COX2-related multicentric mixed-type Castleman's disease in a young man.

BACKGROUND

A 34-year-old male presented with a 5-month history of fatigue, anorexia, low fever, night sweats, and oliguria with edema of the eyelid, exacerbated by abdominal distension and mild diarrhea of 3 weeks duration. Physical examination showed positive signs of ascites, palpable spleen, slight abdominal tenderness and mild rebound tenderness in the lower abdomen, and edema of the lower limbs. Initial laboratory tests revealed abnormal liver biochemistry and increased protein concentration in the urine. Chest X-ray showed minimal pleural effusion in both sides of the thoracic cavity, and ultrasound detected moderate ascites, several small lymph nodes in the retroperitoneum, and mild splenomegaly with widening of the splenic vein. A lymph node biopsy established the diagnosis, and cytokine analysis in the serum revealed COX2 as the possible mediator.

INVESTIGATIONS: Abdominal paracentesis, chest X-ray, abdominal ultrasound, thoracic and abdominal CT scans, gastroscopy, colonoscopy, biopsies of the liver, bone marrow and lymph nodes, immunophenotype staining for lymphocytes, cytokine analysis.

DIAGNOSIS: COX2-related multicentric mixed-type Castleman's disease.

MANAGEMENT: Chemotherapy and COX2 inhibitors.