title: Immunohistochemical studies of c-kit, transforming growth factor-beta, and basic fibroblast growth factor in mast cell disease.
abstract: In 58 patients with mast cell disease (MCD) and three with basophilic leukemia, bone marrow (54 cases) or skin tissue (four cases) was studied immunohistochemically for expression of Kit (c-kit protein), the different isomers of transforming growth factor-beta (TGF-beta), basic fibroblast growth factor (bFGF), and their respective receptors. Kit was expressed in all cases of MCD but in none of basophilic leukemia. Expression pattern of cytokines and their receptors was variable in systemic MCD with (SMCD-HD) or without (SMCD) associated hematologic disorder. However, type I TGF-beta receptor (TGFbeta1R) was not expressed in 30% of SMCD-HD patients or in patients with mast cell leukemia, but the remaining cases of MCD showed near uniform expression. The associated hematologic disorders in TGFbeta1R-negative cases of SMCD-HD were prognostically less favorable than those associated with TGFbeta1R-positive cases of SMCD-HD. The results confirm the diagnostic value of Kit immunohistochemistry in MCD and suggest a biologically relevant heterogeneity in TGFbeta1R expression among patients with SMCD-HD.
authors.affiliation: Division of Hematopathology and Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Jin-Young
authors.initials: JY
authors.lastname: Baek
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
authors.forename: Animesh
authors.initials: A
authors.lastname: Pardanani
authors.forename: Chin-Yang
authors.initials: CY
authors.lastname: Li
mesh_terms: D001853:Bone Marrow; D016207:Cytokines / Q000378:metabolism; D016222:Fibroblast Growth Factor 2 / Q000378:metabolism*; D006801:Humans; D007124:Immunoenzyme Techniques; D007946:Leukemia, Mast-Cell / Q000378:metabolism*; D012515:Mast-Cell Sarcoma / Q000378:metabolism; D008415:Mastocytosis / Q000378:metabolism*; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism*; D012189:Retrospective Studies; D012697:Serine Endopeptidases / Q000378:metabolism; D016212:Transforming Growth Factor beta / Q000378:metabolism*; D053802:Tryptases
chemical_list: D016207:Cytokines; D016212:Transforming Growth Factor beta; D016222:Fibroblast Growth Factor 2; D019009:Proto-Oncogene Proteins c-kit; D012697:Serine Endopeptidases; D053802:Tryptases