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Familial acquired thrombotic thrombocytopenic purpura in siblings – no immunogenetic link with associated human leucocyte antigens
Author(s) -
Gödel Philipp,
Fischer Julia,
Scheid Christoph,
Gathof Birgit S.,
Wolf Jürgen,
Rybniker Jan
Publication year - 2017
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.12831
Subject(s) - immunology , thrombotic thrombocytopenic purpura , thrombocytopenic purpura , medicine , antigen , purpura (gastropod) , platelet , biology , ecology
Acquired immunoglobulin G (IgG)‐mediated thrombotic thrombocytopenic purpura ( TTP ) has not yet been described in non‐twin siblings. We report two cases of acquired TTP in Caucasian sisters with inactive ADAMTS 13 metalloprotease due to ADAMTS 13 autoantibodies suggesting a role of genetic determinants in this life‐threatening disease. However, human leucocyte antigen ( HLA ) class II types presumably associated with acquired TTP were not identified in the patients, indicating that HLA class II typing may not be useful in acquired TTP risk assessment of family members.