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2017 ; 15
(1
): 172
Nephropedia Template TP
gab.com Text
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English Wikipedia
Macrophage activation-like syndrome: an immunological entity associated with
rapid progression to death in sepsis
#MMPMID28918754
Kyriazopoulou E
; Leventogiannis K
; Norrby-Teglund A
; Dimopoulos G
; Pantazi A
; Orfanos SE
; Rovina N
; Tsangaris I
; Gkavogianni T
; Botsa E
; Chassiou E
; Kotanidou A
; Kontouli C
; Chaloulis P
; Velissaris D
; Savva A
; Cullberg JS
; Akinosoglou K
; Gogos C
; Armaganidis A
; Giamarellos-Bourboulis EJ
BMC Med
2017[Sep]; 15
(1
): 172
PMID28918754
show ga
BACKGROUND: A subanalysis of a randomized clinical trial indicated sepsis
survival benefit from interleukin (IL)-1 blockade in patients with features of
the macrophage activation-like syndrome (MALS). This study aimed to investigate
the frequency of MALS and to develop a biomarker of diagnosis and prognosis.
METHODS: Patients with infections and systemic inflammatory response syndrome
were assigned to one test cohort (n?=?3417) and a validation cohort (n?=?1704).
MALS was diagnosed for patients scoring positive either for the hemophagocytic
syndrome score and/or having both hepatobiliary dysfunction and disseminated
intravascular coagulation. Logistic regression analysis was used to estimate the
predictive value of MALS for 10-day mortality in both cohorts. Ferritin, sCD163,
IL-6, IL-10, IL-18, interferon gamma (IFN-?), and tumor necrosis factor alpha
(TNF-?) were measured in the blood the first 24 h; ferritin measurements were
repeated in 747 patients on day 3. RESULTS: The frequency of MALS was 3.7% and
4.3% in the test and the validation cohort, respectively. In both cohorts, MALS
was an independent risk factor for 10-day mortality. A ferritin level above
4420 ng/ml was accompanied by 66.7% and 66% mortality after 28 days,
respectively. Ferritin levels above 4420 ng/ml were associated with an increase
of IL-6, IL-18, INF-?, and sCD163 and a decreased IL-10/TNF-? ratio, indicating
predominance of pro-inflammatory phenomena. Any less than 15% decrease of
ferritin on day 3 was associated with more than 90% sensitivity for unfavorable
outcome after 10 days. This high mortality risk was also validated in an
independent Swedish cohort (n?=?109). CONCLUSIONS: MALS is an independent
life-threatening entity in sepsis. Ferritin measurements can provide early
diagnosis of MALS and may allow for specific treatment.