Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: A case series of 34 patients  

Oleh:

Boulos, Scarlett ; Vaid, Reena ; Aladily, Tariq N. ; Ivan, Doina S. ; Talpur, Rakhshandra

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: JAAD: Journal of the American Academy of Dermatology (keterangan: ada di ClinicalKey) vol. 71 no. 06 (Dec. 2014), page 1117–1126 .
Topik: cutaneous T-cell lymphoma; immunopathology; juvenile onset; mycosis fungoides; narrowband ultraviolet B radiation; presentation; treatment; vitamin-D deficiency

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Perpustakaan FK Nomor Panggil: J15.K Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

Background Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, typically presents in middle-aged to elderly individuals. Objective We sought to study the demographics, clinicopathologic features, treatment response, and prognosis of patients with biopsy-proven MF diagnosed before 20 years of age. Methods Patients were identified from a prospectively collected database for retrospective analysis. Results Of 1902 patients with MF, 34 had juvenile-onset MF: 41% were stage IA, 56% were stage IB, and 3% were stage IIB at diagnosis. The male to female ratio was 1.1:1. The median age of symptom onset was 9 years (range 3-19 years), with a delay in diagnosis between 1 month and 14 years. Patients primarily presented with hypopigmented (53%), hyperpigmented (29%), and pink-violaceous (41%) patches/plaques. Immunohistochemistry revealed 39% with CD8+ immunophenotype, 67% of which had hypopigmented lesions. The phototherapy response rate in 21 patients was 81%. All patients who completely responded to narrowband ultraviolet B phototherapy had hypopigmented MF. Limitations This is a single cancer center study. Conclusion Juvenile-onset MF presents with early-stage disease with an overrepresentation of hypopigmented MF and CD8+ immunophenotype. Narrowband ultraviolet B is an effective treatment option for juveniles, especially for those with the hypopigmented variant.

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