Biopsy-Proven Thrombotic Microangiopathy Secondary to Uncontrolled Hypertension in a Young Man

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Introduction: Thrombotic microangiopathy is characterized by microangiopathic hemolytic anemia,thrombocytopenia,and end organ damage. It is a life-threatening condition that requires immediate treatment with plasma exchange. Case Description: Patient is a 23-year-old male with a significant past history of asthma, DM II, Obesity who presented to the ED with 6 weeks duration of isolated non productive cough. On arrival, he was afebrile with blood pressure of 233/149, pulse rate 108 per minute, respiratory rate 16 per minute. Initial labs were significant for potassium of 2, sodium 130, blood urea nitrogen 35, creatinine 4.06. Urinalysis was significant for protein 3+, blood 3+, and RBC >50, urine protein/creatnine ratio 11.66 mg. Nephrology was consulted for acute kidney injury. During admission he developed sudden onset of complete vision loss in medial left field. MRI showed several foci of abnormal T2 prolongation within white matter. Neurology was consulted and was methylprednisolone was started. Fundoscopy revealed optic nerve head findings consistent with malignant hypertension. Serological markers include ANA, dsDNA, RA, SCL–70, anti-JL–1 were negative. CSF analysis was negative for multiple sclerosis, IgG and oligoclonal bands, and paraneoplastic antibodies. Serology was negative for vasculitis, scleroderma, Sjogren disease, HIV, hepatitis B and C. Serological workups were not consistent with hemolysis, antiphospholipid syndrome and hemolytic uremic syndrome. Ultrasound revealed increased echogenicity in both kidneys compatible with medical renal disease, without any masses or obstruction. Renal artery doppler ultrasound was negative for renal artery stenosis. Echocardiogram showed an ejection fraction of 30-35%. Renal biopsy results were consistent with thrombotic microangiopathy. Patient was considered to have thrombotic microangiopathy secondary hypertensive emergency with end organ damage to brain, eye, heart, and kidney. Discussion: Hypertensive emergency with heart failure with reduced ejection fraction with thrombotic microangiopathy is a rare entity that requires careful clinical diagnosis to prevent further end organ damage. Our patient is a young african american male with uncontrolled hypertension. Prompt diagnosis and control of blood pressure prevents worsening of clinical condition.

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