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What is new about primary cutaneous B cell lymphomas
Maël Blanchard1,2, Wolfram Hoetzenecker3, Gabriela Blanchard1,2
1Department of Dermatology and Venereology, Lausanne University Hospital (CHUV), Lausanne, Switzerland.
Insights
This review details the 2022 World Health Organization classification of primary cutaneous B-cell lymphomas (CBCL), distinguishing indolent from aggressive subtypes. Further research is vital for refining CBCL management and guidelines.
Area of Science:
- Hematology
- Dermatology
- Oncology
Background:
- Primary cutaneous B-cell lymphomas (CBCL) are a diverse group of lymphomas affecting the skin.
- The 2022 World Health Organization classification refines the categorization of CBCL subtypes.
Purpose of the Study:
- To review the clinical, cellular, and molecular characteristics of the five CBCL subtypes.
- To discuss current management and treatment strategies for CBCL.
- To highlight the need for further research in CBCL.
Main Methods:
- Literature review of recent scientific advances.
- Analysis of the 2022 World Health Organization classification of mature lymphoid neoplasms.
- Synthesis of information on clinical, cellular, and molecular features, management, and treatment.
Main Results:
- The 2022 classification distinguishes indolent CBCL (marginal zone, follicle center lymphoma, EBV-positive mucocutaneous ulcer) from aggressive CBCL (diffuse large B-cell lymphoma, leg-type, intravascular large B-cell lymphoma).
- Understanding of CBCL entities has advanced significantly.
- New treatments for systemic B-cell lymphomas offer hope for CBCL.
Conclusions:
- Accurate classification of CBCL is essential for appropriate management.
- High-quality prospective research is crucial to update international guidelines for CBCL.
- Translating advances in systemic lymphoma treatment to CBCL requires specific investigation.
Abstract:
Primary cutaneous B-cell lymphomas (CBCL) are a heterogeneous group of B-cell lymphomas without evidence of extracutaneous disease at the time of diagnosis. The 2022 World Health Organization classification of mature lymphoid neoplasms differentiates the indolent primary cutaneous marginal zone lymphoproliferative disorder, primary cutaneous follicle center lymphoma and Epstein-Barr virus-positive mucocutaneous ulcer, from the more aggressive primary cutaneous diffuse large B-cell lymphoma, leg-type and intravascular large B-cell lymphoma. The new updates in the 2022 classification are based on recent scientific advances in the understanding and characterization of these entities. This article aims to review the main clinical, cellular and molecular features of the five CBCL subsets along with their management and treatment. The exponentially growing evidence for new treatment options for systemic B-cell lymphomas raises expectations for the field of CBCL as well. However, specific prospective high quality research on CBCL is still crucial to further define their management and update international guidelines.
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