Variceal bleeding, hypersplenism, and systemic mastocytosis. Pathophysiology and management
B. E. Sumpio, G. O'Leary and R. J. Gusberg
Department of Surgery, Yale University School of Medicine, New Haven, CT 06510.
Systemic mastocytosis is characterized by an abnormal proliferation of
tissue mast cells. Though rarely a surgical disease, it occasionally
presents as variceal bleeding secondary to portal hypertension.
Ultrastructural studies of the liver and spleen and portal pressure
measurements support the hypothesis that a perisinusoidal intrahepatic
fibrosis is responsible for the increased portal pressure. When variceal
bleeding complicates systemic mastocytosis, shunt surgery is indicated,
with the type of shunt dictated by both hematologic and hemodynamic issues.
Satisfactory blockade of histamine release can be achieved preoperatively
by disodium cromoglycate and/or histamine antagonists to obviate any
systemic effects precipitated by shunting of mast cell-rich splenic blood
into the systemic circulation.