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Thrombotic microangiopathy in patients with phosphatidylserine dependent antiprothrombin antibodies and

Y Kon1, T Atsumi, H Hagiwara

  • 1Department of Medicine II, Hokkaido University Graduate School of Medicine, Kita-ku, Sapporo, Japan.

Clinical and Experimental Rheumatology
|March 11, 2008
PubMed

Insights

Thrombotic microangiopathy (TMA) is a rare condition. This study highlights TMA associated with IgG phosphatidylserine dependent antiprothrombin antibodies (aPS/PT), suggesting a thrombophilia link over ADAMTS-13 deficiency.

Area of Science:

  • Hematology
  • Immunology
  • Rheumatology

Background:

  • Thrombotic microangiopathy (TMA) is a rare disorder characterized by microvascular thrombosis.
  • TMA is occasionally reported in patients with antiphospholipid antibodies or antiphospholipid syndrome, but its exact pathogenesis remains unclear.
  • Antiphospholipid antibodies are a heterogeneous group, and specific antibody types may have distinct clinical associations.

Observation:

  • Two cases of TMA associated with IgG phosphatidylserine dependent antiprothrombin antibodies (aPS/PT) are presented.
  • Case 1 involved a 44-year-old female with systemic lupus erythematosus (SLE) who developed TMA after stroke and ticlopidine treatment, with positive IgG aPS/PT but negative anticardiolipin antibodies (aCL).
  • Case 2 involved a 32-year-old female with SLE who developed TMA during pregnancy after surgical abortion, with positive IgG aPS/PT and lupus anticoagulant (LA) but negative aCL.

Findings:

  • Neither patient had reduced levels of von Willebrand factor cleaving protease (ADAMTS-13), indicating that their TMA was likely due to thrombophilia rather than ADAMTS-13 deficiency.
  • The presence of IgG aPS/PT antibodies was a key finding in both cases, distinguishing them from TMA typically associated with other antiphospholipid antibodies.
  • Diagnostic evaluation ruled out other common causes of TMA, strengthening the association with the specific antibody profile.

Implications:

  • This study suggests that IgG aPS/PT antibodies may play a direct role in the pathogenesis of TMA, particularly in patients with underlying conditions like SLE.
  • The findings underscore the importance of testing for specific antiphospholipid antibodies, such as aPS/PT, in patients presenting with TMA, even when common antibodies like aCL are negative.
  • Successful treatment with plasma exchange in both cases indicates a potential therapeutic strategy for TMA associated with aPS/PT antibodies.

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