Table 3.
Previously described cohorts of adult secondary HLH patients treated with anakinra are summarized
| Author, Year | N | Causes of HLH (%) | HLH-Directed Treatments | Outcomes |
|---|---|---|---|---|
| Dimopoulos, 2020 [22] | 8 | COVID-19 pneumonia/sepsis (100%) | Anakinra 200 mg IV q8, corticosteroids (dosage unspecified) | Five of 8 patients (63%) survived. This reported OS was higher than historical series of patients with sHLH in sepsis |
| Kumar, 2017 [18] | 13 | Autoimmune/rheumatologic (62%), hematologic malignancy (23%), unknown (8%), other (8%) | Anakinra 100 mg subq q12 (given with cyclosporine, IVIG, and corticosteroids in most instances) | Nine of 13 patients (69%) survived, including 7 of 8 (88%) with autoimmune/rheumatologic disease |
| Monteagudo, 2020 [19] | 5 | Autoimmune/rheumatologic (80%), unknown (20%), other | Continuous anakinra infusion (up to 2400 mg/d) | Four of 5 (80%) patients survived. Two patients had previously responded poorly to subq anakinra |
| Sammut, 2020 [20] | 4 | CMV viremia (25%), hematologic malignancy (25%), rheumatologic (25%), unknown (25%) | Anakinra 100 mg subq daily, with corticosteroids (75%), or with HLH-2004 protocol (25%) | Two of 4 (50%) patients survived |
| Wohlfarth, 2019 [21] | 8 | Unknown (38%), hematologic malignancy (25%), autoimmune disease (13%), EBV viremia (13%), other (13%) | Anakinra 100 m–200 mg q8, with IVIG (88%) and/or high-dose CS (62%) | Four of 8 patients (50%) survived hospitalization |
CMV cytomegalovirus, COVID19 coronavirus disease 2019, CS corticosteroid, EBV Epstein–barr virus, HLH hemophagocytic lymphohistiocytosis, IV intravenous, sHLH hemophagocytic lymphohistiocytosis, subq subcutaneous