engleski

Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma : case report

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is described as primary cutaneous proliferation of epidermotropic CD 8+ cytotoxic T-cells. Middleaged to elderly patients are affected. An 80-year-old male patient with this rare lymphoma is presented. In this case, clinical picture was predominated by ulcerated and necrotic nodules in the regions of lower extremities, chest and penis. At onset seven months before hospital admission, the disease first manifested with discrete, weakly infiltrated plaques that evolved to ulcerative and necrotic tumors in the foot and lower leg regions. Ulcerous lesions of the corpus penis developed in the advanced stage of the disease, accompanied by severe pain and clinically resembling fulminant gangrene. On cytology, lymphocytic hyperplasia was detected in the enlarged inguinal lymph nodes. Biopsy specimens were obtained from four tumor infiltrates. In all slides, immunohistochemistry revealed moderately abundant inflammatory infiltrate predominantly consisting of small, medium and large T lymphocytes presenting CD3+, CD2-, CD8+, CD7+, CD4-, CD5-, CD30-, CD56-, granzyme B-, TIA-1 +, in the upper dermis and epidermis. CT of the thorax and abdomen showed enlargement of axillary, mediastinal and inguinal lymph nodes, yet not exceeding 1 cm. There was no enlargement of retroperitoneal lymph nodes. Sternal puncture, bone biopsy, peripheral blood count, liver enzymes and LDH were within the normal limits. Protein electrophoresis showed hypoalbuminemia and hypergammaglobulinemia. Upon consultation with a hematologist, CNOP polychemotherapy was initiated and planned to be administered in six cycles. After first cycle, the patient developed agranulocytosis and the cytostatic doses had to be tapered. Following third chemotherapy cycle, clinical improvement with partial regression of skin lesions was observed. Then the patient abandoned treatment by his own decision, so further course and outcome of the disease remained unknown.

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