Extensive myocardial calcification in septic shock and precursor B-cell acute lymphoblastic leukaemia

A 15-year-old male with Klinefelter syndrome presented with neutropenic septic shock and multiorgan failure. Bone marrow biopsy demonstrated precursor B-cell acute lymphoblastic leukaemia. AEIOP chemotherapy was initiated on Day 6 of admission (prednisone, vincristine, daunorubicin, pegylated asparaginase, methotrexate, and intrathecal cytarabine). At Day 21, during his induction phase, echocardiography demonstrated new widespread myocardial calcification. On apical four chamber views, there was dense calcification of the left and right ventricular myocardium, the mitral annulus, the interventricular septum, and the interatrial septum (Panel A). Involvement of the papillary muscle was seen on the parasternal short axis view (Panel B). The echocardiographic ejection fraction was visually estimated at 50% (see Supplementary data online, Video S1Supplementary data online, Video S1). Computed tomography revealed dense myocardial calcification concentrated around the left ventricle (Panel C). The post contrast cardiac magnetic resonance images demonstrated multifocal areas of delayed enhancement extending from the pericardium to the subendocardium with a small pericardial effusion (Panel D). The left ventricular ejection fraction was measured as 36% with minimal tricuspid and mitral regurgitation (see Supplementary data online, Video S2Supplementary data online, Video S2).

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