Fatal haemophagocytic lymphohistiocytosis and atypical haemolytic uraemic syndrome following coronavirus disease 2019 infection.
Jennifer Khong, Alisa Liberman, Arabella Hammoudeh et al.
✦ AI-curated · Sources linked
Case in brief
A previously healthy woman in her early 30s developed rash, fever and arthralgia three weeks after SARS-CoV-2 infection, followed by rapidly progressive multiorgan failure. Investigations showed severe thrombocytopenia, microangiopathic haemolytic anaemia, acute kidney injury and ferritin >100 000 ng/mL. Kidney biopsy showed thrombotic microangiopathy, supporting atypical haemolytic uraemic syndrome (aHUS); additional laboratory findings and splenomegaly supported concurrent haemophagocytic lymphohistiocytosis (HLH). Despite plasmapheresis, corticosteroids, etoposide, eculizumab and continuous renal replacement therapy, she died seven weeks after infection. Autopsy confirmed both diagnoses.
What made this case unusual
The case featured concurrent aHUS and HLH after COVID-19, with extensive thrombotic microangiopathy and bone marrow haemophagocytosis confirmed at autopsy.
Diagnostic / clinical pearl
In a patient with post-COVID multiorgan failure, thrombocytopenia, microangiopathic haemolysis and acute kidney injury alongside marked hyperferritinaemia and other HLH findings may indicate concurrent aHUS and HLH.
Why it matters
The report illustrates that these overlapping syndromes can accompany a catastrophic course despite multiple therapies.