Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: A case series of 34 patients - 15/11/14
Abstract |
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.
Le texte complet de cet article est disponible en PDF.Key words : cutaneous T-cell lymphoma, immunopathology, juvenile onset, mycosis fungoides, narrowband ultraviolet B radiation, presentation, treatment, vitamin-D deficiency
Abbreviations used : MF, NB, PUVA, TCR, UV
Plan
Supported by the Sherry L. Anderson Research Fund. |
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Conflicts of interest: None declared. |
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Reprints not available from the authors. |
Vol 71 - N° 6
P. 1117-1126 - décembre 2014 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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