Non-Hodgkin's lymphoma with rheumatism-like presentation and multiple cystic and nodular lesions in the liver.

We report the case of a non-Hodgkin's lymphoma in an isolated and not previously diagnosed "adult polycystic liver disease". In this case, a rheumatism-like presentation was associated with the discovery of multiple nodular and cystic lesions in the liver. Of these, the former were related to lymphoma involvement, the latter to pre-existent liver disease. The singularity of the case, not previously reported, and some difficulties met in diagnosing the two pathologies, justify the report.

Long time interval between multiple sclerosis onset and occurrence of primary Sjögren's syndrome in a woman treated with interferon-beta.

Primary Sjören's syndrome with central nervous system involvement can clinically mimic multiple sclerosis (MS). However, SS and MS may coexist. We report here a case of a 48-year-old woman affected by relapsing-remitting MS, good responder to interferon (IFN)-beta 1a, developing sicca complex after 29 years from MS onset. At the age of 48, after 5 years successful treatment with i.m. IFN-beta 1a, xerophtalmia and xerostomia with dysphagia occurred. Autoantibody screening for connective tissue diseases, including anti-ENA, was negative. Schirmer's test showed reduced lacrimal gland function and a minor salivary gland biopsy showed chronic inflammatory infiltration with fibrosis, acinar atrophy and ductal ectasia. According to clinical and pathological findings a diagnosis of SS was made. Other cases of connective tissue diseases after IFN-beta treatment have been described. However, this is, to our knowledge, the first report on the development of primary SS after long time interval from MS onset in a woman treated with IFN-beta. Although there are no evidences about a possible role of IFN-beta in triggering SS yet, a screening for clinical and laboratory signs of SS should be assessed in MS patients during IFN-beta treatment.

Hepato-splenic gammadelta T-cell lymphoma: a rare entity mimicking the hemophagocytic syndrome.

Hepatosplenic gammadelta T-cell lymphoma is a rare histologic type of the peripheral T-cell lymphomas, clinically characterized by predominant involvement of liver and spleen, no or little adenopathy, and an often aggressive course; it affects mainly adolescents and young adults, with a male predominance. Postthymic T-cell malignancies are heterogeneous in their clinical and laboratory features. Among the gammadelta postthymic T-cell lymphomas, two distinct entities (cutaneous and hepatosplenic, respectively) are reported in the literature. The former shows predominant multiple involvement of the skin and subcutaneous tissue; it occurs mostly in older patients and the phenotype is CD3-, CD4-, CD8-. Because of the small number of reports, the course of the disease was unknown. The latter shows a clinical picture characterized by hepatosplenomegaly, no or little adenopathy, and sometimes systemic symptoms (fever, cytopenias likely due to hypersplenism); it presents a peculiar sinusoidal involvement of liver and spleen. The bone marrow histologic feature often reveals a little infiltration, especially sinusoidal and easily underestimated phenotype: CD2+, CD3+, CD7+, CD5-, CD4-, CD8-, CD44+. Few cases of this lymphoma associated by hemophagocytic syndrome are described (Sun, 1990; Kadin, 1981; Jaffe, 1983). We report a case of a young man with a rapid and fatal course in which the more important clinical feature was hemophagocytosis. The diagnosis of lymphoma was very difficult because of paucity of histologic involvement, and only the rearrangement of TCR gamma chain gene by polymerase chain reaction on paraffin sections confirmed a clonal T-cell proliferation.