Primary Cutaneous CD30+ Lymphoproliferative Disorders

Calzado-Villarreal, L.; Polo-Rodríguez, I.; Ortiz-Romero, P.L.

doi:10.1016/S1578-2190(10)70598-9

Article information

Abstract

CD30+ lymphoproliferative disorders are the most common group of cutaneous T-cell lymphomas after mycosis fungoides and its subtypes. This group includes lymphomatoid papulosis and CD30+ anaplastic large-cell lymphoma; these 2 entities are the extremes of a spectrum with numerous intermediate varieties in which it is not possible to establish a clear diagnosis based on clinical and histopathologic criteria. CD30+ lymphoproliferative disorders must be differentiated from other lymphoproliferative diseases with CD30+ cells in the tumor infiltrates, such as mycosis fungoides or Hodgkin disease, and also from other inflammatory conditions or nonhematological neoplasms that can include this cell type, such as pityriasis lichenoides et varioliformis acuta or certain mesenchymal tumors (CD30+ pseudolymphomas). In contrast to their systemic homologues, which arise in the lymph nodes, CD30+ lymphoproliferative disorders generally have a good prognosis. It is very important to exclude the presence of a lymphoma of systemic origin with extralymphatic spread, as the prognosis and treatment are different.

Keywords:

Cutaneous lymphomas

CD30+ lymphoproliferative disorders

Lymphomatoid papulosis

CD30+ anaplastic large-cell lymphomas

Resumen

El segundo grupo más frecuente de linfomas cutáneos de células T son los síndromes linfoproliferativos (SLP) CD30+, por detrás del grupo de la micosis fungoide (MF) y sus variantes. Estos engloban la papulosis linfomatoide y los linfomas anaplásicos de células grandes CD30+, polos de un espectro que dejan en su zona central casos intermedios, donde no se puede establecer con seguridad un diagnóstico en base a criterios clínicos e histopatológicos.

Los SLP CD30+ deben diferenciarse de otros procesos linfoproliferativos que pueden presentar células CD30+ en sus infiltrados tumorales, como la propia MF o la enfermedad de Hodgkin, y también de otras entidades inflamatorias o neoplasias no hematológicas que presenten estas células, como es el caso de la pitiriasis liquenoide y varioliforme aguda o determinados tumores mesenquimales («pseudolinfomas CD30+»).

En general, el pronóstico de estos SLP CD30+ es favorable, lo que les diferencia de sus homólogos sistémicos, de origen ganglionar. Resulta muy importante descartar la presencia de linfoma de origen sistémico con afectación extraganglionar, pues el pronóstico y el tratamiento serán diferentes.

Palabras clave:

Linfomas cutáneos

Síndrome linfoproliferativo CD30+

Papulosis linfomatoide

Linfoma anaplástico de célula grande CD30+

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