Primary cutaneous follicle center lymphoma
- Eric Jacobsen, MD
Eric Jacobsen, MD
- Assistant Professor of Medicine
- Harvard Medical School
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Rein Willemze, MD
Rein Willemze, MD
- Professor and Chairman, Department of Dermatology
- Leiden University Medical Center
- Section Editors
- Timothy Michael Kuzel, MD, FACP
Timothy Michael Kuzel, MD, FACP
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine, Rush University Medical Center
- Chief, Division of Hematology/Oncology/Cell Therapy
- John A Zic, MD
John A Zic, MD
- Section Editor — Cutaneous Lymphoma
- Associate Professor of Medicine/Dermatology
- Vanderbilt University School of Medicine
Primary cutaneous B cell lymphoma (PCBCL) refers to those cases of B cell lymphoma that present in the skin when there is no evidence of extracutaneous disease at the time of diagnosis and after the completion of an initial staging evaluation. The revised 2016 World Health Organization (WHO) classification system defines three main subtypes of PCBCL :
●Primary cutaneous follicle center lymphoma (PCFCL)
●Primary cutaneous large B cell lymphoma, leg type
●Primary cutaneous marginal zone lymphoma (PCMZL), which is included in the broader category of extranodal marginal zone B cell lymphoma
These appear to be unique entities with differing clinical presentations, pathologic features, and prognosis. The approach to treatment also differs between subtypes. This topic review will discuss PCFCL. This entity was previously known as primary cutaneous follicle center cell lymphoma (PCFCCL) under the European Organization for Research and Treatment of Cancer (EORTC) classification and contains cases that may have previously be classified as cutaneous follicle center lymphoma or diffuse large B cell lymphoma under the 2001 WHO classification.
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- CLINICAL FEATURES
- PATHOLOGIC FEATURES
- DIFFERENTIAL DIAGNOSIS
- Reactive infiltrate
- Systemic lymphoma
- - Follicular lymphoma
- - Intravascular lymphoma
- Other cutaneous lymphomas
- - Primary cutaneous diffuse large B cell lymphoma, leg type
- - Primary cutaneous marginal zone lymphoma
- - Cutaneous T cell lymphoma
- TREATMENT AT RELAPSE
- SUMMARY AND RECOMMENDATIONS