1.Effect of simvastatin on production of reactive oxygen species and secretion of IL-1βin macrophages induced by oxLDL
Mengxing JIN ; Hai YAN ; Yanwei CHENG ; Li GUI ; Chunsong HU ; Linjie ZHANG ; Baojun HUANG
Chinese Pharmacological Bulletin 2014;(5):692-695,696
Aim To study the effect of simvastatin on the production of reactive oxygen species ( ROS ) and the secretion of interleukin-1 beta ( IL-1β) in oxidized low density lipoprotein ( oxLDL )-induced macropha-ges. Methods After the murine macrophage J774A. 1 was treated with 0,50,100,200 mg·L-1 oxLDL, the contents of aggregated lipid in macrophages were ob-served and determined by oil red O staining. Then, the oxLDL-primed macrophages were treated with 0 . 5 ,1 . 0μmol·L-1 simvastatin, the production of ROS was de-termined by flow cytometry and the expressions of pro-caspase-1 , cleaved caspase-1 and mature IL-1βon pro-tein level were determined by Western blot. Results The oil red O staining results showed that oxLDL could induce obvious lipid aggregation in macrophages, and reached the saturation point with 100 mg·L-1 concen-tration. Flow cytometry results indicated that oxLDL could induce the production of ROS in macrophages, up to 167% ± 0. 47%, and ROS level decreased to 139% ± 0. 97% in a dose-dependent manner after treatment with simvastatin. Western blot indicated that simvastatin could inhibit the expression of cleaved caspase-1 and mature IL-1β in macrophages triggered by oxLDL;compared with oxLDL group, the expression of cleaved caspase-1 and mature IL-1β decreased in simvastatin treated group, and all results had statistical significance ( P<0. 05 ) . Conclusion In the lipid ag-gregation model of macrophages induced by oxLDL, simvastatin can inhibit the production of ROS, caspase-1 activation, and secretion of IL-1β in macrophages.
2.Clinical observation of allogeneic hematopoietic stem cell transplantation for 20 patients with hematologic malignancies
Pengji PAN ; Jishi WANG ; Zhiqiang SUN ; Yinghao LU ; Mengxing LI ; Peng ZHAO ; Zhengmei LONG
Journal of Leukemia & Lymphoma 2012;21(2):77-82
ObjectiveTo evaluate the efficacy of allogeneic stem cell transplantation (allo-HSCT) in treatment of hematologic malignancies and observe hematopoietic reconstitution, graft versus host disease (GVHD) occurrence,transplant-related complications and the outcome of disease.Methods20 patients with hematologic malignancies cured by allo-HSCT were analyzed retrospectively. 15 males and 5 females patients were enrolled, and the median age was 39(8-59)years. Mobilization of donor’ s stem cells using rhG-CSF program 3 days before transplantation.Conditioning regimen:the patients with HLA-matched used modified Bu/Cy programs,the patients with HLA-mismatched (with 1 to 3 loci mismatched) used the modified Bu/Cy+ ATG program;the patient with T-ALL and the patient with MM used Flu+Bu/Cy program. GVHD prevention programs: mycophenolate mofetil + cyclosporine + short course methotrexate. Results20 patients were successfully engrafted,the median time of absolute neutrophil count (ANC) > 0.5×109/L was 13 (12-17) days,the median time of Plt > 20×109/L was 16(12-23)days, and the hematopoietic reconstitution was rapid in those patients who were transplanted by the donors with the collected amount of CDh cells > 2.5× 106/kg (recipient body weigh) or the collected amount of mononuclear cell > 5.0×10s/kg (recipient body weigh).No severe hemolytic reaction occurred in 11 cases of blood group incompatibility between donor and recipient after transplantation,11 cases (55 %) developed acute GVHD (aGVHD):4 cases Ⅰ degree aGVHD,4 cases Ⅱ degree aGVHD,2 cases Ⅲ degree aGVHD,1 case Ⅳ degree aGVHD,all patients were improved after treatment.All patients attained complete remission (CR) after transplantation.Follow-up 6 (2-14) months,1 patient died in 5 months after transplantation because of leukemia relapse, 1 case died in 4 months after transplantation because of self-disabling autoimmune hemolytic cyclosporine, chronic GVHD(cGVHD)and multiple organ failure,the remaining patients still were in CR state.ConclusionAllo-HSCT is the effective way to treat hematologic malignancies. Engraftment is closely related with the quantity of hematopoietic stem cells from donor.Blood group incompatibility was not an obstacle for transplantion.Relapse,GVHD,infection are the major cause of death after transplantation.
3.Efficacy of hematopoietic stem cell transplantation in the treatment of 110 cases hematologic malignancies
Jishi WANG ; Yanyan YU ; Yinghao LU ; Zhiqiang SUN ; Mengxing LI ; Peng ZHAO ; Runlan XIE ; Zhengmei LONG
Journal of Leukemia & Lymphoma 2012;21(5):273-276
ObjectiveTo observe the efficiency of hematopoietic stem cell transplantation to the treatment of hematological malignancies and explore prevention and treatment of the complications correlated with HSCT. Methods110 patients with hematological malignancies which were treated by HSCT were recruited. 61 patients were treated with autologous peripheral blood hematopoietic stem cell transplantation (auto-PBSCT), 49 patients were treated with allogeneic hematopoietic stem cell transplantation (allo-HSCT).Among them,there were 28 patients were used by all HLA-identical sibling allo-PBSCT,20 patients were used by haploid allogeneic bone marrow and peripheral blood stem cell transplantation, one case of acute lymphoblastic leukemia in children were treated with cord blood stem cell transplantation.Results109(99.1%) patients acquired hemopoietic reconstruction. The median time of neutrophils≥0.5×109/L, and platelets≥20×109/L were 10 days and 12 days in auto-PBSCT,and were 12 days and 15 days in allo-PBSCT.The incidence of Ⅰ-Ⅲ degree of acute GVHD (aGVHD) in allogeneic transplantation was 28.6 %(14/49),however,the incidence of chronic GVHD (cGVHD) was 32.6 %(16/49).The median follow-up time was 36 (1~60) months.84 patients (76.4 %) were disease-free.Among them,73.8 %(45/61) were in auto-PBSCT group,(79.6 %)39/49 were in allo-HSCT group.26 patients (23.6 %) were died.There were 26.2 %(16/61) who were in auto-PBSCT group died of disease relapse,3.3 %(2/61) had disease relapse.There was no transplant-related deaths.18.4 %(9/49) who were in allo-HSCT group died of disease relapse, 6.1%(3/49)had disease relapse, 2.0 %(1/49)died of transplant-related deaths. ConclusionHematopoietic stem cell transplantation is a safe and effective way for the treatment of malignant hematopathy patients,also an important mean for treatment of blood diseases.
4.Effects of electroacupuncture on angiogenesis and cortical VEGF and BDNF expression inrats with focal cerebral ischemia
Mengxing LI ; Yu WANG ; Yunyun GAO ; Xiaowen YAO ; Wei LAN ; Wei TANG
Journal of Acupuncture and Tuina Science 2022;20(2):91-103
Objective: To observe the effect of electroacupuncture (EA) of "concurrent treatment of the brain and heart" on angiogenesis and cortical vascular endothelial growth factor (VEGF) and brain-derived neurotrophic factor (BDNF) in rats with focal cerebral ischemia, and to explore the mechanism of EA in cerebral ischemia treatment. Methods: A total of 108 Sprague-Dawley rats, 27 rats were randomly selected as the sham-operation group, and the rest rats received the right middle cerebral artery occlusion operation for model preparation firstly, and then were divided into a model group, a traditional acupoint group, and a concurrent treatment of the brain and heart group, with 27 rats in each group. In the sham-operation group, only the carotid artery was isolated. EA at Shuigou (CV26), Quchi (LI11), Hegu (LI4), and Zusanli (ST36) in the traditional acupoint group, and EA at Fengfu (GV16), Baihui (GV20), Xinshu (BL15), and Neiguan (PC6) in the concurrent treatment of the brain and heart group were performed 4 h after the operation, once a day, for 14 consecutive days. Rats in the sham-operation group and the model group were identically fixed without any treatment. Before and after treatment, the modified neurological severity score (mNSS), regional cerebral blood flow (rCBF), and CD34 positive expression by immunohistochemistry were measured. The positive protein expression levels of VEGF and BDNF were detected by immunofluorescence, and the mRNA expression levels of VEGF and BDNF were detected by quantitative reverse transcription-polymerase chain reaction (qRT-PCR). Results: Compared with the sham-operation group, the mNSS, rCBF, and ischemic side cortical micro-vessel density (MVD) decreased, and the protein and mRNA expression levels of VEGF and BDNF increased in the model group (P<0.01). Compared with the model group, the mNSS of the two EA groups decreased, and the mNSS of the concurrent treatment of the brain and heart group was lower than that of the traditional acupoint group on the 14th day (P<0.05). Compared with the model group, the rCBF in the two EA groups increased, and the rCBF reached the highest on the 14th day (P<0.05 or P<0.01), and the rCBF in the concurrent treatment of the brain and heart group was higher than that in the traditional acupoint group (P<0.05); the MVD of the two EA groups was higher than that of the model group, and the MVD of the concurrent treatment of the brain and heart group was higher than that of the traditional acupoint group on the 7th and 14th days (P<0.05 or P<0.01). Compared with the model group, the protein and mRNA expression levels of VEGF and BDNF in the two EA groups increased (P<0.01). The VEGF expression level was the highest on the 7th day in the concurrent treatment of the brain and heart group (P<0.05), and the BDNF expression level was higher on the 7th and 14th days than on the 3rd day (P<0.05). The mRNA expression levels of VEGF and BDNF in both EA groups reached the highest on the 7th day (P<0.05 or P<0.01). Conclusion: EA therapy can up-regulate the VEGF and BDNF expression levels and increase the rCBF in the cortex of rats with focal cerebral ischemia, which may be one mechanism of EA in the cerebral ischemia treatment. The therapeutic effect is accumulated with the effective time, and the concurrent treatment of the brain and heart group is superior to the traditional acupoint group in promoting angiogenesis.
5.Correlation of telomere length of bone marrow mononuclear cells with relapse after allogeneic hematopoietic stem cell transplantation for acute myeloid leukemia
Bo DENG ; Jishi WANG ; Yan ZHANG ; Yinghao LU ; Yanju LI ; Yi HUANG ; Mengxing LI ; Ying CHEN ; Rui GAO ; Xiao CHAI ; Yun ZHAN ; Jie XIONG ; Peng ZHAO
Journal of Leukemia & Lymphoma 2023;32(6):335-342
Objective:To investigate the relationship between telomere length of bone marrow mononuclear cells and prognosis of patients with acute myeloid leukemia (AML) who received allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods:Telomere length of bone marrow mononuclear cells before transplantation, after transplantation and before donor mobilization as well as information related to follow-up of 33 AML patients who received allo-HSCT in the Affiliated Hospital of Guizhou Medical University between June 2020 and June 2021 were retrospectively analyzed. Telomere length was detected by using telomeric terminal restriction fragment (TRF) method. Telomere length was compared among patients with different prognoses. The recurrence within 1 year was treated as the gold standard and receiver operating characteristic (ROC) curve was used to analyze the effect of telomere length before transplantation or before donor mobilization in the judgement of the recurrence within 1 year after transplantation. The patients were stratified according to the optimal threshold value of telomere length for patients or donors, and Kaplan-Meier method was used to compare the progression-free survival (PFS) of patients with different stratification, and log-rank test was performed.Results:The median age of 33 patients was 34 years (14-61 years), and there were 17 males and 16 females; 31 patients were initially diagnosed with AML, 1 patient transferred from myelodysplastic syndrome (MDS) to AML, and 1 patient transferred from chronic granulocytic leukemia (CML) to AML; 14 received identical sibling transplantation and 19 received haploidentical sibling transplantation. The median age of the donors was 30 years (20-65 years), including 24 males and 9 females. Telomere length of bone marrow mononuclear cells before mobilization in 33 donors was longer than that in patients before transplantation (33 cases) and at +30 d after transplantation (31 cases) [(6.67±0.31) kb, (6.40±0.33) kb, (6.48±0.33) kb, respectively; all P < 0.05], and the difference between patients before and at +30 d after transplantation was not statistically significant ( t = 0.89, P = 0.378), and the telomere length of bone marrow mononuclear cells in 11 patients +180 d after transplantation was (6.66±0.18) kb. The incidence of acute graft-versus-host disease (aGVHD) after transplantation was 45.5% (15/33), the incidence of infection with clear imaging and pathogenic basis was 39.4% (13/33), the mortality rate within 1 year after transplantation was 3.0% (1/33), and the recurrence rate within 1 year after transplantation was 15.2% (5/33). There were no statistically significant differences in telomere length of donor pre-mobilization bone marrow mononuclear cells between the groups with and without aGVHD and between the infected and non-infected groups (all P > 0.05).Compared with patients who had not relapsed within 1 year after transplantation, telomere length of donor pre-mobilization bone marrow mononuclear cells was shorter in patients who relapsed within 1 year after transplantation [(6.39±0.19) kb vs. (6.72±0.30) kb, t = -3.23, P = 0.011], telomere length was longer in patients before transplantation [(6.75±0.16) kb vs. (6.35±0.36) kb, t = 4.17, P = 0.001]. ROC curve analysis showed that the optimal threshold values for telomere length of pre-transplantation and donor pre-mobilization bone marrow mononuclear cells were 6.48 and 6.42 kb, respectively for patients who relapsed within 1 year after transplantation. PFS in patients with pre-transplantation bone marrow mononuclear cells telomere length < 6.48 kb was better than that in patients with telomere length ≥ 6.48 kb ( P = 0.003); PFS in patients with pre-mobilization bone marrow mononuclear cells telomere length>6.42 kb was better than that in patients with telomere length ≤ 6.42 kb ( P < 0.001). Conclusions:In allo-HSCT for AML, patients have an increased risk of relapse within 1 year after transplantation when their pre-transplantation bone marrow mononuclear cells telomere length is long and the donor bone marrow mononuclear cells telomere length is short.
6.Research on Locating Device for the Entry Point of Intramedullary Nail Based on Inertial Navigation
Chu GUO ; Bobin MI ; Junwen WANG ; Jing JIAO ; Shilei WU ; Tian XIA ; Jingfeng LI ; Guohui LIU ; Mengxing LIU
Chinese Journal of Medical Instrumentation 2024;48(2):179-183
Objective To introduce a locating device for the entry point of intramedullary nail based on the inertial navigation technology,which utilizes multi-dimensional angle information to assist in rapid and accurate positioning of the ideal direction of femoral anterograde intramedullary nails'entry point,and to verify its clinical value through clinical tests.Methods After matching the locating module with the developing board,which are the two components of the locating device,they were placed on the skin surface of the proximal femur of the affected side.Anteroposterior fluoroscopy was performed.The developing angle corresponding to the ideal direction of entry point was selected based on the X-ray image,and then the yaw angle of the locating module was reset to zero.After resetting,the locating module was combined with the surgical instrument to guide the insertion angle of the guide wire.The ideal direction of entry point was accurately located based on the angle guidance.By setting up an experimental group and a control group for clinical surgical operations,the number of guide wire insertion times,surgical time,fluoroscopy frequency,and intraoperative blood loss with or without the locating device was recorded.Results Compared to the control group,the experimental group showed significant improvement in the number of guide wire insertion times,surgical time,fluoroscopy frequency,and intraoperative blood loss,with a statistically significant difference(P<0.01).Conclusion The locating device can assist doctors in quickly locating the entry point of intramedullary nail,effectively reducing the fluoroscopy frequency and surgical time by improving the success rate of the guide wire insertion with one shot,improving surgical efficiency,and possessing certain clinical value.
7.Influence of peripheral blood NLR,MLR and PLR in prognosis of patients with multiple myeloma
Xinlan XIA ; Die LIU ; Lihong AN ; Yan LI ; Peng ZHAO ; Yan ZHANG ; Mengxing LI ; Jishi WANG ; Yinghao LU
Chongqing Medicine 2024;53(15):2261-2268
Objective To investigate the influence of peripheral blood neutrophil-to-lymphocyte ratio (NLR),monocyte-to-lymphocyte ratio (MLR) and platelet-to-lymphocyte ratio (PLR) on the prognosis in the patients with multiple myeloma (MM).Methods A total of 159 newly diagnosed MM admitted and treated in the Affiliated Hospital of Guizhou Medical University from January 2019 to May 2023 were selected as the study subjects.The general clinical data,blood biochemical and marrow routine detection results before the in-itial treatment were collected.NLR,MLR and PLR were calculated.The univariate and multivariate Cox-re-gression model was adopted to analyze the influencing factors.The receiver operating characteristic (ROC) curve was used to analyze the predictive value.The Kaplan-Meier survival curve and Log-Rank test were used to conduct the survival analysis.Results The ROC curve showed that the critical values of NLR,MLR and PLR were 2.682,0.317 and 147.786 respectively.The patients were divided into the high/low NLR groups (n=61,n=98),high/low MLR group (n=76,n=83) and high/low PLR groups (n=59,n=100).The pro-portions of blood calcium<2.5 mmol/L and creatinine<177 μmmol/L in the low NLR group in the low NLR group were higher compared with the high NLR group (P<0.05);the blood calcium,creatinine and DS stage had statistical differences between the low MLR group and high MLR group (P<0.05);blood calcium had statistical difference between the low PLR group and high PLR group (P<0.05).After 3 treatment courses,the complete remission rate in the high NLR group,high MLR group and high PLR group was significantly lower than that in the corresponding low group (P<0.05).The multivariate Cox-regression analysis results showed that hemoglobin<100 g/L and high PLR were the independent risk factors affecting the progress free survival (PFS) stage in the patients with MM (P<0.05).The age>60 years old was the independent risk factors affecting the overall survival (OS) in the patients with MM (P<0.05).Conclusion NLR,MLR and PLR could serve as the assisted tool to evaluate the prognosis in the patients with MM.
8.Risk factors of abnormal urinary albumin/creatinine ratio in people with obesity
Zhe CAO ; Tongyue YANG ; Shiyu LIU ; Mengxing PAN ; Xuyang GONG ; Qianshuai LI ; Jiao WANG ; Lin ZHAO ; Guijun QIN ; Yanyan ZHAO
Chinese Journal of Endocrinology and Metabolism 2024;40(3):186-191
Objective:To explore the clinical characteristics and risk factors of abnormal urinary albumin/creatinine ratio(UACR) in obese population.Methods:Baseline data from 2011 to 2012 in Henan Sub-center of"Risk Evaluation of cAncers in Chinese diabeTic Individuals: A lONgitudinal(REACTION) study"were utilized and those of body mass index≥28 kg/m 2 were screened. The patients were divided into UACR normal group and UACR abnormal group(101 pairs) upon being matched on a 1∶1 basis by age and gender. Multivariate logistic regression analysis, receiver operating characteristic(ROC) curve, and restricted cubic spline(RCS)analysis were performed to explore the risk factors for abnormal UACR. Results:Compared with the normal UACR group, the UACR abnormal group had a higher number of alcohol consumers, a higher prevalence of hypertension, elevated systolic blood pressure, and triglyceride(all P<0.05). Multivariate logistic regression analysis showed that alcohol consumption( P=0.008), systolic blood pressure( P<0.001), triglyceride( P=0.049), and homeostasis model assessment for insulin resistance(HOMA-IR, P=0.033) were independent risk factors for abnormal UACR in obese people. The ROC curve analysis indicated that systolic blood pressure had the strongest diagnostic performance as a single factor(ROC curve area=0.801), and there was no significant difference in diagnostic performance compared to multiple factors combination. RCS analysis results showed that the probability of abnormal UACR increased monotonically with the increase of systolic blood pressure when the systolic blood pressure was between 130 and 158 mmHg(1 mmHg=0.133 kPa). When systolic blood pressure was not in the interval, the probability of abnormal UACR did not change significantly. The results of regression analysis of triglyceride subgroup showed that when triglyceride level was greater than or equal to 5.6 mmol/L, the risk of abnormal UACR level was significantly increased( P=0.029). Conclusion:Systolic blood pressure, triglyceride, HOMA-IR, and alcohol drinking history are independent risk factors for abnormal UACR in obese people. When systolic blood pressure is≥130 mmHg or triglyceride is≥5.6 mmol/L, the risk of abnormal UACR is significantly increased.
9.Effect of Modified Qilang Prescription on Drug-dependent Constipation of Qi-Yin Deficiency Type
Mengxing CAO ; Mengen ZHOU ; Yong LI
Chinese Journal of Experimental Traditional Medical Formulae 2022;28(24):145-152
ObjectiveTo observe the clinical effect of modified Qilang prescription on the treatment of drug-dependent constipation of Qi-Yin deficiency type. MethodIn a randomized double-blind clinical trial, 160 patients with drug-dependent constipation were randomly divided into two groups, namely, the treatment group (80 cases) and the control group (80 cases). The treatment group was treated with modified Qilang prescription and lactulose oral liquid simulator, while the control group was treated with lactulose oral liquid and the simulator of modified Qilang prescription. The course of treatment of both groups was 8 weeks. The scores of main syndromes of constipation, traditional Chinese medicine (TCM) syndromes, patient assessment of constipation symptom (PAC-SYM) scale, and patient assessment of constipation quality of life (PAC-QOL) scale before and after treatment were recorded. The patients were followed up at the 2nd and 4th week after drug withdrawal. ResultAfter treatment, the full analysis set (FAS) analysis showed that the total effective rate was 91.14% (72/79) in the treatment group and 73.33% (55/75) in the control group. The treatment group had a higher total effective rate than the control group (Z=-6.62, P<0.01). The per-protocol set (PPS) analysis showed that the total effective rate was 93.51% (72/77) in the treatment group and 76.39% (55/72) in the control group. The treatment group had a higher total effective rate than the control group (Z=-6.77, P<0.05). After treatment, the FAS and PPS analysis showed that the main syndrome scores of constipation were lower in both groups than those before treatment (P<0.05). Except the syndromes of falling, overexertion defecation, and distension, the scores of other syndromes in the treatment group were lower than those in the control group (P<0.05). The scores of TCM primary syndromes in the two groups were lower than those before treatment (P<0.05), and the scores of the treatment group were significantly lower than those of the control group (P<0.05). The scores of TCM secondary syndromes in the treatment group were significantly lower than those before treatment (P<0.05). The syndrome scores of abdominal distension, fatigue, and lack of sleep in the treatment group were significantly lower than those in the control group (P<0.05). The scores of PAC-SYM scale were improved in both groups after treatment (P<0.05), and the treatment group was superior to the control group in improving fecal syndromes and abdominal syndromes. The scores of PAC-QOL scale were improved in both groups after treatment, and the treatment group was superior to the control group in improving patients’ physiological function, social psychology, anxiety, and satisfaction. After drug withdrawal, the recurrence rate was lower in the treatment group at the 2nd(χ2 =5.65,P<0.05) and 4th week (χ2 =12.37,P<0.01). Conclusionmodified Qilang prescription is effective in the treatment of drug-dependent constipation, and its clinical effect is better than that of lactulose oral liquid. The curative effect of modified Qilang prescription is stable and lasting without obvious adverse reactions.
10.Clinical characteristics and prognosis of 3q26 rearrangements in chronic myeloid leukemia
Lianghui LI ; Li YAO ; Mengxing XUE ; Li HUO ; Ping CAI ; Suning CHEN
Chinese Journal of Clinical Laboratory Science 2019;37(5):349-352
Objective:
To evaluate the clinical characteristics and prognosis of 3q26 rearrangements in chronic myeloid leukemia (CML) patients.
Methods:
The clinical and laboratory data of 1 075 patients with CML diagnosed from 2010 to 2016 were retrospectively analyzed, and they were divided into 3q26 rearrangement positive group (n=19) and 3q26 rearrangement negative group (n=1 056). The expression of EVI1, ABL kinase region mutation and survival time between the two groups were compared. Meanwhile, the prognostic effects of three treatment methods, including tyrosine kinase inhibitors (TKIs), TKIs combined with chemotherapy and allogeneic hematopoietic stem cell transplantation, on the patients with 3q26 rearrangements were compared.
Results:
Most of the patients with 3q26 rearrangements were in the advanced phase (χ 2 =181.233, P<0.01), and the median time to enter the acute phase was shorter (9.5 months). The mutation ratio of ABL kinase region and expression levels of EVI1 in 3q26 rearrangement positive group were significantly higher than that in the negative group (χ 2 =16.758, P<0.01; Z/U=-0.331 9, P<0.01). After treatment with TKIs, the median survival time of the 3q26 rearrangement positive group was significantly shorter than that of the negative group (χ 2 =313.229, P<0.01). The prognosis of the patients treated with hematopoietic stem cell transplantation was better than that with TKIs (P=0.049).
Conclusion
The CML patients with 3q26 rearrangements have a higher risk of sudden change, shorter survival time and poor prognosis. Hematopoietic stem cell transplantation may improve their prognosis.