[Lymphomatoid granulomatosis: differential diagnosis and therapy]

In a 59-year-old patient presenting with fever, malaise and pulmonary masses the diagnosis of lymphomatoid granulomatosis was established histologically by open lung biopsy. Despite an immunosuppressive therapy with cyclophosphamide and prednisolone partial remission was achieved for no longer than 8 weeks. Five months later he died in a septic condition.

In our paper we discuss the present case and give a short review of the literature. Lymphomatoid granulomatosis is a rare angiocentric and angiodestructive condition which shows characteristics of both a lympho-proliferative and a necrotizing aseptic granulomatous process. It involves most frequently lungs, central nervous system and skin. The clinical course is variable, but mortality is high. Malignant lymphoma is a frequent fatal complication. Immunologic dysfunction and EBV-associated B-cell transformation seem to play an important role in the pathogenesis of this disease.

As optimal therapy is unknown, attempts have been made with immunosuppressive therapy according to the Fauci scheme as well as with combined chemotherapy according to the CHOP scheme and radiation. In a recent trial, promising results have been reported for interferon alpha 2b.