Atypical Presentations of Pediatric-acquired Thrombotic Thrombocytopenic Purpura

Amit Ziv, Rima Dardik, Joanne Yacobovich, Yosef Uziel, Ruby Haviv, Einat Avishai, Gili Kenet, Amos Toren, Ortal Barel, Assaf Arie Barg*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Immune thrombotic thrombocytopenic purpura (iTTP) in children is a rare, severe thrombotic microangiopathy. This condition is characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia due to reduced activity of the von Willebrand factor-cleaving protease ADAMTS13. Methods: A retrospective case series evaluating data collected from the medical files of 4 children diagnosed with iTTP. Results: The presented case series depicts a variety of iTTP presentations: 1 case of primary iTTP, 1 case induced by Shiga toxin, 1 associated with RAS-associated autoimmune leukoproliferative disease (RALD), and 1 initial manifestation of systemic lupus erythematosus (SLE). Notably, 2 patients recovered without undergoing plasma exchange. Conclusion: Early ADAMTS13 testing in children with unexplained hemolysis or thrombocytopenia is crucial. The diverse underlying causes, including infections and autoimmune disorders, underscore the complexity of iTTP in the pediatric population. These cases highlight the necessity for personalized treatment approaches that consider each patient's unique clinical situation and potential alternatives or modifications to conventional therapeutic regimens.

Original languageEnglish
Pages (from-to)306-310
Number of pages5
JournalJournal of Pediatric Hematology/Oncology
Volume46
Issue number6
DOIs
StatePublished - 1 Aug 2024

Keywords

  • ADAMTS13
  • immune thrombotic thrombocytopenic purpura
  • pediatric
  • treatment

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