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Increased operative bleeding during orthopaedic surgery in patients with type I Gaucher disease and bone involvement
1Pediatric Orthopaedic Surgery Unit, Schneider Children's Medical Center of Israel, Petah Tiqva, Israel.
Insights
Patients with type I Gaucher disease, particularly those not splenectomized, show high rates of coagulation factor deficiencies and bleeding risk. Preoperative factor assessment and replacement therapy are recommended to prevent excessive bleeding during surgery.
Area of Science:
- Hematology
- Genetics
- Orthopedics
Background:
- Type I Gaucher disease is a genetic disorder associated with bleeding risks.
- Ashkenazi Jewish patients are a significant demographic for Gaucher disease.
- Orthopedic surgery presents unique challenges for patients with bleeding disorders.
Purpose of the Study:
- To identify coagulation abnormalities in type I Gaucher disease patients.
- To assess bleeding risk in Gaucher disease patients undergoing orthopedic surgery.
- To inform preoperative management strategies for preventing surgical bleeding.
Main Methods:
- Retrospective review of coagulation parameters in 28 type I Gaucher disease patients.
- Analysis of patients who underwent orthopedic surgery and those under treatment, including splenectomized and non-splenectomized individuals.
- Documentation of specific clotting factor deficiencies and prothrombin time.
Main Results:
- 81% of treated patients (non-splenectomized) exhibited prolonged prothrombin time.
- Significant deficiencies noted in Factor XI (36.3%), V (31.8%), VIII (27.2%), IX (13.6%), and XII (27.2%).
- Two orthopedic surgery patients experienced excessive bleeding, one with prolonged prothrombin time and another with Factor XI deficiency.
Conclusions:
- Type I Gaucher disease patients, especially non-splenectomized, have a high prevalence of coagulation factor deficiencies.
- Preoperative evaluation of clotting factors is crucial for patients with type I Gaucher disease.
- Replacement therapy may effectively prevent excessive bleeding in this patient population.
Abstract:
To aid clinicians in identifying patients with type I Gaucher disease who are at risk of excessive bleeding, we reviewed the coagulation parameters of six affected patients with bone involvement who underwent orthopaedic surgery at two centers, and of 22 patients under treatment at another, seven of whom had total splenectomy. All patients were of Jewish Ashkenazi origin. Among the latter group, prolonged prothrombin time was noted in 81%. Incidence of clotting factor deficiency were as follows: factor XI, 36.3%; V, 31.8%; VIII, 27.2%; IX, 13.6%; and XII, 27.2%. Most of the abnormalities occurred in the non-splenectomized patients. Two of the six orthopaedic surgery patients had excessive intraoperative and postoperative bleeding. One, who underwent spinal decompression had prolonged prothrombin time, and the other, who had total hip replacement, showed a deficiency of factor XI. The second patient's hemoglobin level was maintained with transfusion of fresh frozen plasma during contralateral hip arthroplasty five months later. We suggest that preoperative evaluation of clotting factors and replacement therapy may prevent excessive bleeding in patients with type I Gaucher disease.
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