Thrombotic microangiopathy in patients with phosphatidylserine dependent antiprothrombin antibodies and antiphospholipid syndrome.
Thrombotic microangiopathy (TMA) is a rare disorder characterized by microvascular thrombosis. TMA has been reported in patients with antiphospholipid antibodies and/or antiphospholipid syndrome but its pathogenesis is not clarified. We present two patients with TMA associated with IgG phosphatidylserine dependent antiprothrombin antibodies (aPS/PT). CASE 1: A 44-year-old Japanese female with systemic lupus erythematosus (SLE) and positive lupus anticoagulant (LA) was started on ticlopidine after having stroke. Four weeks later she developed TMA. IgG/M/A anticardiolipin antibodies (aCL) were negative, but strong positive IgG aPS/PT were detected. CASE 2: A 32-year-old Russian female with SLE was admitted because of hypertension, renal insufficiency and proteinuria at 14 weeks of pregnancy. She developed TMA after surgical abortion. IgG aPS/PT and LA were strongly positive but IgG/M/A aCL were negative. Neither case had von Willebrand factor cleaving protease (ADAMTS-13), suggesting that TMA in those patients was associated with thrombophilia rather than insufficient ADAMTS-13. Both patients were successfully treated with a series of plasma exchange.
Kon
Y
Y
Department of Medicine II, Hokkaido University Graduate School of Medicine, Kita-ku, Sapporo, Japan.
Atsumi
T
T
Hagiwara
H
H
Furusaki
A
A
Kataoka
H
H
Horita
T
T
Yasuda
S
S
Amengual
O
O
Takao
K
K
D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000276:immunology / Q000628:therapy; D001323:Autoantibodies / Q000097:blood*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D010718:Phosphatidylserines / Q000502:physiology*; D010951:Plasma Exchange; D011247:Pregnancy; D011248:Pregnancy Complications; D013927:Thrombosis / Q000150:complications* / Q000276:immunology / Q000628:therapy; D014652:Vascular Diseases / Q000150:complications* / Q000276:immunology / Q000628:therapy
D001323:Autoantibodies; D010718:Phosphatidylserines