Disease-Defining Molecular Features of Primary Cutaneous B-Cell Lymphomas: Implications for Classification and Treatment

Zhang, Yue; LeWitt, Tessa M.; Louissaint, Abner; Guitart, Joan; Zhou, Xiaolong; Choi, Jaehyuk

doi:10.1016/j.jid.2022.07.027

Cited by 5 publications

(4 citation statements)

References 70 publications

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“…PCDLBCL‐LT is a distinct DLBCL that is restricted to the skin with >80% of cases involving the leg 1–5 . While it has been proposed to be similar to other DLBCLs of non‐germinal center B cell/activated B‐cell type (non‐GCB/ABC type), 7–10 some suggest much greater heterogeneity in terms of cell‐of‐origin 11 . Mutational and cytogenetic studies highlighted its distinction from ABC‐type DLBCL, including many with MYD88 L265p mutations 9–12 .…”

Section: Resultsmentioning

confidence: 99%

“…While it has been proposed to be similar to other DLBCLs of non‐germinal center B cell/activated B‐cell type (non‐GCB/ABC type), 7–10 some suggest much greater heterogeneity in terms of cell‐of‐origin 11 . Mutational and cytogenetic studies highlighted its distinction from ABC‐type DLBCL, including many with MYD88 L265p mutations 9–12 . However, many PCDLBCL‐LT have a mutational/cytogenetic profile similar to other DLBCL of MYD 88/MCD/C5 type, a category that also includes many extranodal lymphomas, such as CNS and testis 13,14 .…”

Section: Resultsmentioning

confidence: 99%

“…The major types of PCBCLs have been recognized as distinct entities with specific genetic characteristics distinguishing them from their respective systemic counterparts. [1][2][3][4][5]9 Questions remain about to what extent they should be segregated from nodal or other extranodal lymphomas that they most closely resemble and for some whether they should be further subcategorized. The Panel highlighted the following critical questions.…”

Section: Ucn1: Uncertainties In the Classification Of Pcbclsmentioning

confidence: 99%

“…11 Mutational and cytogenetic studies highlighted its distinction from ABC-type DLBCL, including many with MYD88 L265p mutations. [9][10][11][12] However, many PCDLBCL-LT have a mutational/cytogenetic profile similar to other DLBCL of MYD88/MCD/C5 type, a category that also includes many extranodal lymphomas, such as CNS and testis. 13,14 Furthermore, a controversial separate group of PCDLBCL, term "not otherwise specified" (PCDLBCL-NOS) with morphological features and prognosis slightly different than PCDLBCL-LT, has been recently identified.…”

Section: Should Pcdlbcl-lt Remain a Unique And Distinct Category Of D...mentioning

confidence: 99%

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Identifying and addressing unmet clinical needs in primary cutaneous B‐cell lymphoma: A consensus‐based paper from an ad‐hoc international panel

Quaglino,

Pimpinelli,

Zinzani

et al. 2023

Hematological Oncology

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Primary cutaneous B‐cell lymphomas (PCBCLs) are lymphoproliferative disorders that appear on the skin without evidence of extracutaneous manifestations at the time of diagnosis. There is a lack of evidence‐based guidelines for their clinical management due to the availability of very few large scale studies and controlled clinical trials. Here we present and discuss a series of major unmet clinical needs (UCNs) in the management of PCBCLs by a panel of 16 experts involved in research and clinical practice of PCBCL. The Panel produced recommendations on the appropriateness of the clinical decisions concerning the identified clinical needs and proposed research for improving the knowledge needed to solve them. Recommendations and proposals were achieved by multiple‐step formalized procedures to reach a consensus after a comprehensive analysis of the scientific literature. Recommendations and proposals lay in the domain of classification uncertainties of PCBCL, optimization of diagnosis, optimization of prognosis, optimization of staging and critical issues on therapeutic strategies with particular focus on new treatments. These recommendations are intended for use not only by experts but above all by dermatologists and hematologists with limited experience in the field of PCBCLs as well as general practitioners.

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