Systemic Mastocytosis

Raajit K. Rampal, MD, PhD


April 24, 2020

Systemic mastocytosis (SM) is a group of heterogeneous blood-related disorders that result in an excessive number of mast cells, which can build up in the skin, bone marrow, gastrointestinal tract, liver, spleen, or lymph nodes.

SM can be divided into five subgroups on the basis of mast cell burden, organ involvement, and SM-related organ damage. These subgroups range from indolent SM to mast cell leukemia. Mutations of the receptor tyrosine kinase KIT are known to play a central role in the progression of SM by enabling proliferation and survival of abnormal mast cells in affected tissues. Prognosis varies depending on disease stage and severity.

Dr Raajit Rampal, from Memorial Sloan Kettering Cancer Center in New York, discusses key diagnostic aspects, risk stratification, and the individualization of treatment in advanced SM.


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