scholarly journals Features of hemophagocytic lymphohistiocytosis in the intensive care unit (ICU) : a 260-patient retrospective analysis

2021 ◽  
Author(s):  
Amandine BICHON ◽  
Jérémy BOURENNE ◽  
Jérôme ALLARDET-SERVENT ◽  
Laurent PAPAZIAN ◽  
Sami HRAIECH ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Mortality Rate ◽  
Organ Failure ◽  
Sofa Score ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Icu Mortality ◽  
Specific Therapy ◽  
Specific Population ◽  
Factors Associated ◽  
Intracellular Infection

Abstract Background Adult hemophagocytic lymphohistiocytosis (HLH) is highly lethal in the ICU. The diagnostic and therapeutic emergency that HLH represents is compounded by its unknown pathophysiological mechanisms. Here, we report on a large cohort of adult-acquired HLH in the ICU. We analyzed prognostic factors associated with mortality to define the diagnostic and therapeutic challenges in this specific population, Methods This retrospective study included adult patients diagnosed with HLH in four ICUs in Marseille, France between 2010 and 2020. Patients who fulfilled the HLH-2004 criteria (> 4/8) and/or had an HScore > 169 were diagnosed with HLH. HLH was categorized into four groups according to etiology: sepsis-associated HLH, intracellular infection-associated HLH, malignancy-associated HLH, and idiopathic HLH. Results 260 patients were included: 121 sepsis-associated HLH (47%), 84 intracellular infection-associated HLH (32%), 28 malignancy-associated HLH (11%), and 27 idiopathic HLH (10%). The ICU mortality rate reached 57% (n = 147/260) without a statistical difference between etiological groups. Independent factors associated with mortality in multivariate analysis included age (OR (5 years) = 1.31 [1.16–1.48], p < 0.0001), SOFA score at ICU admission (OR = 1.37 [1.21–1.56], p < 0.0001), degradation of the SOFA score between ICU arrival and HLH diagnosis (Delta SOFA) (OR = 1.47 [1.28–1.70], p < 0.0001), the presence of bone-marrow hemophagocytosis (OR = 5.27 [1.11–24.97], p = 0.04), highly severe anemia (OR = 1.44 [1.09–1.91], p = 0.01), and hypofibrinogenemia (OR = 1.21 [1.04–1.41], p = 0.02). Conclusions In this large retrospective cohort study of critically ill patients, ICU-acquired HLH in adults was associated with a 57% mortality rate, regardless of HLH etiology. Factors independently associated with prognosis included age, presence of hemophagocytosis in bone-marrow aspirates, organ failure at admission, and worsening organ failure during the ICU stay. Whether a rapid diagnosis and the efficacy of specific therapy improve outcome is yet to be prospectively investigated.

scholarly journals High Mortality of HLH in ICU Regardless Etiology or Treatment

2021 ◽  
Vol 8 ◽  
Author(s):  
Amandine Bichon ◽  
Jérémy Bourenne ◽  
Jérôme Allardet-Servent ◽  
Laurent Papazian ◽  
Sami Hraiech ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Mortality Rate ◽  
Organ Failure ◽  
Sofa Score ◽  
Specific Treatment ◽  
Icu Mortality ◽  
Specific Therapy ◽  
Specific Population ◽  
Factors Associated ◽  
Intracellular Infection

Background: Adult hemophagocytic lymphohistiocytosis (HLH) is highly lethal in the ICU. The diagnostic and therapeutic emergency that HLH represents is compounded by its unknown pathophysiological mechanisms. Here, we report on a large cohort of adult HLH in the ICU (ICU-HLH). We analyzed prognostic factors associated with mortality to define the diagnostic and therapeutic challenges in this specific population.Methods: This retrospective study included adult patients diagnosed with HLH in four ICUs in Marseille, France between 2010 and 2020. Patients who fulfilled the HLH-2004 criteria (≥ 4/8) and/or had an HScore ≥ 169 were diagnosed with HLH. HLH was categorized into four groups according to etiology: sepsis-associated HLH, intracellular infection-associated HLH, malignancy-associated HLH, and idiopathic HLH.Results: Two hundred and sixty patients were included: 121 sepsis-associated HLH (47%), 84 intracellular infection-associated HLH (32%), 28 malignancy-associated HLH (11%), and 27 idiopathic HLH (10%). The ICU mortality rate reached 57% (n = 147/260) without a statistical difference between etiological groups. Independent factors associated with mortality in multivariate analysis included age (OR (5 years) = 1.31 [1.16–1.48], p &lt; 0.0001), SOFA score at ICU admission (OR = 1.37 [1.21–1.56], p &lt; 0.0001), degradation of the SOFA score between ICU arrival and HLH diagnosis (Delta SOFA) (OR = 1.47 [1.28–1.70], p &lt; 0.0001), the presence of bone-marrow hemophagocytosis (OR = 5.27 [1.11–24.97], p = 0.04), highly severe anemia (OR = 1.44 [1.09–1.91], p = 0.01), and hypofibrinogenemia (OR = 1.21 [1.04–1.41], p = 0.02).Conclusions: In this large retrospective cohort study of critically ill patients, ICU-HLH in adults was associated with a 57% mortality rate, regardless of HLH etiology or specific treatment. Factors independently associated with prognosis included age, presence of hemophagocytosis in bone-marrow aspirates, organ failure at admission, and worsening organ failure during the ICU stay. Whether a rapid diagnosis and the efficacy of specific therapy improve outcome is yet to be prospectively investigated.

Out-of-Hospital ICU Transfers to an Oncological Referral Center

2016 ◽  
Vol 34 (1) ◽  
pp. 55-61
Author(s):  
Cristina Gutierrez ◽  
Yenny R. Cárdenas ◽  
Kristie Bratcher ◽  
Judd Melancon ◽  
Jason Myers ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Organ Failure ◽  
Critically Ill ◽  
Resource Utilization ◽  
Sofa Score ◽  
Critically Ill Patients ◽  
Level Of Care ◽  
Icu Mortality ◽  
Referral Center ◽  
Patients With Cancer

Objective: To determine resource utilization and outcomes of out-of-hospital transfer patients admitted to the intensive care unit (ICU) of a cancer referral center. Design: Single-center cohort. Setting: A tertiary oncological center. Patients: Patients older than 18 years transferred to our ICU from an outside hospital between January 2013 and December 2015. Measurements and Main Results: A total of 2127 (90.3%) were emergency department (ED) ICU admissions and 228 (9.7%) out-of-hospital transfers. The ICU length of stay (LOS) was longer in the out-of-hospital transfers when compared to all other ED ICU admissions ( P = .001); however, ICU and hospital mortality were similar between both groups. The majority of patients were transferred for a higher level of care (77.2%); there was no difference in the amount of interventions performed, ICU LOS, and ICU mortality between nonhigher level-of-care and higher level-of-care patients. Factors associated with an ICU LOS ≥10days were a higher Sequential Organ Failure Assessment (SOFA) score, weekend admissions, presence of shock, need for mechanical ventilation, and acute kidney injury on admission or during ICU stay ( P < .008). The ICU mortality of transferred patients was 17.5% and associated risk factors were older age, higher SOFA score on admission, use of mechanical ventilation and vasopressors during ICU stay, and renal failure on admission ( P < .0001). Data related to the transfer such as LOS at the outside facility, time of transfer, delay in transfer, and longer distance traveled were not associated with increased LOS or mortality in our study. Conclusion: Organ failure severity on admission, and not transfer-related factors, continues to be the best predictor of outcomes of critically ill patients with cancer when transferred from other facilities to the ICU. Our data suggest that transferring critically ill patients with cancer to a specialized center does not lead to worse outcomes or increased resource utilization when compared to patients admitted from the ED.

Secondary Hemophagocytic Syndrome

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4958-4958
Author(s):  
Vinicius R. P. Mattos ◽  
Michelle P. Silveira ◽  
Flávia M. Ferreira ◽  
Dirceu H. C. Campelo ◽  
Juliano Cordova Vargas ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Mortality Rate ◽  
Diagnostic Criteria ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Hemophagocytic Syndrome ◽  
Bone Marrow Aspiration ◽  
Laboratory Evaluation ◽  
Clinical Aspects ◽  
Solid Organ ◽  
Diagnosis Criteria

Abstract Introduction: Hemophagocytic lymphohistiocytosis (HLH) corresponds to a wide array of potentially fatal hyper-inflammatory diseases involving pathologic immune activation and engulfment of hematopoietic cells by activated macrophages. These disorders have common clinical and laboratorial features, such as severe cytopenias, fever, hepatosplenomegaly and hyperferritinemia, leading to a dismal prognosis when treatment is delayed. Secondary hemophagocytic syndromes may develop as a result of strong immunological activation of the mononuclear phagocyte system by underlying conditions, such as infection, autoimmune diseases, malignancies and metabolic disorders. Mortality rates are high, even with proper treatments, and can reach up to 50%, usually within the first two months of the diagnosis. Diagnosis of this condition is difficult and requires a high degree of suspicion, since the diagnostic criteria are non-specific. Up to 30% of patients with confirmed hemophagocytic syndrome do not show this morphologic aspect in bone marrow examination. We report the data of our institution, regarding the clinical aspects, treatment and outcome of patients with confirmed hemophagocytosis in bone marrow aspiration analysis. Objective: To determine clinical aspects underlying the development of secondary hemophagocytosis and the outcome of patients with this condition. Methods: We retrospectively reviewed all bone marrow aspirations conducted from January, 2012 until December, 2013, regardless of diagnosis. A total of 1682 examinations were performed during this period and reevaluated by three specialists. We found 45 patients with cytological evidence of hemophagocytosis. The medical charts of these patients were reviewed and the following data was retrieved: age, gender, presence of fever and hepatosplenomegaly, underlying disease, past medical history, known underlying immunosuppression, treatment and outcome. Laboratory data was evaluated in the day of the bone marrow aspiration or in the two preceding or following days and included: hemoglobin, leucocytes and platelets counts; ferritin; triglycerides; fibrinogen, lactate dehydrogenase. Diagnosis criteria were defined accordingly to the guidelines of the Hemophagocytic Lymphohistiocytosis Study Group, published in 2004, excluding the soluble CD25 and NK cell activity assays that were unavailable. Results: Median age was 52 years old (range <1 year-72 years) and 58% were male. Twenty-six (57%) had a diagnosis of neoplasia (21 hematological and 5 solid organ malignancies), and 3 patients had recently underwent bone marrow transplantation. Eighteen patients (40%) were receiving immunosuppressive therapy. Evidence of ongoing infection was identified in 34 cases (75.5%), and in 61.8% the agent was identified. Viral infections were commonly associated (26%), and in our case series, cytomegalovirus was the most implicated agent (5 cases). Other virus found were Parvovirus B19, H1N1, Parainfluenza, Herpes-Virus 6 and Epstein-Barr Virus. Bacterial and fungal infections were each responsible for 28,8% of the cases, and in 13 cases (38,2%) the agent was not identified. Among the patients who had complete laboratory evaluation (27 patients), we found that only 37% presented with all diagnostic criteria. Mortality rate was 35.5%, and median survival was 23 months (95% CI 22-60), with most deaths taking place in the first two months. None of the patients received specific treatment, being treated exclusively for the underlying conditions. Conclusion: Secondary hemophagocytic syndrome is a rare yet severe condition, usually associated with a high mortality rate. In most cases, the diagnosis is not suspected and proper treatment not applied. Diagnosis criteria lack specificity and are more useful to the diagnosis of familial forms of HLH. The most common underlying conditions appear to be malignancies, infections and transplant-related immunosuppression. Treatment of the underlying conditions alone still retains large failure rates, and efforts must be made to achieve early diagnosis and employment of therapy. Disclosures No relevant conflicts of interest to declare.

scholarly journals Sepsis induced sequential organ failure assessment score as a prognostic marker in surgical sepsis: a study of 30 cases in 02 years

2021 ◽  
Vol 8 (2) ◽  
pp. 686
Author(s):  
Tushar Nagyan ◽  
Mriganko S. Ray ◽  
Priyanshu M. Varshney ◽  
Sarvpreet S. Malhi ◽  
Naresh A. Modi ◽  
...  
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Mortality Rate ◽  
Sequential Organ Failure Assessment ◽  
Organ Failure ◽  
Critically Ill ◽  
Sofa Score ◽  
Fine Tuning ◽  
Mortality And Morbidity ◽  
Failure Assessment

Background: For the last few decades critical care medicine has been reinventing and fine-tuning organ dysfunction grading to establish a survival scoring system to accurately predict survivality and organ salvageability of critically ill patient in intensive care unit (ICU). The sequential organ failure assessment (SOFA) score assesses the performance of several organ systems in the body and assigns a score, where higher the SOFA score, higher the likelihood of mortality and morbidity. Early prediction of outcome in surgical sepsis is very likely to aid suitable modification of management strategies 13. This may improve prognosis in such patients and prevent mortality to some extent.  Methods: Observational and prospective study of 30 cases, aged>18 years & patients admitted to post-operative ward and surgical intensive care unit (SICU) with suspected surgical infection, and with two or more criteria of SIRS. Results: In this study out of total 30 patients 63.3% patients survived and 36.6% succumbed to their illness. Our study depicted significant increase in mortality rate when the SOFA score was above 12. Ventilated patient showed a higher mortality rate. Delta, mean, total SOFA Score were statistically significant in our study.Conclusions: SOFA score is useful in predicting mortality and morbidity in critically ill patients, because has a strong correlation between a rise in the score and mortality in all stages of admission. In our study, out of 09 patients whose T0 SOFA score was very high (above 12) out of which 03 patients only survived.

scholarly journals Mortality in Escherichia coli bloodstream infections: a multinational population-based cohort study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Melissa C. MacKinnon ◽  
Scott A. McEwen ◽  
David L. Pearl ◽  
Outi Lyytikäinen ◽  
Gunnar Jacobsson ◽  
...  
Keyword(s):  
Escherichia Coli ◽  
Mortality Rate ◽  
Case Fatality ◽  
Population Based ◽  
Day Case ◽  
Fatality Risk ◽  
E Coli ◽  
Factors Associated ◽  
Case Fatality Risk ◽  
Age And Sex

Abstract Background Escherichia coli is the most common cause of bloodstream infections (BSIs) and mortality is an important aspect of burden of disease. Using a multinational population-based cohort of E. coli BSIs, our objectives were to evaluate 30-day case fatality risk and mortality rate, and determine factors associated with each. Methods During 2014–2018, we identified 30-day deaths from all incident E. coli BSIs from surveillance nationally in Finland, and regionally in Sweden (Skaraborg) and Canada (Calgary, Sherbrooke, western interior). We used a multivariable logistic regression model to estimate factors associated with 30-day case fatality risk. The explanatory variables considered for inclusion were year (2014–2018), region (five areas), age (< 70-years-old, ≥70-years-old), sex (female, male), third-generation cephalosporin (3GC) resistance (susceptible, resistant), and location of onset (community-onset, hospital-onset). The European Union 28-country 2018 population was used to directly age and sex standardize mortality rates. We used a multivariable Poisson model to estimate factors associated with mortality rate, and year, region, age and sex were considered for inclusion. Results From 38.7 million person-years of surveillance, we identified 2961 30-day deaths in 30,923 incident E. coli BSIs. The overall 30-day case fatality risk was 9.6% (2961/30923). Calgary, Skaraborg, and western interior had significantly increased odds of 30-day mortality compared to Finland. Hospital-onset and 3GC-resistant E. coli BSIs had significantly increased odds of mortality compared to community-onset and 3GC-susceptible. The significant association between age and odds of mortality varied with sex, and contrasts were used to interpret this interaction relationship. The overall standardized 30-day mortality rate was 8.5 deaths/100,000 person-years. Sherbrooke had a significantly lower 30-day mortality rate compared to Finland. Patients that were either ≥70-years-old or male both experienced significantly higher mortality rates than those < 70-years-old or female. Conclusions In our study populations, region, age, and sex were significantly associated with both 30-day case fatality risk and mortality rate. Additionally, 3GC resistance and location of onset were significantly associated with 30-day case fatality risk. Escherichia coli BSIs caused a considerable burden of disease from 30-day mortality. When analyzing population-based mortality data, it is important to explore mortality through two lenses, mortality rate and case fatality risk.

scholarly journals Establishment of a prognostic model based on the Sequential Organ Failure Assessment score for patients with first-time acute myocardial infarction

2021 ◽  
Vol 49 (5) ◽  
pp. 030006052110119
Author(s):  
Shuai Zheng ◽  
Jun Lyu ◽  
Didi Han ◽  
Fengshuo Xu ◽  
Chengzhuo Li ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Acute Myocardial Infarction ◽  
Intensive Care Unit ◽  
Intensive Care ◽  
Sequential Organ Failure Assessment ◽  
Organ Failure ◽  
Sofa Score ◽  
Cardiovascular Events ◽  
Failure Assessment ◽  
First Time

Objective This study aimed to identify the prognostic factors of patients with first-time acute myocardial infarction (AMI) and to establish a nomogram for prognostic modeling. Methods We studied 985 patients with first-time AMI using data from the Multi-parameter Intelligent Monitoring for Intensive Care database and extracted their demographic data. Cox proportional hazards regression was used to examine outcome-related variables. We also tested a new predictive model that includes the Sequential Organ Failure Assessment (SOFA) score and compared it with the SOFA-only model. Results An older age, higher SOFA score, and higher Acute Physiology III score were risk factors for the prognosis of AMI. The risk of further cardiovascular events was 1.54-fold higher in women than in men. Patients in the cardiac surgery intensive care unit had a better prognosis than those in the coronary heart disease intensive care unit. Pressurized drug use was a protective factor and the risk of further cardiovascular events was 1.36-fold higher in nonusers. Conclusion The prognosis of AMI is affected by age, the SOFA score, the Acute Physiology III score, sex, admission location, type of care unit, and vasopressin use. Our new predictive model for AMI has better performance than the SOFA model alone.

scholarly journals MicroRNA activation signature in patients with hemophagocytic lymphohistiocytosis and reversibility with disease-specific therapy

2016 ◽  
Vol 137 (1) ◽  
pp. 309-312 ◽  
Author(s):  
Janos Sumegi ◽  
Shawnagay Nestheide ◽  
Bruce Aronow ◽  
David Fletcher ◽  
Mehdi Keddache ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Specific Therapy ◽  
Disease Specific

scholarly journals Hemophagocytic Lymphohistiocytosis Complicating Erythroleukemia in a Child with Monosomy 7: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Samin Alavi ◽  
Maryam Ebadi ◽  
Alireza Jenabzadeh ◽  
M. T. Arzanian ◽  
Sh. Shamsian
Keyword(s):  
Bone Marrow ◽  
Case Report ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Chromosomal Abnormalities ◽  
Bone Marrow Aspiration ◽  
Review Of The Literature ◽  
Monosomy 7 ◽  
Persistent Fever ◽  
First Case ◽  
Erythroid Precursors

Herein, the first case of childhood erythrophagocytosis following chemotherapy for erythroleukemia in a child with monosomy 7 is reported. A 5-year-old boy presented with anemia, thrombocytopenia, and hepatosplenomegaly in whom erythroleukemia was diagnosed. Prolonged pancytopenia accompanied by persistent fever and huge splenomegaly and hepatomegaly became evident after 2 courses of chemotherapy. On bone marrow aspiration, macrophages phagocytosing erythroid precursors were observed and the diagnosis of HLH was established; additionally, monosomy 7 was detected on bone marrow cytogenetic examination. In conclusion, monosomy 7 can lead to erythrophagocytosis associated with erythroid leukemia and should be considered among the chromosomal abnormalities contributing to the association.

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