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Clin J Am Soc Nephrol. 2019 Mar 12. pii: CJN.11470918. doi: 10.2215/CJN.11470918. [Epub ahead of print]

Etiology and Outcomes of Thrombotic Microangiopathies.

Author information

1
Service de Néphrologie-hypertension, Dialyses, Transplantation Rénale, Hôpital Bretonneau et hôpital Clocheville.
2
Service d'Hématologie Biologique, Hôpital Bretonneau.
3
Service de Médecine Intensive Réanimation, Hôpital Bretonneau.
4
Service de Maladies Infectieuses, Hôpital Bretonneau.
5
Service d'Hématologie et Thérapie Cellulaire, Hôpital Bretonneau.
6
Équipe de Recherche Labellisée Centre National de la Recherche Scientifique 7001, Université de Tours, Tours, France.
7
Maternité Olympe de Gouges, Hôpital Bretonneau.
8
Laboratoire d'Hématologie-Hémostase, Hôpital Trousseau.
9
Équipe d'accueil7501 and.
10
Service de Médecine interne, Hôpital Bretonneau.
11
Équipe d'accueil4245, François Rabelais University, Tours, France.
12
Institut National de la Santé et de la Recherche Médicale U1246, Hôpital Bretonneau, and.
13
Laboratoire de Santé Publique, Hôpital Bretonneau, Centre Hospitalier Universitaire Tours, Tours, France.
14
Centre Hospitalier Universitaire Pontchaillou, Service de Néphrologie, Rennes, France.
15
Université Rennes 1, Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche 1085, Rennes, France; and.
16
Centre de recherche en Transplantation et immunologie, Unité Mixte de Recherche 1064, Institut National de la Santé et de la Recherche Médicale, Université de Nantes et département de Néphrologie et Immunologie, Centre Hospitalier Universitaire Nantes, Nantes, France.
17
Service de Néphrologie-hypertension, Dialyses, Transplantation Rénale, Hôpital Bretonneau et hôpital Clocheville, halimi@med.univ-tours.fr.

Abstract

BACKGROUND AND OBJECTIVES:

Thrombotic microangiopathies constitute a diagnostic and therapeutic challenge. Secondary thrombotic microangiopathies are less characterized than primary thrombotic microangiopathies (thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome). The relative frequencies and outcomes of secondary and primary thrombotic microangiopathies are unknown.

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS:

We conducted a retrospective study in a four-hospital institution in 564 consecutive patients with adjudicated thrombotic microangiopathies during the 2009-2016 period. We estimated the incidence of primary and secondary thrombotic microangiopathies, thrombotic microangiopathy causes, and major outcomes during hospitalization (death, dialysis, major cardiovascular events [acute coronary syndrome and/or acute heart failure], and neurologic complications [stroke, cognitive impairment, or epilepsy]).

RESULTS:

We identified primary thrombotic microangiopathies in 33 of 564 patients (6%; thrombotic thrombocytopenic purpura: 18 of 564 [3%]; atypical hemolytic and uremic syndrome: 18 of 564 [3%]). Secondary thrombotic microangiopathies were found in 531 of 564 patients (94%). A cause was identified in 500 of 564 (94%): pregnancy (35%; 11 of 1000 pregnancies), malignancies (19%), infections (33%), drugs (26%), transplantations (17%), autoimmune diseases (9%), shiga toxin due to Escherichia coli (6%), and malignant hypertension (4%). In the 31 of 531 patients (6%) with other secondary thrombotic microangiopathies, 23% of patients had sickle cell disease, 10% had glucose-6-phosphate dehydrogenase deficiency, and 44% had folate deficiency. Multiple causes of thrombotic microangiopathies were more frequent in secondary than primary thrombotic microangiopathies (57% versus 19%; P<0.001), and they were mostly infections, drugs, transplantation, and malignancies. Significant differences in clinical and biologic differences were observed among thrombotic microangiopathy causes. During the hospitalization, 84 of 564 patients (15%) were treated with dialysis, 64 of 564 patients (11%) experienced major cardiovascular events, and 25 of 564 patients (4%) had neurologic complications; 58 of 564 patients (10%) died, but the rates of complications and death varied widely by the cause of thrombotic microangiopathies.

CONCLUSIONS:

Secondary thrombotic microangiopathies represent the majority of thrombotic microangiopathies. Multiple thrombotic microangiopathies causes are present in one half of secondary thrombotic microangiopathies. The risks of dialysis, neurologic and cardiac complications, and death vary by the cause of thrombotic microangiopathies.

PODCAST:

This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2019_03_12_CJASNPodcast_19_04_.mp3.

KEYWORDS:

Acute Coronary Syndrome; Anemia; Cognitive Dysfunction; Confidence Intervals; Epilepsy; Escherichia coli; Folic Acid; Glucosephosphate Dehydrogenase Deficiency; Hypertension; Immune System Diseases; Incidence; Logistic Models; Malignant; Neoplasms; Odds Ratio; Pregnancy; Purpura; Retrospective Studies; Shiga Toxin; Sickle Cell; Stroke; Thrombotic Microangiopathies; Thrombotic Thrombocytopenic; heart failure; hospitalization; renal dialysis

PMID:
30862697
DOI:
10.2215/CJN.11470918

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