Primary and secondary cutaneous CD30+lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment

Bekkenk, Marcel W.; Geelen, Françoise A. M. J.; Vader, Pieter; Heule, Freerk; Geerts, Marie-Louise; Vloten, W. A. van; Meijer, Chris J.L.M.; Willemze, Rein

doi:10.1182/blood.v95.12.3653.012k23_3653_3661

Cited by 66 publications

(63 citation statements)

References 25 publications

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“…They are the second most common CTCL about 25% of all CTCLs (53). Clinical presentation is different in both LyP and pcALCL but they have overlapping pathological features (71).…”

Section: Primary Cutaneous Cd30+ T Cell Lymphoproliferative Disordersmentioning

confidence: 99%

An Update on Molecular Biology of Cutaneous T Cell Lymphoma

Walia

Yeung

2020

Front. Oncol.

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Cutaneous T cell lymphomas represent a heterogenous group of lymphoproliferative disorders defined by clonal proliferation of T cells present in the skin. The latest WHO classification in 2016 and WHO-EORTC classification in 2018 has updated the classification of these entities based on the molecular profile. Research in the field of molecular genetics of CTCL has allowed a better understanding of the biology of these tumors and has helped to identify potential targets for therapy that can be tailored to individual patients. In this review, we discuss the latest developments in the molecular profile of CTCLs including biomarkers for diagnosis, prognosis, and potential therapeutic targets. We have also touched upon the utility of various molecular diagnostic modalities. For the purpose of this review, we researched papers in PubMed indexed journals in English literature published in the past 20 years using keywords CTCL, mycosis fungoides, molecular profile, molecular diagnosis, whole genome profile, genomic landscape, TCR clonality.

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“…They are the second most common CTCL about 25% of all CTCLs (53). Clinical presentation is different in both LyP and pcALCL but they have overlapping pathological features (71).…”

Section: Primary Cutaneous Cd30+ T Cell Lymphoproliferative Disordersmentioning

confidence: 99%

An Update on Molecular Biology of Cutaneous T Cell Lymphoma

Walia

Yeung

2020

Front. Oncol.

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“…Lymphomatoid papulosis is categorized as a CD30 + LPD [56]. CD30 is a cell surface marker that can be found on T and B lymphocytes but was first identified on Reed‐Sternberg cells in Hodgkin lymphoma by Stein and coworkers [62].…”

Section: Lymphomatoid Papulosismentioning

confidence: 99%

“…Lymphomatoid papulosis (LyP) is a chronic disorder that is characterized by a waxing and waning disease course, i.e. it is typically self-regressing [56,57]. LyP is considered a rare disease and children and adolescents account for only 4-10 % of LyP cases [56,58].…”

Section: Epidemiology and Clinical Presentationmentioning

confidence: 99%

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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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“…Classification requires clinicopathologic correlation by an experienced dermatologist and pathologist. pcALCL has a good prognosis when compared to systemic ALCL [1, 2], even in the presence of regional lymphadenopathy, and may regress spontaneously [3, 4]. LyP has a characteristic self-resolving, recurring clinical course.…”

Section: Introductionmentioning

confidence: 99%

Clinicopathologic and microenvironmental analysis of primary cutaneous CD30-positive lymphoproliferative disorders: a 26 year experience from an academic medical center in Brazil

et al. 2019

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BackgroundPrimary cutaneous CD30+ lymphoproliferative disorders (pc-CD30-LPD) are a group of clonal T cell lymphoproliferative disorders that despite very similar tumor histology follow different and characteristic clinical courses, suggesting a homeostatic role of the tumor microenvironment. Little is known about tumor microenvironment and there is almost no literature about PD-L1 expression in pc-CD30-LPD.MethodsThis retrospective study presents a fully clinicopathologically characterized series of pc-CD30-LPDs from an academic medical center in Brazil, including 8 lymphomatoid papulomatosis (LyP), 9 primary cutaneous anaplastic large cell lymphoma (pcALCL) and 4 borderline lesions. All the cases were scored for FOXP3+ regulatory T-cells (Treg) and CD8+ cytotoxic tumor infiltrating lymphocytes (TIL) densities, as well as PD-L1 expression in tumor cells and tissue associated macrophages. The CD8+/FOXP3+ ratio was also evaluated.ResultsAmong the 21 cases of pc-CD30-LPD, PD-L1 expression is frequent in both tumor cells and tissue associated macrophages in pc-CD30-LPD across categories, suggesting that the PD-L1 axis may be a common feature of pc-CD30-LPDs. While reactive T cell infiltrates vary widely from case to case, a common feature across pc-CD30-LPDs is higher density of CD8 than FOXP3 + T cells. The distribution of T cells within the lesions however differed between LyP and pcALCL: we found that LyP lesions tend to be permeated by CD8+ and FOXP3+ T cells, whereas pcALCL tend to be surrounded by a rim of CD8+ TIL and FOXP3+ Tregs with relatively lower density infiltrates in the center of the lesion.ConclusionsLyP has a trend to have denser immune cells throughout the lesion, with higher FOXP3+ Treg and CD8+ TIL in the center than the edge comparing with pcALCL. PD-L1+ is frequent in tumor cells and tissue associated macrophages in pc-CD30-LPD. The differential distribution of CD8+ and FOXP3+ TILs in LyP as compared to pcALCL could provide a clue to the relapsing/remitting course of LyP as compared to the less frequent spontaneous regression of pcALCL.

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Primary and secondary cutaneous CD30+lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment

Cited by 66 publications

References 25 publications

An Update on Molecular Biology of Cutaneous T Cell Lymphoma

An Update on Molecular Biology of Cutaneous T Cell Lymphoma

Primary cutaneous T‐cell lymphomas in childhood and adolescence

Clinicopathologic and microenvironmental analysis of primary cutaneous CD30-positive lymphoproliferative disorders: a 26 year experience from an academic medical center in Brazil

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