凝固時間が正常でありながら著明な陰茎および陰嚢の血腫を生じた血友病A

A CASE OF HEMOPHILIA A WITH NORMAL CLOTTING TIME PRESENTING AS LARGE PENILE AND SCROTAL HEMATOMA

緒言

　われわれ臨床医は，家族歴や既往歴に出血性疾患が認められなければ，術前に出血傾向の検査として，血小板数，出血時間，凝固時間，毛細血管抵抗を行なうのが普通である。

　しかし，先天性血液凝固異常の軽症例では，これら検査がすべて正常値を示す場合が稀にみられる。しかも，20〜30歳になつてなんらかの症状を呈するものがあるといわれる^1）。したがつて，極く稀にしか遭遇しないと考えられるが，このような症例であることを知らずに手術を行なつた場合どうすべきかを考えておくことは，臨床医にとつて大切なことと考える。

A 23 years old male patient with mild hemophilia A who developed large penile and scrotal hematoma after sustaining contusion on his right inguinal and scrotal region was reported. His family history was unremarkable. The patient had been well until 11 years old when he developed abnormal surgical bleeding. Since then he had repeated episodes of hemoarthrosis and hemophilia A was first pointed out on complete medical examination when he was 14 years old. Conservative treatment including fresh and preserved whole blood transfusion, administration of Factor VIII and trans-AMCHA alleviated his symptoms.