Abstract:
Hemophagocytic Lymphohistiocytosis (HLH) is
a severe hyperinflammatory syndrome induced by
aberrantly acquired macrophages and cytotoxic T cells.
The primary form is called the Genetic form where as
the secondary acquired form is most common in adults.
Secondary HLH induced by autoinflammatory or
autoimmune disorders is called Macrophage activation
syndrome (MAS or MAS-HLH). Here we present a case
of Systemic lupus Erythematosus presenting for the first
time with MAS-HLH associated with acute pancreatitis,
emphasizing the challenges in early detection and the
need for prompt treatment, given the high mortality
associated with MAS-HLH.
