Linfoma primario cutáneo de células T epidermotropo agresivo CD8+ / Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma

El linfoma de células T epidermotropo agresivo CD8+ o linfoma de Berti es un subtipo infrecuente de los linfomas primarios cutáneos, descripto en 1999. Aún se considera una entidad provisional en la última clasificación de linfomas cutáneos primarios de la Organización Mundial de la Salud. Predomina en los hombres adultos y se manifiesta con pápulas, placas hiperqueratósicas y tumores ulcerados, diseminados y de inicio agudo. En la histopatología, es característica la presencia de un infiltrado de linfocitos atípicos medianos CD8 +, con epidermotropismo de patrón pagetoide. Tiene un comportamiento agresivo y es de mal pronóstico, con una sobrevida a los 5 años menor del 40%. Si bien esta entidad es un reto terapéutico, el tratamiento inicial se basa en poliquimioterapia y/o trasplante de células hematopoyéticas.

The aggressive epidermotropic CD8+ T-cell lymphoma or Berti's lymphoma, is a rare subtype of primary cutaneous lymphomas, first described in 1999. It is still considered a provisional entity by the latest World Health Organization classification of cutaneous lymphomas. Patients are commonly adults with a male predominance and it is characterized by widespread rapid evolving papules, hyperkeratotic annular plaques and ulcerated tumors. Histopathologicfindingsarecharacteristic,withaninfiltrate of medium-sized CD8+ atypical lymphocytes involving the epidermis with a pagetoid pattern. Ithasanaggressivebehaviorandtheprognosisispoor with a 5-yearsurvival less than 40%. Instead these disease represents a therapeutic challenge, the initial treatment consists on polychemotherapy and hematopoietic stem cell transplantation.

Primary cutaneous folliculotropic and lymphohistiocytic anaplastic large cell lymphoma.

BACKGROUND: Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a T-cell lymphoma that presents in the skin and consists of CD30+ anaplastic large lymphocytes with abundant cytoplasm and pleomorphic nuclei. In addition to the classic variant, several other histologic patterns of ALCL have been identified, among them the lymphohistiocytic variant. OBJECTIVE AND METHODS: We describe a case of the lymphohistiocytic variant of PC-ALCL with a marked follicular involvement. The lesion appeared as a single nodule in the face that completely regressed following biopsy. A diffuse infiltration of lymphocytes and macrophages was seen in the dermis associated with hypertrophied hair follicles, follicular mucinosis, and marked folliculotropism, leading to follicular disruption. Cohesive groups of CD30+ large, atypical lymphocytes with a high proliferative index were seen focally. The patient was followed up for 41 months, during which time no relapses occurred. CONCLUSIONS: This case shows that PC-ALCL may present with different histologic features, including a follicular variant, that may mimic both benign and malignant conditions.

Primary cutaneous eosinophil-rich anaplastic large cell lymphoma: report of an unusual case and literature review.

BACKGROUND: Cutaneous, neutrophil-rich anaplastic large cell lymphoma (ALCL) is an uncommon variant of ALCL that may be confused with inflammatory dermatoses. OBJECTIVE AND METHODS: We describe an eosinophil-rich variant of ALCL occurring on the left ear without systemic involvement. The lesion had inflammatory characteristics, which led initially to a histological diagnosis of an inflammatory process. Two months later, a second biopsy diagnosed eosinophil-rich variant of ALCL. The patient underwent radiation therapy, but due to the extensive deformation and mutilation of the ear, the patient elected to have the ear amputated. We discuss the clinicopathological findings and the differential diagnosis CONCLUSIONS: To the best of our knowledge, the occurrence of a cutaneous, eosinophil-rich variant of ALCL has not been previously reported. It is important to alert pathologists to this variant of ALCL so that this possibility may be considered in the early differential diagnosis of inflammatory cutaneous conditions.