Intravenous cyclophosphamide therapy in a case with refractory thrombotic microangiopathic hemolytic anemia and SLE.
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.
Akaogi
Jun
J
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
Akasaka
Noriko
N
Yamada
Hidehiro
H
Hama
Nobuaki
N
Satoh
Minoru
M
Nichols
Cody
C
Ozaki
Shoichi
S
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
D018501:Antirheumatic Agents; D003520:Cyclophosphamide