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De novo thrombotic microangiopathy after kidney transplantation

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Disease definition

A rare renal disease characterized by thrombotic microangiopathy developing de novo in kidney transplant recipients with no evidence of occurrence of the disease prior to transplantation. Precipitating factors include antibody-mediated rejection, immunosuppressive medication, viral infections, and genetic abnormalities in the complement cascade, among others. The condition most commonly occurs within the first 3-6 months post-transplantation. Clinical presentation is highly variable and ranges from a limited form confined to the kidney with relatively good prognosis to a systemic variant consisting of the classic triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury.

ORPHA:244275

Classification level: Disorder

Source: PubMed ID 29157988

Prevalence: Unknown

Inheritance: -

Age of onset: All ages

ICD-10: M31.1

ICD-11: NE8Y

UMLS: C5680696

A summary on this disease is available in Français, Español, Deutsch, Português, Nederlands, Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2021.pdf) - Orphanet Urgences
Diagnostic Keys
Français (2025) - Les clés du diagnostic Logo FSMR
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