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本文报告一例原发性巨球蛋白血症患者,因外周血和明显肿大的脾脏的淋巴细胞,免疫吸附因子Ⅷ,引起出血。脾切除后,因子Ⅷ恢复正常。患者32岁男性,因痔出血、自发性血尿、拔牙后出血、腋下淋巴结肿大,肝脾肿大和轻度贫血而入院。检查发现,视网膜静脉分段,血红蛋白8.9克%,白细胞7600/立方毫米,淋巴细胞75%(许多不典型的),IgM(λ)5670毫克%,凝血酶原时间15秒,部分凝血活酶时间为79秒,出血时间8分(正常:<7分),因子Ⅷ减少至48%,因子Ⅷc5%,因子Ⅷ相关
This article reports a case of patients with primary macroglobulinemia, due to peripheral blood and significantly enlarged spleen lymphocytes, immunosuppressive factor Ⅷ, causing bleeding. After splenectomy, factor Ⅷ returned to normal. The 32-year-old man was admitted to hospital for hemorrhoid hemorrhage, spontaneous hematuria, bleeding after tooth extraction, swollen armpit lymph nodes, hepatosplenomegaly and mild anemia. Examination revealed retinal vein segments with 8.9 grams of hemoglobin, 76,000 cells per cubic millimeter of leukocytes, 75% of lymphocytes (many atypical), 5670 milligrams of IgM (λ), 15 seconds of prothrombin time, partial thromboplastin time 79 seconds, bleeding time 8 points (normal: <7 points), factor Ⅷ reduced to 48%, factor Ⅷ c5%, factor Ⅷ correlation