Peripheral Tcell Lymphoma Unspecified

General: The designation "peripheral T-cell lymphoma, unspecified" (PTCLU) encompasses a heterogeneous group of mature T-cell neoplasms that do not meet criteria for one of the more distinct clinicopathologic entities described below. Overall, PTCLU accounts for approximately half of all mature T-cell lymphomas.83 In most cases, patients present primarily with nodal disease, although extranodal presentations also occur, including those with primary cutaneous disease.

Pathology: The histologic findings in PTCLU are very diverse. There is usually extensive effacement of the normal lymph node architecture by a diffuse proliferation of the malignant cells (Figure 52.15). Some cases consist of small- to intermediate-sized cells with only minimal cytologic atypia, such that distinction from T-zone hyperplasia can be difficult by morphology alone. In most cases, however, the malignant cells are intermediate to large in size, with irregular nuclear contours and vesicular chromatin.83 Frequently, the malignant cells exhibit prominent pale to clear cytoplasm. A subset of cases will include large, Reed-Sternberg-like cells, creating a differential diagnosis that includes Hodgkin's lymphoma.

Immunophenotype: In general, cases of PTCLU will express one or more T-cell antigens. Frequently,

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