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Washington University Experience | VASCULAR | Sickle cell anemia | 1A0 Case 1 History
Case 1 History ---- The patient was a 19 year-old right-handed African-American woman with a history of sickle cell disease (Electrophoresis 1 year prior to death: HbS 86.3%; HbF 10.2%; A2 3.5%) with every other month pain crises (typically right elbow and chest pain), recent stillbirth in 12/06 and hypertension. Beginning on 02/25, she complained of left-sided Tylenol-unresponsive headache, shortness of breath, nausea and vomiting, and chest pain. At the BJH ER on 02/26 she reported headache, weakness and numbness in the left upper extremity which was accompanied by confusion, dysarthria, and gait difficulties. In the ER, she had a seizure, treated with Ativan and intubation. Head CT showed a large intraparenchymal right fronto-parietal lobe hemorrhage with surrounding edema, left midline shift and subarachnoid hemorrhage along the falx. In the ER her blood pressure was 207/150. She was started on Dilantin. An angiogram on 02/26 revealed proximal occlusion of the right ACA and resultant hemorrhagic transformation. The leading hypothesis was that she had a thrombotic stroke with hemorrhagic transformation. Exchange transfusion on 02/27 reduced HbS from 58 to 19.8%. She was oriented to self, year, and location. Her renal function deteriorated (Cr 1.5). On 03/05 she developed respiratory distress. Chest x-ray revealed pulmonary edema vs. pneumonia, and she began receiving antibiotics. Repeat head CT on 03/05 revealed a previously unidentified left frontal intraparenchymal bleed with surrounding vasogenic edema, intraventricular extension and mass effect. She had left common femoral deep venous thrombosis treated with placement of an IVC filter. She developed respiratory distress with tachypnea, decreased oxygen saturations, and no response to treatment. Chest x-ray revealed complete 'white out' of the right lung. On 03/10 there was a sudden decline in her mental status. In the setting of persistent depressed mental status, increasing leukocytosis (WBC 34) and creatinine (Cr 4.6); she was started on dialysis. On 03/26, she experienced respiratory arrest after aspirating. Cardiac pulseless electrical activity (PEA) followed. After the code, she was unresponsive and her pupils were fixed and dilated. Head CT revealed: interval worsening of acute intraparenchymal hemorrhage in the left frontoparietal region, with extension into the ventricular system; hydrocephalus; and cerebellar tonsillar herniation. On 03/29, neurological exam detected no evidence of brainstem function and the patient's family withdrew care. ---- At autopsy the weight of the unfixed brain was 1270g. The cerebellar tonsils appeared deformed bilaterally, consistent with herniation. Coronal sections of the brain showed parenchymal and intraventricular hemorrhages. Leptomeningeal vessels adjacent to areas of hemorrhage show variable and often marked vascular hyperplasia and occlusion. Sub-acute infarcts in the left frontal and right parietal lobes and scattered microinfarcts elsewhere were associated with organizing luminal thrombi. Hemorrhages in the left frontal and right parietal lobes extended throughout the ventricular system.
