Department of Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Department of Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Hum Pathol. 2022 Jun;124:14-23. doi: 10.1016/j.humpath.2022.03.005. Epub 2022 Mar 17.
Histopathological diagnoses are challenging for rare CD3-and CD20-negative extramedullary leukemias/lymphomas. We report 118 cases of CD3- CD20-extramedullary leukemias/lymphomas (2.4% of 4977 cases). CD45 was positive in 68% of cases. Forty-nine (41%) cases were anaplastic large cell lymphomas. Thirty-five (30%) cases were large B-cell lymphomas/plasmablastic lymphomas positive for at least one of the following markers: CD79a, PAX5, CD19, CD138, and MUM1. Nine (8%) cases were peripheral T/NK-cell lymphomas, where at least CD43, CD45RO, or cytotoxic molecules were positive; 4, 3, and 2 cases were extranodal NK/T-cell lymphoma, nasal type, peripheral T-cell lymphoma-not otherwise specified, and adult T-cell leukemia/lymphoma, respectively. The remaining 25 (21%) cases included 11, 8, and 6 cases of myeloid sarcoma, blastic plasmacytoid dendritic cell neoplasm, and B- or NK-cell lymphoblastic leukemia/lymphoma, respectively. For large B-cell lymphoma/plasmablastic lymphoma diagnosis, MUM1 (92%) was the most sensitive marker, followed by CD79a (63%), PAX5 (52%), CD138 (42%), and CD19 (36%). EBER 1 and HHV8 were positive in 32% and 0% of the cases. For peripheral T/NK-cell lymphomas other than ALCL, CD45RO and CD43 were positive in nine cases; however, cytotoxic molecules (TIA1, 86%; granzyme B, 71%) were the most sensitive markers. In conclusion, most cases of the 118 (2.4%) CD3 CD20 extramedullary leukemia/lymphoma were represented by anaplastic large cell lymphomas (41%). The second most frequent group of neoplasia, large B-cell lymphoma/plasmablastic lymphoma (30%), characterized a special diagnostic challenge when B-cell markers were not expressed, requiring immunohistochemistry for multiple B-cell markers and molecular analysis in some cases.
组织病理学诊断对于罕见的 CD3 和 CD20 阴性髓外白血病/淋巴瘤具有挑战性。我们报告了 118 例 CD3-CD20 髓外白血病/淋巴瘤（占 4977 例的 2.4%）。68%的病例 CD45 阳性。49 例（41%）为间变大细胞淋巴瘤。35 例（30%）为大 B 细胞淋巴瘤/浆母细胞淋巴瘤，至少有一种以下标志物阳性：CD79a、PAX5、CD19、CD138 和 MUM1。9 例（8%）为外周 T/NK 细胞淋巴瘤，至少 CD43、CD45RO 或细胞毒性分子阳性；4、3 和 2 例分别为结外 NK/T 细胞淋巴瘤，鼻型、外周 T 细胞淋巴瘤-非特指型和成人 T 细胞白血病/淋巴瘤。其余 25 例（21%）包括 11 例髓样肉瘤、原始浆细胞样树突细胞肿瘤、B 或 NK 细胞淋巴母细胞白血病/淋巴瘤，分别为 8 例、6 例。对于大 B 细胞淋巴瘤/浆母细胞淋巴瘤的诊断，MUM1（92%）是最敏感的标志物，其次是 CD79a（63%）、PAX5（52%）、CD138（42%）和 CD19（36%）。EBER1 和 HHV8 在 32%和 0%的病例中阳性。对于非 ALCL 的外周 T/NK 细胞淋巴瘤，9 例 CD45RO 和 CD43 阳性；然而，细胞毒性分子（TIA1，86%；颗粒酶 B，71%）是最敏感的标志物。总之，118 例（2.4%）CD3-CD20 髓外白血病/淋巴瘤中，大多数（41%）为间变大细胞淋巴瘤。第二个最常见的肿瘤组为大 B 细胞淋巴瘤/浆母细胞淋巴瘤（30%），当 B 细胞标志物不表达时，诊断极具挑战性，需要进行多种 B 细胞标志物的免疫组织化学和某些情况下的分子分析。
