Sinushistiocytosis with massive lymphadenopathy complicated by an obstruction of the vena cava superior and inferior

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disease of the lymph nodes, still of unknown origin. We are reporting the case of a 16 year old boy with SHML which occurred in 1983. Investigations showed a massive lymphadenopathy of the mediastinal and abdominal nodes, causing displacement and compression of surrounding tissue. The patient further developed a blockage of the vena cava superior and inferior, leading to numerous collateral circulation routes iri the upper and lower extremities. The etiology of the venous blockage is still disputed. It is possible that they are the result of compresssion of the major veins. Alternatively, the cause could lie in the disruption of the coagulation system. Finally and more likely, the problem could be the result of fibrosis developing through the healing process.