Acute Cyclophosphamide Hemorrhagic Myopericarditis: Dilemma Case Report, Literature Review and Proposed Diagnostic Criteria

cAse RepoRtA 41-year-old woman presented with dyspnea after receiving cyclophophamide (CYA) 50 mg/kg, for four day, for treatment of refractory Chronic Inflammatory Demyelinating Polyneuropathy (CIDP).Prior to initiation of therapy, she had no history of coronary artery disease or congestive heart failure, and had a structurally normal heart by echocardiography 6 months previously.She had never been placed on prior chemotherapy for her disease (only trials of intravenous immunoglobulin and steroids) and had no other predisposing cardiovascular risk factors.On day 4 of CYA therapy, she developed dyspnea and lower extremity oedema with troponin elevation to 2.08 ng/mL (peak 7.51 ng/mL) and new diffuses low voltage waves on electrocardiogram.Chest radiograph showed progressive pulmonary vascular distention with cardiomegaly.Echocardiogram evidenced diffuse myocardial thickening and moderately sized circumferential pericardial effusion with reduced right ventricular expansion and early right atrial collapse, concerning for early tamponade physiology [Table /Fig -1].She required pericardiocentesis on day 10 for increasing effusion size.Despite effusion drainage, Right Ventricular (RV) systolic function deteriorated on serial echocardiograms although her left ventricular function remained intact.She progressed to multi-organ failure including liver and kidney.Her diagnosis remained in question as competing diagnoses included acute coronary syndrome versus progression of rheumatologic disease.A diagnosis of CYA haemorrhagic myopericarditis could not be established by MRI (due to renal failure) or by biopsy (due to progressive pancytopenia and multi-organ failure).A diagnosis of haemorrhagic myopericarditis was questioned due to focal right ventricular dysfunction with preservation of left ventricle ejection fraction.24 days after day 1 of CYA therapy, she progressed to Pulseless Electrical Activity (PEA) arrest and died.Autopsy revealed cardiomegaly (530 g), acute myocardial necrosis, intra-myocardial extravasation of blood, fibrin, and fibrin-platelet microthrombi within capillaries.The histopathologic findings were compatible with a diagnosis of haemorrhagic myopericarditis and were most prominent in the myocardium of her right ventricle, consistent with the progressive structural RV changes seen on echocardiography.