Acquired hemophilia A requiring plasma exchange and mechanical ventilation

TAKAHASHI Wataru, ICHIKAWA Motoshi, FURUICHI Shiho, NAGASAWA Fusako, ISO Hisako, ARAI Honoka, TSURUMI Shigeharu, HANDA Tomoyuki, TADOKORO Jiro, NAKAMURA Yuko, NAKAMURA Yuka, SASAKI Ko, MITANI Kinuko

doi:10.11406/rinketsu.60.191

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Other Title

血漿交換・呼吸管理を含む集中治療を要した後天性血友病A
症例報告第8回日本血液学会関東甲信越地方会奨励賞血漿交換・呼吸管理を含む集中治療を要した後天性血友病A
ショウレイホウコクダイ8カイニホンケツエキガッカイカントウコウシンエツチホウカイショウレイショウケッショウコウカン・コキュウカンリオフクムシュウチュウチリョウオヨウシタコウテンセイケツユウビョウ A

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Abstract

<p>A 56-year-old man who sustained a right waist injury 1 month ago, reported to our department complaining of pain in the right waist and femur for 1 day. In a computed tomography examination, hematoma of the right iliopsoas muscle was revealed, and arterial embolization was immediately performed but was not effective. Laboratory findings showed hemoglobin levels as 5.4 g/dl, platelet of 20.2×10⁴/µl, prothrombine time of 13.1 s, partial thromboplastin time (APTT) of 81.1 s, and a convex upward curve of the APTT cross-mixing test. The activity of the coagulation factor VIII was <1.0%, but its amount was 120%, and the level of factor VIII inhibitor was 130 Bethesda Unit/ml. Disseminated intravascular coagulation was not noted. Under the diagnosis of acquired hemophilia A, treatment with prednisolone and recombinant activated factor VII was initiated. However, APTT remained prolonged, and intubation and mechanical ventilation were required because of right hemothorax. After steroid pulse therapy and plasma exchange, APTT returned to its normal range, and the inhibitor disappeared. Thus, we finally succeeded in extubation. This case indicated that intensive care may be necessary in the early phase treatment for acquired hemophilia A.</p>

Journal

Rinsho Ketsueki

Rinsho Ketsueki 60 (3), 191-196, 2019

The Japanese Society of Hematology

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