Morbidity and mortality in a national cohort of pediatric patients with hemophagocytic lymphohistiocytosis.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 10062-10062
Author(s):  
Steven William Allen ◽  
Meghan McCormick ◽  
Ram Kalpatthi ◽  
Louis Rapkin
Keyword(s):  
Health Care ◽  
Median Time ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Morbidity And Mortality ◽  
Cell Transplant ◽  
Hematopoietic Stem ◽  
Number Of Patients ◽  
Initial Hospitalization

10062 Background: Despite significant advances in diagnosing and treating hemophagocytic lymphohistiocytosis (HLH) in the pediatric population, survival remains low and the extent of disease morbidity and health care utilization is poorly characterized. Thus, we sought to investigate morbidity, mortality, and the health care burden of HLH in a pediatric population. Methods: We queried the Pediatric Health Information System (PHIS) for children admitted with HLH and treated with etoposide and dexamethasone between 1/1/2004 – 9/30/2018. Collected data included demographics and clinical variables associated with morbidity and mortality. Results: A total of 493 patients (10.5 per 100,000 patients admitted) met inclusion criteria during the study period. The majority of patients (n = 284, 58%) were less than 5 years of age. A total of 331 patients were readmitted after the initial hospitalization, with 29% of readmissions complicated by infections. Median cost for initial hospitalization was $101,906 (IQR: $47,552 – $271,822). A significant number of patients required ICU care during both the initial admission (60%) and readmission (57%). Hematopoietic stem cell transplant (HSCT) was performed in 136 patients (28%) with a median time to HSCT of 126 days (IQR: 75-193 days). Overall mortality was 32% (n = 158), half occurring during the initial admission. There was a trend towards increased mortality in younger age groups. Median time to death during and after the initial admission was 45 days (IQR: 20-84 days) and 198 days (IQR: 108-368 days), respectively. Post-HSCT mortality rate was 35%. Conclusions: This represents the largest cohort of pediatric patients treated for HLH. Overall and post-HSCT mortality was consistent with prior publications. We observed significant morbidity and increased health care resource utilization in our cohort. These findings emphasize the need for novel therapeutic approaches to improve not only patient survival but also long-term quality of life. Planned future analysis of the PHIS data will be aimed at assessing treatment variability, morbidity and mortality depending on treatment, and risk factors associated with mortality in pediatric patients with HLH.

scholarly journals Morbidity, Mortality, and Healthcare Utilization in a National Cohort of Pediatric Patients with Hemophagocytic Lymphohistiocytosis Who Received Hematopoietic Stem Cell Transplant

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2183-2183
Author(s):  
Archana Ramgopal ◽  
Meghan McCormick ◽  
Ram Kalpatthi ◽  
Louis Rapkin ◽  
James Zullo ◽  
...  
Keyword(s):  
Stem Cell ◽  
Resource Utilization ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Hematopoietic Stem Cell Transplant ◽  
Pediatric Patients ◽  
Stem Cell Transplant ◽  
Cell Transplant ◽  
Hematopoietic Stem ◽  
Elapsed Time ◽  
Over Time

Background Hemophagocytic lymphohistiocytosis (HLH) is a severe life threatening hyper-inflammatory syndrome of abnormal immune activation and dysregulation if untreated. The 5-year probability of survival (pSu) obtained from HLH registries and treatment protocols HLH-94 and HLH-2004 ranges from 21%-64%, with improved 5-year pSu of up to 70% following hematopoietic stem cell transplant (HSCT) (Arico et al., Trottestam et al., Bergsten et al.). Despite significant advances in the management of HLH over time, survival remains low and the extent of disease morbidity and healthcare utilization is poorly characterized. In this study, we sought to investigate morbidity, mortality, and the healthcare burden in children and adolescents with HLH who underwent HSCT. Methods Using the Pediatric Health Information System (PHIS) database, we identified patients under the age of 21 years admitted between 01/01/2004 and 09/30/2018 with a primary or secondary ICD-9 or ICD-10 diagnosis codes for HLH, as well as concurrent medication charges for both dexamethasone and etoposide in the same encounter. We then identified the patients who underwent HSCT to further analyze them. We abstracted data on demographics, hospitalizations, HSCT related complications, mortality, resource utilization and costs. Results were summarized using descriptive statistics. Time to HSCT was calculated as elapsed time from the admission date of the initial encounter to the date of the encounter in which there was a procedure code for HSCT. Time to mortality event was calculated as elapsed time from the admission date of the initial encounter to the discharge date of the encounter in which mortality occurred. The PHIS database provides an encrypted patient medical record number; thus, we were able to follow patients over time. This allowed for a better visualization of the patient's hospitalizations trend over 14 years. Results A total of 493 patients met inclusion criteria for HLH during the study period from 52 children's hospitals. The majority of patients (n = 284, 58%) were less than 5 years of age. Of these, 136 patients (28%) underwent HSCT with 155 hospital encounters, including readmissions. The median age at the time HSCT was 2 years (IQR; 0-9 years) and there were 82 males (60%). The median time to HSCT was 126 days (IQR: 75-193 days) and the average length of stay for the initial HSCT hospitalization was 61.1 days. Median initial HSCT hospitalization cost was $463,630 (IQR; 230,795 - 558,533). ICU care was required for 71 (46%) of patients. Overall, 91 (67%) patients developed transplant-related complications, which included infections, sinusoidal obstruction syndrome or graft versus host disease (Table 1). Mortality after HSCT was 22% (n=30) with an increased mortality observed with advanced age at the time of HSCT (Figure 1). The median time to death after the initial HSCT admission was 65 days (IQR; 56-94 days). Conclusion This is a large in-patient cohort of pediatric patients with HLH who underwent HSCT in the US. We observed an improved overall mortality after HSCT in this population compared to previous studies. However, morbidity (particularly from infections) and heath care resource utilization remain high. This stresses the importance of novel therapeutic approaches to improve not only patient survival but also long-term quality of life. Planned future analysis of this database will be aimed at assessing treatment variability; morbidity and mortality by treatment regimen, time to HSCT, and HSCT preparative regimen; and risk factors associated with mortality in pediatric patients with HLH who do and do not undergo HSCT. Disclosures No relevant conflicts of interest to declare.

scholarly journals Comparison of Two Apheresis Instruments (Old vs. New) for Granulocyte Collection: A Single Institutional Experience

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5036-5036
Author(s):  
Fleur M Aung ◽  
Roland Bassett ◽  
Benjamin Lichtiger ◽  
Emil J. Freireich
Keyword(s):  
Platelet Count ◽  
Missing Values ◽  
Pediatric Patients ◽  
Collection Efficiency ◽  
Statistical Tests ◽  
Cell Transplant ◽  
Oncology Patients ◽  
Hematopoietic Stem ◽  
Number Of Patients ◽  
Peripheral Venous Access

Abstract Background: A retrospective analysis was performed to evaluate the collection efficiency of two apheresis devices for granulocytes using the Spectra Optia (TerumoBCT) vs. the Cobe Spectra (Cobe: TerumoBCT) which served as control. Study Design and Methods: A total of 410 granulocyte collections (GCs) were collected from volunteer healthy blood donors (<4%) and family and friends of severely neutropenic oncology patients requiring GCs from July 2014 to November 2015. All healthy male and females donors eligible to donate blood with peripheral venous access were allowed to donate granulocytes after first undergoing Platelet Apheresis with very few exceptions. This was performed to evaluate the donor's ability to undergo a lengthy apheresis procedure. All first time donors were mobilized with G-CSF (600/480 mcg) and/or Dexamethasone 8 mg 12 hours prior to the apheresis. Both instruments were primed with acid citrate-dextrose (ACD-A) and then changed to a coagulation buffer of a 500 mL bag of 6% hydroxyethyl starch (HES) to which 40 mL of trisodium citrate 46.7% was added. The maximum allowed time for the apheresis procedures on both devices was 3 hours. 186 consecutive GCs were performed using the Spectra Optia from March to November 2015 vs. 224 consecutive GCs using the Cobe Spectra device from July 2014 to March 2015 and served as control. The GCs were transfused to both adult and pediatric patients. GC products greater than 500 mL were divided into double/triple or quadruple units based on the total white count of the unit. Hematopoietic stem cell transplant and pediatric patients received irradiated GCs whereas leukemia patients received non-irradiated GCs. Statustical Method: All statistical analysis were performed using R version 3.3.0. The Wilcoxon rank-sum test was used to compare continuous variables between the Spectra Optia and Cobe Spectra. All statistical tests used a significance level of 5%. No adjustments for multiple donations were made. The table summarizes characteristics by group [ Cobe Spectra (COBE) vs. Spectra Optia (SPECTRA)]. The table presents for each variable, by group, the number of patients, the minimum ("Min") and maximum ("Max") values, the quartiles ("q1" and "q2"), the median, mean and the standard deviation ("SD"), along with the number of missing values ("#NA"), if any. For each parameter, there is also a p-value corresponding to a Wilcoxson rank-sum test. Results: From a total of 186 Spectra Optia granulocytapheresis we were able to process and obtain 433 GC units (21 single, 90 double, 72 triple and 4 quadruple units) as compared to the Cobe Spectra where 224 granulocytapheresis yielded 393 GC units (single 85, double 109 and 30 triple units). All of the split/unspilt GCs from both apheresis devices had a minimum wbc count > 1.0 x 10e10. We found the following parameters - total blood volume processed, totall WBC collected, run time, bag wbc, absolute neutrophil count, absolute lymphocyte count, granulocyte collection efficiency, post-stimulation platelet count, absolute platelet count, MPV and number of GCs split were significantly higher ( p = <0.0001) using the fully automated Spectra Optia while volume collected, hematocrit, bag platelets and platelet collection efficiency were significantly higher ( p=<0.0001) using the Cobe Spectra. The age of the Cobe Spectra patients were older on average (p=0.00015) (Table) The weight, height, pre- and post stimulation WBC and pre-stimulation platelet counts of the donors were of no significance. (Table) Conclusion: The ability to achieve higher granulocyte counts per liter of blood processed with a higher granulocyte CE using the fully automated Spectra Optia has allowed us to process and split Granulocyte Units leading to the availability of multiple units thus easing some of the Granulocyte shortages that our severely neutropenic oncology patients currently experience. ReferenceCancelas JA, Padmanabhan A, Le T et al. Spectra Optia granulocyte apheresis collections results in higher collection efficiency of viable, functional neutrophils in a randomized crossover, multicenter trial. Transfusion; 2015; 55: 751-55Leitner GC, Kolovratova K, Horvath M et al. Granulocyte collection using a novel apheresis system eases the procedure and provides concentrates of high quality. Transfusion 2015; 55: 991-5 Table Characteristics by Group Table. Characteristics by Group Disclosures No relevant conflicts of interest to declare.

scholarly journals 577. Incidence and Outcomes of Positive Outpatient Surveillance Blood Cultures in Hematopoietic Stem Cell Transplant (HSCT) Patients with Graft Versus Host Disease (GvHD) On High Dose ≥ 0.5 mg/kg/day (HD) and Low Dose < 0.5mg/kg/day (LD) Steroid Therapy

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S353-S354
Author(s):  
Sarah Perreault ◽  
Molly Schiffer ◽  
Jennifer Zhao ◽  
Dayna McManus ◽  
Francine Foss ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Central Line ◽  
Blood Cultures ◽  
High Dose ◽  
Cell Transplant ◽  
Coagulase Negative Staphylococci ◽  
Hematopoietic Stem ◽  
Surveillance Cultures ◽  
Number Of Patients ◽  
Clinically Significant

Abstract Background Treatment of GvHD with steroids increases the risk of infection in HSCT patients due to additive immunosuppression and may delay the diagnosis of infection due to lack of symptoms. Outpatient surveillance blood cultures in HSCT with GvHD being treated with HD steroids has demonstrated a blood culture positivity rate of 3.5%. Currently, the utility of surveillance cultures in patients receiving LD steroid therapy is unknown. Our practice includes weekly outpatient surveillance cultures for all GvHD patients treated with steroids regardless of the dose. The primary endpoint of this study was to assess the incidence of positive surveillance blood cultures in GvHD patients receiving HD or LD steroids. Secondary endpoints included number of patients treated, hospitalization, 30 day mortality due to infection, and organisms isolated. Methods This was a single-center, retrospective review of GvHD patients at Yale New Haven Hospital between January 2013 and May 2019. Patients were excluded if: lack of signs or symptoms of GvHD, treatment with steroids for any indication other than GvHD, and active GvHD without central line. Cultures from patients receiving antibiotics for concurrent infection were also excluded. Results A total of 71 patients met criteria with 901 blood cultures. On HD, eight patients (14%) had 12 positive cultures (4%), and on LD, 16 patients (25%) had 22 positive cultures (4%) (p=0.15). Treatment occurred in six patients (75%) with four (24%) requiring hospitalization on HD, and 12 patients (75%) with 10 (83%) requiring hospitalization on LD (p=0.45). The median duration of steroid therapy was 93 and 236 days with a median dose of steroids of 1mg/kg/day and 0.15mg/kg/day, respectively. The number of positive cultures/1000 steroid days was 1.2 on HD and 0.5 on LD (RR 2.2). 30 day mortality was only noted in one patient (8%) on LD. The most common organism in both groups was Coagulase-negative staphylococci with all six cultures on HD classified as contaminants and 6/10 cultures requiring treatment on LD. Conclusion Although the relative risk of positive surveillance blood cultures in HD patients compared to LD was twofold higher, there were clinically significant infections identified in the LD group. Disclosures All Authors: No reported disclosures

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