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Primary cutaneous marginal zone lymphoma

Authors
Eric Jacobsen, MD
Arnold S Freedman, MD
Rein Willemze, MD
Section Editors
Timothy M Kuzel, MD, FACP
John A Zic, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

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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Literature review current through: Nov 2016. | This topic last updated: Mon Oct 03 00:00:00 GMT+00:00 2016.
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