scholarly journals Lymphoproliferative malignancies in patients with neurofibromatosis 1

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Christina Bergqvist ◽  
François Hemery ◽  
Arnaud Jannic ◽  
Salah Ferkal ◽  
Pierre Wolkenstein
Keyword(s):  
General Population ◽  
Medical History ◽  
Hodgkin Lymphoma ◽  
Incidence Rate ◽  
Population Based ◽  
Neurofibromatosis 1 ◽  
Population Based Study ◽  
Non Hodgkin Lymphoma ◽  
History Of

AbstractNeurofibromatosis 1 (NF1) is an inherited, autosomal-dominant, tumor predisposition syndrome with a birth incidence as high as 1:2000. A patient with NF1 is four to five times more likely to develop a malignancy as compared to the general population. The number of epidemiologic studies on lymphoproliferative malignancies in patients with NF1 is limited. The aim of this study was to determine the incidence rate of lymphoproliferative malignancies (lymphoma and leukemia) in NF1 patients followed in our referral center for neurofibromatoses. We used the Informatics for Integrated Biology and the Bedside (i2b2) platform to extract information from the hospital’s electronic health records. We performed a keyword search on clinical notes generated between Jan/01/2014 and May/11/2020 for patients aged 18 years or older. A total of 1507 patients with confirmed NF1 patients aged 18 years and above were identified (mean age 39.2 years; 57% women). The total number of person-years in follow-up was 57,736 (men, 24,327 years; women, 33,409 years). Mean length of follow-up was 38.3 years (median, 36 years). A total of 13 patients had a medical history of either lymphoma or leukemia, yielding an overall incidence rate of 22.5 per 100,000 (0.000225, 95% confidence interval (CI) 0.000223–0.000227). This incidence is similar to that of the general population in France (standardized incidence ratio 1.07, 95% CI 0.60–1.79). Four patients had a medical history leukemia and 9 patients had a medical history of lymphoma of which 7 had non-Hodgkin lymphoma, and 2 had Hodgkin lymphoma. Our results show that adults with NF1 do not have an increased tendency to develop lymphoproliferative malignancies, in contrast to the general increased risk of malignancy. While our results are consistent with the recent population-based study in Finland, they are in contrast with the larger population-based study in England whereby NF1 individuals were found to be 3 times more likely to develop both non-Hodgkin lymphoma and lymphocytic leukemia. Large-scale epidemiological studies based on nationwide data sets are thus needed to confirm our findings.

scholarly journals Risk of Seizures in Patients with Organophosphate Poisoning: A Nationwide Population-Based Study

2019 ◽  
Vol 16 (17) ◽  
pp. 3147 ◽  
Author(s):  
Chieh-Sen Chuang ◽  
Kai-Wei Yang ◽  
Chia-Ming Yen ◽  
Cheng-Li Lin ◽  
Chia-Hung Kao
Keyword(s):  
General Population ◽  
Cox Regression ◽  
Population Based ◽  
Organophosphate Poisoning ◽  
Research Database ◽  
Population Based Study ◽  
Increased Risk ◽  
Taiwan National Health Insurance ◽  
History Of

Objective: Previous research has demonstrated that patients with a history of organophosphate poisoning tend to have a higher risk of neurological disorder. However, research on the rate of seizure development in patients after organophosphate poisoning is lacking. This study examined whether individuals with organophosphate poisoning have an increased risk of seizures through several years of follow-up. Patients and Methods: We conducted a retrospective study on a cohort of 45,060 individuals (9012 patients with a history of organophosphate poisoning and 36,048 controls) selected from the Taiwan National Health Insurance Research Database. The individuals were observed for a maximum of 12 years to determine the rate of new-onset seizure disorder. We selected a comparison cohort from the general population that was randomly frequency-matched by age, sex, and index year and further analyzed the risk of seizures using a Cox regression model adjusted for sex, age, and comorbidities. Results: During the study period, the risk of seizure development was 3.57 times greater in patients with organophosphate poisoning compared with individuals without, after adjustments for age, sex, and comorbidities. The absolute incidence of seizures was highest in individuals aged 20 to 34 years in both cohorts (adjusted hazard ratio = 13.0, 95% confidence interval = 5.40−31.4). A significantly higher seizure risk was also observed in patients with organophosphate poisoning and comorbidities other than cirrhosis. Conclusions: This nationwide retrospective cohort study demonstrates that seizure risk is significantly increased in patients with organophosphate poisoning compared with the general population.

scholarly journals Differential cerebrovascular risks in glioblastoma and meningioma patients: a population-based matched cohort study in Wales (United Kingdom)

2021 ◽  
Vol 23 (Supplement_4) ◽  
pp. iv12-iv12
Author(s):  
Michael T C Poon ◽  
Kai Jin ◽  
Paul M Brennan ◽  
Jonine Figueroa ◽  
Cathie Sudlow
Keyword(s):  
Cohort Study ◽  
General Population ◽  
Ischaemic Stroke ◽  
Population Based ◽  
Parametric Models ◽  
Matched Cohort ◽  
Matched Cohort Study ◽  
Hazard Ratios ◽  
History Of

Abstract Aims There is limited evidence on cerebrovascular risks in glioblastoma and meningioma patients. We aimed to compare cerebrovascular risks of these patients with the general population. Method We used population-based routine healthcare and administrative data linkage in this matched cohort study. Cases were adult glioblastoma and meningioma patients diagnosed in Wales 2000-2014 identified in the cancer registry. Controls from cancer-free general population were matched to cases (5:1 ratio) on age (±5 years), sex and GP practice. Factors included in multivariable models were age, sex, index of multiple deprivation, hypertension, diabetes, high cholesterol, history of cardiovascular disease, and medications for cardiovascular diseases. Outcomes were fatal and non-fatal haemorrhagic and ischaemic stroke. We used flexible parametric models adjusting for confounders to calculate the hazard ratios (HR). Results Final analytic population was 16,921 participants, of which 1,340 had glioblastoma and 1,498 had meningioma. The median follow-up time was 0.5 year for glioblastoma patients, 4.9 years for meningioma patients, and 6.6 years for controls. The number of haemorrhage and ischaemic stroke was 154 and 374 in the glioblastoma matched cohort, respectively, and 180 and 569 in the meningioma matched cohort, respectively. The adjusted HRs for haemorrhagic and ischaemic stroke were 3.74 (95%CI 1.87-6.57) and 5.62 (95%CI 2.56-10.42) in glioblastoma patients, respectively, and were 2.42 (95%CI 1.58-3.52) and 1.86 (95%CI 1.54-2.23) in meningioma patients compared with their controls. Conclusion Glioblastoma and meningioma patients had higher cerebrovascular risks; these risks were even higher for glioblastoma patients. Further assessment of these potentially modifiable risks may improve survivorship.

Abstract WMP102: Prevalence and Natural History of Superficial Siderosis: A Population-based Study

Stroke ◽  
2017 ◽  
Vol 48 (suppl_1) ◽  
Author(s):  
Michael Pichler ◽  
Kelly Flemming ◽  
Alejandro Rabinstein ◽  
Robert Brown ◽  
Kejal Kantarci ◽  
...  
Keyword(s):  
Population Based ◽  
Superficial Siderosis ◽  
Mri Findings ◽  
Population Based Study ◽  
Mri Scans ◽  
History Of ◽  
Lobar Hemorrhage ◽  
General Elderly Population ◽  
Cortical Superficial Siderosis

Introduction: Cortical superficial siderosis (cSS) refers to deposition of blood breakdown products along the cerebral cortex, causing characteristic staining patterns seen with iron-sensitive MRI techniques. Cortical superficial siderosis is a relatively rare disorder, but has been linked to cerebral amyloid angiopathy and Alzheimer’s disease. The objective of this study was to determine the frequency and natural history of cSS in the general elderly population. Methods: MRI scans from the Mayo Clinic Study of Aging (MCSA), an ongoing population-based study of elderly residents in Olmsted County, Minnesota, were reviewed by neuroradiologists. Participants with cSS were identified based on linear pattern of hypointensity on gradient recalled echo imaging consistent with cSS. Exclusion criteria were: 1) MRI findings not consistent with cSS or 2) alternative explanation for MRI findings (such as aneurysmal subarachnoid hemorrhage, intracranial surgery, or trauma). Additional data abstracted included extent of cSS, presence of cerebral microbleeds, and clinical outcome. Results: Eleven out of 1,441 participants had MRI scans showing cSS (0.8%). When stratified by age, the frequency was 0.4% in those 50 to 70 years old and 1.1% in those over 70 years old. Six participants had only focal involvement of cSS (restricted to three or fewer sulci) and five had disseminated involvement (affecting more than three sulci). Microbleeds were seen in four of five (80%) participants with disseminated cSS, but none with focal cSS. Five participants (2 focal, 3 disseminated cSS) had follow up MRI scans, with an average follow up of 25 months. There was no further hemorrhage in those with focal cSS. However, all three participants with disseminated cSS experienced additional hemorrhage: one with new microbleeds, one with new microbleeds and lobar hemorrhage, and one with sulcal subarachnoid hemorrhage and lobar hemorrhage. Conclusion: Although rare, cSS may be encountered in the general elderly population. Extent of involvement of cSS and concomitant microbleeds may be important risk factors for progression of disease and intracerebral hemorrhage. The clinical significance of focal cSS occurring in the absence of microbleeds requires further investigation.

scholarly journals Questions on the Bidirectional Relationship Between Primary Sjögren Syndrome and Non-Hodgkin Lymphoma

2020 ◽  
pp. jrheum.201352
Author(s):  
Yun-Tzu Liang ◽  
Pui-Ying Leong ◽  
James Cheng-Chung Wei
Keyword(s):  
Hodgkin Lymphoma ◽  
Population Based ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Population Based Study ◽  
Bilateral Relationship ◽  
Non Hodgkin Lymphoma ◽  
Primary Sjögren Syndrome ◽  
Bidirectional Relationship

We have read with great interest in the article by Wang, et al on the higher incidence of non-Hodgkin lymphoma (NHL) in patients with primary Sjögren syndrome (pSS) and the higher incidence of pSS in patients with NHL1. Thank you for the discovery of the bilateral relationship between pSS and NHL in this nationwide population-based study1.

scholarly journals Spectrum of Second Primary Malignancies in Pediatric and Adult Langerhans Cell Histiocytosis Cases

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 51-52 ◽  
Author(s):  
Richa Parikh ◽  
Ronald S. Go ◽  
Gaurav Goyal
Keyword(s):  
General Population ◽  
Hodgkin Lymphoma ◽  
Solid Tumors ◽  
Adult Population ◽  
Hematologic Malignancies ◽  
Population Based ◽  
Second Primary ◽  
Non Hodgkin Lymphoma ◽  
Increased Risk ◽  
Second Primary Malignancies

Introduction: Langerhans cell histiocytosis (LCH) is a rare MAPK-ERK pathway driven histiocytic neoplasm that occurs in pediatric as well as adult population. Despite improvement in clinical outcomes, there is some data to suggest an increased propensity to develop second primary malignancies (SPMs) in LCH patients. However, population-based studies analyzing the incidence and spectrum of SPMs in pediatric and adult LCH patients are lacking. In this study, we utilized the Surveillance, Epidemiology and End Results (SEER) database to examine the various SPMs occurring among pediatric and adult LCH cases. Methods: We used the November 2018 submission of the SEER 18 registry, which covers ~27.8% of the US population based on the 2010 census, as our database. We used the SEER*Stat version 8.3.6 statistical software to analyze data. We identified cases diagnosed with LCH as their first primary malignancy between 2000 and 2016 using International Classification of Diseases for Oncology edition 3 (ICD-O-3) codes, including LCH NOS (not otherwise specified) (9751/1), LCH (9751/3), LCH, unifocal (9752/1), LCH, multifocal (9753/3), LCH, disseminated, borderline (9754/1) and Disseminated LCH (9754/3). These cases were followed for 180+months, and the standardized incidence ratio (SIR) or relative risk and absolute excess risk (AER) were calculated. We examined the differences in occurrence of SPMs among the pediatric (Age <18 years) and adult population (Age ≥18 years). Additionally, we evaluated the concurrent and prior cancers in LCH patients as an exploratory objective. Results: The study included 1392 cases with LCH (Table 1), with median age at diagnosis 8 years (range newborn - 86 years). Out of these cases, 1205 (87%) were diagnosed as LCH and 186 (13%) as disseminated LCH. 936 cases (67%) were diagnosed at age <18 years (pediatric LCH), while 456 cases (33%) were diagnosed at age ≥18 years (adult LCH). The overall age-adjusted incidence rate for LCH was found to be 1 per 1,000,000. The incidence rate was 2.6 per 1,000,000 in pediatric LCH group and 0.4 per 1,000,000 in the adult LCH group. Out of the entire cohort, 20 (1.4%) cases developed a total of 21 SPMs [SIR 2.07; 95% Confidence Interval (CI): 1.28-3.16]. Median latency period to development of SPMs was 28 months. The pediatric LCH group had an overall higher risk of developing SPMs [SIR 6.42, 95%CI 2.08-14.97] than the general population, especially for hematologic malignancies [SIR 18.76, 95%CI 6.09-43.78], mainly, nodal Hodgkin lymphoma [SIR 60.93, 95%CI 7.38-220.12] and extranodal non-Hodgkin lymphoma [SIR 60.88, 95%CI 1.54-339.2]. No solid tumors were seen in this group. The adult LCH group did not have an overall increased risk of developing SPMs than the general population [SIR 1.71, 95%CI 0.98-2.77], except for Acute Lymphocytic Leukemia (ALL) [SIR 66.29, 95%CI 1.68-369.36] especially 60-119 months from diagnosis of LCH and miscellaneous cancers [SIR 11.43, 95%CI 2.36-33.39] especially 12-59 months after diagnosis of LCH. 62.5% of SPMs that developed in the adult LCH group were solid tumors, however, the overall risk for developing solid tumors was not higher than the general population [SIR 1.2, 95%CI 0.58-2.2], except for carcinoma in-situ of vulva [SIR 62.72, 95%CI 1.59-349.45] 2-11 months from diagnosis of LCH. Overall, tumors of the respiratory system (21%), female breast (13%) and prostate (9%) were the most common malignancies occurring prior to development of LCH whereas tumors of the respiratory system (28%), non-Hodgkin lymphoma (20%) and endocrine system (13%) occurred concurrent to LCH. Conclusion: To our knowledge, this is the first comprehensive population-based study assessing the incidence of SPMs in pediatric and adult LCH. Our study shows that the incidence of LCH is higher in the pediatric age group compared to adults. We found an increased risk for hematologic malignancies, specifically for Hodgkin and non-Hodgkin lymphoma in pediatric LCH compared to the general population. Among adult LCH, however, the risk was higher for development of ALL and carcinoma in-situ of vulva when compared to the general population. Our results may help guide survivorship and surveillance strategies among LCH patients. More studies are needed to understand the molecular underpinning leading to increased SPM formation in LCH patients. Disclosures No relevant conflicts of interest to declare.

scholarly journals Longitudinal risk of herpes zoster in patients with non-Hodgkin lymphoma receiving chemotherapy: A nationwide population-based study

2015 ◽  
Vol 5 (1) ◽  
Author(s):  
Shih-Feng Cho ◽  
Wan-Hsuan Wu ◽  
Yi-Hsin Yang ◽  
Yi-Chang Liu ◽  
Hui-Hua Hsiao ◽  
...  
Keyword(s):  
Herpes Zoster ◽  
Hodgkin Lymphoma ◽  
Population Based ◽  
Population Based Study ◽  
Non Hodgkin Lymphoma
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