Case Presentation:

A 58 year‐old male with Human Immunodeficiency Virus (HIV) and chronic Hepatitis C presented with a three‐day history of worsening dyspnea on exertion. No chest pain, cough, or constitutional symptoms were noted. On physical exam vitals were: BP 134/78, HR 110, RR 22, Temperature 37.8°C, Oxygen saturation 95% on room air. The patient was alert, oriented and had no focal neurological deficits. A chest radiograph showed no acute cardiopulmonary process and a CT angiogram of the chest was negative for pulmonary embolus. Labs were notable for AST 960, ALT 323, INR 3.4, LDH 1852, platelets 26,000, and creatinine 1.8 (baseline 1.4). Warfarin, dapsone, and simvastatin where held on admission. Over the following two days the patient experienced two tonic‐clonic seizures and subsequently became minimally responsive with a fever of 38.3°C. Non‐contrast head CT was unrevealing and repeat labs showed BUN 45, creatinine 3.0, lactate 5.2, hemoglobin 9.1, and schistocytes on peripheral smear. Daily plasmapheresis was started on hospital day 5 for a presumptive diagnosis of Thrombotic Thrombocytopenic Purpura (TTP). By hospital day 8, labs showed platelets 41,000, downtrending LDH, and improved mental status. The patient’s renal function, however, continued to worsen and labs revealed creatinine kinase 31,000 and myoglobin in the urine, consistent with rhabdomyolysis. AST/ALT remained elevated at 794/198. On hospital day 12 the patient’s mental status again declined. Platelets fell to 9000. Workup for acute bleeding was negative. He then had a prolonged third seizure, was intubated for airway protection and transferred to the Medical Intensive Care Unit. On hospital day 13 he expired after a PEA arrest.

Discussion:

TTP is associated with a number of triggers, including HIV. Its pathophysiology involves the formation of thrombi consisting of platelets complexed with von‐Willibrand Factor (vWF) in the microvasculature of the kidneys and central nervous system. Individuals with TTP generally lack the protease ADMAST13 that breaks down large vWF multimers, predisposing them to thrombus formation. The patient’s fever, anemia, thrombocytopenia, acute kidney injury, and altered mental status established the diagnosis of TTP, likely precipitated by HIV. However, rhabdomyolysis and acute liver injury are not typically seen in TTP. Only two case reports documenting rhabdomyolysis and multiple organ dysfunction syndrome (MODS) in the setting of TTP are published. These reports suggest that the sequence of endothelial injury, thrombus formation, and ischemic injury may occur in any organ, including the liver and skeletal muscle.

Conclusions:

This case highlights the pathophysiology of TTP and its potential association with ischemic injury in multiple organ systems, including the liver and skeletal muscle. Prompt initiation of plasmapheresis can treat TTP and may also improve associated ischemic injury in other involved organ systems