Macrophage Activation Syndrome in Two Infants with Multisystem
Inflammatory Syndrome in Children
Abstract
Multisystem inflammatory syndrome in children (MIS-C) associated with
SARS-CoV-2 infection with clinical features of Kawasaki-like disease was
reported in various pediatric centers in late April 2020. Currently,
cases have increased throughout the world with a range of manifestations
from less to greater severity. However, hemophagocytosis has not been
described in patients with MIS-C. We describe two infants diagnosed with
MIS-C who presented Macrophage Activation Syndrome (MAS) with
hemophagocytosis documented in the bone marrow. MIS-C can be complicated
with MAS, the key features for diagnosis are splenomegaly,
hypofibrinogenemia, hypertriglyceridemia and bone marrow
hemophagocytosis. Cytokine storm and MAS in MIS-C may represent part of
the spectrum of the disease and HScore could be of value in order to
give timely and aggressive treatment.