This study suggests that a conservative approach may be safe and appropriate in patients with NFPA if followed up with appropriate imaging surveillance, whether postoperative or without primary surgery.
Further investigations revealed her ADAMTS13 (a metalloproteinase that cleaves von Willebrand factor) activity to be <5, confirming the diagnosis of TTP. ADAMTS13 activity of 68-163% is considered normal and activity of less than 10% is diagnostic of TTP. She is currently monitored as an outpatient and continues to have Rituximab for subclinical relapses. Conclusion Bleeding of any form is an extremely rare and severe presentation of TTP. There are only a few case reports of TTP in children till date but there are no reported paediatric cases of symptomatic bleeding as initial presentation. Acquired TTP is mainly reported in adolescence. TTP is specifically related to severe ADAMTS13 deficiency which is also used to monitor disease activity.
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