Clinical and laboratory analysis of 17 patients with γδT-cell large granular lymphocyte leukemia / 中华血液学杂志

Objective@#To compare the difference of the clinical and laboratory characteristics between γδ T-cell large granular lymphocyte leukemia (γδT-LGLL) and αβ T-cell large granular lymphocyte leukemia (αβT-LGLL) .@*Methods@#The clinical and laboratory characteristics of 17 patients with γδT-LGLL and 91 patients with αβT-LGLL in the department of therapeutic center of anemia of enrolled in our hospital from January 2009 to January 2019 were retrospectively analyzed.@*Results@#The median age of the 17 patients with γδT-LGLL was 54 years (range, 25-73 years) , the most common presenting symptom was anemia. In comparison with αβT-LGLL patients, splenomegaly was common (41% and 44%, respectively) , whereas hepatomegaly (12% and 5%, respectively) and lymphadenopathy (6% and 8%, respectively) were rare. The positive rates of antinuclear antibody (59% and 45%, respectively) were high, whereas the positive rates of rheumatoid factor (6% and 10%, respectively) were rare for both groups. There were no differences on peripheral blood counts between the two groups. However, γδT-LGLL patients were found to be predominantly expressed a CD4−/CD8− phenotype. Steroid therapy with prednisone was used alone as first-line therapy for 1 patient. Cyclosporin A (CsA) was used alone as first-line therapy for 3 patients. CsA in combination with steroids were administered in 13 patients. After 4 months treatment, 2 patients acquired complete response, 4 patients acquired partial response, the overall response was 35%.@*Conclusion@#γδT-LGLL is a rare mature T-lymphocyte proliferative disease. Clinical and laboratory characteristics were quite similar for γδT-LGLL in compare with αβT-LGLL. γδT-LGLL predominantly expressed a CD4−/CD8− phenotype. The data presented here indicate the CsA is an effective option for the first-line treatment of γδT-LGLL.

Red blood cell lifespan detected by endogenous carbon monoxide breath test in patients with polycythemia vera / 中华内科杂志

Objective@#To detect the red blood cell lifespan in patients with polycythemia vera (PV), and explore the influencing factors.@*Methods@#From February 2017 to December 2018, 27 patients with PV at Blood Diseases Hospital, Chinese Academy of Medical Science and 18 normal controls were recruited. Red blood cell lifespan was detected by endogenous carbon monoxide (CO) breath test. The related factors were analyzed.@*Results@#The average red blood cell lifespan of 27 PV patients was 80 (range, 35-120) days (d), which was significantly shorter than that of the normal controls [110.5(69-166) d, P<0.05], namely 35.3 d shorter. The red blood cell lifespan of ten newly diagnosed patients and 17 patients who were treated with hydroxyurea and/or interferon were 98 (35-117) d and 69 (45-120) d, respectively, which were both shorter than that of the normal control (P=0.010, 0.000). Correlation analysis showed that red blood cell lifespan of patients with newly diagnosed PV was associated with JAK2 mutation allele burden (r=0.900, P=0.037), peripheral blood lymphocyte count (r=-0.742, P=0.014) and the level of serum vitamin B12 (r=-0.821, P=0.023).@*Conclusion@#The lifespan of red blood cells in patients with PV is about one-third shorter than normal, and is related to JAK2 mutation allele burden, absolute lymphocyte count, and serum vitamin B12 level.

Red blood cell lifespan detected by endogenous carbon monoxide breath test in patients with polycythemia vera / 中华内科杂志

Objective To detect the red blood cell lifespan in patients with polycythemia vera (PV), and explore the influencing factors. Methods From February 2017 to December 2018, 27 patients with PV at Blood Diseases Hospital, Chinese Academy of Medical Science and 18 normal controls were recruited. Red blood cell lifespan was detected by endogenous carbon monoxide (CO) breath test. The related factors were analyzed. Results The average red blood cell lifespan of 27 PV patients was 80 (range, 35-120) days (d), which was significantly shorter than that of the normal controls [110.5(69-166) d, P<0.05], namely 35.3 d shorter. The red blood cell lifespan of ten newly diagnosed patients and 17 patients who were treated with hydroxyurea and/or interferon were 98 (35-117) d and 69 (45-120) d, respectively, which were both shorter than that of the normal control (P=0.010, 0.000). Correlation analysis showed that red blood cell lifespan of patients with newly diagnosed PV was associated with JAK2 mutation allele burden (r=0.900, P=0.037), peripheral blood lymphocyte count (r=-0.742, P=0.014) and the level of serum vitamin B12 (r=-0.821, P=0.023). Conclusion The lifespan of red blood cells in patients with PV is about one?third shorter than normal, and is related to JAK2 mutation allele burden, absolute lymphocyte count, and serum vitamin B12 level.

Comparison of ifosfamide in combination with liposomal doxorubicin and dexamethasone in the treatment of relapsed/refractory multiple myeloma with or without extramedullary plasmacytoma / 中国医师进修杂志

Objective To compare the clinical effect of ifosfamide in combination with liposomal doxorubicin and dexamethasone (CDD) in the treatment of relapsed/refractory multiple myeloma (MM) with or without extramedullary plasmacytoma (EMP). Methods The clinical data of 71 relapsed/refractory MM patients treated with CDD regimen from January 2011 to December 2016 were retrospectively analyzed, including 48 patients with EMP(group A)and 23 patients without EMP (group B).One cycle of the CDD treatment was 21 d or 28 d and efficacy analysis was performed after every two cycles.Results The overall response rate in group A was 43.8%(21/48)and the complete remission and near complete remission rate was 8.3%(4/48);the overall response rate in group B was 65.2%(15/23)and the complete remission and near complete remission rate was 13.0% (3/23). There were no statistically significant differences between the two groups(χ2=1.203,0.659,P>0.05).The progression-free survival (PFS)time in group A was(8.6 ± 3.3)months, while the PFS time in group B was(7.9 ± 2.5)months and there was no significant difference between the two groups(t=1.009,P>0.05).There was no significant difference about incidence rate of adverse effects between the two groups(P>0.05).Conclusions CDD regimen can be used for the treatment of relapsed/refractory MM with or without EMP, the PFS and drug related adverse effects are similar, especially in patients with EMP.

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>