Primary cutaneous follicle center lymphoma
|Primary cutaneous follicle center lymphoma|
Primary cutaneous follicle center lymphoma (PCFCL) is a type of lymphoma. It was recognized as a distinct disease entity in the 2008 WHO classification.:218 PCFCL had been previously conceived as a variant of follicular lymphoma (FL).:218
Unlike FL, PCFCL is not typically associated with t(14;18) translocation:218 although presence of that translocation does not exclude PCFCL.:314 It is usually not associated with overexpressed Bcl-2.:218
PCFCL represents about 55% to 60% of primary cutaneous B-cell lymphomas (PCBCL);:373 primary cutaneous marginal zone lymphoma and diffuse large B-cell cell lymphoma, leg type are the other primary cutaneous B-cell lymphomas.:373 The cause of PCFCL is unknown.:312
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Surgical removal and/or radiotherapy is given for localized disease.:314 Radiation using multiple radiation fields is given if the disease has wider extent with grouped lesions.:314 For the less common situation of more extensive disease (still confined to skin), rituximab without chemotherapy is used.:314 Intralesional interferon alpha (IFN-α) and intralesional rituximab have been used.:314 Approximately one-third of PCFCL relapse, usually in the skin; treatment is similar to initial management and overall survival remains excellent.
Spread from the skin is unusual, and the prognosis is excellent:218 with a 5-year survival of over 97%.:314 The International Extranodal Lymphoma Study Group identified elevated LDH, more than two skin lesions, and nodular lesions as three prognostic factors, that are used to assess a cutaneous lymphoma international prognostic index (CLIPI), which is prognostic of disease-free status.
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