Rare Cutaneous T-Cell Lymphomas

Damasco, Fabiana; Akilov, Oleg E.

doi:10.1016/j.hoc.2018.08.004

Cited by 14 publications

(9 citation statements)

References 64 publications

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“…Rarely, locally multifocal presentations, involvement of the lower extremities, accompanying symptoms such as pruritus or pain, and occurrences in younger patients are also possible. Generalized disease, rapid progression, or refractory lesions should prompt investigations into alternative diagnoses [5].…”

Section: Discussionmentioning

confidence: 99%

“…Patients usually present later in life, but lesions have been documented between the ages of 6 months and 91 years [4]. Systemic features, disseminated or generalized multifocal disease, or rapidly progressing lesions are inconsistent with SMPLPD [5]. The histopathological hallmarks are a diffuse nonepidermotropic lymphocytic infiltrate of CD4+ and CD3+ mature lymphocytes with associated, variably abundant B cells.…”

Section: Introductionmentioning

confidence: 99%

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The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series

et al. 2020

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Background: Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (SMPLPD) is a provisional entity within the 2016 World Health Organization classification of primary cutaneous lymphomas. The condition is currently classified as a lymphoproliferative disorder to emphasize its benign course and discourage aggressive, systemic treatment modalities. Objective: To provide a relevant synthesis for the dermatological practitioner on the prevalence, presentation, and treatment of SMPLPD. Methods: We conducted an updated systematic literature review and a retrospective chart review of diagnosed cases of SMPLPD from 2 Canadian academic cutaneous lymphoma centers. Results: A total of 23 studies with 136 cases were extracted from the systematic review and 24 patients from our retrospective chart review. SMPLPD proved relatively common accounting for 12.5% of all cutaneous T-cell lymphomas encountered in our cutaneous lymphoma clinics, second in frequency only to mycosis fungoides. The typical clinical presentation was that of an older individual (median age 59 years) with an asymptomatic solitary lesion on their upper extremity. The most common clinical differentials were cutaneous lymphoid hyperplasia, basal cell carcinoma, and lymphoma unspecified. T follicular helper markers were reliably detected. The main treatment modalities were surgical excision, local radiation therapy, and topical or intralesional steroids. Cure was achieved in the vast majority of cases. Conclusions: SMPLPD is an underdiagnosed T-cell lymphoma with an overtly benign clinical course. The condition has an excellent prognosis and responds well to skin-directed therapies. Practitioners should be aware of this condition to avoid aggressive systemic treatments.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series

et al. 2020

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“… 1 PCGD-TCL is aggressive, with a median survival of 15-31 months. 5 However, there have been cases of successful treatment and remission with radiation therapy followed by chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

A rare case of aggressive cytotoxic T-cell lymphoma in a patient on dupilumab

et al. 2022

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“…A similar situation has been described in a short series of intestinal gd lymphomas, where very discrete bowel disease was found after chemotherapy. 18 In summary, secondary cutaneous involvement by MEITL may show histopathologic and immunohistochemical features that could overlap different subtypes of primary cutaneous cytotoxic T-cell lymphomas 19,20 (ie, subcutaneous panniculitic-like T-cell lymphomas, primary cutaneous gd T-cell lymphoma or primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma). In some instances, only the evidence of a primary GI lymphoma or the development of GI symptoms may allow the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Secondary Cutaneous Involvement: A Diagnostic Challenge

Olmos-Alpiste

Vázquez

Gallardo

et al. 2020

The American Journal of Dermatopathology

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A 45-year-old woman presented with a solitary breast nodule that histologically corresponded to a dense dermal/ subcutaneous infiltration of atypical cytotoxic T-lymphocytes (CD3 + , CD8 + , CD56 + , TIA-1+, CD5 2 , CD4 2 , CD30 2 , EBV 2 ), resembling subcutaneous panniculitic T-cell lymphoma. The presence of TCRd gene rearrangement and the absence of bF1 expression let to suspect the diagnosis of primary cutaneous gdT-cell lymphoma. As a consequence of jejunum perforation following chemotherapy treatment, a mucosal atypical lymphoid infiltration with marked epitheliotropism was observed in the resected intestinal sample, and the diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) was finally established. Disease progression appeared with multiple erythematous plaques showing a dense lichenoid atypical cytotoxic T-cell infiltrate with intense epidermotropism, mimicking primary cutaneous epidermotropic aggressive CD8 + T-cell lymphoma. MEITL is an uncommon and aggressive peripheral T-cell lymphoma that often presents in adults with gastrointestinal symptoms. Secondary cutaneous involvement is a rare phenomenon that may show clinicopathologic and immunohistochemical features that overlap with different subtypes of primary cutaneous cytotoxic T-cell lymphomas. In the absence of gastrointestinal symptoms, the diagnosis may be challenging, and only the evidence of underlying MEITL may allow to establish the definite diagnosis.

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Rare Cutaneous T-Cell Lymphomas

Cited by 14 publications

References 64 publications

The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series

The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series

A rare case of aggressive cytotoxic T-cell lymphoma in a patient on dupilumab

Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Secondary Cutaneous Involvement: A Diagnostic Challenge

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