3 These criteria are composed of 6 easily measured clinical and laboratory parameters (fever, splenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia/hypofibrinogenemia, and hemophagocytosis), as well as 3 more specialized tests (genetic testing, NK cell function, and serum soluble interleukin-2 ). 2 The HLH-2004 criteria were developed for pediatric HLH but are commonly used to diagnose adult HLH as well. 1 HLH is difficult to diagnose, as it can mimic sepsis, liver failure, autoimmune disease, malignancy, and other inflammatory/immune-activating conditions. Hemophagocytic syndromes (HPS), including hemophagocytic lymphohistiocytosis (HLH), are life-threatening conditions caused by pathological overactivation of the immune system resulting in hypercytokinemia and multiorgan failure. Additional studies enriched with patients without HLH who have conditions associated with T-cell activation, such as lymphoma and autoimmune lymphoproliferative syndrome, are needed. Serum sIL-2r is a sensitive test for diagnosis of adult HLH, but is not as specific as previously reported in children. Levels above 10 000 U/mL were not associated with worse prognosis in patients with HLH. Higher mean sIL-2r levels were seen in malignancy-associated HLH (20 241 U/mL) compared with infection-associated HLH and macrophage activation syndrome (97 U/mL, respectively P <. Although there was a large indeterminate range for sIL-2r, a level of 2400 U/mL or less was helpful for ruling out HLH (sensitivity, 100%), and more than 10 000 U/mL was helpful for ruling in HLH (specificity, 93%). The optimal threshold for sIL-2r was 2515 U/mL (sensitivity, 100% specificity, 72.5%). The receiver operating characteristic curve demonstrated that sIL-2r is a good to excellent diagnostic test for adult HLH, with an area under the curve (AUC) of 0.90 (95% confidence interval, 0.83-0.97) compared with AUC 0.78 (95% confidence interval, 0.67-0.88) for ferritin. There were 38 patients with HLH and 40 with a non-HLH diagnosis (such as sepsis, liver disease, histiocyte disorders, autoimmune disease, leukemia, or lymphoma). Serum sIL-2r levels were measured by enzyme-linked immunosorbent assay (adult reference range, 241-846 U/mL).
We conducted a single-center retrospective study of 78 consecutive adults who had sIL-2r measured for suspected HLH. Serum soluble interleukin-2 receptor (sIL-2r) is an important disease marker in hemophagocytic lymphohistiocytosis (HLH), but there are no published data on its diagnostic value in adults.
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