20 women were studied. Six women received enzyme replacement therapy during pregnancy. Mean platelet count prior to delivery was 83,000/μl ± 35,000/μl. Fourteen out of 45 (31%) deliveries were complicated by PPH. Neither thrombocytopenia nor enzyme therapy predicted PPH. Twelve out of 13 women with PPH (92.3%) versus 2/7 non-bleeders (28.6%) had impaired platelet aggregation (less than the 3^rd percentile of normal average aggregate size values), when tested by CPA, (odds ratio [OR] 17.8, 95% confidence interval [CI] 2.5; 126.2; p=0.007). Notably, 78.6% of women with impaired CPA aggregation developed PPH during at least one delivery, as opposed to 16.7% of those with normal CPA platelet function tests (OR 11.6, 95% CI 1.7–77.7, p=0.018). In conclusion, women with type 1 Gaucher disease who have abnormal platelet function tests may have an increased risk of PPH.

Keywords

Type 1 Gaucher disease - peripartum bleeding - PPH - platelet function - Cone and platelet analyzer (CPA)

Full Text

References

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