Primary hepatic anaplastic large cell ki-1 lymphoma and celiac disease: a casual association?

Abstract

Although liver is one of the extranodal organs commonly involved in both Hodgkin and non-Hodgkin lymphoma, Primary Hepatic Lymphoma (PHL) is rare. In non-immunocompromised patients, primary hepatic malignant non-hodgkin’s lymphoma is a rare disease, with less than 100 cases reported (1). Anaplastic large-cell lymphomas (ALCL) were first described by Stein et al. in 1985 (2) as large-cell neoplasms with a pleomorphic appearance, subtotal effacement of the lymph node structure and expression of the lymphoid activation antigen CD-30 (ki-1). ALCL frequently involves both lymph nodes and extranodal sites (3). The most common extra-nodal sites affected by ALCL include skin, bone, soft tissue, lung, and liver. However, is extremely rare for ALCL to present as a liver primary lymphoma, and only eight cases have been reported. ALCL accounts for approximately 3% of adult non-Hodgkin lymphomas. The neoplastic cells consistently express CD30 molecule in all variants. Most cases of ALCL are associated with the characteristic chromosomal translocation t[2;5], which results in up regulation of anaplastic lymphoma kinase (ALK) protein. We report a case of a primary hepatic anaplastic large T-cell ki-1 non-Hodgkin lymphoma in a 55-year-old patient with celiac disease.