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A Rare Case of Atypical Hemolytic Uremic Syndrome (HUS) in an Adult Male: A Catastrophic Presentation

Sindhu C Pokhriyal1, Samuel Sule-Saa1, Jemima A Alemonai1

  • 1Internal Medicine, One Brooklyn Health-Interfaith Medical Center, Brooklyn, USA.

Cureus
|July 19, 2024
PubMed

Atypical hemolytic uremic syndrome (HUS) is extremely rare in adults. HUS is characterized by hallmark features of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury. Atypical HUS (aHUS) is caused by uncontrolled complement activation. The complement activation can be triggered by infections such as Streptococcus pneumoniae or influenza, pregnancy, malignancy, cytotoxic drugs, organ transplants, or autoimmune diseases. Genetic mutations and autoantibodies have been found to play a crucial role in the pathogenesis of dysregulated complement activity. The majority of cases of atypical HUS due to invasive S. pneumoniae infection are more commonly seen in children. We present a case of S. pneumoniae HUS (Sp-HUS) presenting with multiorgan failure, disseminated intravascular coagulation (DIC), and limb ischemia in an adult. This case highlights the importance of considering S. pneumoniae HUS (Sp-HUS) in the differential diagnosis of thrombotic microangiopathies (TMA) in adults.

Keywords:
atypicalcomplementhemolytichusstreptococcus pneumoniaesyndromeuremic

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