Table 1.

Etiology of Secondary HLH in Adult Patients

Infection:  Virus:   Most frequent:    EBV, CMV (43% and 9% of cases respectively)    HIV  Less frequent:    Dengue, Hepatitis A, B, HSV-1 and 2, Influenza, Parvovirus, SFTS, VZV, Bk virus, Bunyavirus, Adenovirus, Hantavirus Bacteria: Most frequent:  TBC Less frequent:   Rickettsia, Coxiella burnetti, Brucellosis, Ehrlichia, Babesia, Bartonella henselae, Syphilis Enterococcus faecium, Klebsiella pneumoniae, Haemophilus influenzae, Group G streptococcal abiotrophia defectiva, Acinetobacter baummanii Fungi: Histoplasmosis, Candidiasis, Aspergillosis, Penicillium marneffei, Cunninghamela bertholletiae, Cryptococcus neoformansParasite: Leishmaniasis, Plasmodium, Toxoplasmosis

Malignancy:  Haematological disease: mainly lymphoma  Solid cancer Autoimmune disease:  Most frequent:   SLE, Still disease  Less frequent:   Rheumatoid arthritis, vasculitis, connectivitis   Antiphospholipid syndrome, Goodpasture syndrome   Kikuchi-Fujimoto disease, Inflammatory bowel disease Drug:  Chemotherapy: fludarabine and cyclophosphamide  Checkpoint inhibitors:   Pembrolizumab, Nivolumab, ipilimumab  Lamotrigine, Phenobarbital, Diaminophenylsulfone, BCG vaccination Other:  - Post-transplant (haematological, solid organ transplantation)  - Pregnancy

Abbreviations: HSV, herpes simplex virus; SFTS, severe fever with thrombocytopenia syndrome; VZV, varicella zoster virus; EBV, Epstein-Barr virus; CMV, cytomegalovirus; BK, polyomavirus BK; BCG, Bacille Calmette-Guérin; SLE, systemic lupus erythematosus.