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A3396 - Thrombotic Microangiopathy: A Killer with Many Faces
Author Block: S. Noronha, D. Brown; Mayo Clinic, Rochester, MN, United States.
Introduction: Thrombotic microangiopathies may have multiple presentations and it is important for intensivists to be familiar with the variable presentations of these syndromes. Description: A 45 year old female patient with a medical history significant only for a viral meningitis many years ago, presented to an outside facility with worsening headaches and drowsiness for a week. She was endotracheally intubated for airway protection and an extensive infectious workup was unrevealing. Brain imaging remained non-diagnostic throughout. She had significant laboratory derangements, chiefly thrombocytopenia with platelets at 35,000/microliter, and a conjugated hyperbilirubinemia with a total bilirubin of 13.1 milligram/deciliter (mg/dL). She was diagnosed with hemophagocytic lymphohistiocytosis (HLH), based on a non-confirmatory but suggestive bone marrow biopsy. She received Etoposide and Cyclosporine and developed leukopenia and worsened thrombocytopenia with no improvement in her condition. Her hyperbilirubinemia peaked at a total bilirubin of 30 mg/dL. Her peripheral smear was concerning for thrombotic microangiopathy, but her ADAMTS13 levels were non-diagnostic. Her serum anti-nuclear antibody levels were also elevated to 6.7 units, with an SS-A/Ro antibody elevated to 8.0, but with no clinical features of Sjogren’s disease. The remaining rheumatic disease workup was negative. A repeat bone marrow biopsy ruled out HLH, but demonstrated non-diagnostic panhypoplasia. A prolonged course of high-dose steroids did not significantly change her clinical condition. Several runs of plasmapheresis, however, significantly improved her mental status, which further strengthened the theory that this was a microangiopathy. During her hospitalization, she developed multiple complications including septic shock from a urinary infection, repeated red blood cells and platelet transfusions, renal failure requiring hemodialysis and, ultimately, small bowel perforations. Care was transitioned to comfort in the setting of her bowel perforations and she died soon after. Discussion: This difficult case presented a diagnostic conundrum. Presumptively treated for HLH based on a bone marrow biopsy, our patient was ultimately found to have thrombotic microangiopathy which responded to plasmapheresis. However, she had developed numerous complications as a result of her illness which ultimately resulted in her death. A search of the literature did not reveal any instances of thrombotic microangiopathy consistent with the complicated presentation of our patient. Diagnostic challenges of this nature are often seen in the intensive care unit, and it is important to recognize the multiple manifestations of thrombotic microangiopathies in order to promptly diagnose and provide appropriate treatment.