Systemic mastocytosis (SM) with progessive infiltration of various org
ans by mast cells is a rare disease with a mean survival of 2 years, S
o far there is no established therapy. We describe a patient with prog
ressive mastocytosis who had a clinical response to interferon alpha-2
b 3 x 5 Mio U sc three times a week. The response is stable 3 years af
ter initiation of therapy and includes reduction of abdominal lymphade
nopathy, ascites, decrease of serum and urinary histamine levels as we
ll as pulmonary reticulonodular markings.