Mast Cell Leukemia
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Mast Cell Leukemia (MCL): This is probably the rarest form of leukemia in humans and makes up <1% of cases of systemic mastocytosis. It includes both de novo and secondary forms. Two clinical variants of MCL have been recognized. In acute MCL, there are cytopenias and organ damage due to mast cell infiltrates. When bone marrow criteria for MCL are fulfilled but there is no organ damage, it is labeled as chronic MCL. Chronic MCL remains stable for months to years, but eventually progresses to acute MCL.
Skin lesions of cutaneous mastocytosis are absent. Serum tryptase levels are markedly elevated. Patients may have mediator-related symptoms such as flushing and hypotension. Most patients die within 1 year of diagnosis in acute MCL. Patient with chronic form may show prolonged survival.
Image source Ayman Qasrawi at English Wikipedia, Public domain, via Wikimedia Commons
Skin lesions of cutaneous mastocytosis are absent. Serum tryptase levels are markedly elevated. Patients may have mediator-related symptoms such as flushing and hypotension. Most patients die within 1 year of diagnosis in acute MCL. Patient with chronic form may show prolonged survival.
Image source Ayman Qasrawi at English Wikipedia, Public domain, via Wikimedia Commons
