Table 2. Diagnostic criteria for primary... | American Society of Hematology

Table 2.

Diagnostic criteria for primary diseases

SAIDs-HLH: meet all the following criteria:

1. Meet the HLH diagnosis criteria.
2. Had excessive systemic inflammation, leading to recurrent fever, rashes, increased C-reactive protein and erythrocyte sedimentation rate, and IL-6 overproduction.
3. No evidence of infection, tumor, or specific antibody involvement.

CAEBV-HLH: meet all the following criteria:

1. Meet the HLH diagnosis criteria.
2. Infectious mononucleosis-like symptoms persist or recurrent >3 mo.
3. Evidence of EBV infection.
- • Increased EBV-DNA copy numbers (>500 copies/L) in the peripheral blood and plasma (cell-free).
- • Anti-EBV serological pattern indicated EBV reactivation from a previous infection: positive EBV nuclear antigen-IgG and EBV capsid antigen-IgG antibodies with high avidity.
- • EBV-EBV encoded RNAs in situ hybridization was positive in the affected tissues.
4. Lymph node or bone marrow pathologic biopsy suggests EBV-positive lymphoproliferative disease and exclusion of other possible diagnoses: known immunodeficiency and malignancy.

EBV-HLH: meet all the following criteria:

1. Meet the HLH diagnosis criteria.
2. Evidence of EBV infection.
- • Increased EBV-DNA copy numbers (>500 copies/L) in the peripheral blood and plasma (cell-free).
- • Anti-EBV serological pattern indicated primary or previous EBV infection.
3. CAEBV was excluded.