Mast Cell Leukemia (MCL) is an extremely rare and aggressive form of systemic mastocytosis, a group of disorders characterized by the abnormal proliferation and activation of mast cells, a type of immune cell. Mast cell leukemia is considered one of the most severe subtypes of systemic mastocytosis and is associated with a poor prognosis.
Key points:
Abnormal Mast Cells: In MCL, there is a massive infiltration of malignant mast cells in various tissues and organs, including the bone marrow. These cells are abnormal and can release excessive amounts of chemicals, such as histamine.
Symptoms: Symptoms of MCL are often severe and may include skin lesions, systemic symptoms (e.g., fever, weight loss), organ enlargement, and a risk of anaphylaxis.
Diagnosis: Tests can detect abnormally high number of mast cells in the bone marrow and other tissues. Molecular testing may identify specific genetic mutations.
Prognosis: MCL is considered a highly aggressive condition, and the prognosis is generally poor. Progression of the disease is rapid. Treatment options are limited.
Treatment: The treatment of MCL is challenging due to its aggressiveness. It may involve antihistamines, corticosteroids, and medications to stabilize mast cells. Allogeneic stem cell transplantation (bone marrow transplant) may be considered.