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27. Progress in the diagnosis and treatment of Rosai-Dorfman disease

Author

LIU Ting, CAO Xin-xin

Subjects
rosai-dorfman disease, clinical manifestations, treatment, Medicine
Abstract

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis. Its etiology and pathogenesis are yet incompletely elucidated. The clinical manifestations of RDD are highly heterogeneous. It is easy to be misdiagnosed or missed diagnosis. There is no standard treatment at present. Therefore, the progression in pathogenesis, diagnosis and treatment are reviewed. The treatment of RDD is best tailored to the individual clinical circumstances. Observation is suitable for patients with uncomplicated lymphadenopathy or asymptomatic cutaneous RDD. For RDD patients with extra nodal unifocal disease or organ compression, surgery is recommended. Systemic therapy can be considered for patients with systemic, recurrent or refractory RDD.

Published
2022
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37. International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults

Author

Goyal, Gaurav, Tazi, Abdellatif, Go, Ronald S., Rech, Karen L., Picarsic, Jennifer L., Vassallo, Robert, Young, Jason R., Cox, Christian W., Van Laar, Jan, Hermiston, Michelle L., Cao, Xin-Xin, Makras, Polyzois, Kaltsas, Gregory, Haroche, Julien, Collin, Matthew, McClain, Kenneth L., Diamond, Eli L., and Girschikofsky, Michael

Published
2022
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39. Liver involvement with Langerhans cell histiocytosis in adults.

Author

Chang, Long, Cai, Hua-Cong, Lang, Min, Lin, He, Luo, Ya-Ping, Duan, Ming-hui, Zhou, Dao-bin, Goyal, Gaurav, and Cao, Xin-xin

Subjects
RESEARCH funding, ASPARTATE aminotransferase, TREATMENT effectiveness, RETROSPECTIVE studies, DESCRIPTIVE statistics, ALKALINE phosphatase, BILIRUBIN, MULTIVARIATE analysis, GAMMA-glutamyltransferase, ODDS ratio, MEDICAL records, ACQUISITION of data, ALANINE aminotransferase, LIVER, LANGERHANS-cell histiocytosis, PROGRESSION-free survival, CONFIDENCE intervals, LIVER function tests, MOLECULAR pathology, SERUM albumin, OVERALL survival, SYMPTOMS, ADULTS
Abstract

Background and Aims Liver involvement portends poor prognosis in adults. We aimed to characterize the clinical features, liver function tests, radiologic findings, molecular profiles, therapeutic approaches and outcomes of adults patients with Langerhans cell histiocytosis (LCH) with liver involvement. Methods We conducted a retrospective analysis of all adults with LCH (≥ 18 years) seen at Peking Union Medical College Hospital (Beijing, China) between January 2001 and December 2022. Results Among the 445 newly diagnosed adults with LCH, 90 patients had liver involvement at diagnosis and 22 patients at relapse. The median age was 32 years (range, 18–66 years). Of 112 evaluable patients, 108 had full liver function testing, including alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase (ALP), γ-glutamyl transpeptidase (GGT), and total bilirubin and albumin. Elevated ALP was seen in 63.0% and GGT in 86.1%; 14.8% had elevated bilirubin. Next-generation sequencing of 54 patients revealed frequent BRAF N486_P490 (29.6%), BRAF V600E (18.5%), and MAP2K1 (14.8%). Outcomes After a median 40 months' follow-up (range 1-168 months), 3-year progression-free survival (PFS) and overall survival were 49.7% and 86.6% respectively. In multivariable analyses, ≥3 abnormal liver function tests (HR 3.384, 95% CI 1.550–7.388, P  = .002) associated with inferior PFS; immunomodulatory drug therapy (HR 0.073, 95% CI, 0.010-0.541, P  = .010) correlated with superior PFS versus chemotherapy. Conclusions In summary, elevated GGT and ALP were common in adults with LCH liver involvement. Greater than equal to 3 abnormal liver function tests predicted poor outcomes. Immunomodulatory drug therapy was associated with favorable progression-free survival compared to chemotherapy. [ABSTRACT FROM AUTHOR]

Published
2024
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42. FRG3, a Target of slmiR482e-3p, Provides Resistance against the Fungal Pathogen Fusarium oxysporum in Tomato.

Author

Ji, Hui-Min, Zhao, Min, Gao, Ying, Cao, Xin-Xin, Mao, Hui-Ying, Zhou, Yi, Fan, Wen-Yu, Borkovich, Katherine A, Ouyang, Shou-Qiang, and Liu, Peng

Subjects
FRG3, Fusarium oxysporum, NBS-LRR, disease resistance, microRNA, tomato, wilt disease, Plant Biology
Abstract

The vast majority of plant disease resistance (R) genes encode nucleotide binding site-leucine-rich repeat (NBS-LRR) proteins, which specifically determine the plant immune response and have been demonstrated to be targets of several microRNA (miRNA) families. The fungus Fusarium oxysporum f. sp. lycopersici (FOL) causes vascular wilt disease in tomato worldwide. Here, we explored a possible role for FGR3 in tomato defense against FOL. FRG3 is a predicted NBS-LRR like gene that is targeted by slmiR482e-3p, a member of slmiR482 miRNA family. Northern blot data demonstrated that all seven members of the slmiR482 family were regulated in diverse ways after infection by FOL. The ability of FRG3 to be regulated by slmiR482e-3p was confirmed at the transcript level by co-expression studies in Nicotiana benthamiana. A virus-induced gene silencing (VIGS) approach revealed that FRG3 confers resistance to the Motelle tomato cultivar. Taken together, our study has identified a novel R gene, FRG3, which is targeted by slmiR482e-3p at the transcript level, and is necessary for resistance to tomato wilt disease in planta.

Published
2018

49. Seed population in large Solar Energetic Particle events and the twin-CME scenario

Author

Ding, Liu-Guan, Li, Gang, Le, Gui-Ming, Gu, Bin, and Cao, Xin-Xin

Subjects
Astrophysics - Solar and Stellar Astrophysics
Abstract

It has been recently suggested that large solar energetic particle (SEP) events are often caused by twin CMEs. In the twin-CME scenario, the preceding CME is to provide both an enhanced turbulence level and enhanced seed population at the main CME-driven shock. In this work, we study the effect of the preceding CMEs on the seed population. We examine event-integrated abundance of iron to oxygen ratio (Fe/O) at energies above 25 MeV/nuc for large SEP events in solar cycle 23. We find that the Fe/O ratio (normalized to the reference coronal value of $0.134$) $\leq2.0$ for almost all single-CME events and these events tend to have smaller peak intensities. In comparison, the Fe/O ratio of twin-CME events scatters in a larger range, reaching as high as $8$, suggesting the presence of flare material from perhaps preceding flares. For extremely large SEP events with peak intensity above $1000$ pfu, the Fe/O drop below $2$, indicating that in these extreme events the seed particles are dominated by coronal material than flare material. The Fe/O ratios of Ground level enhancement (GLE) events, all being twin-CME events, scatter in a broad range. For a given Fe/O ratio, GLE events tend to have larger peak intensities than non-GLE events. Using velocity dispersion analysis (VDA), we find that GLE events have lower solar particle release (SPR) heights than non-GLE events, \red{agreeing with earlier results by Reames 2009b.

Published
2016
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50. Large solar energetic particle event that occurred on 2012 March 7 and its VDA analysis

Author

Ding, Liu-Guan, Cao, Xin-Xin, Wang, Zhi-Wei, and Le, Gui-Ming

Subjects
Astrophysics - Solar and Stellar Astrophysics
Abstract

On 2012 March 7, the STEREO Ahead and Behind spacecraft, along with the near-earth spacecraft (e.g. SOHO, Wind) situated between the two STEREO spacecraft, observed an extremely large global solar energetic particle (SEP) event in Solar Cycle 24. Two successive coronal mass ejections (CMEs) have been detected close in time. From the multi-point in-situ observations, it can be found that this SEP event was caused by the first CME, and the second one was not involved. Using the velocity dispersion analysis (VDA), we find that for well magnetically connected point, the energetic protons and electrons are released nearly at the same time. The path lengths to STEREO-B(STB) of protons and electrons have distinct difference and deviate remarkably from the nominal Parker spiral path length, which is likely due to the presence of interplanetary magnetic structures situated between the source and the STB. Also the VDA method seems only to obtain reasonable results at well-connected locations and the inferred energetic particles release times in different energy channels are similar. We suggest that good-connection is crucial for obtaining both accurate release time and path length simultaneously, agreeing with the modeling result of Wang & Qin (2015).

Published
2016
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