Posterior Reversible Encephalopathy Syndrome after Allogeneic Stem Cell Transplantation in Pediatric Patients with Fanconi Anemia, a Prospective Study

Behfar, Maryam; Babaei, Mohammad; Radmard, Amir Reza; Kooraki, Soheil; Farajifard, Hamid; Naji, Parisa; Taebi, Sahar; Hamidieh, Amir Ali

doi:10.1016/j.bbmt.2020.08.021

Cited by 7 publications

(14 citation statements)

References 36 publications

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“…Furthermore, the secondary malignancies and endocrine deficiencies are the late ones. [20][21][22] The allo-HSCT responses vary due to individual diversity and Our data suggest that there are not any statistical differences in aGvHD and cGvHD incidence, considering the numbers of nonhematological congenital malformations (p = 0.27 and 0.9, respectively). There are not any differences with each of the malformations, as well.…”

Section: Discussionmentioning

confidence: 64%

“…Oral mucositis, hemorrhagic cystitis, thrombotic thrombocytopenic purpura, and central nervous system complications such as posterior reversible encephalopathy syndrome are the early allo‐HSCT complications due to the nature of FA. Furthermore, the secondary malignancies and endocrine deficiencies are the late ones 20–22 …”

Section: Discussionmentioning

confidence: 99%

“…Fanconi anemia (FA) is a rare inherited disease caused by DNA repair pathway dysfunction leading to apoptosis and bone marrow failure (BMF). Based on the molecular genetic analysis, 22 different genes with various mutations types identified heretofore which are responsible for FA pathology, including FANC family A to W. FA is not only heterogeneous in genotypes but also phenotypes. It associates with various congenital malformations, progressive BMF, and nonhematological and hematological malignancies.…”

Section: Introductionmentioning

confidence: 99%

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Fanconi anemia phenotypic and transplant outcomes' associations in Iranian patients

Ansari

Behfar

Naji

et al. 2023

Health Science Reports

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Objectives: Fanconi anemia (FA) is a rare, heterogeneous, inherited disorder.Allogeneic hematopoietic stem cell transplantation (HSCT) represents the only therapeutic option to restore normal hematopoiesis. This study reports the outcomes of FA-HSCT patients and identifies factors, including clinical phenotype.Our team examined more than 95% of Iranian FA patients during the last decade. Study Design: One hundred and six FA patients (age range: 2-41) who underwent HSCT from March 2007 to February 2018 were enrolled. Clinical characteristics of genetic disease, pre-HSCT findings, HSCT indication, and long-term follow-up evaluated and recorded. Data were analyzed using SPSS 19.0.Results: The mean follow-up period for survivors was 36 months (range, 1-101). The 3-year overall survival (OS) and disease-free survival were 72.2% and 71.2%, respectively. The 3-year OS rate for patients with limited and extensive malformations was 78.8% and 56.6%, respectively (p = 0.025). Acute graft versus host disease incidence was 60.52% for patients with limited malformations versus 70% for patients with extensive ones (p = 0.49). Chronic graft versus host disease incidence for these two groups was 9.21% and 10%, respectively (p = 0.91).Conclusions: OS was not associated with each of the malformations singly; however, it was lower in the extensive group. The younger age of patients at the HSCT time leads to a higher OS. The differences in FA patients' outcomes and the various genotypes were probably related. These data provide a powerful tool for further studies on genotype-phenotype association with HSCT results.

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Fanconi anemia phenotypic and transplant outcomes' associations in Iranian patients

Ansari

Behfar

Naji

et al. 2023

Health Science Reports

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“…Most MRI changes resolve, but persistent imaging abnormalities and epilepsy can develop ( 44 ). We previously demonstrated that female sex, age >10 years old, acute GVHD, hypertension, immunodeficiency, SCD, T cell leukemia, and CNS leukemia/involvement are linked to poor outcome in children with oncologic/hematologic diseases and PRES ( 10 ); in this population, the main causes of death are underlying diseases, severe infection, MODS, respiratory failure, GVHD, and severe organ toxicity ( 15 , 18 , 25 – 32 ), although in some cases mortality was a direct result of PRES ( 12 , 18 , 24 , 28 , 30 ). In our systematic review, only 6.8% of the deaths were attributable to PRES, and the mortality rate was higher following HSCT than chemotherapy (25.3 vs. 7.7%), indicating that the latter is a safer treatment option for pediatric patients with oncologic/hematologic diseases who develop PRES.…”

Section: Discussionmentioning

confidence: 99%

“…However, PRES has recently been reported in single-or multi-center studies of pediatric oncologic/hematologic diseases such as leukemia, lymphoma, solid tumors, and non-malignant disease after chemotherapy and HSCT, with high morbidity and mortality rates ranging from 2.4 to 22.6% (11,12,15,18,22,(24)(25)(26)(27)(28). In patients with oncologic/hematologic diseases, the main causes of death were underlying diseases, severe infection, multiple organ dysfunction syndrome (MODS), respiratory failure, graft-vs.-host disease (GVHD), and severe organ toxicity (15,18,(25)(26)(27)(28)(29)(30)(31)(32); and several studies found that the deaths were directly attributable to PRES (12,18,24,28,30).…”

Section: Introductionmentioning

confidence: 99%

Management and Clinical Outcome of Posterior Reversible Encephalopathy Syndrome in Pediatric Oncologic/Hematologic Diseases: A PRES Subgroup Analysis With a Large Sample Size

et al. 2021

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This study investigated the management and clinical outcomes along with associated factors of posterior reversible encephalopathy syndrome (PRES) in childhood hematologic/oncologic diseases. We present data from children with hematologic/oncologic diseases who developed PRES after treatment of the primary disease with chemotherapy and hematopoietic stem cell transplantation (HSCT) at 3 medical centers in Changsha, China from 2015 to 2020, and review all previously reported cases with the aim of determining whether this neurologic manifestation affects the disease prognosis. In the clinical cohort of 58 PRES patients, hypertension [pooled odds ratio (OR) = 4.941, 95% confidence interval (CI): 1.390, 17.570; P = 0.001] and blood transfusion (OR = 14.259, 95% CI: 3.273, 62.131; P = 0.001) were significantly associated with PRES. Elevated platelet (OR = 0.988, 95% CI: 0.982, 0.995; P < 0.001), hemoglobin (OR = 0.924, 95% CI: 0.890, 0.995; P < 0.001), and blood sodium (OR = 0.905, 95% CI: 0.860, 0.953; P < 0.001), potassium (OR = 0.599, 95% CI: 0.360, 0.995; P = 0.048), and magnesium (OR = 0.093, 95% CI: 0.016, 0.539; P = 0.008) were protective factors against PRES. Data for 440 pediatric PRES patients with hematologic/oncologic diseases in 21 articles retrieved from PubMed, Web of Science, and Embase databases and the 20 PRES patients from our study were analyzed. The median age at presentation was 7.9 years. The most common primary diagnosis was leukemia (62.3%), followed by solid tumor (7.7%) and lymphoma (7.5%). Most patients (65.0%) received chemotherapy, including non-induction (55.2%) and induction (44.8%) regimens; and 86.5% used corticosteroids before the onset of PRES. Although 21.0% of patients died during follow-up, in most cases (93.2%) this was not attributable to PRES but to severe infection (27.3%), underlying disease (26.1%), graft-vs.-host disease (14.8%), multiple organ dysfunction syndrome (8.0%), and respiratory failure (3.4%). PRES was more common with HSCT compared to chemotherapy and had a nearly 2 times higher mortality rate in patients with oncologic/hematologic diseases than in those with other types of disease. Monitoring neurologic signs and symptoms in the former group is therefore critical for ensuring good clinical outcomes following treatment of the primary malignancy.

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Posterior Reversible Encephalopathy Syndrome after Allogeneic Stem Cell Transplantation in Pediatric Patients with Fanconi Anemia, a Prospective Study

Cited by 7 publications

References 36 publications

Fanconi anemia phenotypic and transplant outcomes' associations in Iranian patients

Fanconi anemia phenotypic and transplant outcomes' associations in Iranian patients

Management and Clinical Outcome of Posterior Reversible Encephalopathy Syndrome in Pediatric Oncologic/Hematologic Diseases: A PRES Subgroup Analysis With a Large Sample Size

Diazepam/immunosuppressants/phenytoin

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