scholarly journals Thrombotic Microangiopathy: Multi-Institutional Review of Pediatric Patients Who Underwent HSCT

Author(s):  
Archana Ramgopal ◽  
Shiva Sridhar ◽  
Jignesh Dalal ◽  
Ram Kalpatthi

Thrombotic microangiopathy (TMA) is a rare but serious complication of hematopoietic stem cell transplantation (HSCT). The purpose of our study is to estimate the incidence and risk factors of TMA in 93 out of a total of 12369 children (0.8%) receiving HSCT. HHV6 infection was an independent risk factor associated with increased mortality in patients with TMA (Hazard Ratio: 2.86 [1.01, 8.39], p=0.05), and our study conducts a review of the literature with the association of HHV-6 and complement activation. Studies exploring the pathophysiology of TMA and its relationship to HSCT are needed to optimize the outcome of pediatric patients.

2021 ◽  
Vol 11 (6) ◽  
pp. 467
Author(s):  
Archana Ramgopal ◽  
Shiva Sridar ◽  
Jignesh Dalal ◽  
Ramasubramanian Kalpatthi

Thrombotic microangiopathy (TMA) is a rare but serious complication of hematopoietic stem cell transplantation (HSCT). The purpose of our study is to estimate the incidence, prevalence, and analyze the risk factors and outcome of TMA in children receiving HSCT. Patients under the age of 21 who underwent HSCT at one of the 42 Pediatric Health Information System (PHIS) hospitals from 2000–2012 were analyzed, including demographics, hospitalizations, TMA, and other HSCT-related complications. From 2000 to 2012, a total of 12,369 unique pediatric patients who received HSCT were identified. Among these, 93 (0.8%) children were identified to have the diagnosis of TMA. TMA was significantly associated with allogeneic HSCT, peripheral blood stem cell trasnplants (PBSCT), cytomegalovirus (CMV), human herpes virus 6 (HHV6), fungal infection, graft-versus-host disease (GVHD), and veno-occlusive disease (VOD) (p = 0.01). Multivariate logistic regression analysis of mortality showed only HHV6 was an independent risk factor associated with increased mortality in patients with TMA (hazard ratio: 2.86 [1.01, 8.39], p = 0.05). The prevalence of TMA in our study is 0.8% with a mortality in our pediatric TMA cohort of 30%, which is in contrast to the higher mortality reported in previously published, small-case series. HHV6 emerged as not only a risk factor for TMA but also as associated with increased mortality in these patients.


2019 ◽  
Vol 51 (1) ◽  
pp. 74-79 ◽  
Author(s):  
Rong Yang ◽  
Runan Zhang ◽  
Yanyue Zhang ◽  
Yaping Huang ◽  
Hanying Liang ◽  
...  

ABSTRACT Objective To assess the rate of, and risk factors for, human cytomegalovirus viremia (HCMV) in donor+/recipient+ (HCMV serostatus matched) hematopoietic stem-cell transplantation (HSCT) recipients. Methods HCMV DNA from 144 donor+/recipient+ HSCT recipients was examined by quantitative polymerase chain reaction (qPCR). Results The cumulative incidence of HCMV viremia was 69.4% (100/144) during the 48 weeks after HSCT. In a multivariate analysis, acute graft-versus-host disease (aGVHD) was discovered to be a risk factor for the occurrence of HCMV viremia (P = .006). The cumulative incidence of HCMV viremia and increasing DNA loads were significantly associated with aGVHD occurrence (P = .001 for each). The occurrence of late-term HCMV viremia was associated with aGVHD (P = .001) and a higher DNA load during the first 12 weeks after HSCT (P = .04). Conclusions aGVHD is a risk factor for HCMV viremia. Recipients with aGVHD who have a high HCMV DNA load should be strictly monitored to prevent HCMV activation.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4870-4870
Author(s):  
Wei Zhao ◽  
Man Chen ◽  
Yanli Zhao ◽  
Hui Wang ◽  
Peihua Lu ◽  
...  

Abstract Background: Air-leak syndrome (ALS) occurs when there is leakage of gas from the alveoli, which results in clinical symptoms including cough and sputum, dyspnea, and hypoxemia. ALS is rare but potentially life-threatening in patients who have received an allogeneic hematopoietic stem cell transplantation (allo-HSCT) and is an independent prognosis factor of patients undergoing HSCT. However, the clinical features and risk factors for post-transplant ALS among pediatric patients have rarely been explored. Aims: This study analyzed the clinical characteristics, risk factors, clinical treatment options and prognosis to provide a scientific basis for ALS prevention and treatment for pediatric patients. Methods: We retrospectively reviewed patients who were diagnosed with ALS following allo-HSCT between January 2013 and December 2019 at the Hebei Yanda Lu Daopei Hospital, and analyzed the role of ALS in the prognosis of the HSCT. Results: A total of 2026 pediatric patients received an HSCT between January 2013 and December 2019. The ALS incidence rate was 1.4% (28 of 2,206 patients) with a survival rate of 64.3% (10 of 28 patients). The median overall survival (OS) time was 429 days (range: 55-1614 days). Sixteen patients were males and 12 were female. The median patients age was 12 years old (range: 1-16 years), and the median follow-up time was 871 days (range: 55-2973 days).We divided ALS into two categories: 15 cases of bronchiolitis obliterans syndrome (BOS) and 13 cases of idiopathic pneumonia syndrome (IPS). There was a significant difference in OS between the twogroups (80% among BOS patients versus 46% among IPS patients; P=0.037). Logical regression analysis showed that gender, an unmatched transplant donor and recipients (P=0.049), time to first occurrence of graft-versus-host disease after transplantation (P=0.021), and dosage of methylprednisolone >0.5mg/kg at the onset of ALS (P=0.049), were independent risk factors for poor prognosis in ALS. In addition, we found fluticasone, azithromycin, and montelukast (FAM) could significantly improve the prognosis following ALS (P=0.005). Compared with IPS, our results showed that some patients with BOS may benefit from imatinib (P=0.055), ruxolitinib (P=0.009), or pirfenidone (P=0.044). Conclusion: ALS is a rare manifestation of pulmonary complications following HSCT among pediatric patients. Our analysis demonstrates that early diagnosis and FAM treatment may improve the survival rate of ALS following HSCT among pediatric patients. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.


2021 ◽  
Author(s):  
Jaspar Kloehn ◽  
Grit Brodt ◽  
Jana Ernst ◽  
Bernd Gruhn

Abstract Purpose Hepatic sinusoidal obstruction syndrome (SOS), which is also known as veno-occlusive disease of the liver, represents a serious complication following hematopoietic stem cell transplantation (HSCT). Our study aimed to investigate important risk factors for SOS in a pediatric population. Methods This retrospective study analyzed 105 children who underwent allogeneic HSCT at our pediatric HSCT center in Jena. The observation period was 12 years and SOS was defined by the modified pediatric Seattle criteria up to day +30 after HSCT. Results 15 out of all 105 patients developed SOS (14.3%). The median time from HSCT to SOS diagnosis was 12 days. The mortality rate of SOS was only 20.0%. In univariate analysis, we identified the significant risk factors of a patient age < 1 year (odds ratio (OR) = 7.25, p = 0.037) and a prior treatment with gemtuzumab ozogamicin (OR = 11.00, p = 0.020). In addition, some laboratory values, which were taken before HSCT, had a significant association to SOS. Ferritin > 1500 ng/mL (OR = 4.00, p = 0.033), ferritin > 2000 ng/mL (OR = 4.69, p = 0.016), ferritin > 2400 ng/mL (OR = 5.29, p = 0.005) and the international normalized ratio (INR) ≥ 1.3 (OR = 5.91, p = 0.009) showed significant results in univariate analysis. The following risk factors could be confirmed in multivariate analysis: prior treatment with gemtuzumab ozogamicin (OR = 9.24, p = 0.048), ferritin > 2400 ng/mL (OR = 5.74, p = 0.023) and INR ≥ 1.3 (OR = 8.02, p = 0.007).Conclusion Our study confirms several risk factors for hepatic SOS following allogeneic HSCT in pediatric patients. In addition, we report for the first time a significant association between high INR before HSCT and hepatic SOS, which consequently could improve the SOS risk evaluation.


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