Hemophagocytic lymphohistiocytosis: A diagnostic conundrum

Grzybowski, Brittany; Vishwanath, Vijay

doi:10.4103/jpn.jpn_140_16

Cited by 23 publications

(6 citation statements)

References 32 publications

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“… 12 Moreover, NK cells regulate the macrophage turnover and provide a negative feedback mechanism to macrophage overabundance and a more tissue-aggressive inflammatory response to infection. 13 The absence of NK-cell–mediated frontline defense is associated with an aggressive secondary hyperinflammatory response driving ARDS in severe COVID-19 patients and forms the hypothetical framework of this study. Here, we evaluated NK-cell counts and activity as well as serum concentrations of associated cytokines and soluble receptors as characteristics of hyperinflammation associated with severe COVID-19.…”

Section: Introductionmentioning

confidence: 96%

Impaired natural killer cell counts and cytolytic activity in patients with severe COVID-19

et al. 2020

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The global pandemic of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)–driven coronavirus disease 2019 (COVID-19) has caused unprecedented human death and has seriously threatened the global economy. Early data suggest a surge in proinflammatory cytokines in patients with severe COVID-19, which has been associated with poor outcomes. We recently postulated that the inflammatory response in patients with severe COVID-19 disease is not inhibited by natural killer (NK) cells, resulting in a “cytokine storm.” Here, we assessed the NK-cell functional activity and the associated cytokines and soluble mediators in hospitalized COVID-19 patients. Significantly impaired NK-cell counts and cytolytic activity were observed in COVID-19 patients when compared with healthy controls. Also, cytokines like interleukin 12 (IL12), IL15, and IL21 that are important for NK-cell activity were not detected systematically. Serum concentrations of soluble CD25 (sCD25)/soluble IL2 receptor α (sIL2-Rα) were significantly elevated and were inversely correlated with the percentage of NK cells. Impaired NK-cell cytolytic activity together with other laboratory trends including elevated sCD25 were consistent with a hyperinflammatory state in keeping with macrophage-activation syndrome. Our findings suggest that impaired counts and cytolytic activity of NK cells are important characteristics of severe COVID-19 and can potentially facilitate strategies for immunomodulatory therapies.

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Section: Introductionmentioning

confidence: 96%

Impaired natural killer cell counts and cytolytic activity in patients with severe COVID-19

et al. 2020

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“…Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening severe systemic hyperinflammatory syndrome characterized by hypercytokinemia with multi-organ dysfunction. Viral etiologies have been recognized as triggers of sHLH and account for approximately 35% of adult cases [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Assessment of the HScore as a predictor of disease outcome in patients with COVID-19

et al. 2021

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Severe coronavirus disease 2019 (COVID-19) accompanies hypercytokinemia, similar to secondary hemophagocytic lymphohistiocytosis (sHLH). We aimed to find if HScore could predict disease severity in COVID-19. HScore was calculated in hospitalized children and adult patients with a proven diagnosis of COVID-19. The need for intensive care unit (ICU), hospital length of stay (LOS), and in-hospital mortality were recorded. The median HScore was 43.0 (IQR 0.0–63.0), which was higher in those who needed ICU care (59.7, 95% CI 46.4–72.7) compared to those admitted to non-ICU medical wards (38.8, 95% CI 32.2–45.4; P = 0.003). It was also significantly higher in patients who died of COVID-19 (105.1, 95% CI 53.7–156.5) than individuals who survived (41.5, 95% CI 35.8–47.1; P = 0.005). Multivariable logistic regression analysis revealed that higher HScore was associated with a higher risk of ICU admission (adjusted OR = 4.93, 95% CI 1.5–16.17, P = 0.008). The risk of death increased by 20% for every ten units increase in HScore (adjusted OR 1.02, 95% CI 1.00–1.04, P = 0.009). Time to discharge was statistically longer in high HScore levels than low levels (HR = 0.41, 95% CI 0.24–0.69). HScore is much lower in patients with severe COVID-19 than sHLH. Higher HScore is associated with more ICU admission, more extended hospitalization, and a higher mortality rate. A modified HScore with a new cut-off seems more practical in predicting disease severity in patients with severe COVID-19.

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“…HLH is diagnosed by the HLH-2004 protocol. Raised ferritin >10000 ng/ml is recognized to be 98% sensitive and 96% specific for HLH; hence, it is a useful screening tool for early detection of HLH, triggering further investigations [ 11 ]. However, clinical conditions such as sepsis or systemic inflammatory response syndromes can show similar characteristics [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…Treatment of HLH involves treatment of the provocative illness if secondary, use of immune-suppressive and modulatory agents, and subsequent stem-cell transplantation. The agents commonly used include dexamethasone, methylprednisolone, methotrexate, intravenous immunoglobulin, and cyclosporine A [ 10 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

Expanded Dengue Syndrome: A Case of Subarachnoid Haemorrhage, Cranial Diabetes Insipidus, and Haemophagocytic Lymphohistiosis

Jayasinghe

Pinto

Arachchi

et al. 2021

Case Reports in Infectious Diseases

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Dengue fever is a mosquito-borne viral infection common in tropical countries with increasing incidence. The clinical manifestations can range from asymptomatic or mild infection to multiorgan failure. The latter is also called “Expanded dengue syndrome,” and it carries a high rate of mortality and morbidity. Intensive care management of such complicated cases is a challenging task for the treating physician, which requires intense monitoring and a multidisciplinary approach for decision making. We report an atypical case of an expanded dengue syndrome presented with subarachnoid haemorrhage associated with moderate thrombocytopenia, cranial diabetes insipidus, and haemophagocytic lymphohistiosis in a young healthy female patient.

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Hemophagocytic lymphohistiocytosis: A diagnostic conundrum

Cited by 23 publications

References 32 publications

Impaired natural killer cell counts and cytolytic activity in patients with severe COVID-19

Impaired natural killer cell counts and cytolytic activity in patients with severe COVID-19

Assessment of the HScore as a predictor of disease outcome in patients with COVID-19

Expanded Dengue Syndrome: A Case of Subarachnoid Haemorrhage, Cranial Diabetes Insipidus, and Haemophagocytic Lymphohistiosis

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