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成人T细胞白血病/淋巴瘤在临床上易与病毒/药物性皮肤疹相混淆,在病理上易被误诊为蕈样肉芽肿/赛塞里综合征。

Adult T-cell leukemia/lymphoma clinically confused with viral/drug skin eruptions and pathologically misinterpreted as mycosis fungoides/Sézary syndrome.

作者信息

AbdullGaffar Badr, Abdulrahman Suad

机构信息

Pathology section, Rashid hospital, Dubai, United Arab Emirates.

出版信息

J Cutan Pathol. 2021 Sep;48(9):1103-1108. doi: 10.1111/cup.13789. Epub 2020 Dec 22.

DOI:10.1111/cup.13789
PMID:33354782
Abstract

Adult T-cell leukemia/lymphoma (ATLL) is a lymphoproliferative disorder of mature CD4-positive T-cell lymphoid cells associated with retrovirus human T-lymphotropic virus type-1 (HTLV-1) with a wide clinical and pathologic spectrum. We report a case of a 53-year-old African man who presented with fever and skin eruptions on the trunk composed of non-itchy erythematous reticulated macules and papules initially suspected for viral exanthem or drug rash. Skin punch biopsy showed a dermal T-cell lymphoid infiltrate with epidermotropism. The patient developed generalized lymphadenopathy and his peripheral blood showed lymphocytosis with atypical lymphocytes with convoluted nuclei. Our initial diagnosis was mycosis fungoides with Sézary syndrome. However, some clinical and histopathologic features were unusual. The acute onset, lack of previous skin lesions, the histomorphologic features of the dermal, nodal and peripheral blood lymphocytes and the geographic origin of the patient raised the suspicion of other T-cell lymphomas, particularly ATLL. This was confirmed by a positive anti-HTLV-1 serology. Our final diagnosis was acute variant ATLL. Different T-cell lymphomas can involve the skin with overlapping clinical, histomorphologic and immunohistochemical features. Some clinical and pathologic features should alarm dermatologists and pathologists to the possibility of ATLL particularly in patients from HTLV-1 endemic geographic areas.

摘要

成人T细胞白血病/淋巴瘤(ATLL)是一种成熟CD4阳性T细胞淋巴细胞增殖性疾病,与逆转录病毒人类T淋巴细胞病毒1型(HTLV-1)相关,具有广泛的临床和病理谱。我们报告一例53岁非洲男性病例,该患者最初表现为发热以及躯干皮肤出现皮疹,皮疹由不痒的红斑网状斑疹和丘疹组成,最初怀疑为病毒疹或药疹。皮肤穿刺活检显示真皮T细胞淋巴细胞浸润伴亲表皮现象。患者出现全身淋巴结肿大,外周血显示淋巴细胞增多,伴有核卷曲的非典型淋巴细胞。我们最初的诊断是蕈样肉芽肿伴塞扎里综合征。然而,一些临床和组织病理学特征并不寻常。急性起病、既往无皮肤病变、真皮、淋巴结和外周血淋巴细胞的组织形态学特征以及患者的地理来源引发了对其他T细胞淋巴瘤的怀疑,尤其是ATLL。抗HTLV-1血清学阳性证实了这一点。我们最终的诊断是急性变异型ATLL。不同的T细胞淋巴瘤可累及皮肤,具有重叠的临床、组织形态学和免疫组化特征。一些临床和病理特征应提醒皮肤科医生和病理科医生注意ATLL的可能性,特别是在来自HTLV-1流行地区的患者中。

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