Hypopigmented interface T‐cell dyscrasia: A form of cutaneous T‐cell dyscrasia distinct from hypopigmented mycosis fungoides

精神障碍 浆细胞失调 蕈样真菌病 病理 医学 皮肤病科 淋巴瘤 免疫学 等离子体电池 免疫球蛋白轻链 抗体 骨髓
作者
Cynthia M. Magro,Joshua W. Hagen,A. Neil Crowson,Yen Chen Liu,Martín C. Mihm,Natalie Drucker,Aminah H. Yassin
出处
期刊:Journal of Dermatology [Wiley]
卷期号:41 (7): 609-617 被引量:17
标识
DOI:10.1111/1346-8138.12458
摘要

Abstract Hypopigmentation in cutaneous T‐cell lymphoproliferative disease should not always be equated with hypopigmented mycosis fungoides ( MF ). A form of hypopigmented pre‐lymphomatous T‐cell dyscrasia falling under the designation of the so‐called hypopigmented interface variant of T‐cell dyscrasia has recently been proposed. The aim of the present study was to establish hypopigmented interface T‐cell dyscrasia as its own entity apart from other T‐cell dyscrasias and MF using a patient case series. Twenty four cases of hypopigmented interface T‐cell dyscrasia were identified in the dermatopathology database of Weill Medical College of Cornell University. There were 17 females and seven males (mean age, 36 years). In children and adolescents, the patients were most commonly of African American extraction. Truncal photo‐protected areas manifesting as large solitary patches or multiple smaller macules were characteristic; disease progression to MF occurred in only one patient. The lesions responded to topical steroids and light therapy. The pathology was defined by a cell poor interface associated with degeneration of keratinocytes and melanocytes, and by lymphocytes whose nuclei showed low‐grade cerebriform atypia, and which expressed a significant reduction in CD 7 and CD 62L expression. In 50% of the cases, the implicated cell type was of the CD 8 subset. Clonality was not identified. Hypopigmented interface T‐cell dyscrasia is a distinct entity separate from and rarely progressive to MF .
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