Gatti Arianna, Franceschetti Silvia, Speziale Valentina, Valli Viviana Beatrice, Draisci Michela, Campidelli Cristina, Brando Bruno, Cuppari Irene, Corso Alessandro
Hematology Laboratory and Transfusion Center Department, ASST Ovest Milanese-Ospedale Legnano, 20025 Legnano (Milan), Italy.
Hematology Department, ASST Ovest Milanese-Ospedale Legnano, 20025 Legnano (Milan), Italy.
J Med Cases. 2025 May;16(5):181-186. doi: 10.14740/jmc5114. Epub 2025 May 28.
Nodal T-follicular helper cell lymphoma angioimmunoblastic type (nTFHL-AI) is a rare and aggressive neoplasm of mature T-follicular helper cells. nTFHL-AI is characterized by polyclonal hypergammaglobulinemia, hemolytic anemia, circulating immune complexes, and cold agglutinins. nTFHL-AI is also often associated with B-cell or plasma cell expansion, mimicking B-cell lymphomas or plasma cell neoplasms. Therefore, the diagnosis of nTFHL-AI can sometimes be challenging and requires a specific immunophenotypic panel. However, the peripheral blood involvement in nTFHL-AI seems rare and has not been frequently addressed in the literature. We report the case of a 54-year-old man with multiple lymphadenopathies, hepatosplenomegaly, and skin rash, complaining of asthenia. Peripheral blood smear showed plasmacytoid cells and red cell rouleaux. A first flow cytometry screening panel of peripheral blood disclosed marked polyclonal plasmacytosis (12%). No mature B lymphocytes were detectable. In the suspicion of an nTFHL-AI, another flow cytometric panel was performed, including CD3, CD4, CD5, CD7, CD8, and CD10. The high-sensitivity flow cytometry analysis disclosed a small circulating population of atypical T cells (0.07%) expressing CD4, CD3, CD5, CD10, partially CD7, and negative for CD8. Moreover, anti-TCRβ-chain constant region 1 (TRBC1) antibody (JOVI-1) was used to confirm the T-cell clonal restriction of this abnormal population. Immunohistochemistry on excised lymph node sections was carried out and confirmed the diagnosis of nTFHL-AI. In this case, the unexpected detection of a small circulating population of nTFHL-AI cells by high-sensitivity flow cytometry has prompted an extensive diagnostic workup leading rapidly to the correct diagnosis.
结节性T滤泡辅助细胞淋巴瘤血管免疫母细胞型（nTFHL-AI）是一种罕见的成熟T滤泡辅助细胞侵袭性肿瘤。nTFHL-AI的特征为多克隆高球蛋白血症、溶血性贫血、循环免疫复合物和冷凝集素。nTFHL-AI还常与B细胞或浆细胞增殖相关，类似B细胞淋巴瘤或浆细胞瘤。因此，nTFHL-AI的诊断有时具有挑战性，需要特定的免疫表型检测组合。然而，nTFHL-AI累及外周血似乎罕见，文献中也未经常提及。我们报告一例54岁男性病例，有多处淋巴结肿大、肝脾肿大和皮疹，伴有乏力。外周血涂片显示浆细胞样细胞和红细胞缗钱状排列。外周血首次流式细胞术筛查检测组合显示明显的多克隆浆细胞增多（12%）。未检测到成熟B淋巴细胞。怀疑为nTFHL-AI时，进行了另一项流式细胞术检测组合，包括CD3、CD4、CD5、CD7、CD8和CD10。高灵敏度流式细胞术分析发现一小群循环中的非典型T细胞（0.07%），表达CD4、CD3、CD5、CD10，部分表达CD7，CD8阴性。此外，使用抗TCRβ链恒定区1（TRBC1）抗体（JOVI-1）确认该异常细胞群的T细胞克隆限制性。对切除的淋巴结切片进行免疫组织化学检查，确诊为nTFHL-AI。在该病例中，通过高灵敏度流式细胞术意外检测到一小群循环中的nTFHL-AI细胞，促使进行了广泛的诊断检查，迅速得出正确诊断。
