BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant sour...BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant source to trigger and sustain the pathophysiology has been proposed to come from the altered gut microbiota and chronic intestinal inflammation. In this case, our serendipitous finding provides convincing evidence that the persistently dysregulated autoimmunity may be generated, at least in a significant proposition of AA patients, by the altered gut microbiota and compromised intestinal epithelium.CASE SUMMARY A 30-year-old Chinese male patient with refractory severe AA experienced a 3-month-long febrile episode, and his fever was refractory to many kinds of injected broad-spectrum antibiotics. When presenting with abdominal cramps, he was prescribed oral mannitol and gentamycin to get rid of the gut infection. This treatment resulted in a quick resolution of the fever. Unanticipatedly, it also produced an excellent hematological response. He had undergone three episodes of recurrence within the one-year treatment, with each recurrence occurring 7-8 wk from the gastrointestinal inflammation eliminating preparations. However,subsequent treatments were able to produce subsequent remissions and consecutive treatments were successful in achieving durative hematological improvements, strongly indicating an etiological association between chronic gut inflammation and the development of AA. Interestingly, comorbid diseases superimposed on this patient(namely, psychiatric disorders, hypertension,insulin resistance, and renal dysfunction) were ameliorated together with the hematological improvements.CONCLUSION Chronic gut inflammation may be responsible for AA pathogenesis. The comorbidities and AA may share a common etiological association.展开更多
Chorioangioma is a tumor of vascular placental origin, the most frequent among primary placental non-trophoblastic tumors;however, it is found in about 1% of placental pathology case studies. Most of the chorioangioma...Chorioangioma is a tumor of vascular placental origin, the most frequent among primary placental non-trophoblastic tumors;however, it is found in about 1% of placental pathology case studies. Most of the chorioangiomas found are small, asymptomatic lesions that are only found postnatally after careful slicing of the placenta?[1]. Larger tumors, particularly those measuring more than 4 cm, are rarely seen in obstetric practice but are clinically significant. In this case report, we will show you the premature with large chorioangioma and we will tell the treatment we take to cure the baby successfully.展开更多
<strong>Introduction:</strong> Chronic renal failure (CRF) is defined as glomerular filtration rate (GFR) less than 60 ml/min/1.73m<sup>2</sup> for at least three (3) months. Anemia is one of i...<strong>Introduction:</strong> Chronic renal failure (CRF) is defined as glomerular filtration rate (GFR) less than 60 ml/min/1.73m<sup>2</sup> for at least three (3) months. Anemia is one of its most common complications. Anemia increases the risk factor for cardiovascular mortality by 18% per gram of hemoglobin loss. <strong>Objectives:</strong> To determine the prevalence and characteristics of this severe anemia, to determine the indications for transfusion, the complications related to this anemia, the evolution and the prognosis of these patients. <strong>Materials and Methods:</strong> This was a descriptive study with retrospective data collection over 18 months (January 1, 2017 to June 30, 2018) that included hospitalized CRF patients. Were included, non-dialyzed chronic renal failure patients with Hb ≤ 5 g/dl hospitalized during the said period. Not included were chronic renal failure patients with an Hb level ≥ 5 g/dl, those followed up and/or hospitalized outside the study period. <strong>Results:</strong> Among 1176 patients, 26 had severe anemia (Hb level ≤ 5 g/dl) on CRF, a prevalence of 2.21%. The mean age was 40 years ± 32.62 with extremes of 15 and 67 years. Seventeen women and 9 men. The etiology of chronic renal failure (CRF) was hypertensive vascular nephropathy in 50% of cases. CRF was end-stage in 18 patients (69.2%). The mean hemoglobin level was 4.10 g/dl ± 0.64 with extremes of 2 and 5 g/dl. The anemia was microcytic hypochromic in 50% and aregenerative (96.2%). The main symptoms were asthenia in 20 cases (76.9%), dizziness in 20 cases (76.9%), exertional dyspnea in 19 cases (73.1%). Signs of cardiac decompensation (n = 12) were jugular turgor 10 cases (38.5%), hepato-jugular reflux 06 cases (23.1%), mitral insufficiency murmur 06 cases (23.1%). The main complication was left ventricular hypertrophy 17 cases (77.3%). There was no correlation between anemia and sex (p = 0.291), age (p = 0.778), malaria (p = 0.158), etiology of CRF (p = 0.26). The evolution after treatment of anemia was favorable in 19 patients (73.1%), unfavorable in 02 patients (7.7%) and 05 deaths (19.2%). The deaths were of cardiovascular cause: left ventricular insufficiency 04 cases, stroke 01 case. <strong>Conclusion:</strong> Anemia is frequent in patients with chronic renal failure and remains an important risk factor for cardiovascular disease and poor general condition.展开更多
This study aimed to analyze the impact of anemia on mortality rate and nutritional recovery, during severely malnourished inpatient treatment. Material and Methods: This was a retrospective cohort study conducted from...This study aimed to analyze the impact of anemia on mortality rate and nutritional recovery, during severely malnourished inpatient treatment. Material and Methods: This was a retrospective cohort study conducted from data of severely malnourished children hospitalized at a feeding the rapeutic center. Pearson’s Chi square test, General linear model, Mortality relatives risks, Kaplan-Meir survival curves have been used. Results: At admission, 85.3% of included malnourished children had anemia (Hb ≤ 11 g/dl) and 10.6% severe anemic (Hb 6 g/dl). Mortality rate did not differed significantly from severely malnourished children with anemia (12.4%) and without anemia (22.2%), p = 0.12. Kaplan Meir survival curves did not differed significantly between the two groups, (p Log Rank = 0.11). From admission to discharge, anthropometric Z-scores means evolution did not differed significantly between severely malnourished children with and without anemia at admission. But anthropometric Z-scores means evolution differed significantly within each group’s subjects: WHZ (between groups: p = 0.74;within subjects: p 0.001), and WAZ (between groups: p = 0.54;within subjects: p 0.001). Conclusion: With a strong respect of current recommendation of anemia treatment of inpatient severely malnutrition management, there is no increased mortality rate in SAM anemic group;and nutritional recovery is significant within subjects of SAM anemic and non anemic children, without difference between groups.展开更多
THYMOMA, a relatively rare epithelial neoplasm with unique clinical and pathologic features, is the most usual diagnosis for a mass located in the mediastinum. It is often associated withautoimmune disorders. The myas...THYMOMA, a relatively rare epithelial neoplasm with unique clinical and pathologic features, is the most usual diagnosis for a mass located in the mediastinum. It is often associated withautoimmune disorders. The myastnema gravls ano pure red cell aplasia are the most common disorders, with the incidences of 40% and 5%, respectively, while the incidence of aplastic anemia is only about 0-1.4%. 1 Thymectomy is hard to perform on patients with severe aplastic anemia（SAA） due to severe pancytopenia.展开更多
Severe anemia during pregnancy remains an acute maternal health problem in low income countries and its management is a challenge. This study aimed to analyze the characteristics of hospitalized pregnant women with se...Severe anemia during pregnancy remains an acute maternal health problem in low income countries and its management is a challenge. This study aimed to analyze the characteristics of hospitalized pregnant women with severe anemia and issue of the management in a regional hospital of a low income country. Material and Methods: This was a retrospective descriptive study from January 1, 2007 to December 31, 2008, at a regional hospital maternity. Data of the 283 pregnancy severe anemia cases have been analyzed using usual Pearson’s Chi square test or Fisher’s exact test. Results: Of the 283 women with pregnancy severe anemia, 98% were illiterate, 98% were household women;majority were in the third trimester (64%);41% had no previous delivery;49% did not had antenatal care. There were no statistics association between hemoglobin values stage and gestational age, gravidity and parity. During the hospitalization, from the 283 women with pregnancy severe anemia, deliveries occurred on 22.6%;abortion on 1%. From the 64 deliveries, 45.3% were alive preterm birth, 20.3% preterm stillbirth, 25.0% at term birth and 9.4% at term stillbirth. Between the 45 alive babies, 91% were low birth weight. In global, from the 283 women, mortality rate was 2.8%. Conclusion:?Severe anemia during pregnancy results in maternal mortality, preterm, low birth and stillbirth even between hospitalized women. As most of the pregnancy anemia risk factors are chronic poverty related factors, intervention must be focused on prevention including health pregnant women iron supplementation and adequate nutritional diet promoting at both health facilities and community level.展开更多
Hematopoietic stem cell transplantation(HSCT)has emerged as a curative strategy for sickle cell anemia(SCA);it is necessary to find markers of SCA clinical severity to spare those SCA patients whose clinical course is...Hematopoietic stem cell transplantation(HSCT)has emerged as a curative strategy for sickle cell anemia(SCA);it is necessary to find markers of SCA clinical severity to spare those SCA patients whose clinical course is mild from the morbidity and mortality associated with HSCT. Haplotypes have been correlated with the severity of clinical manifestations in SCA patients, and fetal hemoglobin(HbF)and socioeconomic status(SeS)have also been described as negative factors. We studied these factors and their impact on clinical manifestations in a population of Southern Brazilian patients attending the Center for Sickle Cell Anemia at Hospital de Clínicas de Porto Alegre/RS, Brazil. Clinical severity was defined as two or more veno-occlusive episodes per year. The βS haplotypes were determined by PCR in 75 SCA patients. Among the 150 βS chromosomes analyzed, 99(66%)were identified as Bantu(Ban), 41(27%)asBenin(Ben), and 10(7%)as other haplotypes. Most patients in our sample(62.7%)belonged to lower SeS groups, precluding meaningful statistical analysis of SeS impact on clinical severity. There was no correlation between haplotypes or HbF level and SCA clinical severity. Gene polymorphisms and environmental issues have to be taken into consideration.展开更多
For:zPlastlc:,nemia(AA)seriousb 10()d dlselse,there are sever1 methods()ftreatment、sueh15 andr()gens,antitlly-mocyte globt一lin(ATG),eyelo、Por一nA(CYA),and bone Inarrow tr:,nsl)lantation(BMT).However, none excePt BM...For:zPlastlc:,nemia(AA)seriousb 10()d dlselse,there are sever1 methods()ftreatment、sueh15 andr()gens,antitlly-mocyte globt一lin(ATG),eyelo、Por一nA(CYA),and bone Inarrow tr:,nsl)lantation(BMT).However, none excePt BMT 15 veryhelpt’ul.l In ellin:l,ATG,eYA,:Ind BMTare not available.Clinie之11 Praetlce in thePast years has shown thnt trzditionalChir一ese medieine(TCM)15 very etetive inAA,2展开更多
Objective： To explore the prognostic factors for very severe aplastic anemia （VSAA） patients treated mainly with Chinese Kidney （Shen）-invigorating drugs （CKID） combined with anti-lymphocyte globulin （ALG） or...Objective： To explore the prognostic factors for very severe aplastic anemia （VSAA） patients treated mainly with Chinese Kidney （Shen）-invigorating drugs （CKID） combined with anti-lymphocyte globulin （ALG） or anti-thymocyte globulin （ATG）. Methods： Twenty-seven VSAh. patients were treated with CSID＋ALG/ ATG therapy in conjunction with cyclosporine A, androgen, hemopoietic growth factor, etc. The relationship of the effectiveness and some factors （age of patients, course of illness, blood and bone marrow figures, etc.） were analyzed. Results： In the 25 evaluated VSAA patients who had been followed up for over 1 year, 9 patients （36.0%） were basically cured, 5 （20.0%） remitted, 6 （24.0%） were markedly improved, and 5 （20.0%） were treated in vain, with the total effective rate of treatment being 80.0% （20/25）. Better clinical therapeutic effects were shown in patients newly diagnosed with VSAA, of male sex （P=0.037）, 〉20 years old （P=0.045）, with an illness course ≤ 1 month （,P=0.048）, with peripheral neutrophil count 〉0.1 × 10^9/L （P=0.023）, and with reticulocyte count 〉10 × 10^9/L （P=0.002）. Platelet count （P=0.620） and bone marrow lymphocyte percentage （P=0.736） showed no correlation with the therapeutic effectiveness. Multi-factor analysis by the Kaplan-Meier procedure on the factors influencing survival showed that rather longer survival times occurred in patients 〉 20 years old, with peripheral neutrophil count ≤〈0.1 × 10^9/L, reticulocyte count ≤10 × 10^9/L, and platelet count 〉 10 × 10^9/L （allP=0.0001）. Bone marrow lymphocyte percentage and the initiation time of ALG/ATG application （from onset of the illness） showed no significant influence on patients＇ survival time （P=0.085 and P=0.935, respectively）. Conclusions： CSKD＋ALG/ATG therapy for treatment of VSAA could enhance the current clinical therapeutic effects and elevate patients＇ survival rate. Conditions including male sex, age 〉20 years, illness course ≤1 month, neutrophil count 〉0.1× 10^9/L, and reticulocyte count 〉10 × 10^9/L are the likely effective indices for predicting favorable therapeutic effectiveness in newly diagnosed VSAA patients.展开更多
Objective：To determine the effect of combined treatment with Chinese medicine（CM） and allogeneic hematopoietic stem ceil transplantation（allo-HSCT） on patients with severe aplastic anemia（SAA）.Methods：Eleven p...Objective：To determine the effect of combined treatment with Chinese medicine（CM） and allogeneic hematopoietic stem ceil transplantation（allo-HSCT） on patients with severe aplastic anemia（SAA）.Methods：Eleven patients were treated with CM plus ailo-HSCT.Nine patients received a conditioning regimen consisting of fludarabine（Flu）,anti-thymocyte globulin（pig ALG）,or anti-lymphocyte globulin（Rabbit ATG） and cyclophosphamide（CY）,and two patients received pig ALG and CY.All patients were treated with Kidney（Shen）-reinforcing,blood-activating,and stasis-removing（KBS） herbal preparation beginning at 1 week before transplantation and ending at 8 weeks after transplantation.Chimerism status was assessed by analyzing short tandem repeat（STR） polymorphisms.Results：All patients recovered hematopoietic function and none had graft failure.The median number of days required for the absolute neutrophil count（ANC） increased to 〉0.5× 10~9/L was 15 days（12-22 days） and for spontaneous platelet recovery to 〉20×10~9/L without post-transplantation transfusion was 17 days（15-27 days）.Nine patients were longterm survivors and achieved full donor chimerism.The overall cumulative incidence of acute graft versus host disease（GVHD） grades Ⅰ-Ⅱ and Ⅲ-Ⅳ was 18.2%（2/11） and 9.1%（1 /11）,respectively.The overall accumulated incidence of chronic GVHD was 27.3%and all patients had limited chronic GVHD.At a median follow-up time of 32 months（range：12-97 months）,9 patients were still alive.The estimated 5-year overall survival（OS） rate was 81.8%.The incidence of treatment-related mortality,2-year post-transplantation,was 18.2%.Two patients died from GVHD after transplantation.Conclusion：Treatment with the KBS formulation may reduce the rate of graft failure and treatmentrelated mortality and improve the rate of OS in SAA patients with allo-HSCT.展开更多
Severe aplastic anemia II(SAA-II)progresses from non-severe aplastic anemia(NSAA).The unavailability of efficacious treatment has prompted the need for haploidentical bone marrow transplantation(haplo-BMT)in patients ...Severe aplastic anemia II(SAA-II)progresses from non-severe aplastic anemia(NSAA).The unavailability of efficacious treatment has prompted the need for haploidentical bone marrow transplantation(haplo-BMT)in patients lacking a human leukocyte antigen(HLA)-matched donor.This study aimed to investigate the efficacy of haplo-BMT for patients with SAA-II.Twenty-two patients were included and followed up,and FLU/BU/CY/ATG was used as conditioning regimen.Among these patients,21 were successfully engrafted,19 of whom survived after haplo-BMT.Four patients experienced grade II–IV aGvHD,including two with grade III–IV aGvHD.Six patients experienced chronic GvHD,among whom four were mild and two were moderate.Twelve patients experienced infections during BMT.One was diagnosed with post-transplant lymphoproliferative disorder and one with probable EBV disease,and both recovered after rituximab infusion.Haplo-BMT achieved 3-year overall survival and disease-free survival rate of 86.4%±0.73%after a median follow-up of 42 months,indicating its effectiveness as a salvage therapy.These promising outcomes may support haplo-BMT as an alternative treatment strategy for patients with SAA-II lacking HLA-matched donors.展开更多
Dear Editor,Haploidentical allogeneic hematopoietic stem cell transplantation(haplo-HSCT),a curative therapy for severe aplastic anemia(SAA)patients,has been used clinically for decades.Two models,not involving ex vit...Dear Editor,Haploidentical allogeneic hematopoietic stem cell transplantation(haplo-HSCT),a curative therapy for severe aplastic anemia(SAA)patients,has been used clinically for decades.Two models,not involving ex vitro T-cell depletion,have been adopted for haplo-HSCT in patients with SAA.The first is referred to as the"Beijing protocol"(Xu et al.,2017),and comprises a conditioning regimen using busulfex(BU),cyclophosphamide(CY).展开更多
文摘BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant source to trigger and sustain the pathophysiology has been proposed to come from the altered gut microbiota and chronic intestinal inflammation. In this case, our serendipitous finding provides convincing evidence that the persistently dysregulated autoimmunity may be generated, at least in a significant proposition of AA patients, by the altered gut microbiota and compromised intestinal epithelium.CASE SUMMARY A 30-year-old Chinese male patient with refractory severe AA experienced a 3-month-long febrile episode, and his fever was refractory to many kinds of injected broad-spectrum antibiotics. When presenting with abdominal cramps, he was prescribed oral mannitol and gentamycin to get rid of the gut infection. This treatment resulted in a quick resolution of the fever. Unanticipatedly, it also produced an excellent hematological response. He had undergone three episodes of recurrence within the one-year treatment, with each recurrence occurring 7-8 wk from the gastrointestinal inflammation eliminating preparations. However,subsequent treatments were able to produce subsequent remissions and consecutive treatments were successful in achieving durative hematological improvements, strongly indicating an etiological association between chronic gut inflammation and the development of AA. Interestingly, comorbid diseases superimposed on this patient(namely, psychiatric disorders, hypertension,insulin resistance, and renal dysfunction) were ameliorated together with the hematological improvements.CONCLUSION Chronic gut inflammation may be responsible for AA pathogenesis. The comorbidities and AA may share a common etiological association.
文摘Chorioangioma is a tumor of vascular placental origin, the most frequent among primary placental non-trophoblastic tumors;however, it is found in about 1% of placental pathology case studies. Most of the chorioangiomas found are small, asymptomatic lesions that are only found postnatally after careful slicing of the placenta?[1]. Larger tumors, particularly those measuring more than 4 cm, are rarely seen in obstetric practice but are clinically significant. In this case report, we will show you the premature with large chorioangioma and we will tell the treatment we take to cure the baby successfully.
文摘<strong>Introduction:</strong> Chronic renal failure (CRF) is defined as glomerular filtration rate (GFR) less than 60 ml/min/1.73m<sup>2</sup> for at least three (3) months. Anemia is one of its most common complications. Anemia increases the risk factor for cardiovascular mortality by 18% per gram of hemoglobin loss. <strong>Objectives:</strong> To determine the prevalence and characteristics of this severe anemia, to determine the indications for transfusion, the complications related to this anemia, the evolution and the prognosis of these patients. <strong>Materials and Methods:</strong> This was a descriptive study with retrospective data collection over 18 months (January 1, 2017 to June 30, 2018) that included hospitalized CRF patients. Were included, non-dialyzed chronic renal failure patients with Hb ≤ 5 g/dl hospitalized during the said period. Not included were chronic renal failure patients with an Hb level ≥ 5 g/dl, those followed up and/or hospitalized outside the study period. <strong>Results:</strong> Among 1176 patients, 26 had severe anemia (Hb level ≤ 5 g/dl) on CRF, a prevalence of 2.21%. The mean age was 40 years ± 32.62 with extremes of 15 and 67 years. Seventeen women and 9 men. The etiology of chronic renal failure (CRF) was hypertensive vascular nephropathy in 50% of cases. CRF was end-stage in 18 patients (69.2%). The mean hemoglobin level was 4.10 g/dl ± 0.64 with extremes of 2 and 5 g/dl. The anemia was microcytic hypochromic in 50% and aregenerative (96.2%). The main symptoms were asthenia in 20 cases (76.9%), dizziness in 20 cases (76.9%), exertional dyspnea in 19 cases (73.1%). Signs of cardiac decompensation (n = 12) were jugular turgor 10 cases (38.5%), hepato-jugular reflux 06 cases (23.1%), mitral insufficiency murmur 06 cases (23.1%). The main complication was left ventricular hypertrophy 17 cases (77.3%). There was no correlation between anemia and sex (p = 0.291), age (p = 0.778), malaria (p = 0.158), etiology of CRF (p = 0.26). The evolution after treatment of anemia was favorable in 19 patients (73.1%), unfavorable in 02 patients (7.7%) and 05 deaths (19.2%). The deaths were of cardiovascular cause: left ventricular insufficiency 04 cases, stroke 01 case. <strong>Conclusion:</strong> Anemia is frequent in patients with chronic renal failure and remains an important risk factor for cardiovascular disease and poor general condition.
文摘This study aimed to analyze the impact of anemia on mortality rate and nutritional recovery, during severely malnourished inpatient treatment. Material and Methods: This was a retrospective cohort study conducted from data of severely malnourished children hospitalized at a feeding the rapeutic center. Pearson’s Chi square test, General linear model, Mortality relatives risks, Kaplan-Meir survival curves have been used. Results: At admission, 85.3% of included malnourished children had anemia (Hb ≤ 11 g/dl) and 10.6% severe anemic (Hb 6 g/dl). Mortality rate did not differed significantly from severely malnourished children with anemia (12.4%) and without anemia (22.2%), p = 0.12. Kaplan Meir survival curves did not differed significantly between the two groups, (p Log Rank = 0.11). From admission to discharge, anthropometric Z-scores means evolution did not differed significantly between severely malnourished children with and without anemia at admission. But anthropometric Z-scores means evolution differed significantly within each group’s subjects: WHZ (between groups: p = 0.74;within subjects: p 0.001), and WAZ (between groups: p = 0.54;within subjects: p 0.001). Conclusion: With a strong respect of current recommendation of anemia treatment of inpatient severely malnutrition management, there is no increased mortality rate in SAM anemic group;and nutritional recovery is significant within subjects of SAM anemic and non anemic children, without difference between groups.
基金Supported by the Key Provincial Talents Program of Jiangsu Province(H201126)the Natural Science Fund for Colleges and Universities of Jiangsu Province(09KJB320015)+1 种基金Key Projects in the National Science&Technology Pillar Program(2008BAI61B02 and 2008ZX09312-026)the Priority Academic Program Development of Jiangsu Higher Education Institutions
文摘THYMOMA, a relatively rare epithelial neoplasm with unique clinical and pathologic features, is the most usual diagnosis for a mass located in the mediastinum. It is often associated withautoimmune disorders. The myastnema gravls ano pure red cell aplasia are the most common disorders, with the incidences of 40% and 5%, respectively, while the incidence of aplastic anemia is only about 0-1.4%. 1 Thymectomy is hard to perform on patients with severe aplastic anemia（SAA） due to severe pancytopenia.
文摘Severe anemia during pregnancy remains an acute maternal health problem in low income countries and its management is a challenge. This study aimed to analyze the characteristics of hospitalized pregnant women with severe anemia and issue of the management in a regional hospital of a low income country. Material and Methods: This was a retrospective descriptive study from January 1, 2007 to December 31, 2008, at a regional hospital maternity. Data of the 283 pregnancy severe anemia cases have been analyzed using usual Pearson’s Chi square test or Fisher’s exact test. Results: Of the 283 women with pregnancy severe anemia, 98% were illiterate, 98% were household women;majority were in the third trimester (64%);41% had no previous delivery;49% did not had antenatal care. There were no statistics association between hemoglobin values stage and gestational age, gravidity and parity. During the hospitalization, from the 283 women with pregnancy severe anemia, deliveries occurred on 22.6%;abortion on 1%. From the 64 deliveries, 45.3% were alive preterm birth, 20.3% preterm stillbirth, 25.0% at term birth and 9.4% at term stillbirth. Between the 45 alive babies, 91% were low birth weight. In global, from the 283 women, mortality rate was 2.8%. Conclusion:?Severe anemia during pregnancy results in maternal mortality, preterm, low birth and stillbirth even between hospitalized women. As most of the pregnancy anemia risk factors are chronic poverty related factors, intervention must be focused on prevention including health pregnant women iron supplementation and adequate nutritional diet promoting at both health facilities and community level.
基金Research and Event Incentive Fund of Hospital de Clinicas de Porto Alegre (FIPEHCPA)
文摘Hematopoietic stem cell transplantation(HSCT)has emerged as a curative strategy for sickle cell anemia(SCA);it is necessary to find markers of SCA clinical severity to spare those SCA patients whose clinical course is mild from the morbidity and mortality associated with HSCT. Haplotypes have been correlated with the severity of clinical manifestations in SCA patients, and fetal hemoglobin(HbF)and socioeconomic status(SeS)have also been described as negative factors. We studied these factors and their impact on clinical manifestations in a population of Southern Brazilian patients attending the Center for Sickle Cell Anemia at Hospital de Clínicas de Porto Alegre/RS, Brazil. Clinical severity was defined as two or more veno-occlusive episodes per year. The βS haplotypes were determined by PCR in 75 SCA patients. Among the 150 βS chromosomes analyzed, 99(66%)were identified as Bantu(Ban), 41(27%)asBenin(Ben), and 10(7%)as other haplotypes. Most patients in our sample(62.7%)belonged to lower SeS groups, precluding meaningful statistical analysis of SeS impact on clinical severity. There was no correlation between haplotypes or HbF level and SCA clinical severity. Gene polymorphisms and environmental issues have to be taken into consideration.
基金Supported by the National Natural Science Foundation of China
文摘For:zPlastlc:,nemia(AA)seriousb 10()d dlselse,there are sever1 methods()ftreatment、sueh15 andr()gens,antitlly-mocyte globt一lin(ATG),eyelo、Por一nA(CYA),and bone Inarrow tr:,nsl)lantation(BMT).However, none excePt BMT 15 veryhelpt’ul.l In ellin:l,ATG,eYA,:Ind BMTare not available.Clinie之11 Praetlce in thePast years has shown thnt trzditionalChir一ese medieine(TCM)15 very etetive inAA,2
文摘Objective： To explore the prognostic factors for very severe aplastic anemia （VSAA） patients treated mainly with Chinese Kidney （Shen）-invigorating drugs （CKID） combined with anti-lymphocyte globulin （ALG） or anti-thymocyte globulin （ATG）. Methods： Twenty-seven VSAh. patients were treated with CSID＋ALG/ ATG therapy in conjunction with cyclosporine A, androgen, hemopoietic growth factor, etc. The relationship of the effectiveness and some factors （age of patients, course of illness, blood and bone marrow figures, etc.） were analyzed. Results： In the 25 evaluated VSAA patients who had been followed up for over 1 year, 9 patients （36.0%） were basically cured, 5 （20.0%） remitted, 6 （24.0%） were markedly improved, and 5 （20.0%） were treated in vain, with the total effective rate of treatment being 80.0% （20/25）. Better clinical therapeutic effects were shown in patients newly diagnosed with VSAA, of male sex （P=0.037）, 〉20 years old （P=0.045）, with an illness course ≤ 1 month （,P=0.048）, with peripheral neutrophil count 〉0.1 × 10^9/L （P=0.023）, and with reticulocyte count 〉10 × 10^9/L （P=0.002）. Platelet count （P=0.620） and bone marrow lymphocyte percentage （P=0.736） showed no correlation with the therapeutic effectiveness. Multi-factor analysis by the Kaplan-Meier procedure on the factors influencing survival showed that rather longer survival times occurred in patients 〉 20 years old, with peripheral neutrophil count ≤〈0.1 × 10^9/L, reticulocyte count ≤10 × 10^9/L, and platelet count 〉 10 × 10^9/L （allP=0.0001）. Bone marrow lymphocyte percentage and the initiation time of ALG/ATG application （from onset of the illness） showed no significant influence on patients＇ survival time （P=0.085 and P=0.935, respectively）. Conclusions： CSKD＋ALG/ATG therapy for treatment of VSAA could enhance the current clinical therapeutic effects and elevate patients＇ survival rate. Conditions including male sex, age 〉20 years, illness course ≤1 month, neutrophil count 〉0.1× 10^9/L, and reticulocyte count 〉10 × 10^9/L are the likely effective indices for predicting favorable therapeutic effectiveness in newly diagnosed VSAA patients.
基金Supported by State Administration of Traditional Chinese Medicine(No.JDZX2012178)2011 Special Research Funds for the Traditional Chinese Medicine Industry(No.201107001)Zhejiang Provincial Traditional Chinese Medicine Administration Bureau Program(No.2007CA025)
文摘Objective：To determine the effect of combined treatment with Chinese medicine（CM） and allogeneic hematopoietic stem ceil transplantation（allo-HSCT） on patients with severe aplastic anemia（SAA）.Methods：Eleven patients were treated with CM plus ailo-HSCT.Nine patients received a conditioning regimen consisting of fludarabine（Flu）,anti-thymocyte globulin（pig ALG）,or anti-lymphocyte globulin（Rabbit ATG） and cyclophosphamide（CY）,and two patients received pig ALG and CY.All patients were treated with Kidney（Shen）-reinforcing,blood-activating,and stasis-removing（KBS） herbal preparation beginning at 1 week before transplantation and ending at 8 weeks after transplantation.Chimerism status was assessed by analyzing short tandem repeat（STR） polymorphisms.Results：All patients recovered hematopoietic function and none had graft failure.The median number of days required for the absolute neutrophil count（ANC） increased to 〉0.5× 10~9/L was 15 days（12-22 days） and for spontaneous platelet recovery to 〉20×10~9/L without post-transplantation transfusion was 17 days（15-27 days）.Nine patients were longterm survivors and achieved full donor chimerism.The overall cumulative incidence of acute graft versus host disease（GVHD） grades Ⅰ-Ⅱ and Ⅲ-Ⅳ was 18.2%（2/11） and 9.1%（1 /11）,respectively.The overall accumulated incidence of chronic GVHD was 27.3%and all patients had limited chronic GVHD.At a median follow-up time of 32 months（range：12-97 months）,9 patients were still alive.The estimated 5-year overall survival（OS） rate was 81.8%.The incidence of treatment-related mortality,2-year post-transplantation,was 18.2%.Two patients died from GVHD after transplantation.Conclusion：Treatment with the KBS formulation may reduce the rate of graft failure and treatmentrelated mortality and improve the rate of OS in SAA patients with allo-HSCT.
基金This work was supported by grants from the National Natural Science Foundation of China(No.81570124 to Jinsong Yan,No.81702683 to Jiajun Xie,No.81773389 to Jing Shao)Science and Technology Innovation Leading Talent Program of Liaoning Province(No.XLYC1902036 to Jinsong Yan)+5 种基金Basic Research on the Application of Dalian Innovation Fund(No.2019J12SN56 to Jinsong Yan)Key R&D projects in Liaoning Province(No.2019JH8/10300027 to Jinsong Yan)Key Project of the Educational Department of Liaoning Province(No.LZ2020003 to Jinsong Yan)the Capital Health Research and Development of Special Project(No.2014-2-5122 to Hengxiang Wang)the Beijing Municipal Science and Technology Project(No.Z151100004015016 to Hengxiang Wang)the Dalian Medical Scientific Research Project(No.1712040 to Yan Yang)。
文摘Severe aplastic anemia II(SAA-II)progresses from non-severe aplastic anemia(NSAA).The unavailability of efficacious treatment has prompted the need for haploidentical bone marrow transplantation(haplo-BMT)in patients lacking a human leukocyte antigen(HLA)-matched donor.This study aimed to investigate the efficacy of haplo-BMT for patients with SAA-II.Twenty-two patients were included and followed up,and FLU/BU/CY/ATG was used as conditioning regimen.Among these patients,21 were successfully engrafted,19 of whom survived after haplo-BMT.Four patients experienced grade II–IV aGvHD,including two with grade III–IV aGvHD.Six patients experienced chronic GvHD,among whom four were mild and two were moderate.Twelve patients experienced infections during BMT.One was diagnosed with post-transplant lymphoproliferative disorder and one with probable EBV disease,and both recovered after rituximab infusion.Haplo-BMT achieved 3-year overall survival and disease-free survival rate of 86.4%±0.73%after a median follow-up of 42 months,indicating its effectiveness as a salvage therapy.These promising outcomes may support haplo-BMT as an alternative treatment strategy for patients with SAA-II lacking HLA-matched donors.
文摘Dear Editor,Haploidentical allogeneic hematopoietic stem cell transplantation(haplo-HSCT),a curative therapy for severe aplastic anemia(SAA)patients,has been used clinically for decades.Two models,not involving ex vitro T-cell depletion,have been adopted for haplo-HSCT in patients with SAA.The first is referred to as the"Beijing protocol"(Xu et al.,2017),and comprises a conditioning regimen using busulfex(BU),cyclophosphamide(CY).
