Table of Contents
Washington University Experience | VASCULAR | Sickle cell anemia | 11A0 Case 11 History
Case 11 History ---- The patient was a 51 year old African-American man with a history of variant sickle cell disease (Hgb mutant S/mutant C), restrictive and obstructive lung disease secondary to SCA, pulmonary hypertension, cigarette smoking, surgical intervention for priapism in 2009, and a recent history of acute chest syndrome requiring ICU admission and intubation in March of 2012. At that time he was also in a pain crisis which resolved with transfusion of 11 units of blood. He was discharged in stable condition after ~2 weeks. At his final admission (June 25, same year) the patient was found unresponsive at home with an estimated down time of 5-10 min, and was pulseless. CPR was started and he was transferred to BJH. He then was coded for another 8-10 min prior to return of pulses. He was acidotic and hypothermic and had hyperkalemia. He was transferred to the Cardiac Care Unit, and was found in ventricular fibrillation arrest, was given CPR with eventual return of rhythm to normal sinus. He was placed on a ventilator, with continuous hemodialysis, and given high dose pressors. The patient began to develop progressive worsening hypotension and shock on multiple pressors and dialysis. The patient's condition worsened and he was found to have absence of heart sounds without respiration and death occurred. A head CT scan was normal. ---- At autopsy her unfixed brain weighed 1240g. Major findings at post-mortem examination showed severe sequelae of sickle cell disease including evidence of vaso-occlusive crisis, generalized multi-organ hypoxia, and right-sided heart failure. Vaso-occlusion was seen throughout many organs as small and medium vessels engorged by tightly packed, refractile, angulated, sickled red blood cells. In conjunction with the occluded vessels, there was evidence of widespread hypoxic-ischemic damage in the liver (zone 3 necrosis), lungs (intra-alveolar pigmented macrophages, pulmonary edema and fibrin deposition), bone marrow (increased erythropoiesis), kidneys (enlarged glomeruli and glomerular thrombi) and cardiomegaly (640g), biventricular hypertrophy, and significant pleural and peritoneal effusions. Other evidence of sickle cell disease included splenic atrophy with congestion by sickled red blood cells and a single focus of subacute microscopic infarction in the thalamus. There was no evidence of acute myocardial infarction, pulmonary embolism, deep venous thrombosis, or infection. ---- Per clinic notes, the patient has a history of hemoglobin SC disease (Hgb SC), the second most common type of sickle cell disease with polymerization when expressed in red blood cells, distorting them and reducing blood flow. Such mutated red blood cells also have an abnormal adhesive affinity for leukocytes and endothelial cells and are trapped in the microvasculature. Patients may develop non-hemorrhagic stroke, pulmonary hypertension, priapism, proteinuria, and renal insufficiency. Pulmonary hypertension has been reported as a particular risk factor for mortality in sickle cell patients.
