Ohe Rintaro, Aung Naing Ye, Shiono Yosuke, Utsunomiya Aya, Kabasawa Takanobu, Tamazawa Nobuyuki, Tamura Yuka, Kato Tomoya, Yamada Akane, Hasegawa Shin, Aizawa Keiko, Inokura Kyoko, Ito Satoshi, Toubai Tomomi, Kato Yuichi, Tsunoda Takahiko, Onami Kosuke, Suzuki Tamio, Ishizawa Kenichi, Yamakawa Mitsunori
Department of Pathological Diagnostics, Yamagata University Faculty of Medicine, Yamagata, Japan.
Department of Neurology, Hematology, Metabolism, Endocrinology and Diabetology, Yamagata University Faculty of Medicine, Yamagata, Japan.
J Clin Exp Hematop. 2018 Mar 16;58(1):1-9. doi: 10.3960/jslrt.17030. Epub 2018 Feb 8.
Blastic plasmacytoid dendritic cell (pDC) neoplasm (BPDCN) is a relatively rare hematological malignancy with significantly complex clinicopathological features that are still unclear. This study aimed to analyze the clinicopathological data of BPDCN and evaluate immunohistochemical detection of minimal bone marrow (BM) involvement. In this study, we examined skin and BM lesions from 6 patients with BPDCN. Neoplastic cells tested positive for CD303 (polyclonal, 100%; monoclonal, 40%) in the skin lesions and for CD303 (polyclonal, 100%; monoclonal, 67%) in the BM clots. Although immunostaining of CD4, CD56, CD123, CD303, and TCLl detected minimal BM involvement in 3 patients, morphological identification was challenging in the BM clots stained with hematoxylin-eosin. In conclusion, our results demonstrate the significance of observing BM smears to detect neoplastic cells and that immunohistochemical examination, including CD303 antibodies, is useful to detect minimal BM involvement. This study is the first to report the expression of thymic stromal lymphopoietin (TSLP) and its receptor in BPDCN cells. Therefore, the TSLP/TSLP receptor axis may be associated with the proliferation of BPDCN, and consequently, the survival of patients.
母细胞样浆细胞样树突状细胞(pDC)肿瘤(BPDCN)是一种相对罕见的血液系统恶性肿瘤,其临床病理特征极为复杂,目前仍不清楚。本研究旨在分析BPDCN的临床病理数据,并评估免疫组化检测骨髓(BM)微小浸润情况。在本研究中,我们检查了6例BPDCN患者的皮肤和BM病变。肿瘤细胞在皮肤病变中CD303检测呈阳性(多克隆,100%;单克隆,40%),在BM凝块中CD303检测呈阳性(多克隆,100%;单克隆,67%)。尽管CD4、CD56、CD123、CD303和TCLl的免疫染色在3例患者中检测到了BM微小浸润,但在用苏木精-伊红染色的BM凝块中进行形态学鉴定具有挑战性。总之,我们的结果表明观察BM涂片以检测肿瘤细胞的重要性,并且包括CD303抗体在内的免疫组化检查对于检测BM微小浸润是有用的。本研究首次报道了胸腺基质淋巴细胞生成素(TSLP)及其受体在BPDCN细胞中的表达。因此,TSLP/TSLP受体轴可能与BPDCN的增殖相关,进而与患者的生存相关。