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Washington University Experience | VASCULAR | Thrombotic Thrombocytopenic Purpura (TTP) | 5A0 Case 5 History
Case 5 History ---- The patient was a former 55-year-old male smoker with a medical history of heart failure (non-ischemic cardiomyopathy) with reduced ejection fraction, severe peripheral artery disease, left deep venous thrombosis (for which he was taking Xarelto), pulmonary embolus, right atrial thrombus, chronic obstructive pulmonary disease, obstructive sleep apnea, and chronic kidney disease, as well as acute leukemia (AML) and ALK-positive anaplastic large cell lymphoma diagnosed in 2009 status post autologous stem cell transplant in 2014. The patient was in his usual state of health until around 8:00 AM on 7/17 when he noticed left facial droop, left upper extremity weakness, and left upper extremity numbness and tingling. He proceeded to an ED where he was also found to have bilateral lower extremity drift, dysarthria, and right gaze preference. His National Institutes of Health Stroke Scale (NIHSS) score was 6. His glucose concentration was 137 mg/dL, and his Anti Factor Xa activity was found to be <0.10 IUnits/mL. A computed tomography angiography of the head at 11:44 AM on 7/17 showed an occlusion of the right middle cerebral artery and a fibrinogen concentration was measured as 159 mg/dL. At 12:16 PM, the patient received tenecteplase for treatment of the ischemic stroke. Laboratory tests drawn at 1:36 PM showed a platelet count of 47 K/cumm. Shortly after, the patient began to complain of a new headache, and a second head CT at 2:15 PM showed interval development of multifocal subarachnoid hemorrhage within the sulci of both cerebral hemispheres. At approximately 3:00 PM, the patient developed an altered mental status and right arm weakness. The NIHSS was 13. Tranexamic acid was administered at 3:11 PM to reverse the effect of tenecteplase. A repeat head CT at 3:17 PM showed interval development of a large focus of intraparenchymal hemorrhage (approximately 20 mL) within the left corona radiata with associated local mass effect including effacement of the sulci of the left frontal lobe, mild effacement of the frontal horn of the left lateral ventricle, and minimal rightward midline shift. There was also a slight interval increase in the amount of multifocal subarachnoid hemorrhage involving the sulci of both frontal lobes, parietal lobes, and right occipital lobe. The patient was intubated for airway protection at 3:34 PM and admitted to the NNICU. The patient was diagnosed with DIC given his hypofibrinogenemia and thrombocytopenia and was transfused a unit of cryoprecipitate at 4:50 PM. At 6:17 PM, a troponin I concentration was found to be 1,744 ng/L, with a peak concentration of 1781 ng/L at 10:24 PM and a subsequent decrease. This was deemed to be the result of an non-ST segment elevation myocardial infarction (NSTEMI) secondary to demand ischemia in the setting of an acute cerebrovascular accident. Evaluation of a peripheral blood smear, including flow cytometry, was negative for recurrent acute leukemia. Ultrasound of the extremities were negative for deep vein thromboses. The patient was also evaluated by neurosurgery upon admission but deemed to not be a candidate for acute surgical intervention due to his platelet count being below 100 K/cumm. The patient was transfused with 5 units of platelets and an additional 2 units of cryoprecipitate with improvement of the platelet count to 119 K/cumm and the fibrinogen concentration to 185 mg/dL by the early morning of 7/19. However, a subsequent decrease in platelet count below 100 K/cumm later that day prevented neurosurgical intervention. On the morning of 7/20, the patient was noted to have unequal, nonreactive pupils (left pupil 1.9 mm, right pupil 5.7 mm and ovoid). right-sided ovoid pupillary changes, and an emergency CT scan of the head showed worsened mass effect and effacement of the basal cisterns on the left concerning for brain herniation. He was given 5% hypertonic saline with improvement in pupillary changes. Afterward, the patient became consistently hypotensive with a mean arterial pressure in the mid to upper fifties, at which point he was started on a norepinephrine drip. The patient also received an additional 3 units of platelets and several units of cryoprecipitate. On 7/21 the patient developed respiratory arrest and pulseless electrical activity. He went through 5 cycles of compressions and was ultimately declared deceased (7/21).
