Haemophagocytic syndrome and COVID-19

被引:61
作者
Retamozo, Soledad [1 ]
Brito-Zeron, Pilar [2 ,3 ]
Siso-Almirall, Antoni [4 ,5 ]
Flores-Chavez, Alejandra [3 ]
Soto-Cardenas, Maria-Jose [6 ]
Ramos-Casals, Manuel [3 ,7 ,8 ,9 ]
机构
[1] Inst Univ Ciencias Biomed Cordoba IUCBC, Inst Modelo Cardiol Privado SRL Cordoba Argentina, Cordoba, Argentina
[2] Hosp CIMA Sanitas, Dept Med, Autoimmune Dis Unit, Barcelona, Spain
[3] IDIBAPS CELLEX, Lab Autoimmune Dis Josep Font, Barcelona, Spain
[4] Inst Investigac Biomed August Pi i Sunyer IDIBAPS, Grp Tranversal Recerca Atencio Primaria, Barcelona, Spain
[5] Consorci Atencio Primaria Salut Barcelona Esquerr, Ctr Atencio Primaria Les Corts, Barcelona, Spain
[6] Hosp Univ Puerta Mar, Dept Internal Med, Cadiz, Spain
[7] Univ Barcelona, Dept Med, Barcelona, Spain
[8] ICMiD, Hosp Clin, Dept Autoimmune Dis, Barcelona, Spain
[9] Hosp Clin Barcelona, Serv Malalties Autoimmunes Sistem, C Villarroel 170, Barcelona 08036, Spain
关键词
COVID-19; Cytokine storm syndrome; Haemophagocytic syndrome; Hyperinflammatory syndrome; Macrophage activation syndrome; Multisystem inflammatory syndrome; CORONAVIRUS INFECTIONS; CYTOKINE STORM; LYMPHOHISTIOCYTOSIS; DISEASE;
D O I
10.1007/s10067-020-05569-4
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Primary and secondary haemophagocytic lymphohistiocytosis (HLH) are hyperferritinaemic hyperinflammatory syndromes with a common terminal pathway triggered by different etiopathogenetic factors. HLH is characterised by a decreased capacity of interferon gamma production with an activated NK phenotype profile similar to other hyperinflammatory syndromes. Viruses are closely linked to the development of HLH as infectious triggers, and the break of tolerance to self-antigens is considered a critical mechanism involved in the development of immune-mediated conditions triggered by viral infections. Emerging studies in patients with COVID-19 are suggesting a key role of monocytes/macrophages in the pathogenesis of this viral infection, and there is a significant overlap between several features reported in severe COVID-19 and the features included in the HLH-2004 diagnostic criteria. Therefore, SARS-Cov-2, as other respiratory viruses, may also be considered a potential etiological trigger of HLH. The frequency of HLH in adult patients with severe COVID-19 is lower than 5%, although this figure could be underestimated considering that most reported cases lacked information about some specific criteria (mainly the histopathological criteria and the measurement of NK cell function and sCD25 levels). Because HLH is a multi-organ syndrome, the diagnostic approach in a patient with severe COVID-19 in whom HLH is suspected must be carried out in a syndromic and holistic way, and not in the light of isolated clinical or laboratory features. In COVID-19 patients presenting with persistent high fever, progressive pancytopenia, and hepatosplenic involvement, together with the characteristic triad of laboratory abnormalities (hyperferritinaemia, hypertriglyceridaemia, and hypofibrinogenaemia), the suspicion of HLH is high, and the diagnostic workup must be completed with specific immunological and histopathological studies.
引用
收藏
页码:1233 / 1244
页数:12
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