Primary cutaneous follicle centre lymphoma epidemiology and demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2] Soroush Seifirad, M.D.[3]

The incidence of primary cutaneous follicle centre lymphoma increases with age; the median age at diagnosis is 51 years. Males are more commonly affected with primary cutaneous follicle centre lymphoma than females.^[1]

According to a large population-based study focused on cutaneous lymphomas (CL) in the United States by Bradford et al. on 3884 Cutaneous lymphomas 9CLs) diagnosed during 2001-2005. Cutaneous T-cell lymphomas (CTCLs) accounted for 71% (age-adjusted incidence rate [IR] = 7.7/1 000 000 person-years), whereas cutaneous B-cell lymphomas(CBCLs) accounted for 29% (IR = 3.1/1 000 000 person-years).^[2]

• Over the past 25 years, the CL incidence rate increased from 5.0/1 000 000 person-years during 1980-1982 to 14.3 during 2001-2003.
• During 2004-2005, the CL IR was 12.7. This recent apparent change could be incomplete case ascertainment or potential levelling off of incidence rates.
• CLs rates vary markedly by race and sex, supporting the notion that they represent distinct disease entities.

• There is no prevalence report primary cutaneous follicle centre lymphoma.
• Primary cutaneous follicle center lymphoma is the most common primary cutaneous B-cell lymphoma.
• It accounts for approximately 10% to 20% of all cutaneous lymphomas and comprises about 50% of primary cutaneous B-cell lymphomas.

• The case-fatality rate/mortality rate of primary cutaneous follicle centre lymphoma is quite low.
• Spread from the skin is unusual, and the prognosis is excellent with a 5-year survival of over 97%.
• 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.

• The incidence of primary cutaneous follicle centre lymphoma increases with age; the median age at diagnosis is 51 years.^[1]

• Most commonly, patients are white men, 50 to 60 years old, although younger patients and women may also develop this lymphoma.

• Primary cutaneous follicle centre lymphoma usually affects individuals of the blacks and non-Hispanic whites followed by Hispanic whites and Asian/Pacific Islanders . Whites of European descent individuals are less likely to develop primary cutaneous follicle centre lymphoma.
• According to Bradford et al. CL incidence rate were highest among blacks and non-Hispanic whites (both 11.5/1 000 000 person-years), followed by Hispanic whites (7.9) and Asian/Pacific Islanders (7.1). The CTCL IR was highest among blacks (10.0/1 000 000 person-years), whereas the CBCL IR was highest among non-Hispanic whites (3.5).^[2]

Males are more commonly affected with primary cutaneous follicle centre lymphoma than females.^[1]

According to Bradford et al. also, males had a statistically significant higher incidence of CL than females (14.0 vs 8.2/1 000 000 person-years, respectively; male-female IR ratio [M/F IRR] = 1.72; P < .001).^[2]

• Theoretically, and according to some studies primary cutaneous follicle centre lymphoma cases are more reported in areas of increased prevalence of the following pathogenes:
  • Borrelia burgdorferi
  • Hepatitis C
  • Human herpesvirus 8
    

• Specifically, the region of southeast Asian countries have a presumable high incidence of cutaneous T cell Lymphoma(CTCL), in particular, Epstein-Baar virus-associated natural killer/T-cell lymphomas, while Cutaneous B cell lymphomas (CBCL) are very uncommon.^[3]

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