Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston, South Carolina, USA.
Diagn Cytopathol. 2021 Feb;49(2):E80-E83. doi: 10.1002/dc.24594. Epub 2020 Aug 27.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare but clinically aggressive hematologic malignancy, believed to originate from plasmacytoid dendritic cells (PDCs) although it possesses multilineage potentials. Due to the dismal prognosis, accurate and rapid diagnosis is critical for early management. The disease usually initially involves skin and bone marrow. Here we report the cytopathologic findings in a case of BPDCN involving the liver in a patient previously diagnosed with BPDCN from skin and lymph node biopsies. The fine-needle aspiration specimen from the liver lesion demonstrates a hypercellular smear of atypical epithelioid cells dispersed singly or in loose groups. These cells have enlarged, eccentric, round to irregular nuclei with fine chromatin. The agranular gray-blue cytoplasm shows delicate wispy cytoplasmic extensions and cytoplasmic microvacuoles. Binucleation is common. The concurrent core biopsy shows that the neoplastic plasmacytoid cells with eccentric nuclei were positive for CD4, CD7, CD43, CD56, and CD68, confirming the diagnosis of BPDCN. Mutations of ASXL1 and TET2, classic for BPDCN, and a complex karyotype were detected in skin, bone marrow, and lymph node specimens. We catalog the heterogeneous pathologic features of this rare disease, emphasizing the clinical and histopathological correlation. The differential diagnoses and review of literature are also included. Awareness of this disease and accurate diagnosis are emphasized to aid early management and potentially produce a better clinical outcome.
原始细胞性浆细胞样树突细胞肿瘤（BPDCN）是一种罕见但具有侵袭性的血液系统恶性肿瘤，被认为起源于浆细胞样树突细胞（PDCs），尽管它具有多能性。由于预后不良，准确快速的诊断对于早期管理至关重要。该疾病通常最初累及皮肤和骨髓。在此，我们报告了一例先前通过皮肤和淋巴结活检诊断为 BPDCN 的患者肝脏受累的 BPDCN 的细胞病理学发现。肝病变的细针抽吸标本显示单个或松散分布的异型上皮样细胞的高细胞涂片。这些细胞具有大而偏心的圆形至不规则核，染色质精细。无颗粒的灰蓝色细胞质显示出细腻的丝状细胞质延伸和细胞质微泡。双核很常见。同时进行的核心活检显示，具有偏心核的肿瘤性浆细胞样细胞对 CD4、CD7、CD43、CD56 和 CD68 呈阳性，证实了 BPDCN 的诊断。皮肤、骨髓和淋巴结标本中检测到 ASXL1 和 TET2 的突变，这是 BPDCN 的经典特征，以及复杂的核型。我们对这种罕见疾病的异质病理特征进行了编目，强调了临床和组织病理学的相关性。还包括鉴别诊断和文献复习。强调对这种疾病的认识和准确诊断，以帮助早期管理并可能产生更好的临床结果。
