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Intervention/management
Strength of
recommendation
Certainty of
evidence
Only irradiated, leukocyte-depleted cellular blood
products
Strong Moderate
Systemic glucocorticoids Strong Moderate
B-cell–depleting therapy for severe refractory
cytopenias, control of EBV-infected B cells, or
significant lymphoproliferation
Strong Low
High-dose IVIG
Do not use splenectomy, unless splenic
sequestration or severe, refractory cytopenias
Conditional Moderate
Granulocyte-colony stimulating factor (G-CSF) to
achieve an ANC of 1000–1500 cells/μl for patients
with recurrent and severe infections
Conditional Low
Do not treat autoimmune neutropenia without
infections
Strong Moderate
Do not treat isolated benign lymphoproliferation
without organ compromise, discomfort, or
significant impact on quality of life (QoL)
Conditional Very low
Radiation sensitivity testing before cancer therapy
in patients with IEI without molecular diagnosis
Conditional Low
Standard treatment regimens of shorter duration Conditional Low
Coordinated treatment by a multidisciplinary team
without expertise in hyperinflammation
Strong Low
Defer HLH treatment in stable patients until
associated malignancy and infection have been
ruled out
