Background: Henoch Schonlein Purpura (HSP) is a common vasculitis in children. Thrombotic events rarely occur. In the extreme end of the spectrum of thrombosis, a condition called thrombotic storm was seen to occur in a patient with HSP.

Aims: This case report aims to discuss the clinical course and management of thrombotic storm as a complication of Henoch-Schonlein Purpura in a female adolescent patient.

Methods: This a case report of a female adolescent patient presenting with thrombotic storm as a complication of Henoch-Schonlein Purpura.

Results: Figure 1a-1c: MRAsContract abdominal CT scan
A previously healthy 14 year old female was diagnosed with HSP and unexpectedly developed multiple sites of thrombosis (deep venous thrombosis, superior sagittal sinus thrombosis and transverse sinus thrombosis). This led to the suspicion of a rare, accelerated thrombotic event known as thrombotic storm. Despite being on enoxaparin, she went on to develop common iliac vein thrombosis, which strengthened the diagnosis of thrombotic storm. Anticoagulation was continued, after which, there was marked clinical improvement. Anticoagulation was continued after discharge, with noted resolution of DVT on the second month of treatment. Further improvement was seen with resolution of common iliac vein and superior sagittal sinus thrombosis on the seventh month of anticoagulation and resolution of transverse sinus thrombosis on the 16^th month of anticoagulation. The patient remains to be asymptomatic.

Conclusions: Thrombotic storm occurring as a complication of a vasculitic disease strengthens the link between inflammation and thrombosis. Since there is no specific test to diagnose thrombotic storm, the clinician’s awareness of this syndrome is key, so that treatment is started immediately upon recognition of the symptoms. Once the thrombotic cascade resolves, prognosis is excellent, however lifelong anti-coagulation is recommended to prevent recurrent thrombosis.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/a-rare-case-of-thrombotic-storm-in-henoch-schonlein-purpura/