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Hemolytic uremic syndrome / Thrombotic thrombocytopenic purpura
Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenic Purpura (TTP) are rare but serious disorders that affect the blood and kidneys. Both conditions involve the formation of small blood clots (microthrombi) in blood vessels, which can block circulation, damage organs, and cause the breakdown of red blood cells (hemolysis) along with a low platelet count (thrombocytopenia). While they share similarities, their underlying causes and presentations may differ.
- Type 1 (Distal RTA): The distal tubule cannot properly excrete acid.
- Type 2 (Proximal RTA): The proximal tubule cannot reabsorb bicarbonate efficiently.
- Type 4 RTA: Usually linked to low aldosterone levels or resistance to its effects, leading to problems with potassium and acid balance.
Causes
- HUS: Shiga toxin-producing E. coli infection (STEC-HUS), genetic abnormalities, certain medications, or other systemic diseases.
- TTP: Autoimmune destruction or genetic deficiency of ADAMTS13 enzyme, infections, pregnancy, cancer, or medications.
Symptoms
- Fatigue, weakness, and paleness (from anemia)
- Unexplained bruising or bleeding (due to low platelets)
- Decreased urine output or blood in urine (kidney involvement)
- High blood pressure
- Confusion, headache, or seizures (if the brain is affected)
- Fever (more common in TTP)
Treatment & Management
Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenic Purpura (TTP) are medical emergencies that require immediate treatment in a hospital setting. In HUS, management is largely supportive and may include intravenous fluids to maintain hydration, blood transfusions to treat anemia, medications to control blood pressure, and dialysis in cases of severe kidney failure. For atypical or genetic forms of HUS, targeted therapies such as eculizumab may be prescribed to prevent further damage. In TTP, urgent plasma exchange (plasmapheresis) is the mainstay of treatment and can be lifesaving, often combined with corticosteroids and immunosuppressive drugs to reduce abnormal clot formation. Newer therapies, such as caplacizumab, may also be used to improve outcomes. With timely diagnosis and prompt intervention, many patients can recover well, though ongoing follow-up is important to monitor kidney and blood health.
Prevention & Care
Good hygiene and food safety practices can reduce the risk of infection-related HUS. Regular follow-up with a hematologist or nephrologist is essential for patients with a history of HUS or TTP to monitor kidney and blood health.