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Sporadic bloody diarrhoea‐associated thrombotic thrombocytopenic purpura‐haemolytic uraemic syndrome: an adult and paediatric comparison
Author(s) -
Karpac Charity A.,
Li Xiaoning,
Terrell Deirdra R.,
Kremer Hovinga Johanna A.,
Lämmle Bernhard,
Vesely Sara K.,
George James N.
Publication year - 2008
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2008.07116.x
Subject(s) - thrombotic thrombocytopenic purpura , medicine , adamts13 , bloody , incidence (geometry) , bloody diarrhea , gastroenterology , pediatrics , diarrhea , surgery , platelet , physics , optics
Summary Although diarrhoea‐associated haemolytic uremic syndrome (HUS) in children is well described, the clinical features of bloody diarrhoea‐associated thrombotic thrombocytopenic purpura (TTP)‐HUS in adults are not documented. Twenty‐one adults, 6·5% of the 322 adults in The Oklahoma TTP–HUS Registry, 1989–2006, have presented with bloody diarrhoea. There were no case clusters. Escherichia coli O157:H7 was identified in five patients, but many patients did not have appropriate studies. The annual incidence was 0·68/10 6 , 10‐fold less than the incidence of diarrhoea‐associated HUS in children in Oklahoma. Two (13%) of 16 patients in whom ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13) was measured had <10% activity. Severe neurological abnormalities (67%) and renal failure (62%) were common; seven patients (33%) died; no survivors have relapsed. Compared to the 38 other Oklahoma Registry patients with ADAMTS13 <10%, frequency of severe neurological abnormalities and death was not different; frequency of renal failure was greater; frequency of relapse was less. Compared to 5999 children with sporadic diarrhoea‐associated HUS in published reports, frequency of renal failure and relapse was not different; frequency of severe neurological abnormalities and death was greater ( P < 0·05 for all differences). Awareness of the continuous occurrence of sporadic bloody diarrhoea‐associated TTP–HUS in adults is important for prompt diagnosis and appropriate management.