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A5321 - Fat Emboli Syndrome in a Patient with Sickle Cell Disease Presenting as Diffuse Alveolar Hemorrhage
Author Block: R. Sangani1, N. Bosch2, E. Klings3; 1Internal Medicine, Boston Medical Center, Boston, MA, United States, 2Pulmonary Fellowship Program, Boston University Department of Medicine, Boston, MA, United States, 3Pulm Ctr/Houseman R304, Boston Univ Sch of Med, Boston, MA, United States.
Introduction: Fat emboli syndrome (FES) is a clinical syndrome that follows an identifiable insult marked by a triad of hypoxemia, neurologic abnormalities and a petechial rash. While the pathogenesis of FES is uncertain, it is a well-reported complication of sickle cell disease (SCD). Here we present an unusual case of FES presenting with diffuse alveolar hemorrhage (DAH).
Case Presentation: A 23 year-old HbSS male presented with 3 days of non- productive cough and left lower extremity pain. On arrival, vital signs were normal, basic laboratory investigation was unremarkable, and chest X-ray (CXR) showed no acute cardiopulmonary process. Treatment for vaso-occlusive crisis was initiated. Within 24 hours of admission, the patient’s oxygen saturation decreased to 82% on room air, he developed a new leukocytosis of 12.4 K/UL and CXR showed new bilateral perihilar opacities consistent with acute chest syndrome (ACS). The patient was transferred to the Medical ICU where invasive mechanical ventilation for acute hypoxic respiratory failure and antibiotics were initiated. He was transfused 5 units packed red cells, followed by an exchange transfusion of 7 units packed red cells with clinical improvement. Over the next 48 hours, his respiratory status improved, and his ventilator support was weaned in preparation for extubation. The next day (HD 3), he developed fever, tachycardia and hemoptysis of bright red blood with worsening hypoxemia. Neurologic exam revealed facial twitching, upward eye deviation, and laboratory evaluation demonstrated a new thrombocytopenia. Emergent bronchoscopy with bronchoalveolar lavage was performed which was consistent with DAH. Vasculitis work-up including antinuclear, anti-neutrophil cytoplasmic, antiphosopholipid, anti-glomerular basement membrane, and anti- streptolysin antibodies were negative. He was diagnosed with FES based upon two major and four minor Gurd criteria. The patient was started on methylprednisolone 250mg three times a day with rapid clinical improvement, extubation, and resolution of both neurologic impairment and hemoptysis over the next 48 hours. The patient fully recovered from this event and returned to employment three months post-discharge.
Conclusion: This is the first reported case of non-traumatic FES associated with DAH in a patient with sickle cell disease and is reflective of a similar case reported in a patient without SCD. In addition to highlighting this rare association, this case also highlights the potential effectiveness of pulse-dose glucocorticoids for FES with DAH. This warrants further study before it can be universally recommended.