Primary Cutaneous T-Cell Lymphomas

Abstract

Primary cutaneous T-cell lymphomas (CTCLs) encompass a clinically and biologically heterogeneous group of non-Hodgkin lymphomas (NHLs) defined by clonal proliferation of skin-homing malignant T lymphocytes and natural killer cells. They account for up to 75% to 80% of all cutaneous lymphomas. The current WHO-EORTC classification of cutaneous lymphomas with primary cutaneous manifestations lists 13 entities. The most common subtypes-mycosis fungoides, Clinical featuresMF is typically a chronic, slowly progressing disease with an indolent evolution. The disease is characterized by the development of patches, plaques or tumors (Figure 1; see Color Figures, page 513). The typical patch is a flat, annular, scaly skin lesion mimicking eczema, psoriasis, or dermatophyte infection. It is often erythematous to violaceous in color and has a predilection for sun-protected areas, including the lower abdomen, upper thighs, buttocks, and female breasts. These initial lesions may spontaneously regress and recur. Patches are highly variable and can give rise to or coexist with plaques, which are raised, palpable skin lesions (Figure 1a and 1b

Diagnostic microRNA profiling in cutaneous T-cell lymphoma (CTCL)

“…3,4 The malignant T cells typically exhibit the phenotype of mature CD4 ϩ memory T cells and are normally of clonal origin. 5 T cells with a malignant phenotype are characterized by epidermotropism and are preferentially present in the upper parts of the skin, whereas T cells with a normal phenotype primarily are detected in the lower portions of the dermis. The epidermal T cells are sometimes found in patterns of Pautrier microabscesses that are collections of T cells adherent to dendritic processes of Langerhans cells.…”

Primary cutaneous CD30+ anaplastic large cell lymphoma: report of a rare case

“…The t(2;5)(p23;q35) translocation and its variants is rarely found in PCALCL. 1,6 The appearance of large atypical cells on histopathology alone in patients with lymphoma does not always connote a poor prognosis. Immunohistochemical markers are essential to know the exact type of lymphoma because the prognosis will largely depend on this.…”

“…6 Primary cutaneous CD30+ lymphoproliferative disorders, accounts for 30% of the CTCL. 1 Under the group of primary cutaneous CD30+ lymphoproliferative disorders, the following diseases are included: primary cutaneous anaplastic large cell lymphoma (PCALCL), lymphomatoid papulosis (LyP) and borderline cases.…”

Linfoma subcutâneo de células T paniculite-símile

“…São definidos como proliferação clonal maligna de linfócitos T ou células natural killer (NK) residentes na pele. [1][2][3][4] A atual classificação da OMS para os LCCT lista 13 entidades. Micose fungoide e suas variações, síndrome de Sézary, linfoma cutâneo de grandes células anaplásicas e papulose linfomatoide representam aproximadamente 95% dos LCCT.…”

“…Micose fungoide e suas variações, síndrome de Sézary, linfoma cutâneo de grandes células anaplásicas e papulose linfomatoide representam aproximadamente 95% dos LCCT. 1,4 O linfoma subcutâneo de células T paniculitesímile (LSCTPS) é um tipo incomum de LCCT que foi reconhecido como entidade clínico-patológica apenas em 1991. Até 2003, menos de 200 casos haviam sido relatados na literatura de língua inglesa.…”