DOI: 10.1161/circ.148.suppl_1.17846 ISSN: 0009-7322

Abstract 17846: Successful Treatment of Recurrent Pericarditis With Rilonacept in the Setting Of ATRA/ATO Use in Acute Promyelocytic Leukemia

Muhammad Majid, Aro Daniela Arockiam, Ankit Agrawal, Ashwin Kumar, Zachary Yaker, Sarah Mudra, Allan L Klein
  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Case Presentation: A 40-year-old female was admitted for a 4-day history of recurrent left-sided pleuritic chest pain. Physical examination was pertinent for tachycardia and presence of a pericardial friction rub. Past medical history included recently diagnosed Acute Promyelocytic Leukemia undergoing active chemotherapy. Complete blood count showed leukopenia. Brain natriuretic peptide was slightly elevated at 241 pg/mL. Electrocardiogram demonstrated sinus tachycardia with diffuse ST-segment elevation and PR-segment depression (Figure 1. A). Echocardiogram showed normal biventricular function with a small posterior and lateral pericardial effusion and small pericardial effusion adjacent to the right ventricle (Figure 1. B). Cardiac magnetic resonance (CMR) imaging showed a moderate circumferential pericardial effusion and increased pericardial signal on T2 STIR imaging and late gadolinium enhancement (LGE) (Figure 1. C, D). Given the constellation of symptoms and findings, the patient was diagnosed with acute pericarditis secondary to differentiation syndrome (DS) suspected to be due to her underlying malignancy and anti-cancer treatments. She was started on aspirin and colchicine. Steroids and rilonacept were later added for incomplete treatment response. On follow-up, she was doing well. Repeat CMR demonstrated mild to moderate pericardial delayed enhancement with no edema and no pericardial effusion (Figure 1. E,F).

Discussion: The presentation of DS can vary, and consensus diagnostic criteria are lacking. Cases of myopericarditis with concurrent pericardial effusions have been reported in the setting of patients undergoing chemotherapeutic treatment with differentiating agents. Management is complicated and requires multi-disciplinary care. Our case illustrates that rilonacept may be considered as an alternative to corticosteroids in the treatment of recurrent pericarditis in a patient with a hematologic malignancy.

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