Age: 51.0
Sex: F
Vaccine: COVID19
Symptoms: Hyperferritinaemia, Hyperkalaemia, Hypocomplementaemia, Hypotension, Hypoxia
Clinical narrative: From admission H&P dated 9/12/21 by Doctor: "History per chart and collateral given patient's encephalopathy. The patient was reportedly in USOH when she began experiencing abdominal pain. She reportedly started a short course of penicillin as an outpatient for this abdominal pain. She presented to the Hospital with worsening abdominal pain, worsening of her chronic low back pain, and chest pain. Mild leukocytosis with elevated ALT and bilirubin on admission. Troponin was elevated to 0.44, but down-trended and deemed to be non-cardiac after evaluation by cardiology. CXR, UA not concerning for infection. CT-AP showed diffuse colonic wall thickening, mild pulmonary interstitial opacities, small bilateral pleural effusions, and a horseshoe kidney. V/Q scan without PE, ultrasound of LE showed patents. TTE with normal EF, RUQUS without biliary dilation, only mild fatty infiltration of the liver. Stool-filled colon - had pain relief with aggressive bowel regimen and bowel movement.    Due to persistent fevers and back pain with elevated procalcitonin, the patient was started empirically on ceftriaxone and vancomycin. Negative CT-spine for infection.   Course complicated by 2x episodes of afib with RVR, the second one requiring amiodarone and beta blocker. She also developed an AKI with Cr rising to peak of 3.56 over the course of days. Intermittent hypotension requiring pressors. Renal consulted, workup concerning for glomerulonephritis, revealed hypocomplementemia, ferritin >80,000, and monoclonal IgG kappa. She refused a bone marrow biopsy. Her urine output dropped to near-anuric, and she became encephalopathic with myoclonus. At this point, she was started on every other day dialysis. No head imaging performed.   Other diagnostic studies included utox (positive for morphine and oxycodone), APAP level (undetectible), Hep C, HepB, C.Diff,    Due to the elevated ferritin and concern for HLH, he was evaluated by hematology, started on dexamethasone. She had hypoxemic respiratory failure requiring bipap that was ultimately weaned to nasal cannula.   Past medical history of major depressive disorder and chronic lower back pain. Notably, she had severe osteomyeletis of the LUE, LLE requiring extensive surgery"   HOSPITAL COURSE:  Patient had an unfortunately complicated hospital course. As above, she was transferred from an outside facility with a constellation of clinical and lab abnormalities including fever of unknown origin, abdominal/chest pain, anuric renal failure requiring iHD, respiratory failure, and encephalopathy as well as cryoglobulinemia, hypocomplementemia and hyperferritniemia (>80K) and developing bicytopenia with concern for, though not diagnosis of, HLH from unclear trigger.  While pursuing workup for unifying diagnosis she developed progressive multiorgan failure as will be discussed below. In regards to diagnostics for underlying diagnosis, with the assistance of multiple consulting services (infectious diseases, heme/onc, rheumatology, nephrology) she underwent broad workup for diagnosis of and trigger of HLH v. Alternative inflammatory/rheumatologic process including bone marrow biopsy (in progress) , rheumatologic workup (notable for low complement levels and reported cryoglobulinemia at OSH), infectious workup via blood/BAL/LP (negative/in progress). Realization that cryoglobulins + at OSH as well as development of chest/abdomen rash and discolored toes raised question of cryoblobulinemic vasculitis. She was empirically treated with 1g IV methylpred then 40mg IV dex/day while awaiting results and diagnostics which unfortunately were not complete prior to her death and remains a question, hopefully to be answered by pending autopsy. Despite gradual improvement in several body systems (hemodynamics, hypoxemia), she developed DIC and pulmonary hemorrhage on the day of her death which ultimately led to her death as described below.    Multiorgan failure will be described below by system:  # Hypoxemic respiratory failure: Intubated for airway protection and hypoxemia upon admission to UWMC. Treated for ARDS with LPV possibly secondary to infection, though notably BAL and other infectious workup remained unremarkable throughout her stay. Hypoxemia improved enough with UF for volume removal and empiric ABX to move towards SBT pt was not able to be extubated. On the day of Patient's death, she developed bloody secretions from ETT so underwent repeat bronchoscopy. BAL diagnostic of alveolar hemorrhage with progressively bloody lavage aliquots. She was re-pulsed with IV methylpred and provided platelet replacement with concern for DAH or pulmonary vasculitis on 9/17 though developed severe pulmonary hemorrhage early on 9/18. Despite MTP and DDAVP to try and stabilize coagulopathy/hemorrhage, she ultimately lost >3L blood from ETT and died following this significant pulmonary hemorrhage (see code note and death note from today's date).    # Shock: Developed distributive shock requiring 3 vasopressors for support early in her stay. She received empiric antimicrobials, high dose steroids and ID workup with no revealing cause for sepsis. Hemodynamics showed signs of improvement leading up to day of death with vasopressors nearly weaned off.    #anuric renal failure- Complicated by anascarca, hyperkalemia and metabolic acidosis. By microscopy related to tubular injury, considered possibly related to cryoglobulinemia v. Vasculitis though unable to pursue renal biopsy due to instability. Required intermittent SLED/SCUF while admitted.    # Encephalopathy: Again, unclear etiology of altered mental status. Initially considered related to sedation and metabolic abnormalities and initial CT head WNL and LP without clear infectious cause (though some studies remain in progress), though remained profoundly encephalopathic following wean of sedatives.  Other considerations have included CNS involvement of primary underlying diagnosis (cryoglobulinemic vasculitis), undiagnosed infection, NCSE, etc.    # DIC # Bicytopenia Likely related to underlying unifying diagnosis. Other considerations included MAHA though no evidence of hemolysis, negative HIT assay, HLH.  Progressive throughout stay requiring cryo transfusion and multiple PLT transfusions on day of death.