Best prognosis: indolent mastocytosis with skin involvement alone.
Poor prognostic groups:
(1) Patients with an associated hematologic disorder have a course dependent on the underlying hematologic disorder.
(2) Mast cell leukemia is associated with a mean survival of less than 6 months.
(3) Lymphadenopathic mastocytosis with eosinophilia is associated with survival of 1-2 years without therapy, but longer survival may be seen with aggressive therapy.
Factors strongly associated with poor survival:
(1) constitutional symptoms (fatigue, weight loss, fever, sweats, ascites)
(2) anemia
(3) thrombocytopenia
(4) abnormal liver function tests with no pre-existing liver disease
(5) cytologic atypia in mast cells with a lobated nucleus
(6) low percentage of fat cells in the bone marrow biopsy
(7) an associated hematologic disorder
Additional factors associated with poor prognosis:
(1) absence of urticaria pigmentosa or other skin involvement
(2) male gender
(3) absence of bone symptoms (bone pain, arthralgias, fracture)
(4) hepatomegaly
(5) splenomegaly
(6) normal bone X-ray findings
