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Thrombocytopenia and bleeding in dental procedures of patients with Gaucher disease.

Givol N, Goldstein G, Peleg O, Shenkman B, Zimran A, Elstein D, Kenet G

Departments of Oral and Maxilo-Facial Surgery, Sheba Medical Center, Tel Hashomer, Israel Pediatric Hematology Institute, Safra Children's Hospital, Sheba Medical Center, Tel Hashomer Thrombosis Unit, National Hemophilia Center, Sheba Medical Center, Tel Hashomer, Israel; Sackler Medical School, Tel Aviv University, Tel Hashomer, Israel Gaucher Clinic, Shaare Zedek Medical Center, Jerusalem, Israel.

Summary.  The risk of bleeding during dental procedures may be increased in patients with Gaucher disease. We aimed to evaluate potential coagulation and platelet function abnormalities and targeted therapy accordingly. Patients with type 1 Gaucher disease who were treated at the Oral and Maxilo-Facial surgery clinic at Sheba Medical Center between 2003 and 2010 comprised the study cohort. Data collected included disease history, enzyme treatment, platelet counts, dental therapy and outcome. Bleeding was defined as excessive bleeding during or immediately following procedure. Coagulation studies and platelet function tests including aggregometry were performed on all patients. Dental procedures (n = 14, including eight teeth extractions, two crown lengthening procedures, one cyst enucleation and three deep dental scaling) of seven patients were studied. Mean platelet count prior to procedure was 73 K ± 14.8 mm(3) . Patients bleeding risk score was calculated according to previous history of bleeding tendency, degree of thrombocytopenia, presence of comorbid coagulopathy and the type of dental procedure. Two patients with highest risk score received prophylactic platelet transfusions, three patients (medium-risk) received DDAVP preprocedure and all received systemic tranexamic acid, which was the only systemic therapy for low-risk patients. Meticulous surgical local haemostasis was applied. No excessive intra-operative or postoperative bleeding occurred. Patients with Gaucher disease who have thrombocytopenia and abnormal platelet function tests may be safely treated if meticulous haemostasis is applied along with systemic therapy as required. Platelet transfusions are not mandatory and should be applied considering the procedure-related risk and the patient's calculated haematological risk for bleeding.

Published 6 May 2011 in Haemophilia.
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