Abstract: TH-PO120
Newly Diagnosed Sickle Cell Disease Complicated by AKI With Bone Marrow Necrosis and Fat Embolism Syndrome Successfully Treated With Plasma Exchange
Session Information
- AKI: Mechanisms - I
November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 103 AKI: Mechanisms
Authors
- Li, Jun, Albert Einstein College of Medicine, Bronx, New York, United States
- Terzibachi, Michel, Albert Einstein College of Medicine, Bronx, New York, United States
- Sharma, Deep, Albert Einstein College of Medicine, Bronx, New York, United States
- Thakkar, Jyotsana, Albert Einstein College of Medicine, Bronx, New York, United States
- Tuider, Laurene, United Health Service Medical Center, Johnson City, New York, United States
- Fisher, Molly, Albert Einstein College of Medicine, Bronx, New York, United States
Introduction
Bone marrow necrosis with fat embolism syndrome (FES) is a devastating complication of sickle cell disease (SCD) that is associated with high mortality. We report an unusual case of an elderly female who presented with severe acute kidney injury (AKI) and features suggestive of thrombotic microangiopathy (TMA) who was found to have hemoglobin SC disease (HbSC) with FES, which was successfully treated with plasma exchange.
Case Description
A 76-year-old African American female with diabetes, hypertension, rheumatoid arthritis presented with altered mental status, hypoxic respiratory failure and septic shock due to pneumonia requiring intubation and vasopressors. Upon admission, she was found to have an elevated serum creatinine of 3.6mg/dl as well as severe anemia (hemoglobin 6.7 g/dL), thrombocytopenia (platelet count 66 k/µL) and elevated transaminases. Additional workup revealed a lactate dehydrogenase of 2553 U/L, haptoglobin <10mg/dl, ADAMTS 13 activity 0.52 IU/mL. Peripheral smear revealed schistocytes, sickle cells and findings of bone marrow necrosis. She was also found to have bilateral femoral head avascular necrosis on CT. Hemoglobin electrophoresis was performed and showed elevated hemoglobin S and hemoglobin C, consistent with HbSC disease. The patient received emergent exchange transfusion for FES in the context of sickle cell crisis. She subsequently received plasma exchange for 5 days and supportive hemodialysis. After treatment, her mental status improved as well as her hematologic parameters and kidney function leading to discontinuation of hemodialysis.
Discussion
Bone marrow necrosis with FES is a rare complication of SCD that can mimic TMA. Interestingly, this syndrome is most often described in HbSC disease, a mild variant of SCD. The present case highlights the importance of not overlooking this diagnosis in patients with SCD who present with TMA-like features and AKI and also shows that plasma exchange is an effective therapeutic strategy.