Treatment of superior vena cava syndrome with recombinant tissue plasminogen activator in a sickle cell patient undergoing bone marrow transplantation

Superior vena cava (SVC) syndrome is a well-recognized clinical entity seen with mediastinal malignancies and intraluminal venous thrombosis. The role of recombinant tissue plasminogen activator (rt-PA) in the resolution of S VC syndrome caused by thrombosis in the bone marrow transplant settings has not been described. The authors report a case of SVC syndrome with good cl inical response in a 16-year-old female with sickle cell disease undergoing an allogeneic bone marrow transplant (BMT) from her HLA identical sibling. Shortly after her transplant, she was found to have significant facial ede ma and swelling above the neck. Concommitantly, her renal function deterior ated with progressive elevation of serum urea nitrogen and creatinine level s, requiring the use of continuous veno-venous hemofiltration. An upper ext remity venogram showed complete SVC obstruction (type III) with apparent in ferior reflux into the azygos system. rt-PA was started at a dose of (0.5 m g/kg/day) for 2 days. There was a dramatic resolution of her symptoms, incl uding significant improvement in renal function with increase in urine outp ut. A repeat venogram showed free flow from the distal tip of the central l ine consistent with a patent superior venn cava. There was no evidence of a ny bleeding manifestations with rt-PA. This report highlights the usefulnes s of rt-PA as a treatment modality for SVC syndrome in rite BMT settings.