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女性,20岁,因头昏、乏力三年余,加重1个月于1989年1月3日入院。自述1982年曾在广东省人民医院诊断“地中海贫血”。体检:贫血貌,躯干及四肢可见散在瘀点及瘀斑,皮肤巩膜无黄染,浅表淋巴结不大,胸骨无压痛,双肺呼吸音粗糙,心界稍向左扩大,心前区可闻及Ⅲ级收缩期吹风样杂音。肝肋下5cm;脾甲乙线13cm,甲戍线17cm,丙丁线7cm;质硬。Hb39g/L,WBC37×10~9/L,原始粒细胞1％,黄疸指数5u,铁蛋白>250ng/mL,高铁血红蛋白还原率65.9％,Hb电泳HbA86.7％,HbA_20.2％,HbH9.8％,抗碱Hb3.3％,包涵体试验(+)。骨髓增生活跃,原始粒细胞5％,骨髓病态造血(粒系核质疏松肿胀,胞浆颗粒增大,红系可见嗜碱、点彩样红细胞,可见小巨核细胞),诊断①α-地中海贫血—血红蛋白H病②MDS—(RAEB)
Female, 20 years old, due to dizziness, fatigue more than three years, one month heavier on January 3, 1989 admission. Readme 1982 in Guangdong Province People’s Hospital diagnosed “thalassemia.” Physical examination: anemia appearance, trunk and limbs visible scattered petechiae and ecchymosis, skin sclera no yellow dye, superficial lymph nodes, sternum no tenderness, lungs rough breathing sound, the heart of the heart to the left to expand slightly, preclinical area can be heard And Ⅲ grade systolic hair-like noise. Liver ribs 5cm; spleen A line 13cm, armor line 17cm, Ding Ding line 7cm; hard. Hb39g / L, WBC37 × 10 ~ 9 / L, primary granulocyte 1%, jaundice index 5u, ferritin> 250ng / mL, methemoglobin reduction rate of 65.9%, Hb electrophoresis HbA86.7%, HbA_20.2%, HbH9. 8%, anti-alkali Hb3.3%, inclusion body test (+). Myeloid hyperplasia is active, the original granulocyte 5%, myelopathic hematopoiesis (granulocytic loose swelling, increased cytoplasmic granules, erythroid visible basophilic, dot-like erythrocytes, visible small megakaryocytes), diagnosis of α-thalassemia- Hemoglobin H disease ②MDS- (RAEB)