Continuous intravenous anakinra for treating severe secondary haemophagocytic lymphohistiocytosis/macrophage activation syndrome in critically ill children

2021 ◽  
Author(s):  
James E.G. Charlesworth ◽  
Shaun Wilson ◽  
Amrana Qureshi ◽  
Esther Blanco ◽  
Amy Mitchell ◽  
...  
Keyword(s):  
Critically Ill ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Critically Ill Children ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Activation Syndrome

scholarly journals Macrophage activation syndrome/haemophagocytic lymphohistiocytosis secondary to Burkholderia cepacia complex septicaemia in an elderly female carrier of X-linked chronic granulomatous disease with extreme lyonisation: ‘cepacia syndrome’ revisited

2019 ◽  
Vol 12 (8) ◽  
pp. e230434 ◽  
Author(s):  
Nicolás Urriola ◽  
Andrew Williams ◽  
Karuna Keat
Keyword(s):  
Chronic Granulomatous Disease ◽  
Burkholderia Cepacia ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Burkholderia Cepacia Complex ◽  
Female Carrier ◽  
Granulomatous Disease ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Stimulating Factor ◽  
Activation Syndrome

X-linked carriers of chronic granulomatous disease (CGD) may become phenotypically affected if substantial skewing from lyonisation occurs. We describe a 73-year-old female carrier with an overt CGD phenotype due to skewed lyonisation, complicated by macrophage activation syndrome (MAS)/haemophagocytic lymphohistiocytosis (HLH) secondary to Burkholderiacepacia complex septicaemia that was successfully treated with a combination of three antibiotics, an antifungal, granulocyte colony stimulating factor, intravenous immune globulin (IVIG) and ciclosporin. Fully phenotypic immunodeficiency is possible in X-linked CGD carriers when skewed lyonisation occurs, rendering such patients to all the same sequelae of CGD such as MAS/HLH. MAS/HLH should be thoroughly excluded when evaluating ‘cepacia syndrome’ in non-CGD patients.

scholarly journals SOLUBLE ST2 AND CD163 AS POTENTIALBIOMARKERS TO DIFFERENTIATE PRIMARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS FROM MACROPHAGE ACTIVATION SYNDROME

2019 ◽  
Vol 11 (1) ◽  
Author(s):  
Zhuo Gao ◽  
Yini Wang ◽  
Jingshi Wang ◽  
Jia Zhang ◽  
Zhao Wang
Keyword(s):  
Healthy Subjects ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Serum Levels ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Soluble St2 ◽  
Tnf Α ◽  
Significant Difference ◽  
Ifn Γ ◽  
Activation Syndrome

Abstract Background and Objective: The differentiation of primary haemophagocytic lymphohistiocytosis (pHLH) and macrophage activation syndrome (MAS) poses a challenge to hematologists. The aim of this study was (1) to compare the levels of soluble ST2 (sST2), sCD163, IL-10, IFN-γ, TNF-α and IL-18 in patients with pHLH and MAS and (2) to investigate whether they can help differentiate the two diseases. Methods: A total of 54 participants were recruited in this study, including 12 pHLH patients, 22 MAS patients and 20 healthy subjects. We measured the levels of sST2 and sCD163 in serum by ELISA. The serum levels of IL-10, IFN-γ, TNF-α and IL-18 were detected using a Luminex 200 instrument. Results: The serum levels of sST2 and sCD163 in MAS patients were markedly higher than that in pHLH patients (363.13 ± 307.24 ng/ml vs 80.75 ± 87.04 ng/ml, P = 0.004; 3532.72 ± 2479.68 ng/ml vs 1731.96 ± 1262.07 ng/ml, P = 0.046). There was no significant difference in the expression of IFN-γ (306.89 ± 281.60 pg/ml vs 562.43 ± 399.86 pg/ml), IL-10 (20.40 ± 30.49 pg/ml vs 8.3 ± 13.14 pg/ml), IL-18 (463.33 ± 597.04 pg/ml vs 1247.82 ± 1318.58 pg/ml) and TNF-α (61.48 ± 84.69 pg/ml vs 106.10 ±77.21 pg/ml) between pHLH and MAS. Conclusion: Patients with pHLH and MAS show some differences in cytokine profiles. The elevated levels of IFN-γ, IL-10, IL-18 and TNF-α can contribute to the diagnosis of HLH, but may not discriminate pHLH from MAS. Levels of sST2 and sCD163 may serve as markers to distinguish pHLH from MAS.

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