Abstract Body: Case Presentation: A 43-year-old male with a past medical history of hypertension presented with symptoms of shortness of breath, cough and chest discomfort. Vital parameters were as follows: heart rate (HR) 100 bpm, blood pressure (BP) 205/105 mmHg, peripheral arterial oxygen saturation (SpO2) 99% on O2 therapy (3 L/min). Laboratory results revealed mild reduction in hemoglobin (12.6 g/dL), thrombocytopenia (71,000/dL), evidence of hemolysis with elevated LDH (1,003 U/L), decreased haptoglobin (<8 mg/dL), and presence of schistocytes on blood smear. Furthermore, severe increase in creatinine (8.3 g/dL), mild hypokalemia (3.1 mEq/L), marked increase in troponin (335 ng/L), elevated aldosterone (72.6 ng/dL) and renin (11.9 ng/mL/hr) were also noted. EKG was suggestive of ST segment depressions in V5-6 and left ventricular hypertrophy. Chest X-ray revealed pulmonary edema, while CT imaging of the head highlighted concerns for PRES. Echocardiogram demonstrated EF of 55-65% with severely increased wall thickness of the left ventricle.
Differential Diagnosis: Due to suspicion for TTP, patient was initiated on corticosteroids and underwent three sessions of plasmapheresis followed by hemodialysis. However, ADAMTS13 activity was found to be at >100% alluding to secondary cause of TMA rather than TTP. Renal biopsy ultimately showed findings consistent with thrombotic microangiopathic changes and focal global glomerulosclerosis.
Treatment and Management: He was treated with furosemide and nicardipine drips for volume and blood pressure control. Hemodialys was initiated for volume overload and antihypertensive regimen was started including amlodipine, carvedilol, doxazosin, hydralazine and losartan.
Outcome and Follow-up: Platelet count normalized during hospitalization. Patient was discharged after 16 days and has remained dialysis dependent. He established follow-up with nephrology and endocrinology.
Teaching Points: This case highlights the importance of prompt differentiation of malignant hypertension-induced TMA from primary TMA including TTP and HUS as treatment strategies differ significantly. Malignant hypertension causes TMA and MAHA through RAAS activation and endothelial injury; therefore, timely blood pressure control is crucial to preventing irreversible multiorgan damage. Accurate diagnosis can be helpful to avoid unnecessary and potentially harmful plasmapheresis and prolonged glucocorticoid use.