Pragmatic differential diagnosis of generalized lymphadenopathy
| Generalized lymphadenopathy . | ||||
|---|---|---|---|---|
| Inflammatory . | Autoimmune . | Infectious . | Neoplastic/clonal . | Miscellaneous/other . |
| iMCD-TAFRO/iMCD-NOS/iMCD-IPL | SLE | Bacterial | BCL | IgG4-RD |
| HLH | ALPS | M. tuberculosis and other Mycobacterium species | TCLs, including AITL | Serum sickness |
| MIS-C/A | RA (majority localized) | Secondary syphilis (early primary is often localized) | Hodgkin lymphoma | Drug-induced hypersensitivity syndrome |
| Kawasaki disease (children) | SS (majority localized) | Atypical infections† | POEMS and POEMS-MCD | Amyloidosis |
| Kikuchi-Fujimoto disease | sJIA, AOSD | Bacterial sepsis | Metastatic carcinomas | Sarcoidosis |
| Kimura disease | Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) | Lymphogranuloma venereum (Chlamydia trachomatis) | Leukemias (ALL/AML/CML/CLL) | Lipid storage diseases |
| VEXAS syndrome | Chancroid (often localized) | Rosai-Dorfman disease | Chronic granulomatous diseases | |
| Inflammatory pseudotumor | Typhoid fever | Erdheim-Chester disease | Angioimmunoblastic lymphadenopathy with dysproteinemia | |
| Viral | LCH | Other granulomatous diseases (silicosis, berylliosis) | ||
| HIV | Kaposi's sarcoma | |||
| Acute hepatitis (HBV, HCV) | ||||
| CMV, EBV | ||||
| HHV8 and HHV8-MCD | ||||
| HHV6 | ||||
| HSV | ||||
| Adenovirus | ||||
| Dengue fever | ||||
| Measles, mumps (often localized), and rubella viruses | ||||
| Other | ||||
| Toxoplasma gondii (toxoplasmosis), Leishmania (leishmaniasis) | ||||
| Histoplasmosis, coccidioidomycosis, cryptococcosis | ||||
| Generalized lymphadenopathy . | ||||
|---|---|---|---|---|
| Inflammatory . | Autoimmune . | Infectious . | Neoplastic/clonal . | Miscellaneous/other . |
| iMCD-TAFRO/iMCD-NOS/iMCD-IPL | SLE | Bacterial | BCL | IgG4-RD |
| HLH | ALPS | M. tuberculosis and other Mycobacterium species | TCLs, including AITL | Serum sickness |
| MIS-C/A | RA (majority localized) | Secondary syphilis (early primary is often localized) | Hodgkin lymphoma | Drug-induced hypersensitivity syndrome |
| Kawasaki disease (children) | SS (majority localized) | Atypical infections† | POEMS and POEMS-MCD | Amyloidosis |
| Kikuchi-Fujimoto disease | sJIA, AOSD | Bacterial sepsis | Metastatic carcinomas | Sarcoidosis |
| Kimura disease | Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) | Lymphogranuloma venereum (Chlamydia trachomatis) | Leukemias (ALL/AML/CML/CLL) | Lipid storage diseases |
| VEXAS syndrome | Chancroid (often localized) | Rosai-Dorfman disease | Chronic granulomatous diseases | |
| Inflammatory pseudotumor | Typhoid fever | Erdheim-Chester disease | Angioimmunoblastic lymphadenopathy with dysproteinemia | |
| Viral | LCH | Other granulomatous diseases (silicosis, berylliosis) | ||
| HIV | Kaposi's sarcoma | |||
| Acute hepatitis (HBV, HCV) | ||||
| CMV, EBV | ||||
| HHV8 and HHV8-MCD | ||||
| HHV6 | ||||
| HSV | ||||
| Adenovirus | ||||
| Dengue fever | ||||
| Measles, mumps (often localized), and rubella viruses | ||||
| Other | ||||
| Toxoplasma gondii (toxoplasmosis), Leishmania (leishmaniasis) | ||||
| Histoplasmosis, coccidioidomycosis, cryptococcosis | ||||
Atypical infections: Rickettsia, Leptospira, Bartonella, Brucella (brucellosis), Francisella tularensis (tularemia, often localized), diphtheria (often localized), Yersinia pestis (often localized, buboes), and Ehrlichia.
AITL, angioimmunoblastic T-cell lymphoma; ALL, acute lymphoid leukemia; ALPS, autoimmune lymphoproliferative syndrome; AML, acute myeloid leukemia; AOSD, adult-onset Still's disease; BCL, B-cell lymphoma; CML, chronic myeloid leukemia; CMV, cytomegalovirus; EBV, Epstein-Barr virus; HBV, hepatitis B; HCV, hepatitis C; HHV8, human herpesvirus 8; HHV8-MCD, human herpesvirus 8-associated multicentric Castleman disease; HHV6, human herpesvirus 6; HLH, hemophagocytic lymphohistiocytosis; HSV, herpes simplex virus; IgG4-RD, immunoglobulin G4-related disease; iMCD-TAFRO/NOS/IPL, idiopathic multicentric Castleman disease-TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis/renal insufficiency, organomegaly)/NOS (not otherwise specified)/IPL (idiopathic plasmacytic lymphadenopathy); LCH, Langerhans cell histiocytosis; MIS-C/A, multisystem inflammatory syndrome in children and adults; RA, rheumatoid arthritis; sJIA, systemic juvenile idiopathic arthritis; SLE, systemic lupus erythematosus; SS, Sjögren's syndrome; TCL, T-cell lymphoma.