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真性红细胞增多症是一种较少见且原因不明的慢性进行性骨髓增生性疾病,也称原发性红细胞增高症。据报导本病引起神经系统并发症约占1/3左右。本文报道真红合并皮层多发梗塞一例。患者,女,55岁,因进行性头昏、头痛、乏力、皮肤红紫7年,加重伴肢体无力4个月于90年1月入院。查体:T36.5c、P88次/分、R21次/分、Bp20/13kPa。面如醉酒容,皮肤、粘膜呈暗红色,以口唇、手掌、眼结膜明显。神志清,表浅淋巴结未扪及。心、肺听诊未见异常。肝剑下3cm,脾肋下4.5cm,胸及四肢骨无压疼。肌力、肌张力正常,无深浅感觉障碍。化验:HB:180g/L、RBC:8.64×1O~(12)/L、WVBC:2.1×1O~9/L、pc:45×1O~9/L、RBC压积:72％、血沉:1小时未下降1毫米,N-ALP:阳性率90％、积分:320分。骨髓:红系增生明显活跃,形态正常。成熟红细胞呈缗钱样排列。粒系:提示核左移、巨核细胞40只/全片、血小板多见。可见畸型血小板。微循环观察:有严重淤滞。脑CT:大脑皮层区可见3个<1.5cm低密度灶,提示“皮层多发性梗塞”。
Polycythemia vera is a rare and unexplained chronic progressive myeloproliferative disorder, also known as essential polycythemia. It is reported that the disease caused neurological complications accounted for about 1/3. This article reports a case of true red merged cortical multiple infarcts. Patients, female, 55 years old, due to progressive dizziness, headache, fatigue, skin purple red seven months, aggravated with limb weakness for 4 months in January 90 admitted. Physical examination: T36.5c, P88 beats / min, R21 beats / min, Bp20 / 13kPa. Facial drunkenness, skin, mucous membrane was dark red, lips, palms, conjunctiva obvious. Conscious, superficial lymph nodes not palpable. Heart, lung auscultation no abnormalities. Under the liver sword 3cm, spleen rib 4.5cm, chest and limb pain without pressure. Muscle strength, muscle tone normal, no sense of depth and sensation. Assay: HB: 180g / L, RBC: 8.64 × 10 ~ 12 / L, WBC: 2.1 × 10 ~ 9 / L, pc: 45 × 10 ~ 9 / L, RBC depressurization: 72%, ESR: 1 Hour did not drop 1 mm, N-ALP: positive rate of 90%, points: 320 points. Bone marrow: erythroid hyperplasia was significantly active, normal morphology. RBC erythrocytes were like money arrangement. Granulocyte line: Prompt nuclear left, megakaryocyte 40 / whole piece, platelet more common. Deformity can be seen platelet. Microcirculation observation: a serious stasis. Brain CT: Cerebral cortical area visible 3 <1.5cm low density lesions, suggesting that “cortical multiple infarction.”