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2015 ; 94
(40
): e1692
Nephropedia Template TP
gab.com Text
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English Wikipedia
Coagulation Disorders and Bleedings in Critically Ill Patients With
Hemophagocytic Lymphohistiocytosis
#MMPMID26448017
Valade S
; Azoulay E
; Galicier L
; Boutboul D
; Zafrani L
; Stepanian A
; Canet E
; Lemiale V
; Venot M
; Veyradier A
; Mariotte E
Medicine (Baltimore)
2015[Oct]; 94
(40
): e1692
PMID26448017
show ga
Reactive hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition
related to a cytokine storm leading to multiorgan dysfunction. A better
understanding of coagulation disorders, frequently reported in HLH patients, may
improve outcomes. Critically ill HLH patients managed in a multidisciplinary
national reference center were retrospectively included. Relationships between
coagulation disorders, severe bleedings, and outcomes were assessed. One hundred
and seventeen patients fulfilled the HLH 2004 criteria. The most common HLH
etiology was hematologic conditions (73%), followed by infectious diseases (20%),
systemic rheumatic diseases (5%), and undetermined HLH etiology (3%). All
patients exerted thrombocytopenia. Coagulation disorders were diagnosed in 79
(68%) patients (61 had hypofibrinogenemia < 1.5 g/L, 51 had prothrombin time
[PT]? 0%). The worst median value throughout ICU stay was 52% (38-65) for PT
with a factor V level of 35% (27-43), 1.59 (1.30-2.09) for the activated partial
thromboplastin time (APTT) ratio, and 2.33 g/L (1.13-3.86) for the fibrinogen
level. Disseminated intravascular coagulation (DIC) was found in 50% of patients.
Coagulation disorders were more frequent in immunocompromised patients, those
with histological/cytological feature of hemophagocytosis, those with the highest
ferritin concentrations, and in patients with HLH not related to infection. These
patients were more prone to receive mechanical ventilation, vasopressors, or
renal replacement therapy. Twenty-six (22%) patients presented severe bleeding
complications, including 5 patients dying from hemorrhagic shock. Strikingly, the
only coagulation parameter significantly associated with severe bleeding was low
fibrinogen with a cutoff value of 2 g/L (P = 0.03). Overall, 33 (28%) patients
died in the ICU and hospital mortality was 44%. Coagulation disorders were
associated with higher mortality, especially fibrinogen < 2 g/L (P = 0.04) and PT
value (P = 0.03). The occurrence of bleeding complications was not associated
with higher risk of hospital death. Risk factors associated with mortality by
multivariate analysis were fibrinogen level < 2 g/L (OR 2.42 [1.08-5.41]), SOFA
score > 6 (OR 3.04 [1.32-6.98]), and age > 46 years (OR 2.26 [1.02-5.04]). Up to
two-third of critically ill HLH patients present with coagulation disorders.
Hypofibrinogenemia or DIC was found in half of the patients and low PT in 40%.
These patients require more life support and have a higher mortality rate.
Fibrinogen < 2 g/L is associated with the occurrence of severe bleeding and
mortality.