Cutaneous lymphoma: Kids are not just little people

Ferenczi, Katalin; Makkar, Hanspaul S.

doi:10.1016/j.clindermatol.2016.07.010

Cited by 24 publications

(36 citation statements)

References 73 publications

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“…While most adult MF patients present with erythematous patches or plaques on sun protected areas of the body, most pediatric MF patients don't have a classic presentation [9]. The majority of children with MF present with atypical variants, with hypopigmented MF being the most prevalent from, seen in over 60 % of all juvenile cases (Table 1) [8,22,23].…”

Section: Epidemiology and Clinical Presentationmentioning

confidence: 99%

Primary cutaneous T‐cell lymphomas in childhood and adolescence

Wohlmuth-Wieser

2021

J Deutsche Derma Gesell

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Summary Primary cutaneous lymphomas are extranodal non‐Hodgkin lymphomas of T‐ or B‐ cell origin, that predominantly affect older patients but have been reported in all age groups and as early as in the first years of life. Diagnosis of cutaneous lymphomas is challenging and requires high clinical suspicion and close collaboration between dermatologists, pediatric oncologists and pathologists. Skin involvement of non‐Hodgkin lymphomas in children or adolescents can either be primary cutaneous or secondary due to an underlying nodal lymphoma. The most common primary cutaneous lymphomas encountered in children are of T‐cell origin, with mycosis fungoides being the most prevalent cutaneous T‐cell lymphoma, followed by CD30+ lymphoproliferative disorders. While cutaneous lymphomas share clinicopathologic characteristics between juvenile and adult forms, there are important differences in terms of clinical presentation, diagnosis and treatment. The hypopigmented variant of mycosis fungoides seems to be overrepresented in the pediatric age group. Prognosis and treatment of mycosis fungoides are stage dependent. The majority of children present with early‐stage disease and respond well to topical corticosteroids and phototherapy.

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Section: Epidemiology and Clinical Presentationmentioning

confidence: 99%

Primary cutaneous T‐cell lymphomas in childhood and adolescence

Wohlmuth-Wieser

2021

J Deutsche Derma Gesell

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Section: Disease Prevalence and Subtypesmentioning

confidence: 99%

“…Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of non-Hodgkin lymphomas arising from the malignant proliferation of skin-homing or skin-resident T cells [1,2]. Although CTCL manifestation in children exists [3], most CTCL typically affect the elderly, with a median age at diagnosis of 55-60 years and an average number of 6.4 new cases per year and per million people [4]. The 2 main subtypes of CTCL include the most frequent, i.e., mycosis fungoides (MF), accounting for approximately 60% of CTCL cases and 50% of all primary cutaneous lymphomas, and the rare leukemic variant Sézary syndrome (SS), representing around 5% of CTCL cases.…”

mentioning

confidence: 99%

Pathogenesis and Therapy of Primary Cutaneous T-Cell Lymphoma: Collegium Internationale Allergologicum (CIA) Update 2020

Bobrowicz

Fassnacht

Ignatova

et al. 2020

Int Arch Allergy Immunol

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Cutaneous T-cell lymphoma (CTCL) is a heterogeneous disease group of unknown etiology with a complex immunological background. As CTCL arises from T cells that have a vital role in the antitumor response, their therapy is largely aimed at reversing the immunological mechanisms leading to or manifesting during this malignancy. Early disease stages can be controlled with skin-directed therapy in most CTCL cases. Still, advanced CTCL has a dismal prognosis and warrants systemic therapy. Despite considerable progress in understanding the pathophysiology of the disease and the numerous systemic treatment options available, longterm remission rates with conventional treatments alone are still low. Allogeneic hematopoietic stem cell transplantation is currently the only curative option for advanced CTCL, including mycosis fungoides and Sézary syndrome. The aims of this review is to summarize the recent findings on the immunology of this heterogeneous disease and to present the advances in its clinical management.

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“…Among CTCL, mycosis fungoides (MF) is the most common and most studied type comprising up to 55% of cases, whereas S ezary syndrome (SS), the leukemic variant, is very rare representing only around 5% of cases. 1,2 MF has a prolonged clinical course that initially presents in photo-protected areas with patches and/or plaques. Three different MF stages are recognized: patches, plaques, and tumor; MF can progress to tumor stage with involvement of other organs, compromising life because of systemic malignant invasion and immunosuppression (Fig.…”

Section: Introductionmentioning

confidence: 99%

Cell signaling in cutaneous T‐cell lymphoma microenvironment: promising targets for molecular‐specific treatment

et al. 2021

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Cutaneous T-cell lymphomas (CTCL) result from the infiltration and proliferation of a population of T cells in the skin, inducing changes in the activity of both T cells and surrounding skin cells. In the CTCL microenvironment, cell interactions mediated by cell signaling pathways are altered. Defining changes in cell signaling enables to understand Tcell deregulations in the CTCL microenvironment and thus the progression of the disease. Moreover, characterizing signaling networks activated in CTCL stages can lead to consider new molecular biomarkers and therapeutic targets. Focusing on mycosis fungoides (MF), the most frequent variant of CTCL, and S ezary syndrome (SS), its leukemic variant, this review highlights recent molecular and genetic findings revealing modifications of key signaling pathways involved in (1) cell proliferation, cell growth, and cell survival such as MAP kinases and PI3K/Akt; (2) immune responses derived from TCR, TLR, JAK/STAT, and NF-kB; and (3) changes in tissue conditions such as extracellular matrix remodeling, hypoxia, and angiogenesis. Alterations in these signaling networks promote malignant Tcell proliferation and survival, T-cell migration, inflammation, and suppression of immune regulation of malignant T cells, making a skin microenvironment that allows disease progression. Targeting key proteins of these signaling pathways, using molecules already available and used in research, in clinical trials, and with other disease indications, can open the way to different therapeutic options in CTCL treatment.

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Cutaneous lymphoma: Kids are not just little people

Cited by 24 publications

References 73 publications

Primary cutaneous T‐cell lymphomas in childhood and adolescence

Primary cutaneous T‐cell lymphomas in childhood and adolescence

Pathogenesis and Therapy of Primary Cutaneous T-Cell Lymphoma: Collegium Internationale Allergologicum (CIA) Update 2020

Cell signaling in cutaneous T‐cell lymphoma microenvironment: promising targets for molecular‐specific treatment

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