Journal List > Korean J Hematol > v.44(3) > 1032827

Lee, Yoon, Lim, Kim, Kim, Kim, Park, Seol, Song, Choi, Chung, and Cho: Post-operative Bleeding due to Acquired Hemophilia Successfully Treated with Recombinant Factor VIIa: Case Report

Abstract

Acquired hemophilia is a rare but potentially life-threatening hemorrhagic disorder caused by the development of autoantibodies against coagulation factor VIII. Concentrates of human factor VIII, desmopressin, activated prothrombin complex concentrates, recombinant activated factor VII can all be used to control episodes of acute bleeding. The recent availability of bypassing agents like recombinant activated factor VII has been shown to be clinically safe and effective as treatment for acute bleeding. In this case report, a 67 year-old male patient with Rh negative blood type developed gross hematuria and bleeding after transurethral resection due to prostatic hypertrophy. After vesicocutaneous fistular reduction operation, post-operative bleeding was presented. The acute bleeding was controlled successfully by the combined treatment with recombinant activated factor VII (Novo seven) and prednisone.

References

1. Hay CR. Acquired haemophilia. Baillieres Clin Haematol. 1998; 11:287–303.
2. Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to factor VIII. Thromb Haemost. 1981; 45:200–3.
crossref
3. Kondo E, Utsumi M, Hattori M, et al. Acquired factor VIII-specific antibody disorder accompanied by a life-threatening retroperitoneal hematoma. Intern Med. 1995; 34:901–3.
crossref
4. Ingerslev J. Hemophilia. Strategies for the treatment of inhibitor patients. Haematologica. 2000; 85:15–20.
5. Hedner U, Erhardtsen E. Potential role for rFVIIa in transfusion medicine. Transfusion. 2002; 42:114–24.
crossref
6. HednerU. Glazer S, Falch J. Recombinant activated factor VII in the treatment of bleeding episodes in patients with inherited and acquired bleeding disorders. Transfus Med Rev. 1993; 7:78–83.
7. Hay CR, Negrier C, Ludlam CA. The treatment of bleeding in acquired haemophilia with recombinant factor VIIa: a multicenter study. Thromb Haemost. 1997; 78:1463–7.
8. Lee JJ, Chung IJ, Park MR, et al. Acquired hemophilia successfully treated with oral immunosuppressive therapy. Korean J Intern Med. 2000; 15:135–7.
crossref
9. Jung SW, Kim S, Youk CM, et al. A case of asymptomatic acquired hemophilia. Korean J Thromb Hemost. 2001; 8:59–62.
10. Kim JH, Lee WI, Yoon HJ, et al. A case of acquired hemophilia A. J Lab Med Qual Assur. 2003; 25:207–9.
11. Chung BH, Kim YJ, Kim MS, et al. A case of acquired hemophilia detected after influenza vaccination. Korean J Med. 2004; 66:298–301.
12. Park SY, Kim JS, Kim Y, et al. Two cases of acquired hemophilia A successfully treated with oral steroid or danazol. Korean J Hematol. 2005; 40:58–63.
crossref
13. Song MH, Ahn SJ, Yoo SH, et al. Total Knee arthroplasty-associated acquired hemophilia: a case report. J Korean Orthop Assoc. 2006; 41:167–9.
crossref
14. Kim MS, Kilgore PE, Kang JS, et al. Transient acquired hemophilia associated with mycoplasma pneumonia pneumonia. J Korean Med Sci. 2008; 23:138–41.
15. Ogata H, Sakai S, Koiwa F, et al. Plasma exchange for acquired hemophilia: a case report. Ther Apher. 1999; 3:320–2.

Fig. 1.
Computed tomographic findings. (A) Hematoma in the left perirenal space with active bleeding at the posterior aspect of left kidney. (B) Hematoma in the pelvic extraperitoneal space.
kjh-44-163f1.tif
Fig. 2.
The change of aPTT in patient treated with recombinant activated Factor VII, Factor VIII, prednisone. After recombinant activated Factor VII was administered, aPTT decreased to normal range.
kjh-44-163f2.tif
Table 1.
Clinical and laboratory characteristics, treatments, clinical outcomes of the patient with acquired hemophilia reported in Korea9–15)
No. case 1 2 3 4 5 6 7 8
Sex F F M F M M F F
Age at diag-nosis (years) 40 34 73 65 82 78 61 33 (months)
Clinical pre-sentation Shoulder pain, gross hematuria No symptom Hematuria Gross hematuria, retroperitoneal bleed Melena No symptome Bleed in total knee arthroplasty site Hematochezia, ecchymoses
FVIII (%) 1.5 0.4 1 2 1 1 1 1
FVIII inhi-bitor (BU) 27.50 9.6 2.7 850 36 2.74 10 2.5
Treatment of acute bleeding ? ? ? Factor VIII ? ? Factor VIII ?
Clinical efficacy ? ? ? None ? ? None ?
Long-term strategy Prednisone, cyclophosph amide Prednisone None Medroxy-prednisone, cyclophosphamide Prednisone, danazol Prednisone Prednisone, cyclophosphamide Prednisone, IVIG
Etiology None None None Influenza vaccination None None Operation Mycoplasma pneumonia
Outcome Remission F/U loss No remission No remission; fatal retroperitoneal bleed: death Remission Remission Remission pneumonia Remission
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