High incidence of gastrointestinal tract disorders and autoimmunity in primary cutaneous marginal zone B-cell lymphomas.

IMPORTANCE To our knowledge, this is the first comprehensive study addressing comorbidities associated with primary cutaneous marginal zone B-cell lymphomas (PCMZLs). OBJECTIVE To determine if patients with PCMZL were at risk for other medical conditions. DESIGN, SETTING, AND PARTICIPANTS Case-control study at a cutaneous lymphoma clinic and a dermatopathologic consultation service at a single academic institution using an extensive questionnaire of illnesses, symptoms, and environmental exposures for 80 sequential patients with PCMZL and 80 matched controls. MAIN OUTCOMES AND MEASURES The frequency of several morbidities was obtained in both groups from data gathered from the questionnaire and corroborated by reviewing medical records when available. RESULTS We found a high incidence of past or present gastrointestinal tract problems in 49 patients (61.2%) with PCMZL compared with 30 participants (37.5%) in the control group (CG) (P = .003). Gastroesophageal reflux was reported in 50.0% (40 vs 27 in the CG, P = .04) and gastric ulcers in 10.0% (8 vs 3 in the CG, P = .13); 20.0% of the cohort had positive Helicobacter pylori serology (16 vs 2 in the CG, P = .003). Colon disorders, including irritable bowel syndrome and inflammatory bowel disease, were more common in the PCMZL cohort (20 vs 7 in the CG, P = .01). Autoimmunity was reported in 20.0% of participants (16 vs 6 in the CG, P = .03). Eight patients had a history of Hashimoto thyroiditis. Three patients had a positive antinuclear antibody. Two had a diagnosis of lupus erythematosus and 1 had Sjögren syndrome. Sicca syndrome was noted in 12.5% (10 vs 3 in the CG, P = .052). A history of noncutaneous malignant neoplasms was observed in 21.3% of the patients (17 vs 8 in the CG, P = .050). Other notable morbidities did not reach statistical significance. CONCLUSIONS AND RELEVANCE Our results indicate a high incidence of systemic conditions in patients with PCMZL, especially involving the gastrointestinal tract, but also autoimmunity and cancer.

[1]  W. Fischbach.  Gastric mucosal-associated lymphoid tissue lymphoma. , 2013, Gastroenterology clinics of North America.

[2]  K. Maclennan,et al.  Differential expression of NF-κB target genes in MALT lymphoma with and without chromosome translocation: insights into molecular mechanism , 2010, Leukemia.

[3]  E. Pescarmona,et al.  Primary cutaneous B‐cell lymphoma is associated with somatically hypermutated immunoglobulin variable genes and frequent use of VH1‐69 and VH4‐59 segments , 2010, The British journal of dermatology.

[4]  Angelica Selim,et al.  TNM Classification System for Primary Cutaneous Lymphomas , 2008 .

[5]  C. Meijer,et al.  Reclassification of 300 primary cutaneous B-Cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers. , 2007, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[6]  E. Athanasiou,et al.  Primary cutaneous MALT‐type lymphoma and Helicobacter pylori: a possible relationship , 2006, Journal of the European Academy of Dermatology and Venereology : JEADV.

[7]  H. Adami,et al.  Autoimmune and chronic inflammatory disorders and risk of non-Hodgkin lymphoma by subtype. , 2006, Journal of the National Cancer Institute.

[8]  M. Duvic,et al.  Primary cutaneous B-cell lymphoma. , 2005, Journal of the American Academy of Dermatology.

[9]  Nicola Pimpinelli,et al.  WHO-EORTC classification for cutaneous lymphomas. , 2005, Blood.

[10]  G. Nuovo,et al.  Cutaneous Immunocytoma: A Clinical, Histologic, and Phenotypic Study of 11 Cases , 2004, Applied immunohistochemistry & molecular morphology : AIMM.

[11]  L. Cerroni,et al.  Absence of Borrelia burgdorferi DNA in cutaneous B‐cell lymphomas from the United States , 2001, Journal of cutaneous pathology.

[12]  H. Kerl,et al.  Infection by Borrelia burgdorferi and cutaneous B‐cell lymphoma , 1997, Journal of cutaneous pathology.

[13]  J. Cayuela,et al.  Lymphomas in Patients With Sjögren's Syndrome Are Marginal Zone B-Cell Neoplasms, Arise in Diverse Extranodal and Nodal Sites, and Are Not Associated With Viruses , 1997 .

[14]  N. S. Mcnutt,et al.  Primary Cutaneous Immunocytoma A B‐Cell Lymphoma that can Easily Be Mistaken for Cutaneous Lymphoid Hyperlasia , 1994, The American journal of surgical pathology.

[15]  H. Blomgren,et al.  Cancer risks in patients with chronic lymphocytic thyroiditis. , 1985, The New England journal of medicine.

[16]  H. Schuurman,et al.  Immunocytoma of the skin simulating lymphadenosis benigna cutis , 1984, Archives of Dermatological Research.

[17]  V. Ninfo,et al.  Helicobacter pylori and gastroesophageal reflux disease: a cross sectional study. , 2011, Hepato-gastroenterology.

[18]  O. Ishikawa,et al.  Primary cutaneous marginal zone B-cell lymphoma infiltrating the frontal bone. , 2007, European journal of dermatology : EJD.

[19]  C. Doglioni,et al.  Infectious agents in mucosa-associated lymphoid tissue-type lymphomas: pathogenic role and therapeutic perspectives. , 2006, Clinical lymphoma & myeloma.

[20]  M. Flaig,et al.  Helicobacter pylori not detected in cutaneous mucosa-associated lymphoid tissue (MALT) lymphomas , 2002, Archives of Dermatological Research.