小儿原发性血小板增多症4例临床及实验室分析

目的探讨小儿原发性血小板增多症(ET)的临床、实验室特征。方法诊断依PVSB标准;行血常规、骨髓细胞形态学、凝血四象、血小板功能等检查。确诊后给予抗凝、活血或马利兰治疗。结果4例患儿中以出血症状诊断2例、血栓症状诊断2例;脾肿大3例;外周血血小板(Plt)724~1028×109/L,易见Plt聚集成堆,可见巨大Plt、畸形Plt;骨髓象巨核细胞明显增多,分类以产板型为主;3例APTT延长,血小板粘附率、聚集功能降低。治疗后症状均缓解,随访至今无恶性转化。结论ET患儿具典型的临床和实验室特征,临床应予以重视,以防误诊。

The clinical and laboratorial analysis of 4 pediatric patients with essential thrombocythemia

Objective To explore the clinical and laboratorial characteristics of pediatric patients with essential thrombocythemia(ET).Methods Pediatric patients with ET were diagnosed according to the PVSB standard,and the routine hematological examination,the cellular morphology of bone marrow,the four index of blood coagulation(PT,Fib,APTT,TT),the platelet function were detected.After diagnosed they were treated with anti-coagulation,blood-Activating or myleran.Results For thc four patients,two were diagnosed by bleeding,others were diagnosed by thrombosis,three cases suffered with splenomegaly;Platelet(Plt) count of peripheral blood were 724~1028×10~9/L,it was easy to observe that Plt were aggregated into cluster,the Bernard-Soulier Plt and abnormal Plt.Megakaryocytes in bone marrow were remarkablely increased and main of them were platelet-forming type;3 case were detected with longer APTT,lower platelet adhesion and aggregation.After treatment all the symptoms were alleviated,and follow-up to now no one was malignantly transformed.Conclusion There are typical characteristics of clinic and laboratory in pediatric patients with ET,which must be paid more attention to lest they be falsely diagnosed.