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A CASE OF EWING'S SARCOMA WITH A CONGENITAL FACTOR VII DEFICIENCY
Abstract number: P-S-216
Lee1 K., Lee1 J.
1Pediatrics, Kyungpook National University School of Medicine, Daegu, South Korea
How-to-cite Lee K, Lee J. A CASE OF EWING'S SARCOMA WITH A CONGENITAL FACTOR VII DEFICIENCY. J Thromb Haemost 2007; 5 Supplement 2: P-S-216
Abstract
Introduction: A congenital factor VII deficiency is a rare disorder and only 16 patients have been reported in Korea. We diagnosed a case of Ewing's sarcoma with a factor VII deficiency in an 11-year-old boy.
Methods: He felt discomfort on his left leg after trauma and visited our hospital at September 2006. We found a mass lesion on his left femur and detected a coagulation disorder during preparation of biopsy. The prothrombin time was prolonged to 59.4 second (INR of 5.3) and plasma activity of factor VII was decreased to 2.0%. He was diagnosed a congenital factor VII deficiency. His elder brother also was diagnosed a congenital factor VII deficiency (3% plasma activity of factor VII) and expired one year ago due to uncontrolled bleeding and sepsis at other hospital. But their parents were asymptomatic and showed normal prothrombin time although the father had decreased level of factor VII at 46%.
Results: He was diagnosed Ewing's sarcoma on biopsy and treated with cyclophosphamide, adriamycin, ifosfamide, etoposide, and vincristine (CCG-7942 protocol). We used a recombinant activated factor VII (rFVIIa) before, during, and after invasive procedures such as biopsy and there was no severe bleeding. He had intermittent nasal bleeding and gross hematuria but it was successfully controlled using rFVIIa. With chemotherapy, gastrointestinal bleeding developed and continued in spite of replacement of rFVIIa. He finally expired.
Conclusions: Most of anticancer drugs can cause mucosal cell damage to lead gastrointestinal bleeding. So we suggest that a preventive replacement of coagulation factor concentrator might be necessary to reduce the risk of severe gastrointestinal bleeding due to chemotherapy for these patients.
To cite this abstract use the following format:
Journal of Thrombosis and Haemostasis 2007; Volume 5, Supplement 2: abstract number
Session Details
| Date: |
01/08/2007
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| Time: |
00:00-00:00
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| Session name: |
XXIst ISTH Congress |
| Subject: |
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| Location: |
Oxford, UK |
| Presentation type: |
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