Cutaneous T-Cell Lymphoid Dyscrasia

Guitart, Joan; Magro, Cynthia M.

doi:10.1001/archderm.143.7.921

Cited by 89 publications

(45 citation statements)

References 96 publications

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“…It has been suggested that between the groups of cutaneous lymphomas on one end and cutaneous pseudolymphomas on the other, a category designated as ‘clonal cutaneous lymphoid hyperplasia’ or ‘cutaneous lymphoid dyscrasia’ can be identified [21, 22]. This cutaneous lymphoproliferative disorder is described as a ‘borderline’ condition with some potential for evolution into malignant lymphoma.…”

Section: Discussionmentioning

confidence: 99%

Solitary Small- to Medium-Sized Pleomorphic T-Cell Nodules of Undetermined Significance: Clinical, Histopathological, Immunohistochemical and Molecular Analysis of 26 Cases

Leinweber

Beltraminelli²,

Kerl³

et al. 2009

Dermatology

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Background: Solitary skin nodules composed of pleomorphic T lymphocytes are often the source of diagnostic problems. Objective: To characterize the clinicopathological features, prognosis and optimal treatment modalities of patients with solitary lymphoid nodules of small- to medium-sized pleomorphic T lymphocytes. Methods: Twenty-six patients were analysed for clinical, histopathological, immunophenotypical, molecular and follow-up data. Results: Lesions were located mainly on the head and neck (n = 16; 61.5%) or trunk (n = 8; 30.8%). Histopathology showed non-epidermotropic nodular or diffuse infiltrates of small- to medium-sized pleomorphic T lymphocytes. Monoclonality was found by PCR in 54.2% of cases (n = 13/24). After a mean follow-up of 79.7 months, a local recurrence could be observed only in 1 patient. Conclusions: Our patients have a specific cutaneous lymphoproliferative disorder characterized by reproducible clinicopathological features. The incongruity between the indolent clinical course and the worrying histopathological features poses difficulties in classifying these cases unambiguously as benign or malignant. We suggest to describe these lesions as ‘solitary small- to medium-sized pleomorphic T-cell nodules of undetermined significance’. Irrespective of the name given to these equivocal cutaneous lymphoid proliferations, follow-up data support a non-aggressive therapeutic strategy.

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Section: Discussionmentioning

confidence: 99%

Solitary Small- to Medium-Sized Pleomorphic T-Cell Nodules of Undetermined Significance: Clinical, Histopathological, Immunohistochemical and Molecular Analysis of 26 Cases

Leinweber

Beltraminelli²,

Kerl³

et al. 2009

Dermatology

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“…PCR-based methods are able to detect clonal rearrangements of the T-cell receptor (TCR) in formalin-fixed, paraffin-embedded biopsy specimens (105, 106). PCR-based methods, while sensitive, should be interpreted with caution, as clonal TCR gene rearrangements may be detected in normal elderly individuals and in patients with benign dermatoses or other disease states (107–111). However, detection of identical clones from two different sites is quite specific for MF (112).…”

Section: Diagnosismentioning

confidence: 99%

Cutaneous T‐cell lymphoma: 2016 update on diagnosis, risk‐stratification, and management

2015

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Disease overview Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). Diagnosis The diagnosis of MF or SS requires the integration of clinical and histopathologic data. Risk-adapted therapy TNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a “risk-adapted,” multi-disciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin-directed therapies is preferred, as both disease-specific and overall survival for these patients is favorable. In contrast, patients with advanced-stage disease with significant nodal, visceral or blood involvement are generally approached with biologic-response modifiers or histone deacetylase inhibitors prior to escalating therapy to include systemic, single-agent chemotherapy. In highly-selected patients, allogeneic stem-cell transplantation may be considered, as this may be curative in some patients.

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“…In retrospect these changes may histopathologically represent early MF, and a clonal TCR rearrangement may or may not be present. Although some of these lesions may ultimately evolve into cutaneous T-cell lymphoma, they are not predictive of this outcome [ 43 ].…”

Section: T-cell Dyscrasiamentioning

confidence: 99%

“…In addition to the triad of erythroderma, lymphadenopathy, and clonal neoplastic T cells in the blood, skin, and/or lymph nodes, the following diagnostic criteria must be met [ 43 ]:…”

Section: Diagnostic Considerationsmentioning

confidence: 99%