髓细胞性白血病皮肤浸润的临床及病理特征

Clinical and pathological characteristics of myelogenous leukemia with skin infiltration

目的 探讨髓细胞性白血病皮肤浸润的临床表现、皮损组织病理及免疫组化特点.方法 分析6例髓细胞性白血病皮肤浸润患者的临床表现、皮损组织病理、免疫组化、实验室检查、治疗及转归.结果 6例中1例以皮肤白血病为首发表现,5例皮肤浸润出现于确诊白血病后的4 ～ 20个月.4例皮疹表现为泛发的红色浸润性丘疹、斑丘疹或结节,2例表现为局部肿物.组织病理示,真皮及皮下脂肪大量肿瘤细胞浸润,免疫组化髓过氧化物酶、CD43、CD45阳性率较高,其他出现阳性的标志物包括CD15、颗粒酶B、白细胞共同抗原、CD68.5例急性髓细胞性白血病均于出现皮肤浸润后60 d至1年内死亡.结论 髓细胞性白血病皮肤浸润一般发生于外周血象与骨髓象改变之后,偶尔也发生于白血病血象和骨髓象改变之前.皮疹常表现为结节或肿物,可局限可泛发,组织病理及免疫组化具有特征性.出现皮肤浸润者生存期短、预后差.

Objective To study the clinical,histopathological and immunohistochemical features of myelogenous leukemia with skin infiltration.Methods This study included 6 patients with myelogenous leukemia with skin infiltration diagnosed at Peking Union Medical College Hospital from July 2002 to July 2011.The clinical,histopathological and immunohistochemical findings in these patients were analyzed retrospectively.Results Leukemia cutis was the initial presentation in 1 patient,and skin infiltration developed within 4 to 20 months after the diagnosis of leukemia in 5 patients.Of these patients,4 presented with generalized erythematous infiltrated papules,maculopapules or nodules,and 2 with localized masses.Biopsy showed massive infiltration of the dermis and subcutaneous fat layer with tumor cells,most of which were positive for myeloperoxidase,CD43 and CD45,and some for CD15,granzyme B,leukocyte common antigen and CD68.Five patients with acute myelogenous leukemia all died within 60 days to 1 year after skin infiltration occurred.Conclusions In patients with myelogenous leukemia,skin infiltration usually occurs after,but occasionally before,the appearance of abnormal haemogram and myelogram,and the presence of skin infiltration is often associated with a poor prognosis and short survival.Myelogenous leukemia with skin infiltration often presents as generalized or localized nodules or masses with characteristic pathological and histochemical manifestations.