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[Hypopigmented mycosis fungoides in children: a clinicopathological study of 6 cases].
Zhonghua Yi Xue Za Zhi. 2010 Dec 14; 90(46):3287-90.ZY

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.

Authors+Show Affiliations

Department of Dermatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

English Abstract
Journal Article

Language

chi

PubMed ID

21223789

Citation

Wang, Tao, et al. "[Hypopigmented Mycosis Fungoides in Children: a Clinicopathological Study of 6 Cases]." Zhonghua Yi Xue Za Zhi, vol. 90, no. 46, 2010, pp. 3287-90.
Wang T, Liu YH, Zheng HY, et al. [Hypopigmented mycosis fungoides in children: a clinicopathological study of 6 cases]. Zhonghua Yi Xue Za Zhi. 2010;90(46):3287-90.
Wang, T., Liu, Y. H., Zheng, H. Y., Sun, Q. N., Jin, H. Z., Li, F., Fang, K., & Yan, Y. (2010). [Hypopigmented mycosis fungoides in children: a clinicopathological study of 6 cases]. Zhonghua Yi Xue Za Zhi, 90(46), 3287-90.
Wang T, et al. [Hypopigmented Mycosis Fungoides in Children: a Clinicopathological Study of 6 Cases]. Zhonghua Yi Xue Za Zhi. 2010 Dec 14;90(46):3287-90. PubMed PMID: 21223789.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Hypopigmented mycosis fungoides in children: a clinicopathological study of 6 cases]. AU - Wang,Tao, AU - Liu,Yue-hua, AU - Zheng,He-yi, AU - Sun,Qiu-ning, AU - Jin,Hong-zhong, AU - Li,Feng, AU - Fang,Kai, AU - Yan,Yan, PY - 2011/1/13/entrez PY - 2011/1/13/pubmed PY - 2011/9/16/medline SP - 3287 EP - 90 JF - Zhonghua yi xue za zhi JO - Zhonghua Yi Xue Za Zhi VL - 90 IS - 46 N2 - 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. SN - 0376-2491 UR - https://www.unboundmedicine.com/medline/citation/21223789/[Hypopigmented_mycosis_fungoides_in_children:_a_clinicopathological_study_of_6_cases]_ L2 - http://journal.yiigle.com/LinkIn.do?linkin_type=pubmed&issn=0376-2491&year=2010&vol=90&issue=46&fpage=3287 DB - PRIME DP - Unbound Medicine ER -