title: Intravenous cyclophosphamide therapy in a case with refractory thrombotic microangiopathic hemolytic anemia and SLE.
abstract: The case of a 27-year-old woman who simultaneously presented with SLE and severe refractory thrombotic microangiopathic hemolytic anemia (TMHA) is reported. She had extremely high levels of platelet-associated IgG (PAIgG), and her TMHA was refractory to plasma exchange and corticosteroid therapy. However, the TMHA was effectively controlled by i.v. cyclophosphamide therapy. ITP and TTP are generally considered distinct diseases; however, TMHA may occur secondary to platelet aggregation via autoimmune mechanisms in certain cases. Immunosuppressive therapy at an early stage of the disease may be beneficial in refractory cases of TMHA with autoimmune features.
authors.affiliation: Division of Rheumatology and Allergy, Department of Internal Medicine, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, Kanagawa, 216-8511, Japan. j2akaogi@marianna-u.ac.jp
authors.forename: Jun
authors.initials: J
authors.lastname: Akaogi
authors.forename: Noriko
authors.initials: N
authors.lastname: Akasaka
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Yamada
authors.forename: Nobuaki
authors.initials: N
authors.lastname: Hama
authors.forename: Minoru
authors.initials: M
authors.lastname: Satoh
authors.forename: Cody
authors.initials: C
authors.lastname: Nichols
authors.forename: Shoichi
authors.initials: S
authors.lastname: Ozaki
mesh_terms: D000328:Adult; D000743:Anemia, Hemolytic / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D018501:Antirheumatic Agents / Q000008:administration & dosage*; D003520:Cyclophosphamide / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D013927:Thrombosis / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; D003520:Cyclophosphamide