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.

Microangiopathic haemolytic anaemia secondary to lupus nephritis: an important differential diagnosis of thrombotic thrombocytopenic purpura

“…Akaogi et al reported a SLE patient with MAHA refractory to PE who responded to intravenous cyclophosphamide therapy. 16 Most of the cases reported with MAHA and SLE who in retrospect had lupus nephritis responded to azathioprine, cyclophosphamide, vincristine and IVIG but none of them treated with mycophenolate mofetil (MMF) as were our patients. MMF has recently been recognized to be an effective management strategy for patients with lupus nephritis.…”