In a small proportion of cases, acute mast cell leukemia may evolve from a more progressive form of systemic mastocytosis.

The common signs and symptoms include fever, headache, flushing of face and trunk.

These former symptoms are due to release of a substance called histamine from neoplastic mast cells.

Abdominal ultrasound or computerized tomography (CT) scanning is used to look for hepatosplenomegaly and lymphadenopathy.

[12] Plasma and urinary histamine levels are frequently elevated in mast cell leukemia.

[14] Although splenectomy has led to brief responses in patients with mast cell leukemia,[4][15] no firm conclusions as to the efficacy of this treatment are possible.

Chemotherapy with combination of cytosine arabinoside and either idarubicin, daunomycin, or mitoxantrone as for acute myeloid leukemia has been used.