Atypical reduction of plasma ADAMTS13 activity by a non-IgG-type inhibitor in a patient with hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli

Abstract

Thrombotic microangiopathies include hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Measurement of plasma levels of "a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13" (ADAMTS13) activity can distinguish HUS from TTP. Reduced plasma ADAMTS13 activity (< 10% normal range) is atypical for HUS, but not for TTP. However, we detected reduced ADAMTS13 activity in a patient with Shiga toxin-producing Escherichia coli-associated HUS caused by non-IgG anti-ADMTS13 autoantibodies. Furthermore, the patient exhibited possible genetic abnormalities associated with atypical HUS. The patient fully recovered after administration of supportive therapy. To the best of our knowledge, very few cases of STEC-HUS with reduced ADAMTS13 activity have been reported; thus far, none have described the presence of non-IgG anti-ADMTS13 autoantibodies. Therefore, we suggest that anti-ADAMTS13 analyses should be performed in patients diagnosed with STEC-HUS, especially in those who present with prolonged healing or unexpected clinical symptoms.

Keywords: A disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13; Atypical hemolytic uremic syndrome; Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome; Thrombotic microangiopathy; Thrombotic thrombocytopenic purpura.

Fig. 1

Patient’s clinical course