Diagnosis of Hereditary TTP Caused by Homozygosity for a Rare Complex ADAMTS13 Allele After Salmonella Infection in a 43-Year-Old Asylum Seeker

A 43-year-old Armenian patient was diagnosed with salmonella infection and thrombotic microangiopathy (TMA). The clinical course was benign with resolution of all laboratory alterations after antibiotic treatment. Constantly deficient ADAMTS13 activity without ADAMTS13 inhibitors and evidence of hom...

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Published inFrontiers in medicine Vol. 8; p. 639441
Main Authors Wendt, Ralph, Kalbitz, Sven, Otto, Felix, Falter, Tanja, Beige, Joachim, Rossmann, Heidi, Lämmle, Bernhard
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 26.02.2021
Subjects
ADAMTS13
cTTP
hereditary TTP
Medicine
TMA
Upshaw Schulman syndrome
hereditary TTP
TMA
ADAMTS13
Upshaw Schulman syndrome
cTTP
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Summary:A 43-year-old Armenian patient was diagnosed with salmonella infection and thrombotic microangiopathy (TMA). The clinical course was benign with resolution of all laboratory alterations after antibiotic treatment. Constantly deficient ADAMTS13 activity without ADAMTS13 inhibitors and evidence of homozygosity for a rare complex ADAMTS13 allele led to the diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP). Half-life of ADAMTS13 after plasma infusion was calculated (27,6h) and double blinded plasma infusion as well as ergometric exercise with and without prior plasma infusion undertaken to investigate suspected smoldering TTP activity.
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These authors share senior authorship
This article was submitted to Hematology, a section of the journal Frontiers in Medicine
Reviewed by: Bipin P. Kulkarni, National Institute of Immunohaematology (ICMR), India; Ioanna Sakellari, G. Papanikolaou General Hospital, Greece
Edited by: Robert W. Maitta, Case Western Reserve University, United States
ORCID: Ralph Wendt orcid.org/0000-0003-1600-658; Bernhard Lämmle orcid.org/0000-0003-4538-5154; Heidi Rossmann orcid.org/0000-0002-7532-8540
ISSN:2296-858X
2296-858X
DOI:10.3389/fmed.2021.639441