Severe inflammatory disorder in trisomy 8 without myelodysplastic syndrome and response to methylprednisolone: A case report.

BACKGROUND: Patients with trisomy 8 consistently present with myeloid neoplasms and/or auto-inflammatory syndrome. A possible link between myelodysplastic syndromes (MDS) with trisomy 8 (+8-MDS) and inflammatory disorders is well recognized, several cases having been reported. However, inflammatory disorders in patients without MDS have been largely overlooked. Generally, Behçet's disease is the most common type in +8-MDS. However, inflammatory disorders with pulmonary involvement are less frequent, and no effective treatment has been established. CASE SUMMARY: A 27-year-old man with recurrent fever, fatigue for > 2 mo, and unconsciousness for 1 day was admitted to our emergency department with a provisional diagnosis of severe pneumonia. Vancomycin and imipenem were administered and sputum collected for metagenomic next-generation sequencing. Epstein-Barr virus and Mycobacterium kansasii were detected. Additionally, chromosomal analysis showed duplications on chromosome 8. Two days later, repeat metagenomic next-generation sequencing was performed with blood culture. Cordyceps portugal, M. kansasii, and Candida portugal were detected, and duplications on chromosome 8 confirmed. Suspecting hematological disease, we aspirated a bone marrow sample from the iliac spine, examination of which showed evidence of infection. We added fluconazole as further antibiotic therapy. Seven days later, the patient's condition had not improved, prompting addition of methylprednisolone as an anti-inflammatory agent. Fortunately, this treatment was effective and the patient eventually recovered. CONCLUSION: Severe inflammatory disorders with pulmonary involvement can occur in patients with trisomy 8. Methylprednisolone may be an effective treatment.

The overexpression of PDGF-BB and its receptor is correlated with lymphatic metastasis in patients with non-small cell lung cancer.

The metastasis of tumors through lymphatic vessels is an important adverse prognostic factor for human cancers. Non-small cell lung cancer (NSCLC) is one of the most common types of lung cancers, and usually occurs with metastasis at an early stage and has a poor prognosis. Lymphatic metastasis is the most common route of lung cancer metastasis, and it requires the interaction of multiple growth factors. Platelet-derived growth factor-BB (PDGF-BB) and platelet-derived growth factor receptor-ß (PDGFR-ß) are related to both angiogenesis and lymphangiogenesis. This study aimed to explore PDGF-BB/PDGFR-ß and its correlations with lymphatic microvessel density (LMVD) and lymph node metastasis in patients with non-small cell lung cancer (NSCLC). A total of 127 Chinese patients with NSCLC pathologically diagnosed from 2009 to 2013 were enrolled in the study. The expression levels of PDGF-BB and PDGFR-ß were measured in all NSCLC tissues using immunohistochemical staining. Further, LMVD stained by D2-40 was evaluated, and the platelet (PLT) count was determined. The expression levels of PDGF-BB and PDGFR-ß in the NSCLC tissues were 73.2% (93/127) and 78.0% (99/127) respectively in 127 patients. The expressions of PDGF-BB and PDGFR-ß and LMVD were associated with TNM stage, lymph node metastasis, and PLT (P < 0.05 or P < 0.01), but not with the patient's age, gender, histological type, histological grade, and tumor size (P > 0.05). It was therefore concluded that the expression levels of PDGF-BB and PDGFR-ß in tumor cells were correlated with TNM stage and lymph node metastasis in Chinese patients with NSCLC and may play a key role in the development of NSCLC.

Association of Rs11615 (C>T) in the excision repair cross-complementing group 1 gene with ovarian but not gynecological cancer susceptibility: a meta-analysis.

BACKGROUND: Evidence suggests that the rs11615 (C>T) polymorphism in the ERCC1 gene may be a risk factor for gynecological tumors. However, results have not been consistent. Therefore we performed this meta- analysis. METHODS: Eligible studies were identified by search of PubMed, MEDLINE and Chinese National Knowledge Infrastructure (CNKI). Odds ratios (ORs) and 95% confidence intervals (CIs) were applied to assess associations between rs11615 (C>T) and gynecological tumor risk. Heterogeneity among studies was tested and sensitivity analysis was applied. RESULTS: A total of 6 studies were identified, with 1,766 cases and 2,073 controls. No significant association was found overall between the rs11615 (C>T) polymorphism and gynecological tumor susceptibility in any genetic model. In further analysis stratified by cancer type, significantly elevated ovarian cancer risk was observed in the homozygote and recessive model comparison (TT vs CC: OR=1.69, 95% CI=1.03-2.77, heterogeneity=0.876; TT vs CT/CC: OR=1.72, 95% CI=1.07-2.77, heterogeneity=0.995). CONCLUSION: The results of the present meta-analysis suggest that there is no significant association between the rs11615 (C>T) polymorphism and gynecological tumor risk, but it had a increased risk in ovarian cancer.

Elevated NKT cell levels in adults with severe chronic immune thrombocytopenia.

The aim of this study was to investigate the frequency of circulating natural killer T (NKT) cells and regulatory T cells (Tregs), as well as serum cytokine profiles, in adult chronic primary immune thrombocytopenia (ITP). The frequency of circulating T cell receptor (TCR) Vα24+Vß11+CD3+ NKT cells and CD4+CD25+CD127-/low Tregs was measured using multi-color flow cytometry. The serum concentrations of 11 cytokines were determined with a cytometric bead assay. The frequency of circulating NKT cells in patients with ITP was 0.13±0.03%, whereas the frequency in healthy controls was 0.07±0.01% of CD3+ (P>0.05). However, the frequency of NKT cells in patients with ITP with platelet counts ≤20×109/l (0.22±0.05%) was significantly higher than that in patients with platelet counts >20×109/l (0.05±0.01%; P<0.05) and that in healthy controls (0.07±0.01%; P<0.05). The frequency of peripheral Tregs was comparable between patients with ITP (3.97±0.44% of CD4+) and healthy controls (3.69±0.31%; P>0.05). No significant differences were observed in the serum concentrations of 11 cytokines between patients with ITP and healthy controls, despite the fact that the serum levels of interleukin (IL)-12p70, IL-8, IL-4, interferon (IFN)-γ and tumor necrosis factor (TNF)-α in patients with ITP were higher than those in the healthy controls. The platelet count was negatively correlated with the frequency of circulating NKT cells in chronic ITP. These results indicate that NKT cells may be involved in ITP with severe thrombocytopenia, and NKT and Tregs may be important in cytokine deregulation in chronic ITP.

Platelet volume indices have low diagnostic efficiency for predicting bone marrow failure in thrombocytopenic patients.

The aim of the present study was to evaluate the predictive efficiency of mean platelet volume (MPV) and platelet size deviation width (PDW) for bone marrow failure (BMF) in thrombocytopenic patients. Platelet count, MPV and PDW data were retrieved from the records of 574 unselected thrombocytopenic patients from between March 2010 and March 2011, of which 182 patients with a platelet count <20×10(9)/l were excluded from further study. A total of 392 valid thrombocytopenic patients were included in the present study and divided into two groups: 124 patients with idiopathic thrombocytopenia purpura (ITP) and 268 with BMF. The predictive efficiency of MPV and PDW were tested for the diagnosis of BMF. Significant differences were observed in the age distribution, platelet count, MPV and PDW between the ITP and BMF groups. The platelet count was positively correlated with MPV and PDW in the patients with ITP but not BMF. The negative-predictive values of MPV and PDW for BMF were 59.3 and 58.9%, respectively, with an MPV threshold of ≥11.0 fl and a PDW threshold of <16.0%. The positive-predictive values of MPV and PDW for BMF were 88.4 and 83.9%, respectively, with an MPV threshold <8.0 fl and a PDW threshold ≥17.5%. The areas under the curves (AUCs) of MPV and PDW were 0.281 and 0.700, respectively, for the diagnosis of BMF. The negative and positive-predictive values of MPV for BMF at different thresholds were not as conclusive as described in previous studies. MPV and PDW do not have sufficient specificity and sensitivity for the diagnosis of BMF in thrombocytopenic patients.