Primary cutaneous follicle center lymphoma
Medical condition
From Wikipedia, the free encyclopedia
Primary cutaneous follicle center lymphoma is a type of lymphoma.[1] It was recognized as a distinct disease entity in the 2008 WHO classification.[2]: 218 PCFCL had been previously conceived as a variant of follicular lymphoma (FL).[2]: 218
Cause
Unlike FL, PCFCL is not typically associated with t(14;18) translocation[2]: 218 although presence of that translocation does not exclude PCFCL.[2]: 314 It is usually not associated with overexpressed Bcl-2.[2]: 218
PCFCL represents about 55% to 60% of primary cutaneous B-cell lymphomas (PCBCL);[3]: 373 primary cutaneous marginal zone lymphoma and diffuse large B-cell cell lymphoma, leg type are the other primary cutaneous B-cell lymphomas.[3]: 373 [4][5] The cause of PCFCL is unknown.[2]: 312
Treatment
Surgical removal and/or radiotherapy is given for localized disease.[2]: 314 [6] Radiation using multiple radiation fields is given if the disease has wider extent with grouped lesions.[2]: 314 [3][5][6] For the less common situation of more extensive disease (still confined to skin), rituximab without chemotherapy is used.[2]: 314 [3][5][6] Intralesional interferon alpha (IFN-α)[7] and intralesional rituximab have been used.[2]: 314 [7] Approximately one-third of PCFCL relapse, usually in the skin; treatment is similar to initial management[6] and overall survival remains excellent.[8]
Prognosis
Spread from the skin is unusual, and the prognosis is excellent[2]: 218 with a 5-year survival of over 97%.[2]: 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.[6]