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

Abstract

Key Points• The optimal cutoff for soluble interleukin-2 receptor in this study was 2515 U/mL (sensitivity, 100%; specificity, 72.5%).• sIL-2r #2400 U/mL is helpful for ruling out HLH (sensitivity, 100%) and .10 000 U/mL is helpful for ruling it in (specificity, 93%). 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 soluble VSIG4 as a surrogate marker for the diagnosis of lymphoma‐associated hemophagocytic lymphohistiocytosis

“…Simultaneously, we also examined the ROC curve of sIL‐2Rα for the diagnosis of L‐HLH in patients with lymphoma; the AUC was 0·94 and the optimal cut‐off was 8033 U/ml, with a highest Youden index of 0·82. The corresponding sensitivity was 94·44% (95% CI, 72·71–99·86%) and the corresponding specificity, 87·18% (95% CI, 72·57–95·70%) (Fig A), values consistent with most other reported results (Lehmberg et al ., ; Hayden et al ., ). Pairwise comparison of the ROC curves demonstrated that there was no significant difference between VSIG4 and sIL‐2Rα for the diagnosis of non‐HLH and L‐HLH in patients with lymphoma ( P = 0·2706, DeLong test).…”

“…Although a few diagnostic guidelines adopted by certain centres exclude the measurement of sIL-2Ra for the diagnosis of HLH, the HLH-2004 guideline is still the most widely cited one (Hayden et al, 2016;Lin et al, 2017). However, high sIL-2Ra levels are found not only in HLH and/or lymphoma, but also in autoimmune lymphoproliferative disorders, cancers and other conditions associated with T-cell activation such as transplantations (Adams et al, 1989;Foley et al, 1998;Bien & Balcerska, 2008;Hayden et al, 2017). In particular, several studies have revealed that IL-2Ra positive cells exist in some cancer tissues such as breast cancer, gastric cancer and colorectal cancer and metastases to lymph nodes (Murakami, 2004).…”

“…The soluble interleukin-2 receptor alpha (sIL-2Ra; also termed sCD25) is a truncated protein shed from the surface of activated CTLs. Thus, in the widely accepted HLH-2004 criteria, prolonged high sIL-2Ra level has been regarded as a surrogate serum marker of activated CTLs under enduring stimulus by pro-inflammtory cytokines for the diagnosis of HLH (Groen-Hakan et al, 2017;Hayden et al, 2017). In contrast, a reliable marker related to activated macrophages remains elusive devoid in clinical practice (Schulert & Grom, 2015;Al-Samkari & Berliner, 2018).…”

Hemophagocytic lymphohistiocytosis in the setting of HELLP Syndrome

“…Soluble IL‐2R (sIL‐2R) came back at 10 580 pg/mL (<1033 pg/mL). sIL‐2R levels of >2515 have a 100% sensitivity and 72.5% specificity for the diagnosis of HLH . She was started on dexamethasone with weekly etoposide per the HLH‐94 protocol .…”

Hämophagozytische Lymphohistiozytose bei unspezifischer Virusinfektion

“…Laborparameter, arterielle Blutgasanalysen und SOFA-Score im VerlaufBestimmung von sIL-2R ist in diesen Fällen sehr sinnvoll und zeigte ab Werten von 2,515 U/ml eine Sensitivität von 100 % und eine Spezifität von 72,5 %[9]. Bei Zweifelsfällen zur Diagnose kann der H-Score (http://saintantoine.aphp.…”