免疫表型分析在诊断无皮肤表现的浆细胞样树突状细胞肿瘤白血病期的不可或缺作用。

Alam Hareem, Saeed Nabiha, Rashid Anila

Section of Hematology & Transfusion Medicine, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan.

Department of Oncology, Aga Khan University Hospital, Karachi, Pakistan.

Leuk Res Rep. 2022 Apr 25;17:100317. doi: 10.1016/j.lrr.2022.100317. eCollection 2022.

Blastic plasmacytoid dendritic cell neoplasm usually presents as skin lesions. Diagnostic error occurs when it primarily presents in leukemic phase without skin involvement. Triad of CD4, CD56 and CD123 immunophenotype expression is essential to avoid misdiagnosis of this rare hematological malignancy. Here we describe a patient who presented in overt leukemic phase of BPDCN highlighting diagnostic challenges encountered that resulted in delayed diagnosis and poor outcome.

母细胞性浆细胞样树突状细胞肿瘤通常表现为皮肤病变。当它主要表现为白血病期且无皮肤受累时，就会发生诊断错误。CD4、CD56和CD123免疫表型表达三联征对于避免误诊这种罕见的血液系统恶性肿瘤至关重要。在此，我们描述了一名表现为母细胞性浆细胞样树突状细胞肿瘤明显白血病期的患者，强调了所遇到的诊断挑战，这些挑战导致了诊断延迟和不良预后。