Primary cutaneous marginal zone 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 M Kuzel, MD, FACP
Timothy M 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 after the completion of an initial staging evaluation. There are 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), this subtype is included in the broader category of extranodal marginal zone B cell lymphoma (see "Clinical manifestations, pathologic features, and diagnosis of extranodal (MALT) and nodal marginal zone lymphomas")
These three subtypes appear to be unique entities with differing clinical presentation, pathologic features, and prognosis. They also require slightly different treatment approaches. This topic review will discuss PCMZL, which is sometimes referred to as skin-associated lymphoid tissue (SALT) lymphoma. Before 2008, classification system, cases of PCMZL may have been classified as primary cutaneous immunocytoma or cutaneous plasmacytoma.
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- Infectious etiology
- - Borrelia burgdorferi
- - Viral etiologies
- Autoimmune disorders
- CLINICAL FEATURES
- PATHOLOGIC FEATURES
- DIFFERENTIAL DIAGNOSIS
- Reactive infiltrate
- Systemic lymphoma
- - Marginal zone lymphoma
- - Other systemic lymphomas
- Other cutaneous lymphomas
- - Primary cutaneous diffuse large B cell lymphoma, leg type
- - Primary cutaneous follicle center lymphoma
- - Cutaneous T cell lymphoma
- - Intravascular lymphoma
- Extramedullary plasmacytoma
- ASSESSING DISEASE RESPONSE
- TREATMENT AT RELAPSE
- SUMMARY AND RECOMMENDATIONS