Demographic patterns of cutaneous T-cell lymphoma incidence in Texas based on two different cancer registries.

Cutaneous T-cell lymohomas (CTCLs) are rare, but potentially devastating malignancies, with Mycosis fungoides and Sézary Syndrome being the most common. In our previous study, we identified and described regions of geographic clustering of CTCL cases in Texas by analyzing ~1990 patients using two distinct cancer registries. In the current work, we describe in detail demographic patterns for this malignancy in our study population and apply logistic regression models to analyze the incidence of CTCL by sex, race, age, and clinical stage at the time of diagnosis. Furthermore, using Fisher's exact test, we analyze changes in incidence over time in the identified Houston communities with unusually high CTCL incidence. While CTCL primarily affects Caucasian individuals >55 years old, we confirm that it presents at a younger age and with more advanced disease stages in African-American and Hispanic individuals. Also, we demonstrate a significant increase in CTCL incidence over time in the identified communities. Spring, Katy, and Houston Memorial areas had high baseline rates. Furthermore, a statistically significant disease surge was observed in these areas after ~2005. This report supplements our initial study documenting the existence of geographic clustering of CTCL cases in Texas and in greater detail describes demographic trends for our patient population. The observed surge in CTCL incidence in the three identified communities further argues that this malignancy may be triggered by one or more external etiologic agents.

Papulosis linfomatoide en una niña de 23 meses / Lymphomatoid papulosis in a 23-month-old child

La papulosis linfomatoide (PL) es un linfoma cutáneo de células T de bajo grado; consiste en una erupción crónica, recurrente y autolimitada de pápulas y pequeños nódulos. Las lesiones predominan en tronco y región proximal de extremidades, pudiendo aparecer en distinto estadio evolutivo simultáneamente. Esta enfermedad afecta principalmente a adultos jóvenes, siendo muy rara en niños. Presentamos un caso de una niña de 23 meses de edad que tras un período de seguimiento de 10 meses presentó dos brotes de lesiones papulonodulares ulceradas, de predominio en tronco y miembros, que remitieron tras aplicación de un corticosteroide tópico. El estudio histopatológico reveló un linfoma T CD30 positivo, compatible con PL tipo A, siendo el reordenamiento de genes del receptor de células T policlonal, tanto en piel como en sangre periférica. Aunque la PL está bien descrita en adultos, el curso clínico, pronóstico, riesgo de linfoma y recomendaciones de seguimiento no han sido establecidas en niños. Las terapéuticas empleadas en adultos no suelen ser bien toleradas en pacientes pediátricos; además, como el tratamiento no modifica el riesgo de malignidad, estos niños requieren un seguimiento continuo a lo largo de su vida por el riesgo permanente de desarrollar otro tipo de linfoma (AU)

Historical perspective on the use of retinoids in cutaneous T-cell lymphoma (CTCL).

Vitamin A and its analogues influence differentiation and proliferation and may also alter immune responses. Limited clinical efficacy of these compounds given alone or as part of a combination therapy has been shown in various types of cutaneous T-cell lymphoma (CTCL), including mycosis fungoides, Sézary syndrome, and prelymphomatous disorders such as parapsoriasis en plaques. Compounds used mostly in small, nonrandomized trials are isotretinoin (13-cis-retinoic acid), etretinate, acitretin, and all-trans-retinoic acid. Clinical responses have been found despite persistent residual disease with atypical lymphocytes in various compartments. The exact mechanism of action of retinoids in CTCL is unclear and depends on the presence of retinoid receptors on the tumor cells, which is variable in different forms of CTCL. Therapies combining retinoids with psoralen-ultraviolet A or with interferons may have a synergistic effect, which deserves confirmation through randomized trials in the future.

Epidemiology and clinical manifestations of cutaneous T-cell lymphoma.

Cutaneous T-cell lymphoma (CTCL) encompasses a constellation of diseases of malignant clonal T lymphocytes that present initially in the skin. Within this disease spectrum, mycosis fungoides, and the Sézary syndrome are best known. Progress in the understanding of the T-cell malignancies now places other clinical entities within this disease classification. The authors review the epidemiology and clinical manifestations of CTCL.