[Mantle cell lymphoma associated with hyper-IgE syndrome].

A 69-year-old woman was admitted with generalized lymph node swelling and huge splenomegaly. CD5(+), Sm-IgM (+) and SmIgD (+) lymphocytes were increased in lymph nodes, spleen and bone marrow, and she was diagnosed as having mantle cell lymphoma. A diagnosis of hyper-IgE syndrome was also made, because IgE was markedly increased (174,780 u/ml) and chronic dermatitis, which was often complicated with infection, occurred repeatedly on her extremities. In this case, interleukin-4 was considered to be one of the factors involved in the hyper-IgE syndrome, because increased IgG1 and reduced IgG2 were observed. Immunological abnormality associated with the hyper-IgE syndrome seemed to contribute to the development malignant lymphoma in this case.