先天性白血病8例临床特征分析

Analysis of Clinical Characteristics of 8 Cases of Congenital Leukemia

目的分析先天性白血病的临床特征和诊治要点。方法回顾性分析8例先天性白血病患儿的临床资料。结果8例患儿中6例伴有脾肿大,5例伴有皮肤紫癜,5例伴有肝脏增大,3例伴有肢端青紫及气促。7例伴有发育畸形:3例21-三体综合征,5例心脏畸形,1例鞘膜积液,1例外阴畸形,1例肛门闭锁。血常规:WBC(143.50±143.40)×10^(9)/L,PLT(171.88±247.2)×10^(9)/L,Hb(111.38±16.45)g/L,所有标本均检测到幼稚细胞。已分型的4例中仅1例为髓系,3例为淋巴细胞白血病,8例患儿均未选择化疗。4例短期内死亡,4例失访。结论先天性白血病是儿科罕见病,预后较差。血常规是发现该病的线索,先天性白血病患儿多伴有脾大及发育畸形等表现。化疗及干细胞移植可能是有效的解决方案。

Objective To analyze the clinical features and main points of diagnosis and treatment of congenital leukemia.Methods The clinical data of 8 children with congenital leukemia were retrospectively analyzed.Results 6 of the 8 children were accompanied by splenomegaly,5 with skin purpura,5 with liver enlargement,and 3 with acral cyanosis and shortness of breath.7 cases were associated with developmental malformations:3 cases of trisomy 21 syndrome,5 cases of cardiac malformation,1 case of hydrocele,1 exception of vaginal deformity,and 1 case of anal atresia.WBC:(143.50±143.40)×10^(9)/L,PLT:(171.88±247.2)×10^(9)/L,Hb(111.38±16.45)g/L,all specimens detected naive cells.Of the 4 cases that had been typed,only 1 was myeloid,3 were lymphocytic leukemias,and 8 patients did not choose chemotherapy.4 patients died shortly and 4 were lost to follow-up.Conclusion Congenital leukemia is a rare pediatric disease with a poor prognosis.Blood routine is the clue to discover the disease.Congenital leukemia is often accompanied by splenomegaly and developmental malformations.Chemotherapy and stem cell transplantation may be effective solutions.