International Journal of Hematology

DOI: 10.1007/s12185-018-2462-y Pages: 1-4

Successful hemostatic management of major surgery for cervical spondylotic myelopathy in a patient with severe factor XI deficiency

1. Gunma University Graduate School of Medicine, Department of Hematology

2. Yokohama City University Graduate School of Medicine, Department of Human Genetics

3. Gunma University Hospital, Department of Orthopaedic Surgery

4. Gunma University Hospital, Clinical Laboratory Center

5. Gunma University Graduate School of Medicine, Department of Clinical Laboratory Medicine

Correspondence to:
Yoshiyuki Ogawa
Tel: +81-27-220-8166
Email: yo-ogawa@gunma-u.ac.jp

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Abstract

Factor XI deficiency (FXID) is a rare bleeding disorder caused by mutations in the F11 gene. Spontaneous bleeding in patients with factor XI deficiency is rare, but major bleeding may occur after surgery or trauma. The basic method for hemostatic treatment is replacement of the missing factor using FXI concentrate or fresh frozen plasma (FFP). We report the case of a 72-year-old male with severe FXID who underwent a laminoplasty under sufficient, but minimal, FFP transfusion. Through detailed monitoring of activated partial thromboplastin time (APTT) and FXI activity at the perioperative period, we succeeded in hemostatic management of major surgery without significant blood loss and fluid overload. From the course of this case, we found that measuring FXI activity is superior to measuring APTT. Furthermore, we identified a novel homozygous mutation in F11 [NM_000128.3:c.1041C > A:p.(Tyr347*)] by whole exome sequencing.

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