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患者男,13岁。因反复皮肤黄染10余年,入院。患儿自3岁时,其皮肤、巩膜轻度黄染,小便呈酱油色;7岁后常鼻衄、脾脏肿大,血小板在5～7万/mm~3左右,多次治疗无好转。入院时体检:体温36.8℃,心率90次/分,血压14/8kPa慢性病容,皮肤巩膜轻度黄染,无出血点;全身浅表淋巴结未触及,五官端正,双肺(-),心尖区可闻及Ⅰ—Ⅱ级SM,肝肋下、剑下均为3cm,质中;脾肋下6cm,质硬,表面光滑,四肢及神经系统(-)。血象:Hb79g/L,WBC5.2×10~9/L,NC59%,LC38%,PC25×10~9/L,纲织红细胞28%。对患儿及其父母进行血红蛋白检查,结果见附表。
Patient male, 13 years old. Due to repeated skin yellow dye more than 10 years, admitted to hospital. Children from 3 years old, the skin, scleral mild yellow dye, urine was soy sauce color; after 7 years of age often nasal, splenomegaly, platelets in 5 to 70,000 / mm ~ 3, multiple treatment no improvement. Physical examination on admission: body temperature 36.8 ℃, heart rate 90 beats / min, blood pressure 14 / 8kPa Chronic disease, skin scleral mild yellow dye, no bleeding; systemic superficial lymph nodes not touched, facial features, lungs (-), apex Can be heard and Ⅰ-Ⅱ grade SM, liver ribs, the sword are 3cm, quality; spleen ribs 6cm, hard, smooth, limbs and nervous system (-). Blood: Hb79g / L, WBC5.2 × 10 ~ 9 / L, NC59%, LC38%, PC25 × 10 ~ 9 / L, reticulocyte 28%. Hemoglobin examination of children and their parents, the results see Schedule.