Iluta Sabina, Termure Dragos-Alexandru, Petrushev Bobe, Fetica Bogdan, Badea Mindra-Eugenia, Moldovan-Lazar Madalina, Lenghel Manuela, Csutak Csaba, Roman Andrei, Pasca Sergiu, Zimta Alina-Andreea, Jitaru Ciprian, Tomuleasa Ciprian, Roman Rares-Calin
Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy, 400337 Cluj-Napoca, Romania.
Department of Hematology, Ion Chiricuta Clinical Cancer Center, 400124 Cluj-Napoca, Romania.
Diagnostics (Basel). 2020 Aug 24;10(9):629. doi: 10.3390/diagnostics10090629.
Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is the rarest subtype of primary cutaneous lymphoma, accounting for approximately 2% of cutaneous lymphomas. The rarity of primary cutaneous PTCL-NOS means that there is a paucity of data regarding clinical and histopathological features and its clinical course. This malignancy is an aggressive and life-threatening hematological malignancy that often presents mimicking other less severe plaque-like skin conditions. Due to the nonspecific nature of these lesions, CD4-positive cutaneous T-cell lymphoma (CTCL) is often misdiagnosed as either mycosis fungoides or Sezary syndrome. We describe a patient who presented with a large tumoral mass in the right frontal area, with involvement of the right upper eyelid and the ocular globe, causing loss of vision greatly impacting the quality of life. Biopsy revealed primary cutaneous PTCL-NOS, treated successfully with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) plus etoposide combination chemotherapy. As elderly patients are indicated to receive attenuated doses of chemotherapy, CHOP-based regimens represent viable options.
外周T细胞淋巴瘤,非特殊类型(PTCL-NOS)是原发性皮肤淋巴瘤中最罕见的亚型,约占皮肤淋巴瘤的2%。原发性皮肤PTCL-NOS的罕见性意味着关于其临床和组织病理学特征及其临床病程的数据很少。这种恶性肿瘤是一种侵袭性且危及生命的血液系统恶性肿瘤,常表现为类似其他不太严重的斑块样皮肤疾病。由于这些病变的非特异性,CD4阳性皮肤T细胞淋巴瘤(CTCL)常被误诊为蕈样肉芽肿或塞扎里综合征。我们描述了一名患者,其右额部出现一个巨大的肿瘤性肿块,累及右上眼睑和眼球,导致视力丧失,严重影响生活质量。活检显示为原发性皮肤PTCL-NOS,采用环磷酰胺、阿霉素、长春新碱和泼尼松(CHOP)加依托泊苷联合化疗成功治疗。由于老年患者需接受减量化疗,基于CHOP的方案是可行的选择。