title: Slowly progressive systemic mastocytosis with high mast-cell burden and no evidence of a non-mast-cell hematologic disorder: an example of a smoldering case?
abstract: A 43-year-old man with extensive systemic mastocytosis with poor prognostic indicators but no overt hematologic abnormality is described. This patient's clinical presentation and course are consistent with the newly proposed 'smoldering mastocytosis' category. Long-term follow-up of patients is needed to determine whether they may be at higher risk for progression into more aggressive categories.
authors.affiliation: Room 11C205, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 10 Center Drive, MSC-1881, Bethesda, MD 20892, USA. cakin@niaid.nih.gov
authors.forename: C
authors.initials: C
authors.lastname: Akin
authors.forename: L M
authors.initials: LM
authors.lastname: Scott
authors.forename: D D
authors.initials: DD
authors.lastname: Metcalfe
mesh_terms: D000328:Adult; D019943:Amino Acid Substitution; D014408:Biomarkers, Tumor / Q000032:analysis; D001851:Bone Diseases, Metabolic / Q000209:etiology; D001853:Bone Marrow / Q000473:pathology; D018801:CD2 Antigens / Q000032:analysis; D002452:Cell Count; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D004252:DNA Mutational Analysis; D018450:Disease Progression; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D008297:Male; D008407:Mast Cells / Q000737:chemistry / Q000473:pathology; D008415:Mastocytosis / Q000097:blood / Q000150:complications / Q000235:genetics / Q000473:pathology*; D020125:Mutation, Missense; D010198:Pancytopenia / Q000209:etiology; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D017463:Receptors, Complement 3b / Q000032:analysis; D015375:Receptors, Interleukin-2 / Q000032:analysis; D013163:Splenomegaly / Q000209:etiology; D014582:Urticaria Pigmentosa / Q000097:blood / Q000473:pathology
chemical_list: D014408:Biomarkers, Tumor; D018801:CD2 Antigens; D017463:Receptors, Complement 3b; D015375:Receptors, Interleukin-2; D019009:Proto-Oncogene Proteins c-kit