case_images: PMC9872489_01
case_images: Serial laboratory evaluation for patient from first episode of macrophage activation syndrome (MAS) through follow-up. Trend in ferritin, fibrinogen and platelet count in patient during and after hospitalisations for MAS. Treatments are shown above graph. Arrows represent intravenous methylprednisolone pulse therapy (1 g/day). Circles denote days of emapalumab infusion. TMP/SMX, trimethoprim/sulfamethoxazole.
case_images: rmdopen-2022-002611f01.jpg
case_images: PMC9872489_01_rmdopen-2022-002611f01.jpg
case_images: F1
case_images: He was admitted and treated with intravenous methylprednisolone 1000 mg daily for 5 days and clinically stabilised, but his neutropenia, lymphopenia, thrombocytopaenia and hypofibrinogenaemia did not improve (figure 1). His labs continued to improve (figure 1) and he was discharged on emapalumab two times weekly, continued canakinumab every 4 weeks and prednisone 60 mg two times per day. With this, he exhibited progressive improvement in his thrombocytopaenia and hypofibrinogenaemia (figure 1) and was discharged to complete a 28-day course of ruxolitinib and a tapering dose of prednisone.