PMC5771920_01
Evolution of laboratory results during treatment. Etoposide was given at a dose of 150 mg/m2 (white squares) or 75 mg/m2 (black squares). Dose reductions were performed because of liver failure and/or myelotoxicity. Dexamethasone was started at a dose of 10 mg/m2. This dose was halved every 2 weeks, but re-escalation was necessary due to relapse HLH after 4 weeks. Afterward, it was tapered without complications. Ciclosporin was started at a dose of 2 mg/kg twice daily, and trough levels of 150-200 ng/mL were maintained. Rituximab was given at a dose of 375 mg/m2 once a week until clearance of PCR EBV. The red rhombus indicates the relapse of HLH triggered by a flare-up of invasive aspergillosis.
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Extreme hyperferritinemia (102,514 mug/L) and elevated triglycerides (921 mg/dL) were noticed (Fig. 1). The dexamethasone dose was re-escalated, etoposide was added back, and ciclosporin was associated (Fig. 1).
(A) Bone marrow slide showing active hemophagocytosis with an increased number of macrophages carrying debris of all three hematopoietic cell lines. (B) Abdominal CT scan showing multiple hematomas with compression of the duodenum leading to subobstruction which necessitated duodenal bypass surgery.
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Bone marrow biopsy showed active hemophagocytosis with an increased number of macrophages carrying debris of all three hematopoietic cell lines (Fig. 2A). Further recovery was prolonged by compression of abdominal hematomas on surrounding structures (Fig. 2B).