Fatal nontraumatic splenic rupture in hemophilia and the kasabach-merritt syndrome

Fatal nontraumatic splenic rupture is documented in two patients, one with classical hemophilia and the other with the Kasabach-Merritt syndrome. Splenic rupture was due to multiple hemangioma-like lesions in both patients. These lesions apparently were acquired, possibly as a result of infection and disordered coagulation. The mechanism of rupture of the lesions could not be determined. The rarity of nontraumatic splenic rupture in these two syndromes probably explains why splenectomy was not done in either patient even though, in retrospect, evidence for subcapsular splenic hemorrhage was present for 2 and 16 days before death from rupture of the splenic capsule, respectively. Although splenectomy is more hazardous in patients with impaired coagulation, the situation also is more urgent in such patients since exsanguination occurs so rapidly following splenic capsular rupture (20 minutes and 90 minutes, respectively, in the present two patients). Patients with impaired coagulation who develop evidence of subcapsular splenic hemorrhage should be evaluated quickly and definitively, using splenic angiography if appropriate. Once subcapsular splenic hemorrhage has been confirmed, splenectomy should be seriously considered.