cutaneous T cell lymphoma


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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 ?
Abbreviations: Pt: patient number; ClinDx: clinical assessment; TNMB: tumor-node-metastasis-blood; LN: lymph node; MG: T cell clonality by molecular testing; CTCL: cutaneous T cell lymphoma; MF: mycosis fungoides, patch (PT), plaque (PQ), tumor (T) phases; MF-E: erythroderma preceded by MF; NOS: not otherwise specified; SS: Sezary syndrome; PathDx: histological assessment; DL: dermatopathic lymphadenitis.
The various types of malignancy associated with PEO are T and B cell lymphoma, leukemia, adenocarcinoma of gut and cutaneous T cell lymphoma.1,4,5 There are reports of PEO associated with AIDS and drug ingestion.6,7 The association of PEO with malignancy and laboratory features like circulating Sezary cells, hypereosinophilia and raised IgE levels point towards the heterogenous nature of this entity.8 When associated with lymphoma, the dermatosis may appear several years before the diagnosis of malignancy or vice versa, so the association of two pathologies in a possible nosological overview has been stressed.9
Some authors have suggested that PEO may predispose to or is in fact an early form of cutaneous T cell lymphoma.10 The treatment of PEO is difficult and response to different agents is variable.
Extracorporeal photochemoimmunotherapy in cutaneous T cell lymphomas. Ann NY Acad Sci.
Mycosis fungoides (MF) is the most common entity among all cutaneous T cell lymphomas (CTCL) and shows different clinical courses based on its clinical presentation.
Genomic aberrations and survival in cutaneous T cell lymphomas. The Journal of Investigative Dermatology.