Figure 1.

A hypothetical framework illustrating conditions seen in critically ill patients meeting 5 of 8 Hemophagocytic Lympho Histiocytosis criteria that require consideration for different consultative services and therapeutic approaches. Sepsis induced MODS has decreased NK cell numbers with normal cytolytic ( ) function, decreased CD8 cell numbers, and activated macrophages. Non-malignant lymphoproliferative disorders have normal NK cell numbers without cytolytic activity ( ), with lymphoproliferation and macrophage activation. Malignant lymphoproliferative ( ) disorders have failed cytolytic activity and macrophage activation. Macrophage Activation Syndrome disorders have normal NK cell numbers with reduced cytolytic activity ( ), decreased suppressor T cells, and activated macrophages.

Abbreviations: MODS – Multiple Organ Dysfunction Syndrome; NK – natural killer; IVIG – intravenous immune globulin; AKI – acute kidney injury; HLH – Hemophagocytic Lymphohistiocytosis; EBV – Epstein Barr Virus; BMT – Bone Marrow Transplantation