Transient factor VIII inhibitor in a hemophilia patient after staphylococcal septic shock syndrome

Kazuhiko Yamamoto, Kenji Niiya, Terunobu Shigematu, Toru Kiguchi, Katsuto Takenaka, Katsushi Shinagawa, Fumihiko Ishimaru, Kazuma Ikeda, Midori Shima, Mine Harada

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Abstract

We report a transient type I factor VIII inhibitor that arose in a 30-year-old hemophilia patient just after staphylococcal septicemia. This situation usually occurs early in the course of substitution therapy with factor VIII concentrate in hemophilia patients. Although disseminated intravascular coagulation and acute respiratory distress syndrome developed after septic shock, the patient recovered following intravenous administration of antibiotics (meropenem and gentamycin), an antithrombin preparation, high-dose methylprednisolone, and recombinant factor VIII concentrate (rFVIII). During this therapy, however, activated partial thromboplastin time gradually lengthened. On the seventh day of hospitalization, intracranial hemorrhage occurred with right hemiplegia, even though the substitution therapy had continued at the same dosage (30 U/kg per day) of rFVIII. At that point, 4 Bethesda units of the type I inhibitor against factor VIII were detected in the plasma. Increased amounts (46 U/kg per day) of rFVIII and prednisolone were administered, and hypothermic therapy was initiated. Following these treatments, the patient's general condition gradually improved, and within 25 days the inhibitor titer dropped to undetectable levels and did not recur during treatment. These clinical findings suggest that the staphylococcal septic shock may have acted as a trigger in the development of transient factor VIII inhibitor in this patient.

Original languageEnglish
Pages (from-to)517-519
Number of pages3
JournalInternational Journal of Hematology
Volume72
Issue number4
Publication statusPublished - Dec 1 2000

All Science Journal Classification (ASJC) codes

  • Hematology

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    Yamamoto, K., Niiya, K., Shigematu, T., Kiguchi, T., Takenaka, K., Shinagawa, K., Ishimaru, F., Ikeda, K., Shima, M., & Harada, M. (2000). Transient factor VIII inhibitor in a hemophilia patient after staphylococcal septic shock syndrome. International Journal of Hematology, 72(4), 517-519.