hydroxocobalamin intranasal (AST726) / TG Therap - LARVOL DELTA

Secondary Hemophagocytic Lymphohistiocytosis Due to Kikuchi Fujimoto Disease/Systemic Lupus Erythematosus Overlap (ATS 2019) - "...Working diagnosis was Hemophagocytic Lymphohistiocytosis (HLH) in setting of low complement levels (C3: 33;C4: 4), elevated liver enzymes (AST 726;ALT 337), pancytopenia, NK 18, fibrinogen 132, and ferritin 20,512...He was discharged to long-term acute care hospital and is doing well on Hydroxychloroquine.DiscussionGiven the patient’s family history of SLE, meeting SLE SLICC criteria and HLH criteria, a diagnosis of secondary HLH due to KFD/SLE overlap was proposed...Unlike KFD without SLE, when steroids don’t have an established role, our patient had both diseases and responded to steroids. This case of secondary HLH with KFD/SLE overlap had a better prognosis than expected with primary HLH."