本文介绍1例岗期性粒细胞减少症(CN)患者发生全身继发性淀粉样变性,似为迄今的第一个“典型”病例。患者女性,24岁。因周期性发热伴疱疹性口炎、齿龈肿胀、腹痛、腹泻于1973年9月1日入院。出生两周时患全身脓疱疮,此后每隔21天即有发热,历时数天。入院时回盲部压痛、中度缺铁性贫血、白细胞减少,血小板计数正常。血清总蛋白7.4克%,A53.8%,α_1 2.3%,α_2 8.6%,β8.1%,γ27.6%;IgG2150mg%,IgA 375 mg%,IgM 290 mg%(正常各为900～1700、120～400、40～160);血清异常免疫球蛋白 This article describes a case of patients with idiopathic granulocytopenia (CN) in patients with systemic secondary amyloidosis, seems to date the first “typical” cases. Patient female, 24 years old. Due to periodic fever with herpetic stomatitis, swollen gums, abdominal pain, diarrhea admitted on September 1, 1973. Two weeks after birth, there is systemic impetigo, and every 21 days thereafter there is fever which lasts for several days. Ileocecal tenderness on admission, moderate iron deficiency anemia, leukopenia, normal platelet count. Serum total protein of 7.4 g%, A53.8%, α_1 2.3%, α_2 8.6%, β8.1%, γ27.6%; IgG250mg%, IgA 375 mg%, IgM 290 mg% (normal 900 ~ 1700, 120 ~ 400,40 ~ 160); serum abnormal immunoglobulin