Primary cutaneous follicle center lymphoma
| Primary cutaneous follicle center lymphoma | |
|---|---|
| Other names | PCFCL |
| Specialty | Hematology/oncology |
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
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]
See also
- Cutaneous B-cell lymphoma
- Primary cutaneous large B-cell lymphoma
- Follicular lymphoma
References
- ISBN 9789283224310. Archived from the originalon October 12, 2013.
- ^ ISBN 9780721600406.
- ^ ISBN 9780781791168.
- PMID 23957984.
- ^ PMID 22710899.
- ^ S2CID 10733762.
- ^ PMID 18567836.
- PMID 23957985.