Value of hemophagocytosis in the diagnosis of hemophagocytic lymphohistiocytosis in critically ill patients

Nyvlt, Peter; Schuster, Friederike S.; Ihlow, Jana; Heeren, Patrick; Spies, Claudia; Hiesgen, Josephine; Schenk, Thomas; von Brünneck, Ann‐Christin; Westermann, Jörg; Brunkhorst, Frank M.; La Rosée, Paul; Janka, Gritta; Lachmann, Cornelia; Lachmann, Gunnar

doi:10.1111/ejh.14185

Cited by 3 publications

References 43 publications

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The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset still’s disease, and COVID-19 cytokine storm

Goubran,

Spaner,

Stukas

et al. 2024

Sci Rep

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Cytokine storm syndromes such as hemophagocytic lymphohistiocytosis (HLH), Adult-onset Still’s disease (AOSD), and COVID-19 cytokine storm (CCS) are characterized by markedly elevated inflammatory cytokines. However clinical measurement of serum cytokines is not widely available. This study examined the clinical utility of C-reactive protein (CRP) and ferritin, two inexpensive and widely available inflammatory markers, for distinguishing HLH from AOSD and CCS. This single centre retrospective study included 44 secondary HLH patients, 14 AOSD patients, and 13 CCS patients. Baseline CRP and ferritin measured within 72 h of diagnosis and before administration of corticosteroids or other anti-inflammatory therapies were analyzed. The median CRP in HLH patients was lower than AOSD (71 mg/L vs. 172 mg/L, p < 0.001) and CCS (71 mg/L vs. 121 mg/L, p = 0.0095) patients. Serum ferritin levels were lower in CCS compared to HLH (1,386 µg/L vs. 29,019 µg/L, p < 0.001) and AOSD (11,359 µg/L vs. 29,019 µg/L, p = 0.035). A CRP < 130 mg/L when combined with an HScore > 136 improves the specificity of HScore alone for HLH from 85.2 to 96.3%. Adding CRP < 130 mg/L to ferritin > 15,254 µg/L increases specificity for HLH from 88.9 to 100%. This study demonstrates that median CRP is lower in HLH than in AOSD and CCS, and median ferritin is lower in CCS than in HLH or AOSD. This study demonstrates the clinical utility of these widely available inflammatory markers for distinguishing between different cytokine storm syndromes. Supplementary Information The online version contains supplementary material available at 10.1038/s41598-024-82760-6.

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The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset still’s disease, and COVID-19 cytokine storm

Goubran,

Spaner,

Stukas

et al. 2024

Sci Rep

View full text Add to dashboard Cite

show abstract

The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset Still’s disease, and COVID-19 cytokine storm

Goubran,

Spaner,

Stukas

et al. 2024

Preprint

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Objectives Cytokine storm syndromes such as hemophagocytic lymphohistiocytosis (HLH), Adult-onset Still’s disease (AOSD), and COVID-19 cytokine storm (CCS) are characterized by markedly elevated inflammatory cytokines. However clinical measurement of serum cytokines is not widely available. This study examined the clinical utility of C-reactive protein (CRP) and ferritin, two inexpensive and widely available inflammatory markers, for distinguishing HLH from AOSD and CCS. Methods This single centre retrospective study included 44 secondary HLH patients, 14 AOSD patients, and 13 CCS patients. Baseline CRP and ferritin measured within 72 hours of diagnosis and before administration of corticosteroids or other anti-inflammatory therapies were analyzed. RESULTS The median CRP in HLH patients was lower than AOSD (71 mg/L vs. 172 mg/L, p < 0.001) and CCS (71 mg/L vs. 121 mg/L, p = 0.0095) patients. Serum ferritin levels were lower in CCS compared to HLH (1,386 µg/L vs. 29,019 µg/L, p < 0.001) and AOSD (11,359 µg/L vs. 29,019 µg/L, p = 0.035). A CRP < 130 mg/L when combined with an HScore >136 improves the specificity of HScore alone for HLH from 85.2% to 96.3%. Adding CRP < 130 mg/L to ferritin >15,254 µg/L increases specificity for HLH from 88.9% to 100%. CONCLUSIONS This study demonstrates that median CRP is lower in HLH than in AOSD and CCS, and median ferritin is lower in CCS than in HLH or AOSD. This study demonstrates the clinical utility of these widely available inflammatory markers for distinguishing between different cytokine storm syndromes.

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Predicting Secondary Hemophagocytic Lymphohistiocytosis in Adult Patients with Scrub Typhus and Its Prognostic Significance

Sharma,

Saini,

Biswal

et al. 2024

Indian Journal of Critical Care Medicine

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A bstract Objective Secondary hemophagocytic lymphohistiocytosis (sHLH) is an increasingly recognized complication in patients with scrub typhus, potentially contributing to substantial mortality despite appropriate antibiotic treatment. This study aims to determine the prevalence and prognosis of sHLH and identify diagnostic factors in adult patients with scrub typhus in North India. Methods This prospective cohort study was conducted at PGIMER, Chandigarh, from August 2021 to November 2023. sHLH was defined as an HScore of 200 or above. The diagnostic performance of biomarkers such as ferritin, fibrinogen, triglycerides, and C-reactive protein was assessed through receiver operating characteristic curve analysis, evaluating area under the curve (AUC), sensitivity, and specificity. Results Out of 150 patients (mean age 39 years, 54% female), 28 (18.7%) were diagnosed with sHLH. Those presenting with high-grade fever, seizures, high pulse rate, hepatomegaly, splenomegaly, cytopenia, and significant hepatic dysfunction were more likely to have sHLH. Ferritin demonstrated the highest diagnostic utility (AUC 0.83), compared to fibrinogen (AUC 0.72), triglyceride (AUC 0.67), and C-reactive protein (AUC 0.69). The optimal cutoff for ferritin was 2000 ng/mL, with a sensitivity of 90% and a specificity of 66%. Higher ferritin thresholds (6000 ng/mL and 10000 ng/mL) increased specificity to 88% and 95%, respectively. Patients with sHLH often presented with multi-organ failure, necessitating mechanical ventilation and vasopressor support. In-hospital mortality was significantly higher in sHLH patients than in those without (21.4% vs 6.6%, p = 0.025). Conclusion Early detection of sHLH using the HScore and ferritin significantly influences the management of scrub typhus, underscoring the necessity for tailored therapeutic strategies to improve patient outcomes. How to cite this article Selvam S, Tuli A, Yuvasai KP, Saini S, Erla SR, Kaur J, et al . Predicting Secondary Hemophagocytic Lymphohistiocytosis in Adult Patients with Scrub Typhus and Its Prognostic Significance. Indian J Crit Care Med 2024;28(9):823–831.

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Value of hemophagocytosis in the diagnosis of hemophagocytic lymphohistiocytosis in critically ill patients

Cited by 3 publications

References 43 publications

The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset still’s disease, and COVID-19 cytokine storm

The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset still’s disease, and COVID-19 cytokine storm

The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset Still’s disease, and COVID-19 cytokine storm

Predicting Secondary Hemophagocytic Lymphohistiocytosis in Adult Patients with Scrub Typhus and Its Prognostic Significance

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