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

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

  1. ↑ Swerdlow, Steven H.; International Agency for Research on Cancer; World Health Organization (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours 2 (4th ed.). International Agency for Research on Cancer. ISBN 9789283224310.
  2. 1 2 3 4 5 6 7 8 9 10 11 12 Jaffe, ES; Harris NL, Vardiman JW, Campo E, Arber, DA. (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
  3. 1 2 3 4 Armitage, JO; Mauch PM; Harris NL; et al. (2010). "Chapter 24". Non-Hodgkin Lymphomas (2nd ed.). Lippincott Williams & Wilkins. ISBN 9780781791168.
  4. ↑ Suárez AL, Pulitzer M, Horwitz S, et al. (2013). "Primary cutaneous B-cell lymphomas: part I. Clinical features, diagnosis, and classification". J. Am. Acad. Dermatol. 69 (3): 329.e1–13; quiz 341–2. doi:10.1016/j.jaad.2013.06.012. PMID 23957984.
  5. 1 2 3 Sokol L, Naghashpour M, Glass LF (2012). "Primary cutaneous B-cell lymphomas: recent advances in diagnosis and management". Cancer Control 19 (3): 236–44. PMID 22710899.
  6. 1 2 3 4 5 Wilcox RA (2015). "Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 90 (1): 73–6. doi:10.1002/ajh.23863. PMID 25535037.
  7. 1 2 Senff NJ, Noordijk EM, Kim YH, et al. (2008). "European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas". Blood 112 (5): 1600–9. doi:10.1182/blood-2008-04-152850. PMID 18567836.
  8. ↑ Suárez AL, Querfeld C, Horwitz S, et al. (2013). "Primary cutaneous B-cell lymphomas: part II. Therapy and future directions". J. Am. Acad. Dermatol. 69 (3): 343.e1–11; quiz 355–6. doi:10.1016/j.jaad.2013.06.011. PMID 23957985.
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