Primary cutaneous follicle center lymphoma

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

Primary cutaneous follicle center lymphoma
Other namesPCFCL
SpecialtyHematology/oncology

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

Diagnosis

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]

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. Jaffe ES, Harris NL, Vardiman JW, Campo E, Arber, DA (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN 9780721600406.
  3. 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. Sokol L, Naghashpour M, Glass LF (2012). "Primary cutaneous B-cell lymphomas: recent advances in diagnosis and management". Cancer Control. 19 (3): 236–44. doi:10.1177/107327481201900308. PMID 22710899.
  6. Wilcox RA (2015). "Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management" (PDF). Am. J. Hematol. 90 (1): 73–6. doi:10.1002/ajh.23863. PMID 25535037.
  7. 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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