Adult suspected primary hemophagocytic syndrome complicated with aggressive natural killer cell leukemia: report of one case and review of literature / 白血病·淋巴瘤

Objective:To improve the understanding of adult primary hemophagocytic syndrome (HPS) with aggressive natural killer cell leukemia (ANKL).Methods:The clinicopathological data of one adult patient with suspected primary HPS complicated with ANKL in Huiqiao Medical Center, Nanfang Hospital of Southern Medical University in October 2017 were retrospectively analyzed, and literatures were reviewed.Results:A 21-year-old male patient presented with persistent fever, hemocytopenia, splenomegaly, low fibrinogen, a significant increase in ferritin, hemophagocytes in bone marrow, decreased natural killer (NK) cell activity, and increased soluble CD25. Flow cytometry detection showed that the expression of NK cells was abnormal, and there were familial lysosomal trafficking regulator (LYST) and UNC13D gene defects. He was suspected of primary HPS complicated with ANKL. The patient was given 4 courses of EPOCH+PEG-Asp (etoposide, dexamethasone, vindesine, cyclophosphamide, doxorubicin hydrochloride liposome, pegaspargase) regimen chemotherapy, 20 mg of citalopidine twice a week maintenance therapy and matched unrelated hematopoietic stem cell transplantation. After 35 months of follow-up, he got sustained remission.Conclusions:Even if there are secondary causes of adult HPS, it is necessary to screen out related genes to avoid misdiagnosis. HPS patients with ANKL progress rapidly, and the early mortality is high. EPOCH+ PEG-Asp regimen induction therapy and allogeneic hematopoietic stem cell transplantation should be used as early as possible after diagnosis.

Effect of simulated microgravity on proliferation and differentiation of the human megakaryocyte cell / 实用医学杂志

Objective To investigate the effect of simulated microgravity on the proliferation and differentiation of the human megakaryocyte cells in vitro. Methods The fourth generation rotating cell culture system (RCCS-4) was used to generate the simulated microgravity environment. The cell viability was assessed by trypan blue staining method. The proliferation of cells was assessed by cell counting method and CCK8 method. The CD41+/CD61+ cells rate and the cells cycle were detected by flow cytometry. The expression levels of thrombopoietin receptor (c-mpl) and transcription factors were detected with RT-PCR. Results After 24, 48, 72 h, culture under simulated microgravity resulted in a significant decrease in the cell number , proliferative activity, cells in the G2/M phase and levels of c-mpl mRNA expression in comparison with that under the normal gravity (P < 0.05). After 48 h and 72 h culture, CD41+/CD61+ cells ratio decreased and RUNX-1 mRNA expression was down-regulated in cells of the group SMG compared with that of the group NG (P < 0.05). Conclusion Microgravity can inhibit the proliferation and differentiation of human megakaryocyte cells in vitro. The mechanism may be that TPO/c-mpl pathway was inhibited by down regulating the expression of c-mpl which transcriptional inhibition lead to.

Clinical characteristics and outcomes of 31 patients with primary bone lymphoma / 南方医科大学学报

<p><b>OBJECTIVE</b>To investigate the clinical characteristics, outcomes and prognostic factors of primary bone lymphoma (PBL).</p><p><b>METHODS</b>We retrospectively analyzed 31 consecutive patients with the diagnosis of PBL initially treated at our hospital between 1992 and 2010. Kaplan-Meier method was used for survival analysis and Cox regression model used for analyzing the prognostic factors.</p><p><b>RESULTS</b>The median age of the patients was 46 years. The most common sites of involvement were the femur (29%) and the spine (29%). Sixteen (52%) patients underwent chemoradiotherapy, and the other 15 (48%) received chemotherapy. With a median follow-up of 49 months, the patients showed an overall response rate of 94% (including a complete response rate of 68% and a partial response rate of 26%). The median overall survival (OS) of the patients was 71 months (95% CI: 36-106 months) with a median progression-free survival (PFS) of 47 months (95% CI: 30-64 months). Univariate analysis identified the use of rituximab, radiotherapy, and an international prognostic index (IPI) score of 0-2 as the favorable prognostic factors for OS and PFS. Multivariate analysis showed that the use of rituximab and IPI score were independent prognostic factors of the OS and PFS, and radiotherapy was the predicting factor for PFS but not for OS.</p><p><b>CONCLUSION</b>The use of rituximab can improve the OS or PFS of patients with PBL, and radiotherapy offers additional benefits for PFS but not for OS.</p>

Decitabine for treatment of myelodysplastic syndrome in an elderly patient and review of literature / 南方医科大学学报

Findings in epigenetic changes in meylodysplastic syndromes (MDS) and the development of demethylating drugs provide a new approach to the treatment of MDS. We used standard-dose decitabine for treatment of MDS in an elderly patient with an International Prostate Symptom Score (IPSS) of moderate risk group 2, and achieved a complete response in the first course. We report our experience with this case and review the relevant literatures.