The differential diagnosis of hemophagocytic lymphohistiocytosis (HLH), including triggering and mimicking conditions
| Malignant . | Infectious . | Inflammatory . | Storage/metabolic . | Vascular . | Iatrogenic . |
|---|---|---|---|---|---|
| BCL HL TCL CLL MDS AML ALL CML Myelofibrosis HCC | EBV CMV HIV Leishmania COVID-19 Atypical infections* West Nile virus Tuberculosis Aspergillus Bacterial sepsis | SJIA Still's disease SLE ALPS Sarcoidosis GCA Kawasaki disease PIMS Kikuchi disease | Gaucher's disease LPI Wolman's disease Lysosomal acid lipase deficiency GSD type 1 Niemann-Pick disease | TTP HUS HELLP DIC | CAR-T BITES ICB DRESS |
| Malignant . | Infectious . | Inflammatory . | Storage/metabolic . | Vascular . | Iatrogenic . |
|---|---|---|---|---|---|
| BCL HL TCL CLL MDS AML ALL CML Myelofibrosis HCC | EBV CMV HIV Leishmania COVID-19 Atypical infections* West Nile virus Tuberculosis Aspergillus Bacterial sepsis | SJIA Still's disease SLE ALPS Sarcoidosis GCA Kawasaki disease PIMS Kikuchi disease | Gaucher's disease LPI Wolman's disease Lysosomal acid lipase deficiency GSD type 1 Niemann-Pick disease | TTP HUS HELLP DIC | CAR-T BITES ICB DRESS |
Atypical infections: Rickettsia, Leptospira, Bartonella, Brucella, and Ehrlichia.
ALPS, autoimmune lymphoproliferative syndrome; AML, acute myeloid leukemia; BCL, B-cell lymphoma; BITE, bispecific T-cell engager; CART, chimeric antigen receptor T cell therapy; CLL, chronic lymphocytic leukemia; CML, chronic myeloid leukemia; CMV, cytomegalovirus; DIC, disseminated intravascular coagulation; DRESS, drug reaction with eosinophilia and systemic symptoms; EBV, Epstein Bar virus; GCA, giant cell arteritis; GSD, glycogen storage disease; HCC, hepatocellular carcinoma; HELLP, emolysis, elevated liver enzymes and low platelet count; HL, Hodgkin lymphoma; HUS, hemolytic uremic syndrome; ICB, immune checkpoint blockade; LPI, lysinuric protein intolerance; MDS, myelodysplastic syndrome; PIMS, pediatric inflammatory multisystem syndrome; SLE, systemic lupus erythematosus; SJIA, systemic juvenile arthritis; TCL, T-cell lymphoma; TTP, thrombotic thrombocytopenic purpura.