Molecular pathogenesis of cutaneous lymphomas

Stadler, Rudolf; Stranzenbach, René

doi:10.1111/exd.13701

Cited by 15 publications

(16 citation statements)

References 48 publications

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“…Chronic stimulation leads to the activation of T helper cells, followed by proinflammatory cytokines release and chronic inflammation, that will finally lead to a clonal malignant T-cell with continuous expansion [24,25]. Impaired skin reactivity to antigens was also described from asbestos exposure [26], but, on the present experimental data, we cannot assign it to the MF development. We are aware that a potential relation between cutaneous lympho-proliferative disorders and asbestos needs further epidemiological data gathering which is not an easy task since mycosis fungoides occurs only in 3.6-5.6 per million individuals [27].…”

Section: Alexandra Maria Rașcu Gabriela Neicu Agripina Rașcu Marincontrasting

confidence: 52%

Are there still other asbestos-related malignancies to be discovered?

Rașcu

Neicu

Oțelea

2018

Romanian Journal of Occupational Medicine

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Mycosis fungoides is one of the most common forms of cutaneous T-cell lymphoma. Its diagnosis is sometimes challenging and quite difficult for the physician, because its onset clinical appearance is similar to other skin diseases. Although there are a few hypotheses about mycosis fungoides’ etiology, they aren’t fully understood and still need confirmation. We report the case of a 68 years old patient diagnosed with mycosis fungoides, who has been exposed to asbestos fibers. This case is one of the few reported cases of association between asbestos and mycosis fungoides. There is no data exploring the causal relation between asbestos exposure and mycosis fungoides but common biological mechanisms could represent an argument. If occupational exposure to asbestos will be confirmed in larger studies, a new research-field of asbestos-related diseases needs to be opened.

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Section: Alexandra Maria Rașcu Gabriela Neicu Agripina Rașcu Marincontrasting

confidence: 52%

Are there still other asbestos-related malignancies to be discovered?

Rașcu

Neicu

Oțelea

2018

Romanian Journal of Occupational Medicine

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“…However, a negative clonality test does not exclude a diagnosis of MF . Newly emerging advanced molecular technology using high throughput TCR sequencing of the CDR3 (complementary determining regions) of TCR‐beta and TCR‐gamma genes, may be helpful in discriminating early MF from other inflammatory dermatoses; however, access to testing is limited. The majority (4/5) of cases in our cohort who had molecular studies performed showed the presence of a neoplastic T‐cell clone by TCR PCR studies.…”

Section: Discussionmentioning

confidence: 99%

“…26,27 Diagnostic discordance in early MF has been noted in approximately 20% of cases. 9 This may lead to vague, non-specific and inconclusive pathology interpretations of early stage lesions of MF 28 39 ; however, access to testing is limited. The majority (4/5) of cases in our cohort who had molecular studies performed showed the presence of a neoplastic T-cell clone by TCR PCR studies.…”

Section: Discussionmentioning

confidence: 99%

Mycosis fungoides with spongiosis: A potential diagnostic pitfall

et al. 2019

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Background Mycosis fungoides (MF) is characterized by epidermotropic atypical lymphocytes in the absence of spongiosis. However, we describe an unusual presentation of MF with spongiosis, mimicking benign inflammatory dermatoses and highlight the importance of pathologic interpretation for diagnostic confirmation. Methods A cross‐sectional study of consecutive patients diagnosed with MF with spongiosis was conducted. The clinical, histopathologic, immunophenotypic, and molecular genetic features of cases were reviewed. Results We identified nine cases of MF (age range 34‐82 years; mean 75 years), with an initial diagnosis of dermatitis (6/9), psoriasis (4/9), or other inflammatory dermatoses (2/9). Pruritus, pain, and blisters were common clinical manifestations. The most common areas of involvement were the extremities (8/9). Epidermotropism with spongiosis was a central histopathological feature in all cases. Conclusion These cases highlight prominent spongiosis in MF and overlap with common benign inflammatory dermatoses. We present these cases to show the diagnostic pitfalls associated with spongiotic presentations of MF. Dermatitis, psoriasis, and other inflammatory skin conditions not responsive to standard therapy warrant further work‐up including biopsy to rule out MF. Multiple skin biopsies and review by a dermatopathologist with expertise in the diagnosis of cutaneous lymphoma is highly recommended.

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“…[19] These studies were summarized by R. Stadler and R. Stranzenbach to give a precise view of the current knowledge in the understanding of the molecular pathogenesis of CTCL. [20] These genetic changes lead to T cells that show apoptosis deficits and a consequent continuous expansion. During this process, the malignant cells show increased differentiation into T helper type 2 cells with corresponding cytokine production of interleukin 4 and 13 and resistance to normal cell control mechanisms.…”

Section: Comple X Mutational L Andsc Ape In C Tclmentioning

confidence: 99%

Molecular pathogenesis of cutaneous lymphoma–Future directions

Stadler

Hain

Cieslak

et al. 2020

Experimental Dermatology

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Cutaneous T-cell lymphomas (CTCLs) are non-Hodgkin lymphomas of mainly mature skin homing CD4 + T cells. [1] The current WHO classification comprises eight main types of cutaneous T-cell lymphomas. The most frequent subtype is Mycosis fungoides (MF) with about 60% of all cases. Sézary syndrome (SS) is another well-known CTCL subform including 5% of all CTCL cases. [2,3] The diagnosis of CTCL is based on a combination of clinical, histopathological and molecular features and is challenging especially in early stages of the disease. As a result, the median time between initial symptoms and primary diagnosis is 3-4 years. [4,5] The disease progression is greatly diverse between patients. For about two-third of MF patients, the disease stays indolent in early stages with limited skin lesions and a normal life expectancy. Around 6% per year progress to advanced stages with tumors on the skin

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Molecular pathogenesis of cutaneous lymphomas

Cited by 15 publications

References 48 publications

Are there still other asbestos-related malignancies to be discovered?

Are there still other asbestos-related malignancies to be discovered?

Mycosis fungoides with spongiosis: A potential diagnostic pitfall

Molecular pathogenesis of cutaneous lymphoma–Future directions

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