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OBJECTIVE@#To analyze the characteristics and prognosis of acute leukemia(AL) with SET-NUP214 fusion gene.@*METHODS@#The clinical data of 17 patients over 14 years old newly diagnosed with SET-NUP214 positive AL admitted in Institute of Hematology and Blood Diseases Hospital from August 2017 to May 2021 were analyzed retrospectively.@*RESULTS@#Among the 17 SET-NUP214 positive patients, 13 cases were diagnosed as T-ALL (ETP 3 cases, Pro-T-ALL 6 cases, Pre-T-ALL 3 cases, Medullary-T-ALL 1 case), AML 3 cases (2 cases M5, 1 case M0) and ALAL 1 case. Thirteen patients presented extramedullary infiltration at initial diagnosis. All 17 patients received treatment, and a total of 16 cases achieved complete remission (CR), including 12 cases in patients with T-ALL. The total median OS and RFS time were 23 (3-50) months and 21 (0-48) months, respectively. Eleven patients received allogeneic hematopoietic stem cell transplantation(allo-HSCT), with median OS time of 37.5 (5-50) months and median RFS time of 29.5 (5-48) months. The median OS time of 6 patients in chemotherapy-only group was 10.5 (3-41) months, and median RFS time of 6.5 (3-39) months. The OS and RFS of patients with transplantation group were better than those of chemotherapy-only group (P=0.038). Among the 4 patients who relapsed or refractory after allo-HSCT, the SET-NUP214 fusion gene did not turn negative before transplantation. While, in the group of 7 patients who have not relapsed after allo-HSCT till now, the SET-NUP214 fusion gene expression of 5 patients turned negative before transplantation and other 2 of them were still positive.@*CONCLUSION@#The fusion site of SET-NUP214 fusion gene is relatively fixed in AL patients, often accompanied by extramedullary infiltration. The chemotherapy effect of this disease is poor, and allo-HSCT may improve its prognosis.
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Humanos , Adolescente , Leucemia-Linfoma Linfoblástico de Células T Precursoras , Estudios Retrospectivos , Leucemia Mieloide Aguda/terapia , Trasplante de Células Madre Hematopoyéticas , Enfermedad Aguda , Pronóstico , Leucemia-Linfoma de Células T del Adulto/terapia , Proteínas de Complejo Poro NuclearRESUMEN
Objective: To investigate the clinical characteristics, diagnosis, treatment and prognosis of therapy-related myeloid neoplasms (t-MNs) after successful treatment for acute promyelocytic leukemia (APL) . Methods: Clinical data of 4 patients, diagnosed as t-MNs secondary to APL at Hematology Hospital of Chinese Academy of Medical Sciences from October 2012 to January 2019, were collected retrospectively. T-MNs related literature was reviewed. Results: The 4 cases were all females, with the median age 42 (range 40-53) years old at the diagnosis of APL. Regarding the induction and consolidation regimens, 3 patients received all-trans retinoid acid (ATRA) and arsenic trioxide (ATO) combined with anthracycline/anthraquinone and/or cytosine. One patient only received ATRA and other auxiliary drugs. Alkylating agents were not administrated. The 4 patients developed t-MNs 40 to 43 months after complete remission (CR) of APL, including 1 case of therapy-related myelodysplastic syndrome (t-MDS) and 3 cases of acute myeloid leukemia (t-AML) . The PML-RARα fusion genes were all negative when t-MNs developed. The three patients with t-AML were treated with 3 to 4 re-induction regimens, one of whom underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) after complete remission (CR) . One patient with t-MDS received hypomethylating agents. After a median follow-up of 54.5 (48-62) months, 2 patients with t-AML died, the median overall survival after t-MN was 12 (5-18) months. From 1989 to 2018, a total of 63 t-MN cases were reported in the literature. Therefore, 67 cases were analyzed when four patients in our center were added, including 27 males and 40 females with median age 52.5 (15-76) years. The median latency was 39 (12-126) months and the median overall survival after diagnosis of t-MN was 10 (1-39) months. Conclusions: Although rare, t-MNs may occur after successful control of APL. There are no existing guidelines for prevention and treatment of t-MNs, which have very poor prognosis. If cytopenia or other abnormalities of peripheral blood cells develop after 3 years of APL, t-MNs should be considered as a differential diagnosis.
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Adulto , Femenino , Humanos , Persona de Mediana Edad , Protocolos de Quimioterapia Combinada Antineoplásica , Arsenicales , Leucemia Mieloide Aguda , Leucemia Promielocítica Aguda/terapia , Neoplasias Primarias Secundarias , Óxidos , Estudios Retrospectivos , Resultado del Tratamiento , TretinoinaRESUMEN
<p><b>OBJECTIVE</b>To compare the efficacy and safety of 3 different regimens, namely MAC, FLAG and CAG, as the re-induction chemotherapy for acute myeloid leukemia(AML) patients with primary induction failure and relapse.</p><p><b>METHODS</b>The clinical data of 156 AML patients with primary induction failure and relapse, except patients with acute promyelocytic leukemia(APL), treated with any of the above 3 regimens in our center from January 2008 to April 2016 were analyzed retrospectively. According to the treatment regimens, 156 patients were divided into MAC group (n=60), FLAG group (n=45) and CAG group (n=51). The complete remission(CR), partial remissison(PR), overall survival(OS), disease-free survival(DFS) and adverse events during the treatment were analyzed, so as to compare and evaluate the efficacy and safety of the 3 different regimens.</p><p><b>RESULTS</b>After 1 course of re-induction chemotherapy, CR in MAC group was significantly higher than that in FLAG and CAG group (55.4% vs 34.1% vs 34.0%)(P<0.05). The OS was not statistically significantly different among 3 groups (P>0.05) with a median OS of 11 months, 5.46 months and 10.2 months, respectively. The myelosuppression was the main adverse event with no significant difference among the groups(P>0.05). More patients treated with MAC regimen underwent febrile neutropenia (93.3% vs 86.7% vs 64.7%)(P<0.001). However, the incidence of fatal infections was not signicantly different among 3 groups(5% vs 8.9% vs 5.9%)(P>0.05).</p><p><b>CONCLUSION</b>Compared with FLAG and CAG regimen, the MAC regimen can enable more AML patients with primary induction failure and refractory to achieve CR without increasing severe adverse events,therefore,this regimen may provide a opportunity for patients to recieve hematopoietic stem cell transplantation.</p>
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<p><b>OBJECTIVE</b>To evaluate the long-term clinical effect of autologous peripheral blood mononuclear cells (PB-MNC) on critical limb ischemia (CLI) in patients with thromboangiitis obliterans (TAO) patients.</p><p><b>METHODS</b>The clinical data of 22 patients with CLI caused by TAO from July 2004 to May 2013 were analyzed retrospectively, 22 patients were divided into 2 groups; out of them 12 cases in one group were treated with granulocyte colony-stimulating factor (G-CSF)-mobilized autologous peripheral blood mononuclear cells (auto-PBMNC group), 10 cases in another group received conservative treatment (CT group). The log-rank test was used to compare the long-term outcomes in auto-PBMNC group and CT group.</p><p><b>RESULTS</b>The wound healing rate (P=0.016) and CLI-free rate (P=0.013) were significantly higher in PB-MNC group compared with that in CT group. No difference was found in amputation rates between the 2 groups (major amputation: P=0.361, minor and major amputation: P=0.867). No patients died or no serious adverse events occurred during the follow-up period.</p><p><b>CONCLUSION</b>The auto-PBMNC therapy can significantly promote the wound healing, and protect against CLI in TAO patients, but the risk of amputation is not low in comparison with conservative treatment.</p>
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Humanos , Amputación Quirúrgica , Extremidades , Factor Estimulante de Colonias de Granulocitos , Farmacología , Isquemia , Terapéutica , Leucocitos Mononucleares , Trasplante , Estudios Retrospectivos , Tromboangitis Obliterante , Terapéutica , Trasplante Autólogo , Resultado del Tratamiento , Cicatrización de HeridasRESUMEN
<p><b>OBJECTIVE</b>To evaluate the safety and therapeutic effect of high-dose daunorubicin-based (HD-DNR) chemotherapy in the treatment of acute leukemia (AL).</p><p><b>METHODS</b>The clinical data of 25 AL patients, including 14 cases for induction chemotherapy, 8 for consolidation chemotherapy and 3 for reinduction therapy, which were treated with HD- DNR (DNR dosage of 90 mg/m(2)× 3 d) between June 2010 and August 2012 in our hospital were retrospectively analyzed, the adverse reaction of chemotherapy, especially cardiac toxicity and therapeutic effect were evaluated.</p><p><b>RESULTS</b>Most of the adverse reactions were mild, including cardiac toxicity, and no patient discontinued therapy because of HD-DNR related toxicities. Grade 3 or higher adverse reactions occurred only in the infection (56%) and diarrhea (12%). Withdrawal or dose reduction due to strong adverse reactions was not observed in all patients. Adverse reactions of infections (92%), lower ejection fraction(52.6%), diarrhea (48%), nausea (36%), vomiting (36%), dental ulcer (36%) and myocardial ischemia (32%) were relatively more common. The median time of neutrophil count reached to ≥ 0.5 × 10(9)/L and platelet ≥ 20 × 10(9)/L were both 21 days(ranged 9-31 and 9-38 days). Nine patients were complicated with infections before chemotherapy and 14 after chemotherapy, mainly occurred in gastrointestinal tract and respiratory system. Gastrointestinal, liver and kidney toxicity was slight. The cardiac ejection decreased in 10 cases, but only 1 reached grade 2 without clinical symptoms. Of the 14 AL patients for induction chemotherapy, 13 achieved hematological complete remission. Eight patients received HD-DNR as consolidation chemotherapy remained complete remission, while 3 refractory/relapsed patients remained non-remission.</p><p><b>CONCLUSION</b>The adverse reaction of HD-DNR based chemotherapy for AL treatment was mild, no obvious cardiac adverse reaction occurred. The treatment dose of DNR at 90 mg/m(2) × 3 d can be safely and effectively used to treat acute leukemia.</p>
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Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Enfermedad Aguda , Protocolos de Quimioterapia Combinada Antineoplásica , Usos Terapéuticos , Daunorrubicina , Usos Terapéuticos , Leucemia , Quimioterapia , Estudios RetrospectivosRESUMEN
<p><b>OBJECTIVE</b>To study the role of the feeder layer cells as niche in the process of expansion of late endothelial progenitor cell in vitro.</p><p><b>METHODS</b>We cultured mononuclear cells (MNC)from human peripheral blood (PB)on the plate with the feeder layer cells which were irradiated late endothelial progenitor cells(EPC)or human umbilical vein endothelial cells (HUVEC) by EGM-2. After 21 days, the numbers of obtained late EPC colonies were counted separately, and their surface antigen of the late EPC was verified by fluorescence-activated cell sorter (FACS) analysis, and their ability of forming vessel structure with Matrigel in vitro. The differentiation of single stem cell on the feeder layer cell was traced by video-microscopy.</p><p><b>RESULTS</b>After 21 days of culture,(40.0±3.9)and(39.3±3.1)late EPC colonies that MNC of a hundred milliliter PB were cultured, respectively, on the feeder layer cells of EPC and HUVEC were much more than (2.0±1.3) colonies cultured on without the feeder layer cells (all P <0.05). These cells also expressed CD31,CD34,eNOS,FLt-1,P1H12,Sendo,VE cadherin,and CD117, as shown by FACS analysis. Furthermore, they formed vessel structure with Matrigel in vitro. The video-microscopy showed the asymmetric cell division was participated by the feeder layer cell during the expansion of single stem cell.</p><p><b>CONCLUSION</b>The massive expansion of late EPC can be achieved by the provision of the feeder layer cells, which may be involved in the stem cell asymmetric cell division.</p>
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Humanos , Comunicación Celular , Técnicas de Cultivo de Célula , Diferenciación Celular , Proliferación Celular , Microambiente Celular , Células Endoteliales , Biología Celular , Células Nutrientes , Sangre Fetal , Biología Celular , Células Endoteliales de la Vena Umbilical Humana , Biología Celular , Células Madre , Biología Celular , Trofoblastos , Biología CelularRESUMEN
<p><b>OBJECTIVE</b>To analyze the efficacy and its correlation with species of transplant cells of autologous mobilized peripheral blood (PB) mononucleated cells (MNCs) transplantation on 59 patients with lower limbs ischemia.</p><p><b>METHODS</b>Fifty-nine patients were evaluated with symptoms scores and after that their PBMNCs were mobilized and collected and then injected into the ischemic area at equal distance. They effectiveness and scores were evaluated at 7th day and 4th month after therapy. The correlation of CD34(+) cells and of MNCs with effectiveness was analysed respectively, and formula for correlations between them and effectiveness was calculated.</p><p><b>RESULTS</b>After MNCs injection, the effectiveness was observed both at 7th day and 4th month. The correlation of MNCs with effectiveness was stronger than that of CD34(+) cells (the effectiveness was represented by nimodipine value), According to the formula of nimodipine value, the value of the latter = 0.484 + 1.055 × CD34(+) cells number and the former = 0.288 + 0.401 × MNCs number with a correlation coefficient of R = 0.461 (P = 0.047) and R = 0.473 (P = 0.000) respectively.</p><p><b>CONCLUSION</b>Autologous mobilized PBMNCs number is a better indicator for effectiveness than CD34(+) cells number.</p>
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Femenino , Humanos , Masculino , Persona de Mediana Edad , Isquemia , Cirugía General , Extremidad Inferior , Monocitos , Trasplante , Trasplante de Células Madre de Sangre Periférica , Métodos , Enfermedades Vasculares Periféricas , Cirugía General , Trasplante AutólogoRESUMEN
The study was aimed to explore the characteristics of clinical manifestation, laboratory indicators and bone marrow examination of SLE patients with anemia. 60 SLE patients with anemia were analyzed for their clinical manifestation, laboratory indicators and bone marrow examination in comparison with 40 contemporaneous SLE patients without anemia. The results indicated that there were significant differences in clinical manifestations of fatigue between the SLE patients with anemia and those without anemia. The detection rate of the decreased Plt and C4 and the percentages of eosinophils, early normoblast, polychromatic normoblast and orthochromatic normoblast in bone marrow were all higher than that in those without anemia. The ANA with titer 1:320 in SLE patients complicated by anemia was lower than that in those without anemia. In conclusion, the clinical manifestation, experimental examination and bone marrow findings were significantly different between the SLE patients with anemia and without anemia.
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Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Anemia , Diagnóstico , Autoanticuerpos , Examen de la Médula Ósea , Lupus Eritematoso Sistémico , DiagnósticoRESUMEN
<p><b>OBJECTIVE</b>To analyse the clinical feature and natural course of essential thrombocythemia (ET).</p><p><b>METHODS</b>A retrospective analysis was conducted in ET patients treated in our hospital during May 1980 to December 2006.</p><p><b>RESULTS</b>Four hundred and thirty eight patients (201 males and 237 females with a median age of 48 years) were diagnosed. Hemorrhage occurred in 101 cases (23.1%), thrombosis in 86 cases (19.6%), and both hemorrhage and thrombosis in 13 cases (3.0%). Splenomegaly occurred in 150 cases and hepatomegaly occurred in 60 cases. One hundred and forty-nine cases (34%) had no symptoms at diagnosis and 145 cases (33.1%) confirmed by routine blood tests due to other diseases. The median platelet count at diagnosis was 1000 x 10(9)/L [(533 -3740) x 10(9)/L]. Bone marrow biopsy was performed in 255 cases which showed mainly increase of enlarged mature megakaryocytes with hyper-lobulated nuclei and local proliferation of reticular fiber was revealed in 51 cases. JAK2V617F mutation was detected in 90(78.9%) of 114 patients studied. Karyotype analysis was performed in 180 cases and 6 (3.3%) had clonal chromosomal aberrations. Two hundred and sixty-one patients were followed up over 12 months with a median of 60 months (range from 12 to 300 months). Seventeen cases (6.5%) evolved into marrow fibrosis (MF) and one case into polycythemia vera (PV). One case evolved into PV 6 years and then MF 20 years after diagnosis of ET. Three cases developed acute monocyte leukemia (M5), myelodysplastic syndrome (MDS) and multiple myeloma (MM), respectively.</p><p><b>CONCLUSIONS</b>ET is a chronic myeloproliferative disorder characterized predominantly by thrombocytosis and hemorrhage. The percentage of asymptomatic cases is high. The prognoses for most cases were good with a few cases may evolve into MF.</p>
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Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Estudios de Seguimiento , Pronóstico , Estudios Retrospectivos , Trombocitemia Esencial , Genética , PatologíaRESUMEN
<p><b>OBJECTIVE</b>To investigate the in vivo effect of modified platelet factor 4 (PF4)-p17-70 cDNA on tumor angiogenesis in nude mice.</p><p><b>METHODS</b>The p17-70 cDNA was cloned into the AdEasy system to transfect packing cell line 293 and produce viral particles encoding p17-70cDNA (Ad p17-70). The integration of p17-70 cDNA was confirmed by RT-PCR and the P17-40 peptide Western blot. The biological activity of purified recombinant adenovirus was determined by umbilical veinal endothelial cell proliferation assay in vitro and in vivo tumor angiogenesis suppression of nude mice bearing human head and neck carcinoma.</p><p><b>RESULTS</b>p17-70 significantly inhibited in vitro proliferation of endothelial cells being 58% lower than that of empty vector and reduced tumor volume in vivo. The tumor mass was (0.086 +/- 0.054) g, (0.171 +/- 0.076) g and (0.195 +/- 0.067) g, the tumor volume was (16.7 +/- 5.2) mm(3), (36.5 +/- 23.7) mm(3) and (41.5 +/- 12.2) mm(3) in p17-70 cDNA transfected group, empty vector group and PBS group, respectively. Immunohistochemical staining demonstrated a decreased number of blood vessels in the tumors.</p><p><b>CONCLUSION</b>P17-70 peptide mediated by adenoviral vector could inhibit the endothelial proliferation in vitro and the tumor growth in vivo.</p>
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Animales , Femenino , Humanos , Ratones , Adenoviridae , Genética , Proliferación Celular , Células Endoteliales , Biología Celular , Terapia Genética , Métodos , Vectores Genéticos , Ratones Endogámicos BALB C , Ratones Desnudos , Neoplasias Experimentales , Patología , Terapéutica , Neovascularización Patológica , Terapéutica , Factor Plaquetario 4 , Genética , Transfección , Venas Umbilicales , Biología CelularRESUMEN
<p><b>OBJECTIVE</b>To evaluate the clinical efficacy of mobilized autologous peripheral blood stem cells (PBSC) transplantation in a 48 years old patient with lower limb arteriosclerosis obliterans (ASO).</p><p><b>METHODS</b>rhG-CSF 600 micro g/d for 5 days to mobilize stem cells. On the fifth day, PBSC were collected with a Version 4 blood-cells separator. Three hours late, the PBSC were intramuscularly injected into the ischemic areas of the two lower limbs (3 x 10(9) cells per limb). The clinical and laboratory findings were monitored every week for 3 months. Forty-four days after the implantation, left lower limb with severe ASO was given an additional implantation of the same number of cells as the first time.</p><p><b>RESULTS</b>The peripheral blood CD(34)(+) cells were increased from 0.18% to 0.75% after 5 days of rhG-CSF mobilization. Three months after the first stem cell transplantation, severe pain lameness, local cool-feeling and ulcer were improved, and ABI increased from 0.49, 0.69 to 0.50, 0.85, the amplitude of blood flow and laser Doppler blood perfusion were also significantly improved (P < 0.01). At the same time, digital subtraction angiographic scores for new collateral vessel formation were showed as + 3(rich). No related complication or adverse effect were observed during the 3-month observation.</p><p><b>CONCLUSION</b>Transplantation of mobilized autologous PBSC might be a simple, safe, and effective method for the treatment of ASO.</p>