Micosis fungoide granulomatosa con linfadenopatías sarcoidales no neoplasicas: reporte de caso y revisiónde literatura / Granulomatous mycosis fungoides associated to sarcoid-like limphadenopathies non neoplasic

RESUMEN

Presentamos el caso de una paciente mujer quien desarrolla un episodio de eritrodermia y adenopatías, luego de muchos años, lesiones localizadas en placa compatibles con micosis fungoide granulomatosa presentando una reacción granulomatosa sarcoidal en ganglios durante un periodo de remisión de la enfermedad. La extensión extra cutánea se puede observar en un tercio de los pacientes con micosis fungoide yse asocia a transformación a linfoma anaplásico de células grandes CD30. Otras explicaciones al desarrollo de granulomas sarcoideos son el desarrollo de sarcoidosis concomitante, asociada o no al linfoma y reacciones sarcoidosis like (en respuesta a antígenos o citoquinas tumorales; en relacióna drogas como la bleomicina, contraste). Algunos autores proponen la existencia de un síndrome linfoma sarcoidosis caracterizado por sarcoidosis activa crónica que inicia el cuadro linfoproliferativo luego de la sarcoidosis. En el caso de nuestra paciente se desarrollan las adenopatías durante un periodo de remisión de la enfermedad en las que no se observa infiltración linfomatoide neoplasica, esto nos orienta a pensar que se trata de una reacción sarcoidosis like ganglionar secundaria a la liberación de citoquinas y antígenos tumorales luego de la radioterapia o que podría tratarse de un caso de sarcoidosisasociada incipiente.

ABSTRACT

Here we report the case of a female patient who developed an episode of eritrodermia and adenopathies, after many years focus injuries in plate compatible with granulomatous mycosis fungoides presenting a granulomatous sarcoid reaction in ganglia during the sickness remission period. The extra cutaneous extension can be present in one third of the patients with mycosis fungoides and associated with the big cells CD 30+ anaplastic large cell lymphoma. Other explanations to the granulomatous sarcoides development are the sarcoidosis consistent development, associated or not to the lymphoma and reactions sarcoidosis-like (in response to antigens or tumoral cytokines; related to drugs like bleomicine, contrast). Some authors propose the existence of a syndrome sarcoidosislymphoma characterized by chronic active sarcoidosis that starts the lymphoproliferative scheme after the sarcoidosis. In our case, the patient develops the adenopathies during a sickness remission period which does not show neoplasic lymphomatoid infiltration; that suggest being a sarcoidosis reaction like ganglionar secondary to the cytokines and tumoral antigens liberation after radiotherapy that mey be a incipient case of sarcoidosis associated. Here we report the case of a female patient who developed an episode of eritrodermia and adenopathies, after many years focus injuries in plate compatible with granulomatous mycosis fungoides presenting a granulomatous sarcoid reaction in ganglia during the sickness remission period. The extra cutaneous extension can be present in one third of the patients with mycosis fungoides and associated with the big cells CD 30+ anaplastic large cell lymphoma. Other explanations to the granulomatous sarcoides development are the sarcoidosis consistent development, associated or not to the lymphoma and reactions sarcoidosis-like (in response to antigens or tumoral cytokines; related to drugs like bleomicine, contrast). Some authors propose the existence of a syndrome sarcoidosis-lymphoma characterized by chronic active sarcoidosis that starts the lymphoproliferative scheme after the sarcoidosis. In our case, the patient develops the adenopathies during a sickness remission period which does not show neoplasic lymphomatoid infiltration; that suggest being a sarcoidosis reaction like ganglionar secondary to the cytokines and tumoral antigens liberation after radiotherapy that mey be a incipient case of sarcoidosis associated.