Both adult T-cell leukemia/lymphoma (ATL) and human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) can be induced by HTLV-1, but concurrent development has been rarely reported. We present the case of a 55-year-old female who developed cranial nerve symptoms after a 20-year history of HAM/TSP. Although multiple white matter lesions were observed on brain magnetic resonance imaging, no abnormalities were seen on a systemic computed tomography scan. Quantitative flow-cytometric analysis of cell populations in the cerebrospinal fluid (CSF) revealed that most of the infiltrating cells were not inflammatory cells, but HTLV-1-infected CD4+ CADM-1+ T-cells completely lacking CD7 expression. As stepwise downregulation of CD7 is correlated with disease progression from HTLV-1 carrier to aggressive ATL, the CSF cells were classified as aggressive ATL; these cells exhibited a more progressed phenotype than those in peripheral blood (PB). HAM/TSP disease activity was estimated to be low. From these and other examinations, we made a diagnosis of acute-type ATL, which unusually developed in the central nervous system at initial onset prior to systemic progression. In ATL cases with a challenging diagnosis, immunophenotypic characterization of CSF and PB is valuable for differential diagnosis and understanding disease status.

中文翻译：

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脑脊液的免疫表型分析揭示了 HAM/TSP 患者的 CNS 中 ATL 的同时发展。

成人 T 细胞白血病/淋巴瘤 (ATL) 和人类 T 细胞白血病病毒 1 型 (HTLV-1) 相关的脊髓病/热带痉挛性截瘫 (HAM/TSP) 均可由 HTLV-1 诱导，但同时发展已很少报道。我们介绍了一名 55 岁女性的病例，她在 20 年的 HAM/TSP 病史后出现颅神经症状。尽管在脑磁共振成像中观察到多个白质病变，但在全身计算机断层扫描中未发现异常。脑脊液 (CSF) 中细胞群的定量流式细胞术分析显示，大多数浸润细胞不是炎症细胞，而是 HTLV-1 感染的 CD4+ CADM-1+ T 细胞，完全缺乏 CD7 表达。由于 CD7 的逐步下调与从 HTLV-1 携带者到侵袭性 ATL 的疾病进展相关，CSF细胞被归类为侵袭性ATL；这些细胞表现出比外周血 (PB) 中更先进的表型。HAM/TSP 疾病活动度估计较低。通过这些检查和其他检查，我们诊断出急性型 ATL，它在全身性进展之前的初始发作时不寻常地在中枢神经系统中发展。在具有挑战性诊断的 ATL 病例中，CSF 和 PB 的免疫表型特征对于鉴别诊断和了解疾病状态很有价值。在全身进展之前的初始发作时，在中枢神经系统中异常发展。在具有挑战性诊断的 ATL 病例中，CSF 和 PB 的免疫表型特征对于鉴别诊断和了解疾病状态很有价值。在全身进展之前的初始发作时，在中枢神经系统中异常发展。在具有挑战性诊断的 ATL 病例中，CSF 和 PB 的免疫表型特征对于鉴别诊断和了解疾病状态很有价值。