儿童双表型白血病的临床特征及预后分析

目的分析儿童双免疫表型白血病的临床及生物学特征，评价儿童急性白血病双免疫表型与治疗相关因素及预后的临床重要性。方法自1998年1月1日至2003年5月31日进入XH 99治疗方案的所有新诊治的急性白血病(AL)患儿，诊断采用MICM分型诊断，治疗分别按AML XH 99、ALL XH 99危险度分类标准进行分层治疗。用流式细胞仪进行免疫表型分析，根据免疫表型结果将患儿分为4组，伴有/无髄系相关抗原表达的急性淋巴细胞白血病(My+ALL/My-ALL)以及伴有/无淋系相关抗原表达的急性髓系白血病(Ly+AML/Ly-AML)。生存分析采用Kaplan Meier方法；生存率之间的比较采用log rank检验；临床及生物学特征与治疗相关因素的分析采用χ2检验或Fisher精确概率法(双尾)。结果①174例提供免疫分型的ALL患儿中，My+ALL患儿34例，占19.54%，其与My-ALL组患儿除在B ALL组患儿达缓解时间有统计学差异外(P<0.05)，其它临床、生物学特征及治疗反应均无统计学差异(P>0.05)；两组患儿5年无事件生存(EFS)率分别为\(61.76±8.33)%与(68.03±5.55)%\],log rank检验两组患儿5年EFS率无统计学差异(P=0.0526)。②74例提供免疫分型结果的AML患儿中，Ly+AML患儿18例，占24.32%，其与Ly-AML组患儿临床、生物学特征及治疗反应无统计学差异(P>0.05)；两组患儿5年EFS率分别为\(39.82±13.59)%与(51.29±9.70)%\],log rank检验两组患儿5年EFS率无统计学差异(P=0.3164)。结论双免疫表型对儿童白血病预后无明显影响，可用同样现行的化疗方案治疗这部分患儿。

Clinical features and prognosis analysis of children with lymphoid associated and myeloid associated antigen positive acute leukemia:a report from the AL XH 99.

AbstractObjectiveTo analyse the clinical features of children with myeloid /lymphoid antigen positive acute leukemia and assess the clinical significance between the bi immunophenotypic leukemia and associated therapeutic factors as well as prognosis.MethodsFrom January 1998 to May 2003,193/75 patients with newly diagnosed ALL/AML were enrolled on protocol ALL XH 99/AML XH 99.Immunophenotyping was measured with the use of flow cytometry.According to the McAbs used,the patients were divided into four subgroups,including the patients with positive expression of lymphoid/myeloid associated antigens and those of negative expression.The probability of event free survival(pEFS) was estimated by Kaplan Meier analysis and the distributions of pEFS were compared using the log rank test.Chi square analysis or Fisher exact test were used to compare differences in the distribution of biologic presenting features.Results1.The proportion of children with ALL expressing one or more of the myeloid associated antigens was 19.54%.There were no significant differences at clinical features and response to treatment except time of complete remission with B lineage acute lymphoblastic leukemia in positive or negative expression of myeloid associated antigens acute lymphoblastic leukemia.Patients with positive expression of myeloid associated antigens were not significantly better than those with negative expression of My ALL ［(61.76±8.33)% vs (68.03±5.55)%,P=0.0526］.2.The proportion of children with AML expressing one or more of the lymphoid associated antigens was 24.32%.In French American British (FAB) M2,lymphoid associated antigen CD19 was expressed by blast cells in most patients and APL was char acterized by the absence of HLA DR,lymphoid associated antigens CD19 and CD7.There were no significant difference at clinical features and response to treatment except FAB in positive or negative expression of lymphoid associated antigens (CD19,CD7) acute myeloid leukemia.Patients with positive expression of lymphoid associated antigens (CD19,CD7) were not significantly better than those with negative expression of Ly AML ［(39.82±13.59)% vs (51.29±9.70)%,P=0.3164］.ConclusionThere is no significant effect on bi immunophenotypic leukemia,and patients can be treated with lymphoid/myeloid associated antigen in contemporary risk adapted therapy.