Articles

Severe ADAMTS13 deficit with a thrombotic thrombocytopenic purpura in a case of inaugural systemic lupus erythematosus with antiphospholipid syndrome

BJH - volume 16, issue 2, april 2025

L. Dassy MD, D. Aktan MD, A. Jaspers MD, PhD, S. Shalchian-Tehran MD, V. Delvaux MD, PhD, F. Maraite MD, F. Pitance MD, J-M. Minon MD, PhD, O. Kaye MD, PhD

SUMMARY

ADAMTS13, an important enzyme in the regulation of von Willebrand factor, may be deficient, leading to a thrombotic thrombocytopenic purpura, a subtype of thrombotic microangiopathy causing thrombocytopenia, organ failure and haemolytic anaemia. This condition may also be associated with antiphospholipid syndrome and/or systemic lupus erythematosus. We report a case of ischaemic neurological lesions due to an inaugural systemic lupus erythematosus and antiphospholipid syndrome, with the demonstration of a decreased ADAMTS13 activity without biological signs of thrombotic microangiopathy, associated with a possible COVID-19 trigger.

(BELG J HEMATOL 2025;16(2):70–7)

Read more