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      Soluble interleukin-2 receptor is a sensitive diagnostic test in adult HLH.

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          Abstract

          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. We conducted a single-center retrospective study of 78 consecutive adults who had sIL-2r measured for suspected HLH. Serum sIL-2r levels were measured by enzyme-linked immunosorbent assay (adult reference range, 241-846 U/mL). 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). 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. The optimal threshold for sIL-2r was 2515 U/mL (sensitivity, 100%; specificity, 72.5%). 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%). Higher mean sIL-2r levels were seen in malignancy-associated HLH (20 241 U/mL) compared with infection-associated HLH and macrophage activation syndrome (9720 and 5008 U/mL, respectively; P < .05). Levels above 10 000 U/mL were not associated with worse prognosis in patients with HLH. Serum sIL-2r is a sensitive test for diagnosis of adult HLH, but is not as specific as previously reported in children. Additional studies enriched with patients without HLH who have conditions associated with T-cell activation, such as lymphoma and autoimmune lymphoproliferative syndrome, are needed.

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          Author and article information

          Journal
          Blood Adv
          Blood advances
          American Society of Hematology
          2473-9529
          2473-9529
          Dec 12 2017
          : 1
          : 26
          Affiliations
          [1 ] Division of Hematology, Department of Medicine, and.
          [2 ] Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.
          [3 ] Division of Experimental Anesthesiology, University Hospital Ulm, Ulm, Germany; and.
          [4 ] Division of Immunology and.
          [5 ] Division of Bone Marrow Transplant and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
          Article
          2017/012310
          10.1182/bloodadvances.2017012310
          5728644
          29296904
          7bd0aca3-cb6a-44d3-9e9f-fb091ab122c4
          History

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