High-Yield Clinical Immunolgy: Immunodeficiencies due to immune dysregulation (Hemophagocytic Lymphohistiocytosis & Autoimmune Lymphoroliferative Syndrome)

These (HLH & ALPS) are the major syndromes of innate and adaptive immune dysregulation respectively resulting in primary immunodeficiency. For the discussion of complement system dysregulation refer to episode on innate immune deficiencies of innate immune system. Also compare HLH with MAS (macrophage activation syndrome) discussed in pharmacotherapy of Juvenile Rheumatoid Arthritis