Primary Cutaneous B-Cell Lymphomas
Primary B-cell lymphoma comprises approximately 20% of all primary cutaneous lymphomas and less than 1% of all NHLs. This disease ranks second among extranodal NHLs, after primary gastrointestinal NHLs.
Smith at al analyzed data from the Surveillance, Epidemiology, and End Results (SEER) registry collected between 1973 and 2001. The age-adjusted incidence rate of PCBCL was 3.9 per million population. This study also suggested that the incidence rates steadily increase with age at diagnosis from 0.09 per million in patients < 30 years old to 10.8 per million in patients ≥ 80 years old (120-fold increase; P < .001). The male-to-female ratio was 1.4:1 for the entire PCBCL group. Low-grade PCBCLs (PCMZL, PCFCL) usually manifest in middle-age populations, with a median age of 53 and 58 years, respectively. PCDLBCL, LT is a disease of elderly persons, with a median age > 70 years. The male-to-female ratio was 1.4 and 2.1 for PCMZL and PCFCL, respectively, and 0.5 for PCDLBCL, LT. No significant geographic or ethnic differences in the occurrence of PCBCL were reported in the reviewed literature.
Although the etiology of PCBCL is not well understood, several reports from Europe revealed an association between PCMZL with Borrelia burgdorferi (Bb). Jelić et al reported that 12 of 22 patients (55%) with PCBCL had a positive serology for Bb. Goodlad et al detected Bb-specific DNA sequences in 7 of 20 patients with PCBCL (5 PCMZL, 5 PCFCL, and 2 PCDLBCL, LT). A limited number of cases of PCBCL positive for Bb were treated with antibiotics and showed resolution of lesions. However, three studies that included patients from Europe did not confirm this hypothesis. Takino et al analyzed 60 cases of PCMZL from East Asia, Germany, and the United States using multiple clinicopathologic characteristics. All 60 cases were negative for Bb DNA or for API2-MALT1 fusion. Goteri at al tested 73 paraffin-embedded tissue samples from patients with PCBCL using polymerase chain reaction (PCR) and showed no evidence of the presence of Bb-specific sequences. Schöllkopf et al analyzed 3,055 patients with NHL and 3,187 controls in a Danish-Swedish case-controlled study. A positive history of a tick bite or antibodies against Borrelia infection in serum was found in 1,579 patients and 1,358 controls. Statistical analysis suggested that the overall risk of NHL was not associated with a self-reported history of tick bite or the presence of anti-Borrelia antibodies. Interestingly, in analyses of NHL subtypes, both parameters were associated with an increased risk of mantle cell lymphoma.
Epidemiology
Primary B-cell lymphoma comprises approximately 20% of all primary cutaneous lymphomas and less than 1% of all NHLs. This disease ranks second among extranodal NHLs, after primary gastrointestinal NHLs.
Smith at al analyzed data from the Surveillance, Epidemiology, and End Results (SEER) registry collected between 1973 and 2001. The age-adjusted incidence rate of PCBCL was 3.9 per million population. This study also suggested that the incidence rates steadily increase with age at diagnosis from 0.09 per million in patients < 30 years old to 10.8 per million in patients ≥ 80 years old (120-fold increase; P < .001). The male-to-female ratio was 1.4:1 for the entire PCBCL group. Low-grade PCBCLs (PCMZL, PCFCL) usually manifest in middle-age populations, with a median age of 53 and 58 years, respectively. PCDLBCL, LT is a disease of elderly persons, with a median age > 70 years. The male-to-female ratio was 1.4 and 2.1 for PCMZL and PCFCL, respectively, and 0.5 for PCDLBCL, LT. No significant geographic or ethnic differences in the occurrence of PCBCL were reported in the reviewed literature.
Etiology
Although the etiology of PCBCL is not well understood, several reports from Europe revealed an association between PCMZL with Borrelia burgdorferi (Bb). Jelić et al reported that 12 of 22 patients (55%) with PCBCL had a positive serology for Bb. Goodlad et al detected Bb-specific DNA sequences in 7 of 20 patients with PCBCL (5 PCMZL, 5 PCFCL, and 2 PCDLBCL, LT). A limited number of cases of PCBCL positive for Bb were treated with antibiotics and showed resolution of lesions. However, three studies that included patients from Europe did not confirm this hypothesis. Takino et al analyzed 60 cases of PCMZL from East Asia, Germany, and the United States using multiple clinicopathologic characteristics. All 60 cases were negative for Bb DNA or for API2-MALT1 fusion. Goteri at al tested 73 paraffin-embedded tissue samples from patients with PCBCL using polymerase chain reaction (PCR) and showed no evidence of the presence of Bb-specific sequences. Schöllkopf et al analyzed 3,055 patients with NHL and 3,187 controls in a Danish-Swedish case-controlled study. A positive history of a tick bite or antibodies against Borrelia infection in serum was found in 1,579 patients and 1,358 controls. Statistical analysis suggested that the overall risk of NHL was not associated with a self-reported history of tick bite or the presence of anti-Borrelia antibodies. Interestingly, in analyses of NHL subtypes, both parameters were associated with an increased risk of mantle cell lymphoma.
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