Primary cutaneous follicle center lymphoma
Primary cutaneous follicle center lymphoma (PCFCL) 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
Contents
Biology
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
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]
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]
See also
References
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