A51-25 Severe Refractory Lactic Acidosis Secondary to Veno Occlusive Sickle Cell Crisis Requiring Exchange Transfusion

Abstract Introduction Most common acute complication of sickle cell disease include acute anemia and Vaso-occlusive phenomena due to numerous vascular effects beyond simple obstruction. These include acute vaso-occlusive pain, acute chest syndrome, stroke, kidney infarction, dactylitis or bone infarction, myocardial infarction, complications related to pregnancy, priapism, and venous thromboembolism. We reported a case of 31-year-old female patient with sickle cell disease who developed Veno occlusive crisis in the form of acute deep vein thrombosis and acute chest syndrome with severely elevated lactic acid levels in the absence of shock or significant hypoxia that resolved after exchange blood transfusion. Case Presentation 31-year-old female with past medical history sickle cell anemia (Hemoglobin S of 60%), diabetes mellitus type 2, history of deep vein thrombosis on Apixaban who presented to the emergency department for 3-day history of chest pain, shortness of breath for 2 days and right leg swelling. Complete blood count (CBC) showed Hemoglobin 5.5 g/dL, White blood cell counts 35 X10*3/uL, Platelet count 60 X 10*3/uL. Complete metabolic panel showed normal creatinine and liver panel. The serum bicarbonate level was 6 mmol/L and venous pH 7.08. Lactate level 17.4 mol/L. Ultrasonography of the right lower extremity noncompressible common femoral, femoral, popliteal, gastrocnemius, posterior tibial veins with hypoechoic intraluminal content and absence of Doppler signal consistent with acute deep vein thrombosis. Computed Tomography Angiogram chest was negative for segmental pulmonary embolism but demonstrated ground glass opacities with mosaic attenuation, multifocal scarring and atelectasis. 2D echocardiogram showed ejection fraction of 60%, normal right ventricular systolic function and no wall motion abnormalities. Despite blood transfusion and intravenous fluid resuscitation patient continued to have persistent lactic acidosis and respiratory distress requiring intubation and mechanical ventilation. Blood lactate levels normalized after initiation of exchange blood transfusion. Discussion Blood transfusion in sickle cell disease (SCD) improves oxygen delivery and can lower the percentage of sickle hemoglobin (Hb S) leading to reduction in vaso-occlusion and reducing morbidity from complications of SCD such as stroke and acute chest syndrome. Exchange transfusion involves removing some of the patient’s own blood and transfusing allogeneic blood, thereby lowering the concentration of Hb S through dilution (to 30 percent). In our patient with severely elevated Hb S level with nonocclusive crisis in the form of DVT, acute chest syndrome and severe metabolic/lactic acidosis despite adequate resuscitation and oxygen delivery, exchange transfusion resulted in normalization of lactic acid level. This abstract is funded by: None