acute mast cell leukemia

Acute mast cell leukemia is a rare form of systemic mastocytosis. In this condition, a high number of immature and abnormal mast cells are found in the bone marrow. This disease is defined by the presence of at least 20% atypical mast cells in bone marrow aspirate smears. In many cases, people with this condition may also have a significant number of these cells in their blood. At diagnosis, many individuals show signs of organ damage, known as C-findings, which include blood cell count problems (cytopenias), enlarged liver (hepatomegaly), fluid in the abdomen (ascites), high blood pressure in the portal vein (portal hypertension), enlarged spleen (splenomegaly), bone lesions, and malabsorption. It is important to note that skin lesions are usually absent with this disease.

Common features in acute mast cell leukemia include signs of organ damage. These can include low blood cell counts, an enlarged liver and spleen, fluid build-up in the abdomen, problems with blood flow in the liver area (portal hypertension), skeletal lesions, and issues with nutrient absorption. Most individuals do not have skin lesions at the time of diagnosis.

Acute mast cell leukemia involves a high percentage of atypical, immature mast cells in the bone marrow. The specific reasons why these cells become abnormal are not clearly provided in the available information. Information about genetic causes or other risk factors is currently limited for this condition.

Information about treatment is currently limited for this condition.

The prognosis for acute mast cell leukemia is generally poor, with many people experiencing significant organ damage at diagnosis. Detailed information about long-term outcomes and quality of life is not provided in the current sources.

Information about current research and future directions is currently limited for this condition.