Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report

Table 2 Diagnostic criteria for hemophagocytic lymphohistiocytosis: HLH-2004 - revised diagnostic guidelines for HLH10

Diagnosis of HLH can be established if one of the two criteria below are met
1 A molecular diagnosis consistent with HLH (i.e. reported mutations found in either PRF1 or MUNC13-4), or
2 Diagnostic criteria for HLH are fulfilled (i.e. at least five of the eight criteria listed below are present)
(A) Persistent fever
(B) Splenomegaly
(C) Cytopenia (affecting ≥ 2 of 3 lineages in the peripheral blood)
(i) Hemoglobin < 90g/L (in infants < 4 wk: < 100g/L)
(ii) Platelets < 100 × 109/L
(iii) Neutrophils < 1.0 × 109/L
(D) Hypertriglyceremia and/or hypofibrinogenemia
(i) Fasting triglycerides ≥ 3.0 mmol/L (i.e. ≥ 265 mg/dL)
(ii) Fibrinogen ≤ 1.5g/L
(E) Hemophagocytosis in bone marrow, spleen or lymph nodes, no evidence of malignancy
(F) Serum ferritin ≥ 500 µg/L (i.e. 500 ng/mL)
(G) Low or absent natural killer cell activity (according to local laboratory reference)
(H) Increased serum sIL2Rα (according to local laboratory reference)

HLH: Hemophagocytic lymphohistiocytosis.