Systemic mast cell disease a rare condition with an abnormal proliferation of mast cells which may infiltrate the bone marrow, liver, spleen, lymph nodes and skin. Most patients have a good prognosis, but a subset of patients show a progressive form the disease with death within a few years of onset.


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


To read more or access our algorithms and calculators, please log in or register.