Mast cell leukemia
The majority of reported cases present in patients with preexisting malignant mast cell disease. Approximately one-third of malignant mastocytosis patients with acute leukemia develop mast cell leukemia. MCL may also occur de novo in patients without a prior history of mastocytosis. MCL may occur in patients of all ages.
Some MCL patients may be asymptomatic. Clinical presentation is variable. The most common symptoms are fever, weight loss, and weakness. Hepatosplenomegaly and peripheral lymphadenopathy are usually present. Other common symptoms include back pain, bone pain, flushing, pruritus, nerve compression, peptic ulcers, anemia, and hypotension. The majority of patients will not present with cutaneous lesions.
Common treatment options include chemotherapy and immunotherapy. Other possible treatment options include stem cell transplants, bone marrow transplants, and splenectomy (in cases of splenomegaly with hypersplenism).
Prognosis is poor as patients typically develop multiorgan failure. The median survival is 6 months.
C94.30 – Mast cell leukemia not having achieved remission
110002002 – Mast cell Leukemia