原发性血小板增多症并发多脏器栓塞和格林巴利综合征救治及文献复习

The treatment of essential thrombocythemia complicated with multi-organ embolism and Guillain-Barré syndrome and relative literature review

目的观察血小板高达2060×109/L的原发性血小板增多症(Essential thrombocythemia,ET)并发多脏器栓塞、多组颅神经受损并因呼吸肌麻痹导致呼吸停止的重症格林巴利综合征(Guillain-Barré syndrome,GBS)的临床特点,并总结救治体会。方法根据患者的临床特征、外周血象、骨髓象、影像学检查和脑脊液检查明确诊断;羟基脲、高三尖杉酯碱抑制血小板生成,潘生丁、阿司匹林抗血小板聚集,肝素钠抗凝,复方丹参和低分子右旋糖酐改善微循环,治疗ET伴多脏器栓塞。呼吸机辅助呼吸,甲基泼尼松龙联合大剂量丙种球蛋白冲击,抑制淋巴细胞DNA合成和封闭巨噬细胞Fc受体(Fc receptor,FcR)治疗重症GBS。地塞米松鞘内注射抗炎,小剂量环磷酰胺(Cytoxan,CTX)静脉滴注,增强对免疫细胞的抑制作用。结果治疗1周后血小板降至正常范围,颅神经损伤症状和双上肢肌力开始恢复,第14天脱离呼吸机。治疗30d双上肢肌力达4级,双下肢2级。至发病第60天,患者四肢肌力完全恢复。结论患者体内巨噬细胞不断吞噬大量的血小板及其碎片,产生抗鞘磷脂抗体,是机体免疫活化的基础;外在感染等因素诱发下,嗜酸性粒细胞、巨噬细胞等抗原提呈细胞被激活,导致患者体液和细胞免疫活化,介导对脊神经的脱髓鞘性损伤,是ET继发多脏器栓塞和GBS的重要免疫机制。羟基脲联合高三尖杉酯碱能迅速抑制巨核细胞和血小板的生成,呼吸机辅助呼吸支持治疗,甲基泼尼松龙联合大剂量丙种球蛋白冲击能迅速起到抗炎作用,有效地改善神经损伤症状,地塞米松鞘内注射可以促进药物进入脊髓屏障,小剂量CTX静脉滴注能增强对糖皮质激素耐药患者的疗效。

Objective To investigate the clinical manifestation and treatment effects of essential thromboeythemia （ET）, which platelet quantity was 2 060×10^9/L, and complicated with multi-organ embolism and Guillain-Barre syndrome （GBS）. Methods The diagnosis was performed on the basis of clinical feature, platelet quantity, bone marrow and image analysis. The ET complicated with multi-organ embolism was treated with drugs of hydroxyurea （HU）, homoharringtonine （HH）, agacore, aspirin, heparin sodium, co-dan-shen root and low molecular dextran, and so on. The GBS was treated with aided breath by using breathing apparatus and the drugs of metastab and highter dosage beriglobin impacted, decacortin intrathecal injection and Cytoxan （CTX）. Results After patient was treated for 7 days, his platelet quantity was at normal range,the breathing apparatus was removed on the 14th day. His injury of cranial nerve was recovered on the 30th day, and on the 60th day his limbs myodynamia was complete .recovered. Conclusion It is immune activation basis that macrophage catched platelet to yield anti-sphingomyelin antibodies, then because of infection, eosinophil and macrophage were activated to result in activation of humoral immunity and cellular immunity,to mediate demyelination damage of spinal nerve in the patient suffered from ET. The HU and HH can inhibit megakaryocyte and platdet proliferation quickly. It is very important that to aided breath with breathing apparatus, to treat with metastab and higher dosage beriglobin pulsely coald play anti-inflammation and effectively ameliorated symptom of nerve injury. Decacortin intrathecal injection could enhance the drugs concentration in patient＇s spinal eord barrie, and using CTX could enhance patient＇s response against glucoeorticoid resistance.