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CASTLEMAN'S DISEASE. Kristine Juntunen, M.D., Basil Varkey, M.D. Medical College of Wisconsin, Milwaukee, WI.
Case: 73 yo white male presents with fever, anorexia and dry cough unresponsive to course of Zithromax. Patient returns to ER with persistent cough, odynophagia and nausea. Physical exam reveals low grade temperature, tachypnea, hypoxia and patient in respiratory distress. Chest x-ray shows mediastinal widening and cat scan reveals a 2cm left hilar mass with multiple mediastinal lymph nodes. Transthoracic biopsy reveals angiofollicular hyperplasia also known as Castleman's disease.
Discussion: Castleman's disease was first described by Frank Castleman in a group of patients with localized mediastinal lymph node enlargement. It has 2 histologic types including the hyaline vascular and the plasma cell variant. The plasma cell type is often found in patients with multicentric disease which presents as a systemic illness with disseminated lymphadenopathy. The hyaline vascular type is a localized disease, 70% of which are intrathoracic. The pathogenesis is unknown however certain theories suggest overproduction if IL-6 causing autocrine proliferation of fibroid dendritic cells. The localized type is curable by surgery while the multicentric variant is less responsive overall with only minimal improvement with chemotherapy, radiation, and steroids.
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