[Hypopigmented mycosis fungoides in children: a clinicopathological study of 6 cases].

ABSTRACT

OBJECTIVE: To explore the clinical and histopathological manifestations, therapy and prognosis of hypopigmented mycosis fungoides (HMF) in children. METHODS: Six pediatric cases of HMF from February 2008 to October 2009 were reviewed and analyzed at our hospital. RESULTS: There were 5 males and 1 female with a average age of 11 years old (range 9-14). The lesions appeared poorly-demarcated hypopigmented macula and patches disseminated over the trunk and extremities, scales and infiltrated papules over the hypopigmented lesions or invasive erythemas arose and enlarged gradually. Histologically, there was an epidermal infiltration of atypical lymphocytes. The immunohistochemical staining revealed a pattern of T lymphoma and a predominance of CD8+ T cell. All patients were of T2N0M0/IB except for one at the stage of T1N0M0/IA. Two patients received narrow band ultraviolet B (NB-UVB), one NB-UVB plus alpha interferon, another NB-UVB plus alpha interferon and topical steroid, one Chinese traditional herbal regimen while the last one none. And NB-UVB alone or plus alpha interferon achieved a clinical complete response (CR). The herbal regimen failed to cure the lesions but achieved no response. The non-treated patient progressed gradually. The average follow-up was 16 months (range 9-28). The progression-free rate was 83.3% and the overall survival rate 100%. CONCLUSIONS: Visceral and lymph note involvements are rare in HMF of children. The prognosis of early-stage treatment is satisfactory. NB-UVB alone or plus alpha interferon achieves a clinical CR in most early-stage patients.