Nephrology & Renal Medicine

Atypical Hemolytic Uremic Syndrome (aHUS)

ICD-10 Code

D59.35

Ultra-rare complement-mediated microangiopathy driven by genetic mutations in alternative complement pathway regulators (e.g., Factor H, Factor I, Membrane Cofactor Protein). Leads to uncontrolled complement activation, causing diffuse microvascular endothelial damage, severe thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure.

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents with clinical triad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and acute kidney injury (AKI). Symptoms include fatigue, pallor, dark urine, and oliguria. No history of prodromal bloody diarrhea. Recent triggers noted: [viral infection/pregnancy/vaccination/none].

Clinical Examination Findings

Patient appears pale and ill-appearing. Vital signs: [BP/HR/Temp]. Skin: Presence of petechiae or ecchymosis. Edema: Peripheral edema noted in lower extremities. Mental status: Alert and oriented, but may show signs of confusion if severe uremia is present.

Treatment Protocol

Immediate initiation of complement inhibition therapy (e.g., Eculizumab or Ravulizumab). Supportive care includes blood transfusions (if severe anemia), dialysis for refractory AKI, and monitoring of LDH, haptoglobin, and platelet counts. Vaccination against Neisseria meningitidis is mandatory prior to or during treatment.

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