Hyperinflammatory Syndrome of Unknown Origin Complicated by Shock, DIC, Intracranial Hemorrhage and Spontaneous...

Hyperinflammation syndrome is a life-threatening condition of heterogeneous etiology. Common manifestations include fever, organomegaly, leukopenia or leukocytosis and malaise. We would like to present a rare case of hyperinflammatory syndrome of unknown origin complicated by shock, severe coagulopathy, spontaneous splenic rupture, and intracranial hemorrhage. A 24-year-old man with no past medical history was admitted to intensive care unit presenting with hemodynamic instability, severe acidosis, splenomegaly, bi-cytopenia, with significantly elevated inflammatory markers. Broad spectrum microbiology, hematology and genetic tests were performed to identify a trigger. Due to presence of both sepsis and hemophagocytic lymphohistiocytosis features (HLH), sepsis-HLH overlap syndrome was suspected. Aggressive treatment (broad-spectrum antibiotics, immunomodulation therapy, therapeutic plasma exchange and renal replacement therapy) was instantly initiated. Despite that, shock and profound disseminated intravascular coagulopathy exacerbated. Unexpected splenic rupture and intracranial hemorrhage required urgent surgical intervention, along with amputation of distal foot phalanges due to severe ischemia. Treatment in the intensive care unit lasted 40 days. One year after a discharge from ICU, patient still required neurological rehabilitation and orthopedic treatment. We do hope that this case will make clinicians more aware of HLH and sepsis-HLH overlap syndrome. Further research is required to improve treatment of severe hyperinflammation.