ULTRASOUND PRESENTATION OF PRIMARY MULTICENTRIC CASTLEMAN'S DISEASE IN A 39 YEAR OLD PATIENT

Problemstellung: Castelman disease (cd) is a rare disorder characterized by a proliferation of lymphoid tissue and was originally described by Benjamin Castleman 50 years ago. It may occur anywhere along the lymphatic chain but the mediastinum is the most common primary location. While in most cases a unicentric manifestation is seen a multricentric form of castleman's disease is known since 1978. The etiologic understanding of cd has strongly improved since the understanding of its association with the human immunodeficiency virus (hiv) and human herpes virus 8 (hhv 8) infection however the exact pathogenesis is still not known.

Methoden: We herein describe the case of a 39 year old mal patient with known hiv infection who was admitted to our clinic with a decreasing general condition of health, fever, and diarrhea.

Ergebnisse: Ultrasonography (us) was performed shortly after submission and demonstrated multiple echogenic abdominal lymph nodes of a maximal size of 38mm. The lymphatic mass presented with a smooth surface, without clear signs of vascularization, and was located along the abdominal aorta, at splenic and hepatic hilus, and iliacal. For further investigation an us-guided biopsy of an iliacal lymph node was performed and histologic examination led to the diagnosis of a primary multicentric castleman disease.

A therapeutic approach with interferone α and chemotherapy led to a distinct reduction of tumor mass and to a continous improvement of the patient's state of health.

Schlussfolgerungen: Differentiated ultrasonographic examination of lymphatic tissue can be helpful in the diagnosis of rare lymphoproliferative disorders.