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Background@#Rixubis (recombinant factor IX, nonacog gamma) is indicated for the control and prevention of bleeding episodes, perioperative management, and routine prophylaxis in hemophilia B patients. This real-world, postmarketing surveillance study aimed to evaluate the safety and effectiveness of Rixubis in adult and pediatric hemophilia B patients in South Korea. @*Methods@#This prospective, observational, multicenter study (clinicaltrials.gov identifier: NCT029 22231) was conducted in hemophilia B patients between April 2015 and April 2019, who were observed for up to 6 months after the initiation of Rixubis treatment. Safety was evaluated based on the number and severity of adverse events (AEs) and serious AEs (SAEs). Hemostatic effectiveness was assessed by physicians and patients by using a four-point scale and rated as excellent, good, fair, or no response based on treatment type. @*Results@#In all, 58 patients were enrolled from four centers by seven physicians during the study period. The safety and effectiveness analysis sets included 57 and 54 patients, respectively. Overall, 11 AEs were reported in eight patients (14.0%), of which three were SAEs and occurred in three patients (5.3%). All 11 AEs were reported as unexpected and mild in severity, with no anaphylactic reaction, and 10 AEs (90.9%) resolved. The majority of AEs (10) were unrelated to Rixubis. Of the 142 hemostatic effectiveness assessments, 123 (86.6%) were reported as good or excellent. @*Conclusion@#Rixubis demonstrated an acceptable safety and effectiveness profile in the treatment of bleeding, perioperative management, and prophylaxis in hemophilia B patients in a real-world setting in South Korea.
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Background@#Rixubis (recombinant factor IX, nonacog gamma) is indicated for the control and prevention of bleeding episodes, perioperative management, and routine prophylaxis in hemophilia B patients. This real-world, postmarketing surveillance study aimed to evaluate the safety and effectiveness of Rixubis in adult and pediatric hemophilia B patients in South Korea. @*Methods@#This prospective, observational, multicenter study (clinicaltrials.gov identifier: NCT029 22231) was conducted in hemophilia B patients between April 2015 and April 2019, who were observed for up to 6 months after the initiation of Rixubis treatment. Safety was evaluated based on the number and severity of adverse events (AEs) and serious AEs (SAEs). Hemostatic effectiveness was assessed by physicians and patients by using a four-point scale and rated as excellent, good, fair, or no response based on treatment type. @*Results@#In all, 58 patients were enrolled from four centers by seven physicians during the study period. The safety and effectiveness analysis sets included 57 and 54 patients, respectively. Overall, 11 AEs were reported in eight patients (14.0%), of which three were SAEs and occurred in three patients (5.3%). All 11 AEs were reported as unexpected and mild in severity, with no anaphylactic reaction, and 10 AEs (90.9%) resolved. The majority of AEs (10) were unrelated to Rixubis. Of the 142 hemostatic effectiveness assessments, 123 (86.6%) were reported as good or excellent. @*Conclusion@#Rixubis demonstrated an acceptable safety and effectiveness profile in the treatment of bleeding, perioperative management, and prophylaxis in hemophilia B patients in a real-world setting in South Korea.
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BACKGROUND: This study aimed to survey the clinical spectrum of diffuse large B-cell lymphoma (DLBCL) in terms of epidemiology, pathologic subtypes, stage, and prognostic index as well as treatment outcomes. METHODS: In 2007-2008, 13 university hospitals evenly distributed in the Korean peninsula contributed to the online registry of DLBCL at www.lymphoma.or.kr and filed a total of 1,665 cases of DLBCL recorded since 1990. RESULTS: Our analysis showed a higher prevalence of DLBCL in male than in female individuals (M:F=958:707), and extranodal disease was more common than primary nodular disease (53% vs. 47%). Among the 1,544 patients who had been treated with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or rituximab-CHOP (R-CHOP) therapy with or without radiation, 993 (63.9%) were alive, with 80% free of disease, 417 were dead (26.8%), with 13% free of disease, and 144 (9.3%) were lost to follow-up, with 23% free of disease. Age below 60 years, stage at diagnosis, international prognostic index (IPI) score regardless of age, and addition of rituximab to CHOP therapy in low- and low-intermediate-risk groups according to IPI scores significantly increased survival duration. CONCLUSION: The epidemiology, clinical spectrum, and biological behavior of DLBCL in Korea are similar to those observed in Western countries, and the advent of rituximab improved survival.
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Femenino , Humanos , Masculino , Anticuerpos Monoclonales de Origen Murino , Protocolos de Quimioterapia Combinada Antineoplásica , Linfocitos B , Ciclofosfamida , Doxorrubicina , Hospitales Universitarios , Corea (Geográfico) , Perdida de Seguimiento , Linfoma , Linfoma de Células B , Prednisolona , Prevalencia , Vincristina , RituximabRESUMEN
Although uncommon, acquired hemophilia A (HA) is associated with a high rate of mortality due to severe bleeding. In spite of many hypotheses regarding the cause of acquired HA, there is as yet no established theory. In this study, we investigated the possibility that mutation(s) in the F8 gene may be correlated with the development of inhibitory autoantibodies. Direct sequencing analysis was performed on all 26 exons of the F8 gene of 2 patients exhibiting acquired HA. Both patients were found to share a common point mutation (c.8899G>A) in the 3'-untranslated region (3'-UTR) of exon 26. This is the first report on the genotyping of F8 in the context of acquired HA.
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Humanos , Autoanticuerpos , Exones , Hemofilia A , Hemorragia , Mutación PuntualRESUMEN
Dermatomyositis (DM) is a rare and idiopathic inflammatory myopathy with characteristic cutaneous manifestations. There is a well-recognized association between DM and cancers. In Korea, several DM cases have been reported to be associated with stomach cancer, breast cancer, acute lymphoblastic leukemia, lung cancer, and tonsil cancer. However, an association between DM and lymphoma in Korea has not been reported up to now. We report a case of DM who developed diffuse large B-cell lymphoma 1 year and 8 months later.
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Neoplasias de la Mama , Dermatomiositis , Corea (Geográfico) , Neoplasias Pulmonares , Linfoma , Linfoma de Células B , Miositis , Leucemia-Linfoma Linfoblástico de Células Precursoras , Neoplasias Gástricas , Neoplasias TonsilaresRESUMEN
Many AML-associated chromosomal abnormalities, such as t(8;21), t(15;17), inv(16), t(9;11), t(9;22) and t(6;9) are well known. The chromosomal aberration of t(16;21)(p11;q22) in AML is rare and it is known to be associated with poor prognosis, young age (median age, 22 yr), and involvement of various subtypes of the French-American-British classification. We report here 2 AML patients with t(16;21)(p11;q22), proved by conventional cytogenetics and/or reverse transcription (RT)-PCR. Erythrophagocytosis by leukemic blasts was observed in both of the cases. One patient was a 24 yr-old male with acute myelomonocytic leukemia. His karyotype was 46,XY,t(16;21)(p11;q22),del(18)(p11.2) and RT-PCR revealed the TLS/FUS-ERG fusion transcripts. Although he received allogeneic peripheral blood stem cell transplantation after the first remission, he died 9 months after the initial diagnosis due to relapse of the disease and graft-versus-host disease. The other patient was a 72 yr-old male with acute myeloid leukemia without maturation. His karyotype was 45,XY,-16,add(21)(q22) and the presence of t(16;21)(p11;q22) was detected by RT-PCR. He was transferred to another hospital with no more follow-up. We suggest that the presence of t(16;21)(p11;q22) and/or TLS/FUS-ERG fusion transcripts has to be considered in cases of AML with erythrophagocytosis.
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Anciano , Humanos , Masculino , Adulto Joven , Cromosomas Humanos Par 16/genética , Cromosomas Humanos Par 22/genética , Enfermedad Injerto contra Huésped/diagnóstico , Cariotipificación , Leucemia Mieloide Aguda/diagnóstico , Proteínas de Fusión Oncogénica/genética , Proteína FUS de Unión a ARN/genética , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Translocación GenéticaRESUMEN
BACKGROUND: On performing umbilical cord blood (UCB) transplantation, faster engraftment may lead better clinical outcome. Because transplanted viable cell count in UCB is related to the engraftment, accurate evaluation of viability of CD34+cells in cryopreserved UCB has clinical implication. We examined the difference in viability of cells in cryopreserved UCB according to the duration of cryopreservation and different methods. METHODS: A total of 60 UCB samples which were cryopreserved for 1 to 4 years were used in this study. Viability of cryopreserved cells were examined with trypan blue exclusion assay, DNA contents analysis, caspase-3 activation test, intracellular esterase activity and Annexin-V/PI staining. RESULTS: After thawing the cryopreserved UCB, 89% of the total MNCs and 84% of CD34+cells were viable as identified by trypan blue exclusion assay. In the CD34+cell population, the cell death rate was found to be 47% by Annexin-V/PI staining and less than 5% by DNA contents analysis. However, cspase-3 activity failed to document apoptosis. The intracellular esterase activity test also showed a cell death rate of about 10~20% at 2, 4, and 6 hours after thawing. CONCLUSION: Viable cells in UCB should be measured by several compensatory techniques rather than a single method. Discordance among Annexin-V/PI staining versus trypan blue exclusion, DNA contents analysis, and the caspase-3 activation test or intracellular esterase activity should be clarified in order to apply these techniques for actual cord blood transplantation.
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Apoptosis , Caspasa 3 , Recuento de Células , Muerte Celular , Criopreservación , Diminazeno , ADN , Sangre Fetal , Trasplantes , Azul de TripanoRESUMEN
BACKGROUND: Acute leukemias co-expressing myeloid and lymphoid antigens but does not meet the criteria for biphenotypic acute leukemia (BAL) is common, however its clinical significance is not fully defined. METHODS: In this study, clinical features of 68 co-expressing (myeloid and lymphoid) acute leukemias diagnosed between January 2000 and December 2006 were studied and compared with those of a control group of patients (pure AML or ALL). RESULTS: Age, gender, initial Lactate dehydrogenase (LDH) level and cytogenetics were not different between the co-expressing group and the control group. But, the initial bone marrow blast percent was significantly higher in the co-expressing group (70% vs. 54.5%, P=0.003). Fifty five percent (16/29) of ALL and 30% (52/172) of AML patients showed myeloid and lymphoid markers concomitantly. The lymphoid antigen positive AML (Ly+AML) patients showed significantly shorter survival rates than pure AML patients (4 year survival rate, 17.6% vs. 45.6%, P=0.002). However hematopoietic stem cell transplantation (HST) abrogated the difference (4 year survival rate, 54.7% vs. 50.6%, P=0.894). In ALL patients, survival rate was not affected by myeloid antigen co-expression (4 year survival rate 26.1% vs. 20%, P=0.954). CONCLUSION: Co-expression of lymphoid markers in AML should be regarded as a poor prognostic factor and more aggressive treatment such as HST should be considered.
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Humanos , Médula Ósea , Citogenética , Trasplante de Células Madre Hematopoyéticas , Inmunofenotipificación , L-Lactato Deshidrogenasa , Leucemia , Leucemia Bifenotípica Aguda , Pronóstico , Tasa de SupervivenciaRESUMEN
BALT(bronchial associated lymphoid tissue) lymphomas are a distinct subgroup of low-grade B-cell extranodal non-Hodgkin's lymphoma, which are classified as a marginal-zone lymphomas. The majority of the patients are asymptomatic or their pulmonary lesions is often discovered incidentally on a routine chest radiograph. A 50-year-old man was admitted for an the evaluation of cough, dyspnea and fever. His chest CT showed ground glass appearance with interlobular septal thickening in both lower lobes, right middle lobe and left lingular division. He had been initially diagnosed with lipoid pneumonia and was kept under observation. However, his chest lesion showed continuous progression and a video-associated thoracoscopy was performed His pulmonary lesion was confirmed histologically to be a BALT(bronchial associated lymphoid tissue) lymphoma. We report a case of a BALT lymphoma, which was initially misdiagnosed as lipoid pneumonia.
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Humanos , Persona de Mediana Edad , Linfocitos B , Tos , Disnea , Fiebre , Vidrio , Tejido Linfoide , Linfoma , Linfoma de Células B , Linfoma no Hodgkin , Neumonía , Radiografía Torácica , Toracoscopía , Tórax , Tomografía Computarizada por Rayos XRESUMEN
BALT(bronchial associated lymphoid tissue) lymphomas are a distinct subgroup of low-grade B-cell extranodal non-Hodgkin's lymphoma, which are classified as a marginal-zone lymphomas. The majority of the patients are asymptomatic or their pulmonary lesions is often discovered incidentally on a routine chest radiograph. A 50-year-old man was admitted for an the evaluation of cough, dyspnea and fever. His chest CT showed ground glass appearance with interlobular septal thickening in both lower lobes, right middle lobe and left lingular division. He had been initially diagnosed with lipoid pneumonia and was kept under observation. However, his chest lesion showed continuous progression and a video-associated thoracoscopy was performed His pulmonary lesion was confirmed histologically to be a BALT(bronchial associated lymphoid tissue) lymphoma. We report a case of a BALT lymphoma, which was initially misdiagnosed as lipoid pneumonia.
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Humanos , Persona de Mediana Edad , Linfocitos B , Tos , Disnea , Fiebre , Vidrio , Tejido Linfoide , Linfoma , Linfoma de Células B , Linfoma no Hodgkin , Neumonía , Radiografía Torácica , Toracoscopía , Tórax , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: It is still obscure how dendritic cells (DCs) can orchestrate whole immune reactions according to the host age. We studied changes of murine splenic DCs after total body irradiation (TBI), with regards to age. METHODS: Young (8~14 wk) and old (12~16 mo) C57Bl/6 mice were irradiated with a dose of 1,100 cGy and were assessed 6 h later for phenotypic and functional changes of the DCs. The mean fluorescence intensities and cytokine producing cell proportions were analyzed with the student's t-test. RESULTS: Interleukin-12 (IL-12), interferon (IFN gamma) and tumor necrosis factor (TNF alpha) producing classical DCs (cDCs) were more numerous in the young untreated mice than in the old mice. However, the number of these cells decreased in the young mice and increased in the old mice after TBI. IL-12, IFN gamma and TNF alpha producing plasmacytoid DCs (pDCs) were more frequent in the old mice than in the young mice before TBI both mice showed an increased frequency of cells producing these cytokines after TBI. Overall, the highest numbers of cDCs and pDCs producing IL-12, IFN gamma and TNF alpha were present in the old mice after TBI. In both the cDC and pDC populations, the old mice had a higher frequency of IL-10+ cells prior to TBI. After irradiation, the young mice had a higher frequency of IL-10+ cells. CONCLUSION: With TBI, the DCs showed dramatic differences between young and old mice. Young mice turned to an immuno-suppressive response whereas the old mice changed to an immuno-stimulation of DCs after TBI. From these dramatic aging effects, we hope to explain the different frequencies and severities of acute GvHD after allogeneic hematopoietic stem cell transplantation according to host age.
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Animales , Ratones , Envejecimiento , Citocinas , Células Dendríticas , Fluorescencia , Trasplante de Células Madre Hematopoyéticas , Esperanza , Interferones , Interleucina-12 , Factor de Necrosis Tumoral alfa , Irradiación Corporal TotalRESUMEN
BACKGROUND: Immunoglobulin heavy chain (IgH) gene rearrangement has been known to be a useful marker for determining the clonality as well as detecting minimal residual disease in B cell malignancies. This study was performed to establish single polymerase chain reaction (PCR) methods for the detection of IgH gene rearrangements in formalin-fixed, paraffin-embedded tissue of patients with B cell lymphoma and determine the type of JH segments used. METHODS: We obtained formalin-fixed, paraffin-embedded tissue sections of 44 patients diagnosed with B cell lymphoma at Ajou University Hospital from January 2005 to January 2007 and reviewed medical records retrospectively. After the extraction of DNA, PCR was performed using VH3 and JHPST primers to detect the third complementarity determining region (CDR3) gene of IgH. Sequence analysis of the PCR products was also done in 23 patients. RESULTS: The CDR3 gene rearrangements were detected in 26 (59%) out of 44 patients with B cell lymphoma. Sequence analysis of the amplified CDR3 gene was successful in 16 (70%) of 23 patients. JH3, JH4, JH5, and JH6 segments were used for CDR3 gene rearrangements in 3 (25%), 4 (33%), 1 (8%), and 4 (33%) patients with diffuse large B cell lymphoma, respectively. CONCLUSION: Although there are some limitations due to a low sensitivity less than 60%, single PCR using consensus primers could be an effective tool for the detection of CDR3 gene rearrangements in routine laboratory settings. Furthermore, sequence analysis of the CDR3 PCR products will provide basic information necessary for further studies.
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Humanos , Regiones Determinantes de Complementariedad , Consenso , ADN , Formaldehído , Reordenamiento Génico , Cadenas Pesadas de Inmunoglobulina , Inmunoglobulinas , Linfoma de Células B , Registros Médicos , Neoplasia Residual , Reacción en Cadena de la Polimerasa , Estudios Retrospectivos , Análisis de SecuenciaRESUMEN
Idiopathic retroperitoneal fibrosis is a relatively rare disease that is characterized by the proliferation of fibrous tissue with an inflammatory process in the retroperitoneal cavity. It can cause an obstruction and compression of the ureter, abdominal aorta, and finally progress to renal failure. During the initial stages, the histology shows active inflammation. However, in the late stages, fibrous scarring occurs and the tissue becomes relatively avascular and acellular. Increased 18F-FDG accumulation was observed in our patient at the time of disease onset, which was attributed to the presence of inflammatory cells and actively metabolizing fibroblasts. We describe two patients with idiopathic retroperitoneal fibrosis, who were examined by 18F-FDG-PET, and discuss the efficiency of positron emission tomography in the diagnosis and management of idiopathic retroperitoneal fibrosis patients.
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Humanos , Aorta Abdominal , Cicatriz , Diagnóstico , Fibroblastos , Fluorodesoxiglucosa F18 , Inflamación , Tomografía de Emisión de Positrones , Enfermedades Raras , Insuficiencia Renal , Fibrosis Retroperitoneal , UréterRESUMEN
BACKGROUND: Myelodysplastic syndromes (MDS) are clonal hematologic stem cell disorders characterized clinically and morphologically by ineffective hematopoiesis. A consensus-defined French-American-British (FAB) classification and International Prognostic Scoring System (IPSS) for predicting outcome and planning therapy in MDS has been developed, but its prognostic value in a large and independent series remains unproven. So we investigate clinical characteristics and prognosis of MDS, according to French-American-British (FAB) classification and International Prognostic Scoring System (IPSS). METHODS: A retrospective analysis of 50 patients who were diagnosed as myelodysplastic syndrome at Ajou University Hospital was performed from November, 1994 to April, 2003. The patients with secondary MDS were excluded. All patients were classified according to the FAB classification and calculated prognostic scores for IPSS. Patients were evaluated for clinical features and for blood and bone marrow findings at the time of diagnosis, and were followed up for survival and leukemic progression. Survival curves were based on the Kaplan-Meier method. All reported p values less than or equal to 0.05 were regarded as stastistically significant. RESULTS: The peak age was in the fifth decade and the male to female ratio was 1.5:1. RA (36%) was observed most frequently. Thereafter, RAEB-t (26%), RAEB (24%), RARS (12%) and CMML (2%) were observed, respectively. The initial symptoms on admission were fever (24%), dizziness and headache (16%), general weakness (16%), hemorrhage (14%), dyspnea (12%), abdominal pain (4%) and vomiting (4%). Cytogenetic studies were performed in 34 patients with MDS. They were classified as good, intermediate, poor group by chromosome score of IPSS. The median survival was 16.4 months for the good group, 15 months for the intermediate, 10.3 months for the poor. The median survival according to FAB classified groups were RA (33.8 mo), RARS (12.5 mo), RAEB (16.4 mo), RAEB-t (6.7 mo) and CMML (1.3 mo). Survival according to IPSS scoring system were 67.2 months for low, 27.1 months for intermediate-1, 10.3 months for intermediate-2 and 6.0 months for high groups. These data were statistically significant (p<0.05). CONCLUSIONS: In our experiencies, FAB and IPSS classification would be good predictors in clinical outcomes. But, because of the heterogeneity of MDS, large multicenter studies will be needed to define the issue of a new classification for these disorders.
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Femenino , Humanos , Masculino , Dolor Abdominal , Anemia Refractaria con Exceso de Blastos , Médula Ósea , Clasificación , Citogenética , Diagnóstico , Mareo , Disnea , Fiebre , Cefalea , Hematopoyesis , Hemorragia , Síndromes Mielodisplásicos , Características de la Población , Pronóstico , Estudios Retrospectivos , Células Madre , VómitosRESUMEN
BACKGROUND: Numerous cell surface proteins of leukemia cells such as CD33 and CD52 have been identified as diagnostic and therapeutic targets. Thus the profiling of the cell surface proteome and proteins restricted to specific leukemia(s) can provide a way to identify novel targets for leukemia diagnosis and therapy. However, there is a lack of data pertaining to the comprehensive analysis of surface membrane proteins because there are few effective strategies for profiling surface membrane proteomes. METHODS: We report on the application of quantitative proteomic techniques that incorporate affinity-capture and purification on monomeric avidin columns to identify all biotinylated cell surface proteins from leukemia cell lines. RESULTS: An analysis of a subset of biotinylated proteins among the different human leukemia cell lines using matrix-assisted laser desorption ionization and tandem mass spectrometry identified, among others, some widely expressed proteins in leukemia cells, such as CD11a, CD11c, CD18, CD31, CD44, and CD147, as well as a set of proteins identified as chaperone proteins, including HSP90, GRP78, GRP75, HSP70, HSP60 and protein disulfide isomerases. On the basis of their known functional roles, several of these proteins may participate in the progression of leukemogenesis and should be considered as potential markers of leukemia. CONCLUSION: Comprehensive profiling of the leukemia cell surface proteome provides an effective approach for the identification of commonly occurring proteins as well as proteins with restricted expression patterns to a specific cell line.
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Humanos , Avidina , Línea Celular , Diagnóstico , Leucemia , Proteínas de la Membrana , Membranas , Proteína Disulfuro Isomerasas , Proteoma , Espectrometría de Masas en TándemRESUMEN
BACKGROUND: The response rates and survival following allogeneic bone marrow transplantation (BMT) or immunosuppressive treatment were compared in severe aplastic anemia (SAA) and the prognostic factors related with survival identified. METHODS: Medical data of SAA patients, treated with BMT or immunosuppressive therapy (IST) at the Ajou University Hospital, between January 1995 and December 2005, were retrospectively analyzed. RESULTS: A total of 43 patients were evaluable; 18 (41.9%) were treated with IST (antithymocyte globulin plus cyclosporine A plus steroid) and 25 (58.1%) with allogeneic BMT. In the IST group, the response rate was 77.8% (2 complete and 12 partial remissions), with two treatment failures. As later complications, acute myeloid leukemia developed in 1 patient and myelodysplastic syndrome developed in 2. In the BMT group, the response rate was 92.0% (18 complete and 5 partial remissions) (P<0.001). Six patients developed grade II to III acute graft-versus-host-disease (GVHD) and 3 developed chronic GVHD. The median survival time in all patients was 60.27 months, and the 5-year survival rates were 61.0 and 81.9% in the IST and BMT groups, respectively (P=0.144). The factors influencing the overall survival were an age under 40-years and a positive treatment response. CONCLUSION: This study shows that allogeneic BMT, compared to IST, resulted in good response andoverall survival rates in patients with SAA. However, the overall survival rate between the two groups was statistically insignificant. Our study suggests that younger age SAA patients, with HLA-matched BMT donors, may benefit more from allogeneic BMT.
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Humanos , Anemia Aplásica , Trasplante de Médula Ósea , Médula Ósea , Ciclosporina , Leucemia Mieloide Aguda , Síndromes Mielodisplásicos , Estudios Retrospectivos , Tasa de Supervivencia , Donantes de Tejidos , Insuficiencia del TratamientoRESUMEN
There haves been few reports of cytomegalovirus (CMV) gastroenteritis following allogeneic stem cell transplantation, especially in pediatric cases. Herein, we report a case of CMV gastroenteritis in a 10-year-old boy with relapsed juvenile myelomonocytic leukemia who had undergone a donor leukocyte infusion. He presented with a skin rash, diarrhea and abdominal pain following a donor leukocyte infusion. Cramping abdominal pain persisted, even after treatment of the acute graft-versus-host disease. Therefore, gastroduodenoscopy and a gastric mucosal biopsy were performed, after which CMV gastroenteritis was diagnosed. The boy recovered after treatment with ganciclovir and intravenous immunoglobulin.
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Niño , Humanos , Masculino , Dolor Abdominal , Biopsia , Citomegalovirus , Diarrea , Exantema , Ganciclovir , Gastroenteritis , Enfermedad Injerto contra Huésped , Inmunoglobulinas , Leucemia Mielomonocítica Juvenil , Leucocitos , Calambre Muscular , Trasplante de Células Madre , Donantes de TejidosRESUMEN
A twenty-year-old man developed pruritic papules on his right forearm on the 25th day after an allogeneic bone marrow transplantation from an HLA-matched related donor. The skin lesion turned out to be lymphomatoid papulosis, both histologically and immunophenotypically, not a GVHD skin lesion. Lymphomatoid papulosis is a chronic lymphoproliferative disease of the skin, characterized by recurrent crusts of pruritic papules, which initially appearing on the upper trunk and both extremities. The lesions heal spontaneously within 2~8 weeks, usually leaving slightly depressed oval scars. Histologically, the lesions show wedge-shaped dense dermal infiltrates of lymphoid cells, with numerous eosinophils, neutrophils and atypical lymphocytes. As much as 50% of the infiltrates show atypical lymphocytes, and the dermal vessels may show endothelial swelling, fibrin deposition and red blood cell extravasation. We are reporting a case of spontaneously healing CD56+ lymphomatoid papulosis, in the patient who received bone marrow transplantation, is reported.
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Humanos , Trasplante de Médula Ósea , Médula Ósea , Cicatriz , Eosinófilos , Eritrocitos , Extremidades , Fibrina , Antebrazo , Linfocitos , Papulosis Linfomatoide , Neutrófilos , Piel , Donantes de TejidosRESUMEN
BACKGROUND: To compare the mobilizing effects and toxicities of two different doses of cyclophosphamide (CY) plus lenograstim (glycosylated G-CSF), we performed a prospective randomized study by enrolling patients suffering with either high-risk Non-Hodgkin's lymphoma (NHL) or breast cancer undergoing ablative chemotherapy. METHODS: The NHL patients received 4 cycles of CHOP and the breast cancer patients received 2-3 cycles of FAC (FEC) adjuvant chemotherapy. Then, the patients were randomly allocated to receive CY 4 g/m2 (arm A) or 1.5 g/m2 (arm B) in combination with lenograstim. Large volume leukapheresis was carried out and it was continued daily until the target cell dose of 2x10 (6) CD34+ cell/kg was reached. RESULTS: Twenty-seven patients were enrolled in the study. The median number of leukaphereis sessions actually performed was 2.5 sessions in arm A and 3 sessions in arm B. The target cell dose was obtained with the median number of one leukapheresis session in both arms of the study (p=0.09). The collected number of CD34+ cells in the leukapheresis products was higher in arm A than arm B (22.4 vs. 9.9x10 (6) /kg, respectively, p=0.05). Grade III or IV leukopenia was present in 14/15 patients (94%) in arm A and in 1/12 patients (8%) in arm B (p< 0.0001). Grade III or IV thrombocytopenia was present in 8/15 patients (54%) in arm A, but this was not present in any patients of arm B (p=0.0004). Neutropenic fever occurred in 6/15 patients (40%) in arm A, and in 1/12 patients (8%) in arm B (p=0.09). The hematological recovery of the leukocytes and platelets after transplantation was not statistically different between the two doses. CONCLUSION: Low-dose CY plus lenograstim is a safe and effective mobilizing regimen.
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Persona de Mediana Edad , Masculino , Humanos , Femenino , Adulto , Acondicionamiento Pretrasplante , Células Madre/efectos de los fármacos , Proteínas Recombinantes/administración & dosificación , Estudios Prospectivos , Agonistas Mieloablativos/administración & dosificación , Linfoma no Hodgkin/tratamiento farmacológico , Leucaféresis , Movilización de Célula Madre Hematopoyética , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Quimioterapia Combinada , Ciclofosfamida/administración & dosificación , Quimioterapia Adyuvante , Neoplasias de la Mama/tratamiento farmacológicoRESUMEN
BACKGROUND: In combination with standard-dose CHOP (cyclophosphamide, vincristine, adriamycin, and prednisolone), the addition of rituximab produces a better clinical response in the treatment of aggressive B-cell non-Hodgkin's lymphoma (NHL) than CHOP alone. METHODS: Thirty-four patients with previously untreated diffuse large B-cell NHL received at least three or four cycles of rituximab 375 mg/m2 or 500 mg per dose on day 1 of each cycle in combination with CHOP chemotherapy. RESULTS: The median age of patients were 61.5 years (range, 28-83 years). After the end of therapy, twenty-five patients (73.5%) experienced a complete response, four patients (11.8 %) had a partial response, and two patients (5.9%) were classified as having progressive disease. The median follow-up duration was 9.4 months (range, 0.2-19.5 months) and 1-year overall survival and progression free survival was 84.8+/-8.7% and 80.3+/-9.4%, respectively. Two patients (5.9%) experienced fever, myalgia, and skin eruption due to rituximab. Neutropenia of grade 3 or 4 occurred in thirty-one patients (91.2%). CONCLUSION: The benefits of rituximab in combination with CHOP chemotherapy include high response rates and good tolerance. However, further prospective, randomized studies are needed to draw definitive conclusions.