ASPARAGINASE ASSOCIATED INTRA-CARDIAC THROMBUS PRESENTING AS SEPSIS IN AN ADOLESCENT PATIENT DURING ALL INDUCTION THERAPY

Background: The incidence of pegaspargase induced thrombotic complications in pediatric patients with acute lymphoblastic leukemia (ALL) is 5.2%, with the majority of thromboses induced by asparaginase occurring in the venous system. Drug-induced intracardiac thrombosis is very rare and, if noted, usually develops within the right atrium in relation to central lines. Case Presentation: A 14-year-old female was diagnosed with B-cell ALL. At the time of diagnosis, her echocardiogram revealed a mild congenital dysplastic mitral valve with underdevelopment of the posterior leaflet, but cardiac function was not affected. The patient was started on a 4-drug induction and received 2 doses of pegaspargase. She was readmitted to the hospital on induction day 25 with diffuse body aches, generalized weakness, mildly elevated lipase, hyperbilirubinemia, pancytopenia, and severe hypo-albuminemia (1.6 gm/dl). Her direct bilirubin measured 1.7 mg/dL and amylase measured 289 U/L. On induction day 38, the patient developed a fever of 39.2°C. She became very ill looking and pale, but was oriented and alert. She stated that breathing was harder than earlier that morning. A chest x-ray was ordered to assess increasing O2 requirement and bilateral opacities were found at the base of the lungs. Her heart rate was 130 bpm and blood pressure was 100/77 mmHg. After being transferred to intensive care, a stat echocardiogram was ordered due to suspected sepsis-induced cardiogenic shock. However, upon examination, the echocardiogram demonstrated an echo bright mass along the lateral wall of the left ventricle (LV), consistent with an LVT. The thrombus extended to the mitral valve causing severe acute mitral regurgitation leading to cardiogenic shock requiring pressors and inotropic support. The patient was initially started on heparin infusion to treat the LVT due to contraindications for surgical intervention including thrombocytopenia, neutropenia, and active cytomegalovirus infection. She later underwent LV thrombectomy and mitral valvuloplasty. She improved significantly after surgery and was transferred to the rehabilitation unit. Conclusion: This patient demonstrated a unique presentation to pegaspargase associated thrombus formation. Given the rareness of cardiogenic shock secondary to intra-cardiac thrombosis during pediatric ALL therapy, the clinical picture can be mistaken with septic shock. Having a high index of suspicion may prompt early evaluation with echocardiogram, which can make an immense difference in the management and outcome of a patient.