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:
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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.
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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.
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Diagnosis: Tests can detect abnormally high number of mast cells in the bone marrow and other tissues. Molecular testing may identify specific genetic mutations.
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Prognosis: MCL is considered a highly aggressive condition, and the prognosis is generally poor. Progression of the disease is rapid. Treatment options are limited.
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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.