Adolescent acquired thrombotic thrombocytopenic purpura: An analysis of the Pediatric Health Information System database

Cohen, Clay T; Zobeck, Mark; Kim, Taylor O; Sartain, Sarah E.; Raffini, Leslie; Srivaths, Lakshmi

doi:10.1016/j.thromres.2022.12.011

Cited by 2 publications

(4 citation statements)

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“…Pediatric iTTP may result in severe morbidity or death. The most extensive pediatric iTTP registries have reported mortality rates ranging from 6% to 9% and relapse rates between 20% and 24% 2,19 . In line with these findings, among our patients, there were no instances of mortality and only 1 patient relapsed.…”

Section: Discussionsupporting

confidence: 90%

“…The most extensive pediatric iTTP registries have reported mortality rates ranging from 6% to 9% and relapse rates between 20% and 24%. 2,19 In line with these findings, among our patients, there were no instances of mortality and only 1 patient relapsed. Notably, outcomes in the pediatric age category appear more favorable than those documented in adult registries.…”

Section: Clinical Presentations and Precipitating Factorssupporting

confidence: 90%

“…1 The prevalence of childhood and adolescent TTP is ~1 case per million children. 2 TTP is caused by reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), which may be hereditary or secondary to autoantibodies directed against ADAMTS13. 3 The initial clinical description by Moschowitz, 4 noting the classical pentad of findings (microangiopathic hemolytic anemia, thrombocytopenia, fever, neurological manifestations, and renal failure), is rarely encountered in the modern era due to heightened clinical awareness and early diagnosis.…”

mentioning

confidence: 99%

“…The annual incidence of TTP is about 1 new case per million people of which less than 10% are in the pediatric age group 1 . The prevalence of childhood and adolescent TTP is ~1 case per million children 2 . TTP is caused by reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), which may be hereditary or secondary to autoantibodies directed against ADAMTS13 3 …”

mentioning

confidence: 99%

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Atypical Presentations of Pediatric-Acquired Thrombotic Thrombocytopenic Purpura

Ziv,

Dardik,

Yacobovich

et al. 2024

Journal of Pediatric Hematology/Oncology

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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.

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Section: Discussionsupporting

confidence: 90%

Section: Clinical Presentations and Precipitating Factorssupporting

confidence: 90%