cutaneous T cell lymphoma

(redirected from Cutaneous T-cell lymphomas)

cutaneous T cell lymphoma

Mycosis fungoides Oncology A heterogeneous group of relatively uncommon NHLs, which first appear on the skin Epidemiology ±1000 cases occur/yr, US, affecting ages 50-60; ♂:♀ ratio, 2:1; black:white, 2:1 Clinical Long–4-10 yrs Hx of eczematous rashes, which may evolve to parapsoriasis en plaque or poikiloderma atrophicans vasculare  Pathology Epidermotropism, paracortical proliferation in lymph nodes, periarterial proliferation in spleen; aggregates of epithelioid cells, prominent vascular channels, BM involvement Management Nitrogen mustard, BCNU, PUVA therapy, RT Response rate 80% if T1; 30–60% if T2; 20% if T3. See Redman syndrome, Sézary syndrome.
References in periodicals archive ?
"Cutaneous T-cell lymphomas like mycosis fungoides and Sezary syndrome are chronic skin malignancies characterized by relapsing and remitting behavior and progressive resistance to treatments.
Lymphomatoid papulosis (LyP) is a chronic papulonecrotic or papulonodular skin disease with histologic features suggestive of a malignant lymphoma.1 The term lymphomatoid papulosis originally was used by Macaulay in 1968 to describe "a self-healing rhythmical paradoxical eruption, histologically malignant but clinically benign." However, the classification system for cutaneous lymphomas has evolved rapidly, and, during consensus meetings in 2003-2004, the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification grouped lymphomatoid papulosis among the indolent cutaneous T-cell lymphomas. These classifications were updated in 2008.2-4
Non-mycosis fungoides cutaneous T-cell lymphomas: report of the 2011 Society for Hematopathology/European Association for Haematopathology Workshop.
Miranda et al., "Primary cutaneous T-cell lymphomas showing gamma-delta ([gamma][delta]) phenotype and predominantly epidermotropic pattern are clinicopathologically distinct from classic primary cutaneous [gamma][delta] T-cell lymphomas," American Journal of Surgical Pathology, vol.
Caption: Cutaneous T-cell lymphomas are non-Hodgkin lymphomas.
Primary cutaneous T-cell lymphomas (CTCLs) are a heterogeneous group of lymphomas that maybe present in the skin without any evidence of extracutaneous disease at the time of diagnosis [1, 2].
Mycosis fungoides (MF) and Sezary syndrome (SS) are indolent cutaneous T-cell lymphomas (CTCL) with an approximately 90% 5-year survival rate for MF and 20% to 40% survival rate for SS, depending on the criteria used to define SS [1, 2].
ZOLINZA, an oral anticancer drug, was approved in Japan for the treatment of cutaneous T-cell lymphomas. Under a distribution agreement signed in 2011, Taiho Pharmaceutical Co Ltd will promote and distribute ZOLINZA in Japan, Merck said.
In contrast, primary cutaneous ALCL accounts for approximately 10% of the cutaneous T-cell lymphomas, affects predominantly older adults and is rare in children.
A T-Cell receptor [gamma] polymerase chain reaction assay using capillary electrophoresis for the diagnosis of cutaneous T-cell lymphomas. Diagn Mol Pathol 2002;11:5966.