engleski

CASTLEMAN DISEASE:A CASE REPORT

Castlemans disease (CD) was described from Benjamin Castleman in 1954. The disease today is enumerated among lymphoproliferative disorders and has unknown etiology, but a interleukin-6 (IL-6) dysregulation and a reaction to viral antigens (HHV8) especially in patients with immunodeficiency is suspected. It is observed in adult and young people, in male or female with equal frequency ; the appearance in childhood is extremely rare. The disease shows various clinical and histological pictures, with a localized type (involvement of one lymph node group) described more frequently than the multicentric one. Histological examination distinguish a "hyaline-vascular type" that represents approximately the 91%, a plasma cell type" that represents approximately the 9% has an aggressive clinical outcome, and the "mixed types". Initial symptoms are nearly absent, but not for the plasma cell type. We describe the unusual clinical case of a male patient 35 years old that referred us for abdominal pain. All laboratory tests were normal except significantly increased values of liver enzimes. CT showed a retroperitoneal mass with highest diameter of 12x7cm, which was compressing aorta, and especially vena cava. The biopsy of this lesion suspected a lymphoproliferative disease. Difficult surgical procedure was performed through medial laparatomy by multidisciplinary team of surgeons(abdominal and vascular).Extraction of whole tumor was done and subsequent histological examination showed hyaline vascular type of CD.

castleman

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