Journal issues > Issue 2, April 2025 > Severe ADAMTS13 deficit with a thrombotic thrombocytopenic purpura in a case of inaugural systemic lupus erythematosus with antiphospholipid syndrome

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

By: 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)

To read the full artice you have the following options:

Click here to log in. Register for a free subscription*

* (only possible for healthcare professionals with RIZIV/INAMI/MATRICULE working in the field of Hematology in Belgium and Luxembourg with prescription authorisation)

Purchase this article for €3.03

** (for healthcare professionals with prescribing authorisation working outside the field of Hematology in Belgium and Luxembourg as well as those without prescribing authorisation working as healthcare professional in Hematology in Belgium and Luxembourg, plus persons without prescription authorisation working outside Belgium and Luxembourg, plus healthcare professionals with prescribing authorisation outside Belgium and Luxembourg plus non-healthcare professionals from outside or inside Belgium or Luxembourg)

Top