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Published on: October 14, 2021
[Cutaneous B-cell lymphoma. Classification and diagnostics]
E Geissinger1, P Adam, H K Müller-Hermelink
1Institut für Pathologie, Universität Würzburg. geissinger@mail.uni-wuerzburg.de
Insights
Primary cutaneous B-cell lymphomas, including PCFCL, PCMZL, and PCLBCL, are classified by WHO-EORTC. Distinguishing these from systemic lymphomas requires staging, while pathologists aid in differentiating neoplastic from reactive lymphoproliferations.
Area of Science:
- Dermatology
- Hematology
- Oncology
Context:
- The WHO-EORTC classification identifies three primary cutaneous B-cell lymphomas: PCFCL, PCMZL, and PCLBCL (leg type).
- PCFCL and PCMZL are indolent lymphomas with favorable prognoses.
- PCLBCL (leg type) exhibits an aggressive clinical course.
Purpose:
- To outline the classification of primary cutaneous B-cell lymphomas.
- To emphasize the need for staging to differentiate primary cutaneous lymphomas from secondary skin involvement by systemic lymphomas.
- To highlight the role of pathologists in distinguishing neoplastic from reactive cutaneous lymphoproliferations.
Summary:
- Primary cutaneous B-cell lymphomas (PCFCL, PCMZL, PCLBCL) are distinct entities in the WHO-EORTC classification.
- Accurate diagnosis necessitates staging to rule out secondary systemic lymphoma involvement.
- Histological and immunohistochemical findings alone are often insufficient for definitive diagnosis.
Impact:
- Improved diagnostic accuracy for cutaneous lymphomas.
- Facilitation of appropriate staging and treatment strategies.
- Enhanced understanding of the differential diagnosis in cutaneous lymphoproliferative disorders.
Abstract:
Primary cutaneous B-cell lymphomas include cutaneous follicle centre lymphoma (PCFCL), cutaneous marginal zone B-cell lymphoma (PCMZL) and cutaneous diffuse large B-cell lymphoma (PCLBCL) "leg type" which are the three main types in the new WHO-EORTC classification for cutaneous lymphomas. PCFCL and PCMZL are indolent lymphomas with an excellent prognosis while PCLBCL shows an aggressive clinical course. All three types must be distinguished from a secondary skin involvement by systemic lymphomas. Since histological and immunohistochemical findings are not decisive, making this distinction requires appropriate staging procedures. In contrast, the pathologist can make an important contribution to the differential diagnosis between neoplastic and reactive cutaneous lymphoproliferations.
