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Cutaneous Lymphoma: Kids are not just little people

Research paper by Katalin Ferenczi, Hanspaul S. Makkar

Indexed on: 05 Oct '16Published on: 10 Jul '16Published in: Clinics in Dermatology



Abstract

Cutaneous T-cell lymphomas (CTCL) are non-Hodgkin's lymphomas that predominantly affect older patients. Onset of cutaneous lymphoma in childhood is rare but it can present as early as the first decade of life. In both adults and children, the diagnosis of cutaneous lymphoma can be challenging as inflammatory dermatoses can mimic CTCL, both clinically and histologically. The clinicopathologic manifestations can be similar in adults and younger individuals, however, differences in the prevalence of certain CTCL variants among age groups exist. Whereas the classic, Alibert-Basin-type mycosis fungoides (MF) and Sézary syndrome represent the overwhelming majority of adult cutaneous T-cell lymphomas, in younger individuals, certain mycosis fungoides variants, such as hypopigmented MF is over-represented, and Sézary syndrome is extremely rare. CD30+ lymphoproliferative diseases, which include lymphomatoid papulosis (LYP) and primary cutaneous anaplastic large cell lymphoma (PCALCL) represent the second most common subtype of CTCL in both adults and children; however, in the pediatric population most of these are represented by LYP and PCALCL is very rare. The prognosis is stage dependent and the most significant prognostic factor in MF is the extent of skin involvement and the presence or absence of extracutaneous disease. The overwhelming majority of pediatric patients present with early stage disease and progression to more advanced stages, such as tumor stage, erythrodermic MF and large cell transformation, which can be seen in adults, are very rare. The choice of treatment regardless of age is dependent on the extent of skin involvement and the presence or absence of extracutaneous disease.

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