title: Non-Hodgkin's lymphoma in systemic sclerosis: case and literature review.
abstract: The occurrence of non-Hodgkin's lymphoma in systemic sclerosis is an uncommon event. In our scleroderma cohort, a case of primary gastric B-cell lymphoma was diagnosed in 2007. The patient was a 45-year-old woman suffering from late systemic sclerosis sine scleroderma in whom non-Hodgkin's lymphoma presented with progressive weight loss, later with gastrointestinal symptoms. Subsequently, we retrospectively analyzed the charts of 251 systemic sclerosis patients consecutively admitted to our Unit from 2000 to 2008 to search for other non-Hodgkin's lymphoma cases (prevalence, 0.49%). Then we performed a Pubmed search for "systemic sclerosis & non-Hodgkin's lymphoma," limited to the English language. Twenty detailed cases of such an association were found, pointing out the following: non-Hodgkin's lymphoma seems to be associated to old age, female sex, diffuse cutaneous subset and early disease; B-cell lymphoma subtypes are the majority; the interval between systemic sclerosis and lymphoma onset is usually short; systemic sclerosis could present as a paraneoplastic syndrome in some cases. We concluded that, although rare, the association of systemic sclerosis and non-Hodgkin's lymphoma may not be coincidental and the clinician should be aware of the risk for lymphoproliferative disorders in scleroderma patients. This is the first description of non-Hodgkin's lymphoma in systemic sclerosis sine scleroderma. The insidious onset of gastric lymphomas, mimicking the most common features of gastrointestinal involvement in systemic sclerosis is underlined.
authors.affiliation: Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Via Pansini 5, 80131, Naples, Italy. serenavettori@libero.it
authors.forename: Serena
authors.initials: S
authors.lastname: Vettori
authors.forename: Stefania
authors.initials: S
authors.lastname: Staibano
authors.forename: Massimo
authors.initials: M
authors.lastname: Mascolo
authors.forename: Gennaro
authors.initials: G
authors.lastname: Ilardi
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008228:Lymphoma, Non-Hodgkin / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D015995:Prevalence; D012189:Retrospective Studies; D000069283:Rituximab; D012595:Scleroderma, Systemic / Q000150:complications* / Q000453:epidemiology; D012737:Sex Factors; D014057:Tomography, X-Ray Computed; D014750:Vincristine / Q000008:administration & dosage; D055815:Young Adult
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone