急性髓细胞白血病微分化型并噬血细胞综合征的临床研究

目的探讨急性髓细胞白血病微分化型(AML-M0)并噬血细胞综合征(HLH)的临床特征及诊断。方法报告2例近期我院收治的AML-M0并噬血细胞综合征,并复习相关文献。结果2例均以反复发热、肝、脾、浅表及深部淋巴结明显肿大、血象改变为首发症状,流式细胞仪检查骨髓CD13+,CD33+,CyMPO+均阳性,有CD34、HLA-DR、TdT、CD117等早期抗原表达。结论肝、脾、浅表及深部淋巴结明显肿大伴血象改变易误诊为淋巴瘤合并HLH,诊断主要依靠免疫组化,电镜或流式细胞术MPO阳性对诊断AML-M0最有意义。

Clinical study on minimally differentiated acute leukemia with hemophagocytic lymphohistiocytosis

Objective To study the clinical feature and diagnosis of minimally differentiated acute leukemia(AML-M0) with hemophagocytic lymphohistiocytosis(HLH). MethodsWe reported two cases of AML-M0 with HLH with review of the literature. ResultsThe initial symptom of these two cases was repeated fever, enlarged liver, spleen, superficial and deep lymph nodes and blood cell changes. The immunofiuorescencc assay revealed the positive stare of CD13+, CD33+, CyMPO+. Early antigens like CD34, HLA-DR, TdT, CD 117 were expressed. ConclusionCases with obvious enlarged liver, spleen, superficial and deep lymph nodes and blood cell changes are usually misdiagnosed as lymphoma with HLH. The diagnosis is relied on the immunohistochemistry and positive stain of MPO with electiroscopy or immunofluorescence assay is most meaningful.