Table 2.

Red flags, adapted from Novotny et al. [6]

Predisposing illness (cancer, chronic/latent infection, autoimmune disease, immune suppression)

Familial history of HLH

Asian descent

Persistent/elevated febrile state

Hepatomegaly, splenomegaly, lymphadenopathy

Organ involvement, DIC

≥1 Cytopenia

Hyperferritinaemia

Hypertriglyceridaemia

Hepatic function alteration, elevated LDH

Hyponatraemia

Hypofibrinogenaemia during febrile/inflammatory state

HScore >169 points

BM score ≥10 points

BM score, Bone Marrow score; DIC, disseminated intravascular coagulation; HLH, haemophagocytic lymphohistiocytosis; LDH, lactate dehydrogenase.