Age: 24.0
Sex: M
Vaccine: COVID19, COVID19
Symptoms: ADAMTS13 activity assay, Apraxia, Bilirubin conjugated increased, Blood bilirubin increased, Blood creatinine increased
Clinical narrative: Patient is a  24 y.o. male, with no known medical history, who received more than a vaccination few weeks ago the presented to the outside hospital for worsening general malaise since he received the COVID vaccine and acute neurological symptoms with focal deficit.  Symptoms was noted by his mother around 10:00 p.m. on 06/19/2021 with right arm weakness, facial droop, tongue deviation.  On presentation to the outside hospital emergency room patient was found to be severely encephalopathy with moaning, eyes deviation,   unable to follow commands, diaphoretic but no focal deficit. He was intubated and sedated for airway protection.  Initial workup with CT head without contrast was negative for acute finding.  CT abdomen pelvis without contrast showed splenomegaly.  Hemoglobin was 8.8, platelet 24, LDH 2856, creatinine 1.27, BUN 23, total bili 3.5, direct 0.5.  UDS negative.  Transferring provider suspicious for TTP.  Patient is been transferred to Hospital for further care.  Given high mortality associated with TTP, patient was initiated on plasma exchange on 6/20/2021.ADAMTS activity and inhibitor assay were ordered.  He received second dose of Covid-19 vaccine ten days ago. 6/21/21: Trialysis catheter place and initiated plasmapheresis yesterday.  CBC being followed closely with improvement in platelet count to 37K this AM, but down to 28K this afternoon.  Hemoglobin low requiring transfusion support. 24-year-old man with no prior medical history and quit 19 vaccine about 10 days ago.  On the morning of June 19, 2021 he presented to Hospital where he was intubated.  Patient had been having progressive malaise and there was concern for focal neurological deficits.  He was intubated in the emergency room and later on transferred to Hospital for further evaluation and treatment.  He had a normal CT angiogram of head but found to have significant hemolysis and profound thrombocytopenia.  MRI of the brain did not show any hemorrhage however it did show possible hypoxic brain damage in cortical and subcortical gray matter.  Upon decreasing his sedation patient became significantly restless and agitated and had to be Re sedated heavily.  Neurology consult was called.  His initial labs showed hemoglobin 7.9, hematocrit 23.6, platelet count 16000, RBC was 2.73.  Neutrophil predominance was seen.  PT PTT INR were normal and fibrinogen was normal D-dimer was elevated 3574. Patient was given multiple units of platelet transfusion and later on started on steroids and started on plasmapheresis. Last 24 hours: His MRI of the brain was negative for any mass mass effect or or signs of infarct MR venogram did not show any cerebral venous sinus thrombosis.  His EEG did show generalized background slowing which appeared to be moderate pointing to hours systemic/metabolic encephalopathy. Patient is continued on plasmapheresis and steroids and this is day 4 of plasmapheresis. 6/24/21: Day 5 of plasma exchange with platelet count stable at 75K, will await repeat CBC.  Patient extubated and alert.  No bleeding reported.   6/25/21: Day 6 of plasma exchange.  ADAMTS 13 activity resulted <5 and inhibitor >8BU consistent with an acquired TTP picture with higher likelihood for refractory disease.  Platelet count dropped to 40K this AM, concerning for refractory disease.  Will initiate Rituxan following PEX today.   6/26/21:  Received Rituxan overnight 6/25 - 6/26.  Had some initial complications with elevation of HR.  Treatment stopped and restarted/continued at slow rate for duration of infusion.   6/27/21:  Plasma exchange held for 24 hours post Rituxan.  Restarted without complications.   6/28/21:  Day 8 plasma exchange.  Tolerating without complications.  Platelet count improved today to 39,000.  No bleeding reported.  Scheduled for plasma exchanged twice today.   6/29/21:  Day 9 plasma exchange.  Will move to daily exchanges starting today given increase in platelet count.   6/30/21:  Day 10 plasma exchange.  Tolerating without complication.  ADAMTS 13 and inhibitor ordered, results pending.   7/1/21:  Day 11 plasma exchange.  Has been tolerating without complication.  ADAMTS 13 activity/inhibitor levels redrawn early this morning.  Plan for cycle #2 Rituxan tomorrow. Peripheral smear showing less schistocytes.    7/2/21:  Day 12 plasma exchange held.  Cycle #2 Rituxan given and with plans to restart PEX 24 hours after completion.  Has tolerated plasma exchange without complications thus far.   7/3/21: Hgb 7.6 g. Platelets 289,000. LDH 223. Continues on daily plasma exchanges.  7/4/21: Hgb 9.7 g. Platelets 321,000. LDH 221.  7/5/21: Hgb 8.3 g. Platelets 318,000. LDH 175.   7/6/21: Hgb 9.1 g. Platelets 283,000. Haptoglobin up to 100. LDH 184. ADAMTS13 inhibitor and activity level sent off. 7/7/21: Hgb 9.2 g. Platelets 300,000. LDH 224. ADAMTS13 activity from 07/02 came back at 30%.  7/8/21: Hgb 9.1 g. Platelets 244,000. LDH 162. Began holding plasmapheresis. 7/9/21: Hgb 9.9 g. Platelets 254,000. Received Rituxan. Tolerated well. LDH 194. 7/10/21: Hgb 10.3 g. Platelets 249,000. Prednisone changed to 90 mg. LDH 199. 7/11/21: Hgb 10.2 g. Platelets 250,000. LDH 205. Plasmapheresis performed. Tolerated well.  DISCHARGED 7/13 with #4 Rituxan scheduled for 7/16/21 as outpatient.