Dataset Information

Sporadic Burkitt lymphomas of children and adolescents in Chinese: a clinicopathological study of 43 cases.

ABSTRACT:

Background

To investigate the clinical and pathologic features as well as the MYC translocations of childhood Burkitt lymphoma (BL) from China.

Methods

Fourty-three cases of childhood BL were retrospectively investigated in morphology, immunophenotype, genotype, treatments and survival analysis.

Results

Clinically, there was a marked male predominance in sex distribution (M: F=9.75:1); abdomen was the most frequent extranodal sites of involvement (46.5%), followed by jaws and facial bones (16.3%). Two third of the patients were in stageI ~ II. Morphologically, 69.76% of the cases showed classical histologic features, while 30.24% of them showed greater nuclear pleomorphism in size and shape. Five cases (11.6%) were positive for EBER1/2. Thirty-one of the 40 cases (77.5%) had the aberration of IGH/MYC translocation while 7 (17.5%) had non-IGH/MYC translocation. Thirty patients (69.7%) received operation and/or chemotherapy while 13 patients (30.3%) received no treatment. Twenty-seven patients (62.8%) died of the tumor, 16 alive, with the average survival time 4.9 and 48.7 months respectively. High IPI, advanced clinical stage, increased serum level of LDH and no chemotherapy received as well as tumor size≥10 cm were related to the lower survival rates of the tumor.

Conclusions

Several differences were showed in this group of BL, including a much higher ratio of male patients, more cases in stageII, clinically inconsistent treatment and a very poor outcome.

Virtual slides

The virtual slide(s) for this article can be found here http://www.diagnosticpathology.diagnomx.eu/vs/1552295877710135.

SUBMITTER: Bi CF

PROVIDER: S-EPMC3414834 | biostudies-literature | 2012 Jun

REPOSITORIES: biostudies-literature

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Publications

Sporadic Burkitt lymphomas of children and adolescents in Chinese: a clinicopathological study of 43 cases.

Bi Cheng-Feng CF Tang Yuan Y Zhang Wen-Yan WY Zhao Sha S Wang Xiao-Qing XQ Yang Qun-Pei QP Li Gan-Di GD Liu Wei-ping WP

Diagnostic pathology 20120622

<h4>Background</h4>To investigate the clinical and pathologic features as well as the MYC translocations of childhood Burkitt lymphoma (BL) from China.<h4>Methods</h4>Fourty-three cases of childhood BL were retrospectively investigated in morphology, immunophenotype, genotype, treatments and survival analysis.<h4>Results</h4>Clinically, there was a marked male predominance in sex distribution (M: F=9.75:1); abdomen was the most frequent extranodal sites of involvement (46.5%), followed by jaws a ...[more]

PMID: 22726497

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Project description:BackgroundFamilial hypercholesterolemia (FH) is an inherited disorder mainly marked by increased low-density lipoprotein cholesterol (LDL-C) concentrations and a heightened risk of early-onset arteriosclerotic cardiovascular disease (ASCVD). This study seeks to characterize the genetic spectrum and genotype‒phenotype correlations of FH in Chinese pediatric individuals.MethodsData were gathered from individuals diagnosed with FH either clinically or genetically at multiple hospitals across mainland China from January 2016 to June 2024.ResultsIn total, 140 children and adolescents (mean age of 6.00 years) with clinically and genetically diagnosed FH were enrolled in the study, with 87 distinct variants identified in the LDLR, APOB and PCSK9 genes. Among the variants, 11 variants were newly identified worldwide, with 9 classified as "pathogenic" or "likely pathogenic", and 2 classified as "variants of uncertain significance". Additionally, the 5 most common variants in the study were c.1448G > A (p.W483*), c.1879G > A (p.A627T), c.1216C > A (p.R406R), and c.1747C > T (p.H583Y) in the LDLR gene, as well as c.10579C > T (p.R3527W) in the APOB gene, accounting for 49.29% (69/140) of all patients. These variants are primarily observed in the Asian or Chinese population and are distinct from those present in Caucasian groups. In this cohort, 105 patients were diagnosed with heterozygous FH (HeFH), while 35 were diagnosed with homozygous FH (HoFH). Finally, only 28.57% of the patients (40/140) were using lipid-lowering medications with 33.33% of HoFH patients initiating treatment after the age of 8. Additionally, only 3 compound heterozygous patients (2.14%) underwent liver transplantation because of significantly high lipid levels.ConclusionThis study reveals the variable genotypes and phenotypes of children with FH in China and illustrates that the genotypes in the Chinese population differ from those in Caucasians, providing a valuable dataset for the clinical genetic screening of FH in China. Furthermore, the older age at diagnosis and treatment highlights the underdiagnosis and undertreatment of Chinese FH pediatric patients, suggesting that early identification should be improved through lipid or genetic screening, and that more timely and regular pharmacological treatments should be implemented.

Dataset Information

Sporadic Burkitt lymphomas of children and adolescents in Chinese: a clinicopathological study of 43 cases.

Background

Methods

Results

Conclusions

Virtual slides

Publications

Sporadic Burkitt lymphomas of children and adolescents in Chinese: a clinicopathological study of 43 cases.

Similar Datasets

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