title: Neurofibromatosis type I and smoldering multiple myeloma: a case report.
abstract: We present a case of a 64-year-old woman with neurofibromatosis (NF1) and smoldering multiple myeloma (SMM). SMM was diagnosed 9 years ago when the asymptomatic patient was found to have mild anemia, IgA paraproteinemia, hypogammaglobulinemia, osteopenia without any lytic bone lesions and bone marrow plasmacytosis. During follow-up period she remained stable in an excellent clinical condition without requiring any therapy for almost 4 years. Forty-two months after diagnosis she had a femoral fracture and since then biphosphonates have been administered intravenously, once monthly. Subsequent evaluations of the disease showed a dramatic reduction of IgA paraprotein to below half the initial value. We will discuss the probable pathogenesis of plasma cell dyscrasia in NF1 patients, as well as the likely antimyeloma activity of biphoshonates.
authors.affiliation: Department of Hematology, Venizelion General Hospital, Heraklion, Crete, Greece.
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Danilatou
authors.forename: Demetra
authors.initials: D
authors.lastname: Liapi
authors.forename: Maria
authors.initials: M
authors.lastname: Psyllaki
authors.forename: Aria
authors.initials: A
authors.lastname: Chatzivasili
authors.forename: Irini
authors.initials: I
authors.lastname: Chronaki
authors.forename: Polyvios
authors.initials: P
authors.lastname: Heliakis
mesh_terms: D050071:Bone Density Conservation Agents / Q000008:administration & dosage* / Q000009:adverse effects; D004164:Diphosphonates / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D005264:Femoral Fractures / Q000139:chemically induced; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D008875:Middle Aged; D009101:Multiple Myeloma / Q000097:blood / Q000188:drug therapy* / Q000209:etiology* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000097:blood / Q000150:complications* / Q000188:drug therapy* / Q000473:pathology; D010266:Paraproteins / Q000032:analysis
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D007070:Immunoglobulin A; D010266:Paraproteins