Abstract:
:Mast cell disease (MCD), a proliferation of mast cells (MC), is occasionally associated with hematologic malignancies. Neoplastic MC have activating c-kit mutations. c-kit is a receptor tyrosine kinase required for the development, proliferation, and survival of MC. Interaction of c-kit with its ligand stem cell factor induces dimerization, receptor phosphorylation, and signal transduction. The most common c-kit mutation detected in neoplastic MCD is Asp816Val, which results in ligand-independent autophosphorylation of the receptor leading to MC proliferation. We describe the rare occurrence of MCD associated with acute myeloid leukemia, report a novel c-kit mutation Asp816 His, and discuss the pathogenesis of MCD associated with hematologic malignancies.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Pullarkat VA,Pullarkat ST,Calverley DC,Brynes RKdoi
10.1002/1096-8652(200012)65:4<307::aid-ajh10>3.0.ckeywords:
subject
Has Abstractpub_date
2000-12-01 00:00:00pages
307-9issue
4eissn
0361-8609issn
1096-8652pii
10.1002/1096-8652(200012)65:4<307::AID-AJH10>3.0.Cjournal_volume
65pub_type
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