title: Aggressive systemic mastocytosis.
abstract: Systemic mastocytosis is a rare and occasionally aggressive condition that raises major diagnostic challenges. We report a case in a 72-year-old patient in whom the diagnosis of malignant mastocytosis required two bone marrow smears and three bone marrow biopsies examined using specific staining techniques. Despite interferon therapy, a mast-cell sarcoma of the sternum developed 1 year after symptom onset, followed 1 year later by acute myeloblastic leukemia, which was rapidly fatal.
authors.affiliation: Rheumatology department du CHU Dupuytren, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France.
authors.forename: Rachida
authors.initials: R
authors.lastname: Inaoui
authors.forename: Barbara
authors.initials: B
authors.lastname: Petit
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
authors.forename: Philippe
authors.initials: P
authors.lastname: Bertin
authors.forename: Richard
authors.initials: R
authors.lastname: Trèves
mesh_terms: D000368:Aged; D019386:Alendronate / Q000627:therapeutic use; D001854:Bone Marrow Cells / Q000201:enzymology / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D015470:Leukemia, Myeloid, Acute / Q000473:pathology; D008407:Mast Cells / Q000201:enzymology / Q000473:pathology; D012515:Mast-Cell Sarcoma / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000150:complications / Q000473:pathology* / Q000628:therapy; D009378:Neoplasms, Multiple Primary; D015663:Osteoporosis, Postmenopausal / Q000188:drug therapy; D011241:Prednisone / Q000627:therapeutic use; D012697:Serine Endopeptidases / Q000378:metabolism; D053802:Tryptases
chemical_list: D016898:Interferon-alpha; D012697:Serine Endopeptidases; D053802:Tryptases; D011241:Prednisone; D019386:Alendronate