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1.
Ann Hematol ; 2024 Jul 22.
Artigo em Inglês | MEDLINE | ID: mdl-39037588

RESUMO

Thrombosis in myeloproliferative neoplasms (MPNs) is an important clinical problem, and risk-stratified management is essential. To identify the clinical characteristics of thrombosis in patients with MPNs, a nationwide multi-institutional retrospective analysis (JSH-MPN-R18) was conducted. The aim of the present study was to perform a sub-analysis of JSH-MPN-R18 findings to clarify the predictive parameters for thrombosis among complete blood count (CBC) results. Among the patients enrolled in JSH-MPN-R18, those with essential thrombocythemia (ET; n = 1152) and polycythemia vera (PV; n = 456) were investigated. We analyzed and compared CBC parameters between patients with and those without any thrombotic events using Welch's T-test. Statistical analyses were performed using the R statistical software. Thrombotic events were observed in 74 patients with ET. In multivariate analysis, only the neutrophil ratio was slightly but significantly higher for ET patients with thrombosis than for those without (p < 0.05). Of note, the absolute neutrophil count (aNeu) was considered a useful predictive tool for thrombosis among patients classified as low-risk according to the revised International Prognostic Score of Thrombosis for Essential Thrombocythemia. Among PV patients, those with thrombosis showed significantly higher hematocrit and aNeu than did those without thrombosis. As a thrombosis-associated factor, the neutrophil ratio was slightly but significantly elevated in patients with ET. This myeloid skew might reflect a higher value of JAK2 V617F allelic frequency in patients with ET with thrombosis; this was not clarified in JSH-MPN-R18. Further accumulation of evidence, including genetic information for JAK2 and other passenger mutations, is warranted.

2.
Eur Spine J ; 33(1): 77-83, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37889328

RESUMO

PURPOSE: This cross-sectional study serves two main purposes. Firstly, it aims to validate the preoperative Japanese Core Outcome Measures Index for the Neck (COMI-Neck) in patients with cervical spondylotic myelopathy (CSM) and ossification of the posterior longitudinal ligament (OPLL). Secondly, it seeks to elucidate differences in preoperative quality of life (QOL) between these two cervical pathologies using patient-reported outcome measures (PROMs). METHODS: A total of 103 preoperative patients (86 with CSM and 17 with OPLL) scheduled for cervical spine surgery were included in the study. Validated PROMs, including the Japanese COMI-Neck, Neck Disability Index (NDI), EuroQol-5 Dimension-3 level (EQ-5D-3L), and SF-12v2, were used to assess QOL. Baseline demographic and clinical data were collected, and statistical analyses were performed to compare the PROMs between CSM and OPLL groups. RESULTS: The Japanese COMI-Neck demonstrated good construct validity, with positive correlations with NDI and negative correlations with EQ-5D-3L and SF-12v2. Comparison of preoperative PROMs between CSM and OPLL groups revealed differences in age, body mass index, and EQ-5D-3L scores. The CSM group had higher NDI scores for concentration and lower EQ-5D-3L scores for self-care compared to the OPLL group. CONCLUSIONS: This study validated the preoperative Japanese COMI-Neck in CSM and OPLL patients and identified specific QOL issues associated with each condition. The findings highlight the importance of considering disease-specific QOL and tailoring treatment plans accordingly. Further research should include postoperative assessments and a more diverse population to enhance generalizability.


Assuntos
Ossificação do Ligamento Longitudinal Posterior , Doenças da Medula Espinal , Espondilose , Humanos , Vértebras Cervicais/cirurgia , Estudos Transversais , Ligamentos Longitudinais , Ossificação do Ligamento Longitudinal Posterior/cirurgia , Ossificação do Ligamento Longitudinal Posterior/complicações , Osteogênese , Avaliação de Resultados em Cuidados de Saúde , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , Doenças da Medula Espinal/cirurgia , Doenças da Medula Espinal/complicações , Espondilose/cirurgia , Espondilose/complicações , Resultado do Tratamento
3.
Eur Spine J ; 33(6): 2198-2205, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38679673

RESUMO

PURPOSE: The primary objective was to validate the construct validity of the Japanese Core Outcome Measures Index (COMI) in preoperative patients aged 60 years or older undergoing lumbar spine surgery for lumbar spinal stenosis (LSS) and lumbar disk herniation (LDH). Additionally, as a secondary aim, we explored the impact of these diseases on quality of life (QOL). METHODS: The analysis included 199 preoperative patients aged 60 and above who were scheduled for lumbar spine surgery. To assess QOL, Japanese versions of the COMI, Oswestry Disability Index (ODI), EuroQol-5 Dimension-3 Level (EQ-5D-3L), and SF-12v2 were employed. The study assessed the validity of the COMI and compared demographic and clinical characteristics between the LSS (147 cases) and LDH (52 cases) groups. It used multivariate covariance analysis (MANCOVA) to examine the impact of diseases (LSS and LDH) on each patient-reported outcome measure while considering covariates. RESULTS: Compared to the LSS group, the LDH group showed more difficulty with the COMI summary score (LSS/LDH [mean]: 6.9/8.1, p < 0.001), ODI score (46.8/57.4, p < 0.001), and EQ-5D utility (0.53/0.43, p < 0.001). The LDH group also reported more difficulties in the COMI-function, COMI-symptom-specific well-being, COMI-disability, ODI-personal care, ODI-social life, and SF-12v2-bodily pain subscales. MANCOVA demonstrated that these results were not influenced by covariates such as gender and medical history. CONCLUSIONS: This study highlights the distinct impact of LSS and LDH on preoperative QOL in older patients undergoing lumbar spinal surgery. Tailored interventions are essential to address the specific challenges posed by these conditions and improve patient-centered outcomes and postoperative recovery.


Assuntos
Deslocamento do Disco Intervertebral , Vértebras Lombares , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , Estenose Espinal , Humanos , Estenose Espinal/cirurgia , Deslocamento do Disco Intervertebral/cirurgia , Deslocamento do Disco Intervertebral/complicações , Masculino , Feminino , Idoso , Vértebras Lombares/cirurgia , Pessoa de Meia-Idade , Japão , Idoso de 80 Anos ou mais , Avaliação da Deficiência , População do Leste Asiático
4.
Cytotherapy ; 25(11): 1212-1219, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37354150

RESUMO

BACKGROUND AIMS: The prognostic impact of platelet recovery after autologous hematopoietic cell transplantation (AHCT) on clinical outcomes remains to be elucidated. We aimed to clarify the impact of platelet recovery on clinical outcomes, risk factors of delayed platelet recovery and the necessary dose of CD34+ cells for prompt platelet recovery in each patient. METHODS: Using a nationwide Japanese registry database, we retrospectively analyzed clinical outcomes of 5222 patients with aggressive non-Hodgkin lymphoma (NHL) or multiple myeloma (MM). RESULTS: At a landmark of 28 days after AHCT, a delay of platelet recovery was observed in 1102 patients (21.1%). Prompt platelet recovery was significantly associated with superior overall survival (hazard ratio [HR] 0.32, P < 0.001), progression-free survival (HR 0.48, P < 0.001) and decreased risks of disease progression (HR 0.66, P < 0.001) and non-relapse/non-progression mortality (HR 0.19, P < 0.001). The adverse impacts of a delay of platelet recovery seemed to be more apparent in NHL. In addition to the dose of CD34+ cells/kg, disease status, performance status and the hematopoietic cell transplant-specific comorbidity index in both diseases were associated with platelet recovery. We then stratified the patients into three risk groups according to these factors. For the purpose of achieving 70% platelet recovery by 28 days in NHL, the low-, intermediate- and high-risk groups needed more than 2.0, 3.0 and 4.0 × 106 CD34+ cells/kg, respectively. In MM, the low-risk group needed approximately 1.5 × 106 CD34+ cells/kg, whereas the intermediate- and high-risk groups required 2.0 and 2.5 × 106 CD34+ cells/kg to achieve about 80% platelet recovery by 28 days. CONCLUSIONS: A delay of platelet recovery after AHCT was associated with inferior survival outcomes.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Linfoma não Hodgkin , Mieloma Múltiplo , Humanos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Mieloma Múltiplo/terapia , Estudos Retrospectivos , Linfoma não Hodgkin/terapia , Linfoma não Hodgkin/etiologia , Plaquetas , Antígenos CD34 , Transplante Autólogo
5.
Acta Haematol ; 145(6): 582-591, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35016184

RESUMO

INTRODUCTION: Chromosomal abnormalities (CAs) have been identified as important factors in determining the biological features and prognostic value of multiple myeloma (MM). MYC gene-related abnormalities (MYC GAs) are one of the CAs, but their unfavorable impact has not been fully investigated in daily clinical practice. METHODS: This study retrospectively analyzed the prognostic impact of MYC GAs on 81 patients through fluorescence in situ hybridization analysis in our institute. RESULTS: MYC GAs were associated with poor overall survival (hazard ratio [HR], 3.08; 95% confidence interval [CI]: 1.23-7.73; p = 0.017), progression-free survival (PFS) (HR, 2.96; 95% CI: 1.58-5.53; p < 0.001), and time to next treatment (TNT) (HR, 2.11; 95% CI: 1.13-3.93; p = 0.018) in the median follow-up of 34.7 months. Furthermore, MYC GAs with an additional chromosome 8 (MYC-Ch8(+)) were associated with shorter PFS (HR, 3.15; 95% CI: 1.38-7.2; p = 0.0064), whereas MYC GAs without an additional chromosome 8 (MYC-Ch8(-)) were associated with shorter PFS (HR, 3.62; 95% CI: 1.51-8.68; p = 0.004) and shorter TNT (HR, 3.72; 95% CI: 1.41-9.81; p = 0.0078). CONCLUSION: These findings could help identify high-risk patients with MM. Further prospective studies are needed to confirm the significance of MYC GAs for the MM prognostic effect.


Assuntos
Mieloma Múltiplo , Humanos , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/genética , Mieloma Múltiplo/tratamento farmacológico , Genes myc , Prognóstico , Hibridização in Situ Fluorescente , Estudos Retrospectivos , Aberrações Cromossômicas
6.
Rinsho Ketsueki ; 63(3): 177-181, 2022.
Artigo em Japonês | MEDLINE | ID: mdl-35387929

RESUMO

Herein we report a case of successful treatment of secondary graft failure due to poor graft function (PGF) using eltrombopag. A 25-year-old woman with aplastic anemia (stage 3) underwent allogeneic bone marrow transplantation (BMT) from her HLA-matched brother. Neutrophil engraftment was achieved on day 17, but she remained dependent on platelet transfusion. Chimerism analysis showed complete donor type, but she also became dependent on red blood cell transfusion later. Eltrombopag was administered on day 253 after BMT, after which she exhibited hematopoietic recovery, resulting in the withdrawal of transfusion dependency. Blood counts continued to be stable after eltrombopag was discontinued. The use of eltrombopag enabled outpatient treatment and induced hematopoietic recovery without significant side effects. Eltrombopag may be an effective and safe option for PGF after BMT.


Assuntos
Anemia Aplástica , Transplante de Células-Tronco Hematopoéticas , Adulto , Feminino , Humanos , Masculino , Anemia Aplástica/etiologia , Benzoatos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hidrazinas/uso terapêutico , Pirazóis
7.
Rinsho Ketsueki ; 63(12): 1653-1656, 2022.
Artigo em Japonês | MEDLINE | ID: mdl-36653139

RESUMO

A 65-year-old man was diagnosed with Philadelphia chromosome-positive acute lymphoblastic leukemia with no initial central nervous system (CNS) involvement. Complete remission was achieved after the induction therapy. However, during consolidation therapy, he developed septic shock and pneumocystis pneumonia, leading to interruption in chemotherapy and allogeneic transplantation. Subsequently, he achieved complete molecular remission and ponatinib maintenance therapy was initiated. Two years later, he developed left leg paralysis and was diagnosed with isolated CNS relapse; however, radiation therapy improved CNS lesions and paralysis. Thus, ponatinib maintenance therapy alone is inadequate in preventing CNS relapse in patients who have not completed systemic chemotherapy for CNS relapse prevention.


Assuntos
Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras , Masculino , Humanos , Idoso , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Doença Crônica , Sistema Nervoso Central , Recidiva , Paralisia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
8.
Blood ; 134(10): 814-825, 2019 09 05.
Artigo em Inglês | MEDLINE | ID: mdl-31270105

RESUMO

Monocyte-derived fibrocytes recently garnered attention because the novel pathogenesis of myelofibrosis (MF), and suppression of fibrocyte differentiation by serum amyloid P remarkably improved MF. We previously revealed that human fibrocytes highly expressed signaling lymphocytic activation molecule F7 (SLAMF7) compared with macrophages and that SLAMF7high monocytes in the peripheral blood (PB) of MF patients were significantly elevated relative to those in healthy controls (HCs). In this study, we evaluated SLAMF7high monocyte percentage in the PB of HCs, myeloproliferative neoplasm (MPN) patients with MF, and MPN patients without MF by using a cross-sectional approach. We found that MPN patients with MF who harbored JAK2V617F had a significantly elevated SLAMF7high monocyte percentage, which correlated positively with the JAK2V617F allele burden. In addition, the serum concentration of interleukin-1ra (IL-1ra) was significantly correlated with the SLAMF7high monocyte percentage and JAK2V617F allele burden. These findings suggest that both SLAMF7high monocytes and IL-1ra could be useful noninvasive markers of MF onset. Furthermore, the JAK2V617F allele burden of SLAMF7high monocytes was significantly higher than that of SLAMF7low monocytes and could be a potential target of elotuzumab (Elo), an anti-SLAMF7 antibody used for treating multiple myeloma. Elo independently inhibited differentiation of fibrocytes derived not only from HCs but also from MF patients in vitro. Elo also ameliorated MF and splenomegaly induced by romiplostim administration in humanized NOG mice. In conclusion, an increase of SLAMF7high monocytes with higher JAK2V617F allele burden was associated with the onset of MF in MPN patients harboring JAK2V617F, and Elo could be a therapeutic agent for MPN patients with MF who harbor JAK2V617F.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Janus Quinase 2/genética , Monócitos/patologia , Mielofibrose Primária/tratamento farmacológico , Mielofibrose Primária/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Substituição de Aminoácidos , Contagem de Células Sanguíneas , Proliferação de Células , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Terapia de Alvo Molecular , Monócitos/metabolismo , Mutação de Sentido Incorreto , Fenilalanina/genética , Mielofibrose Primária/sangue , Mielofibrose Primária/patologia , Família de Moléculas de Sinalização da Ativação Linfocitária/metabolismo , Valina/genética
9.
Rinsho Ketsueki ; 62(1): 20-24, 2021.
Artigo em Japonês | MEDLINE | ID: mdl-33551420

RESUMO

Patients with refractory ascites that develops >3 months after allogenic stem cell transplantation typically have a poor prognosis. We present the case of a 61-year-old man who developed refractory massive ascites approximately 3 months after cord blood transplantation (CBT) and showed complete and spontaneous remission from ascites after 18 months. The patient complained of severe bloating and needed weekly paracentesis to manage the fluid levels. Laboratory tests indicated that the ascites was caused by liver fibrosis. After the patient underwent Keisuke-Matsusaki cell-free and concentrated ascites reinfusion therapy (KM-CART), we were able to decrease the frequency of paracentesis treatments. We planned a transjugular liver biopsy, but the patient contracted pneumocystis pneumonia before the procedure could be performed. Although the pneumonia improved, the ascites worsened again. However, weekly paracentesis spontaneously stopped the progression of ascites and eventually resolved it completely, resulting in the patient's survival.


Assuntos
Ascite , Transplante de Células-Tronco de Sangue do Cordão Umbilical , Leucemia Mieloide Aguda , Ascite/etiologia , Ascite/terapia , Humanos , Leucemia Mieloide Aguda/terapia , Cirrose Hepática/complicações , Cirrose Hepática/terapia , Masculino , Pessoa de Meia-Idade , Paracentese
10.
Biol Blood Marrow Transplant ; 26(11): 2105-2114, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32784070

RESUMO

The outcomes of 7/8 allele-matched unrelated bone marrow transplantation (7/8 UBMT) and umbilical cord blood transplantation (UCBT) have been improving. We retrospectively analyzed adults with acute leukemia who underwent their first 7/8 UBMT or UCBT in Japan. Between January 2008 and December 2017, a total of 4150 patients were recorded, including 488 who underwent 7/8 UBMT and 3662 who underwent UCBT. Only 32 patients with 7/8 UBMT had graft-versus-host-disease (GVHD) high-risk HLA mismatched pairs. Overall survival at 3 years was 54% for 7/8 the UBMT group and 46% for the UCBT group, a nonsignificant difference in multivariate analysis (hazard ratio [HR], 1.01; 95% confidence interval [CI], .88 to 1.17; P = .89). The 7/8 UBMT and UCBT groups showed a similar nonrelapse mortality rate (HR, 1.16; 95% CI, .96 to 1.45; P = .16) and relapse rate (HR, .85; 95% CI, .71 to 1.02; P = .08). However, the UCBT group had a lower risk of grade II-IV acute GVHD (HR, .76; 95% CI, .65 to .88; P < .001) and chronic GVHD (HR, .77; 95% CI, .66- .91; P = .002) compared with the 7/8 UBMT group. In stratified analyses combining disease risk with conditioning intensity, 7/8 UBMT showed superior overall survival to UCBT in standard risk and myeloablative conditioning (HR, .72; 95% CI, .56 to .93; P = .014). Both 7/8 UBMT and UCBT are appropriate alternative donor procedures. The stem cell source can be selected on the basis of disease risk, patient tolerability, or concerns regarding GVHD.


Assuntos
Transplante de Células-Tronco de Sangue do Cordão Umbilical , Doença Enxerto-Hospedeiro , Adulto , Alelos , Transplante de Medula Óssea , Humanos , Japão , Estudos Retrospectivos , Doadores não Relacionados
11.
Am J Hematol ; 95(4): 343-353, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31849102

RESUMO

The major limitation of cord blood transplantation (CBT) for adults remains the delayed hematopoietic recovery and higher incidence of graft failure, which result in a higher risk of early mortality in CBT. We evaluated early overall survival (OS), non-relapse mortality (NRM), neutrophil engraftment, acute graft-vs-host disease, and cause of early death among 9678 adult patients who received single-unit CBT in Japan between 1998 and 2017. The probability of OS at 100 days was 64.4%, 71.7%, and 78.9% for the periods 1998 to 2007, 2008 to 2012, and 2013 to 2017, respectively (P < .001). The cumulative incidences of NRM at 100 days during the same period were 28.3%, 20.8%, and 14.6%, respectively (P < .001). The cumulative incidences of neutrophil engraftment were also improved during the same period (P < .001). The most common cause of death within 100 days after CBT was bacterial infection in 1998 to 2007 and primary disease in the latter two time periods. Across the three time periods, the proportions of deaths from bacterial and fungal infection, graft failure, hemorrhage, sinusoidal obstructive syndrome, and organ failure decreased in a stepwise fashion. Landmark analysis of OS and NRM after 100 days showed that OS did not change over time in the multivariate analysis. Our registry-based data demonstrated a significant improvement of early OS after CBT for adults over the past 20 years. The landmark analysis suggested that improvement of early mortality could lead to an improvement of long-term OS after CBT.


Assuntos
Transplante de Células-Tronco de Sangue do Cordão Umbilical/mortalidade , Adolescente , Adulto , Idoso , Transplante de Células-Tronco de Sangue do Cordão Umbilical/efeitos adversos , Transplante de Células-Tronco de Sangue do Cordão Umbilical/métodos , Transplante de Células-Tronco de Sangue do Cordão Umbilical/tendências , Feminino , Seguimentos , Rejeição de Enxerto , Sobrevivência de Enxerto , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/mortalidade , Humanos , Incidência , Infecções/mortalidade , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/etiologia , Insuficiência de Múltiplos Órgãos/mortalidade , Neutrófilos , Análise de Sobrevida , Resultado do Tratamento , Adulto Jovem
12.
Rinsho Ketsueki ; 61(1): 3-10, 2020.
Artigo em Japonês | MEDLINE | ID: mdl-32023599

RESUMO

Recently, monocyte-derived fibroblast-like cells, called fibrocytes, garnered attention as involved in the novel pathogenesis of various fibrotic diseases. They also play a role in the induction of myelofibrosis (MF). Neoplastic fibrocytes are overrepresented in the bone marrow of patients with primary MF, and the suppression of fibrocyte differentiation by serum amyloid P has been shown to remarkably improve MF. Further, thrombopoietin (TPO) or a TPO receptor agonist directly induces fibrocyte differentiation, and fibrocyte elimination reversed the MF phenotype in a murine model. Human fibrocytes highly express signaling lymphocytic activation molecule-F7 (SLAMF7) compared with macrophages. Myeloproliferative neoplasm (MPN) patients harboring JAK2V617F with MF had a significantly elevated SLAMF7high monocyte percentage, which correlated positively with the JAK2V617F allele burden. Furthermore, the JAK2V617F allele burden and the tendency to differentiate into fibrocytes of SLAMF7high monocytes was significantly higher than that of JAK2V617Flow monocytes and could be a potential target of elotuzumab (Elo), an anti-SLAMF7 antibody used to treat multiple myeloma. Elo independently inhibited the differentiation of fibrocytes derived not only from healthy controls but also from MF patients in vitro. Elo also ameliorated MF and splenomegaly induced by romiplostim administration in humanized NOG mice. Thus, Elo could be a therapeutic agent for MPN patients harboring JAK2V617F with MF.


Assuntos
Transtornos Mieloproliferativos , Mielofibrose Primária , Animais , Medula Óssea , Humanos , Macrófagos , Camundongos , Monócitos
13.
Rinsho Ketsueki ; 61(6): 598-604, 2020.
Artigo em Japonês | MEDLINE | ID: mdl-32624531

RESUMO

This retrospective study evaluated the outcomes of patients treated with combination of bendamustine and rituximab (BR) for recurrent indolent B-cell lymphoma from January 2011 to February 2018 in our department. The cohort included 36 males and 27 females, and majority of the patients (59%) were between 51 and 70 years of age. The disease types were follicular lymphoma (FL) and mantle-cell lymphoma in 42 (67%) and 15 (24%) patients, respectively. Median progression-free survival (PFS) was not reached in patients with FL who completed BR therapy. The analysis of patients who received BR therapy revealed that the number of CD4-positive lymphocytes remained around 200/µl even five years after the end of treatment. BR therapy was a useful treatment option for recurrent indolent B-cell lymphoma, especially in patients with FL, and completion of BR therapy appeared to be important for improved PFS. Furthermore, attention should be paid for potential infections for at least five years after BR therapy because cell-mediated immunodeficiency may become apparent after treatment.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma de Células B , Idoso , Cloridrato de Bendamustina , Feminino , Humanos , Linfoma de Células B/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Estudos Retrospectivos , Rituximab
14.
Rinsho Ketsueki ; 60(12): 1647-1651, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31902815

RESUMO

A 69-year-old woman presented to National Defense Medical College hospital for suspected nephrotic syndrome due to weight gain of 30 kg in 3 weeks and bilateral lower leg edema. However, her urinalysis showed microproteinuria, which excluded nephrotic syndrome. Computed tomography revealed severe systemic edema, pleural effusion, ascites, and enlarged cervical and axillary lymph nodes. Histological examination of axillary lymph node specimen showed a typical architecture of angioimmunoblastic T-cell lymphoma. One course of CHOP chemotherapy regimen was administered which improved the lymph nodes and systemic edema. The patient achieved complete remission after 6 courses of CHOP. Because serum vascular endothelial growth factor (VEGF) level was elevated before the treatment and normalized after the treatment, increased vascular permeability mediated by VEGF was hypothesized to have caused the systemic edema. In addition, VEGF secretion from Epstein-Barr virus (EBV)-infected cells was likely associated with the patient's clinical condition because B lymphocytes stained with CD20 were positive for Epstein-Barr virus-encoded small RNAs (EBERs) and VEGF.


Assuntos
Linfadenopatia Imunoblástica , Linfoma de Células T , Idoso , Edema , Feminino , Herpesvirus Humano 4 , Humanos , Fator A de Crescimento do Endotélio Vascular
15.
Biol Blood Marrow Transplant ; 24(4): 840-848, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29196081

RESUMO

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative therapeutic option for patients with chronic myelomonocytic leukemia (CMML). We retrospectively compared the post-transplantation outcomes of 159 patients with CMML who underwent allo-HSCT using 4 types of donor sources: HLA-matched related donor graft, unrelated bone marrow (U-BM), unrelated cord blood (U-CB), and HLA-mismatched related donor graft. The median patient age at allo-HSCT was 54 years (range, 16 to 75 years). In multivariate analyses, the use of HLA-matched related donor grafts correlated with better overall survival than U-BM (hazard ratio [HR], 2.05; 95% confidence interval [CI], 1.21 to 3.48; P = .008), U-CB (HR, 3.80; 95% CI, 2.07 to 6.95; P < .001), or HLA-mismatched related donor grafts (HR, 6.18; 95% CI, 2.70 to 14.15; P < .001). Mortality after the relapse or progression of CMML did not significantly differ among the 4 types of donor source. Transplantation-related mortality was highest in recipients of U-CB (HR, 3.32; 95% CI, 1.33 to 8.26; P = .010). In patients with CMML, allo-HSCT using an alternative donor may contribute to durable remission; however, further improvements in transplantation-related mortality are required for this type of transplantation.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Leucemia Mielogênica Crônica BCR-ABL Positiva/mortalidade , Leucemia Mielogênica Crônica BCR-ABL Positiva/terapia , Adolescente , Adulto , Fatores Etários , Idoso , Aloenxertos , Intervalo Livre de Doença , Feminino , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida
16.
Haematologica ; 103(11): 1835-1842, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-29976734

RESUMO

The purpose of this trial was to evaluate the efficacy of 2-year consolidation therapy with nilotinib, at a dose of 300 mg twice daily, for achieving treatment-free remission in chronic myeloid leukemia patients with a deep molecular response (BCR-ABL1IS ≤0.0032%). Successful treatment-free remission was defined as no confirmed loss of deep molecular response. We recruited 96 Japanese patients, of whom 78 sustained a deep molecular response during the consolidation phase and were therefore eligible to discontinue nilotinib in the treatment-free remission phase; of these, 53 patients (67.9%; 95% confidence interval: 56.4-78.1%) remained free from molecular recurrence in the first 12 months. The estimated 3-year treatment-free survival was 62.8%. Nilotinib was readministered to all patients (n=29) who experienced a molecular recurrence during the treatment-free remission phase. After restarting treatment, rapid deep molecular response returned in 25 patients (86.2%), with 50% of patients achieving a deep molecular response within 3.5 months. Tyrosine kinase inhibitor withdrawal syndrome was reported in 11/78 patients during the early treatment-free remission phase. The treatment-free survival curve was significantly better in patients with undetectable molecular residual disease than in patients without (3-year treatment-free survival, 75.6 versus 48.6%, respectively; P=0.0126 by the log-rank test). There were no significant differences in treatment-free survival between subgroups based on tyrosine kinase inhibitor treatment before the nilotinib consolidation phase, tyrosine kinase inhibitor-withdrawal syndrome, or absolute number of natural killer cells. The results of this study indicate that it is safe and feasible to stop tyrosine kinase inhibitor therapy in patients with chronic myeloid leukemia who have achieved a sustained deep molecular response with 2 years of treatment with nilotinib. This study was registered with UMIN-CTR (UMIN000005904).


Assuntos
Quimioterapia de Consolidação , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Leucemia Mielogênica Crônica BCR-ABL Positiva/mortalidade , Pirimidinas/administração & dosagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Pirimidinas/efeitos adversos , Indução de Remissão , Taxa de Sobrevida
17.
Gan To Kagaku Ryoho ; 45(13): 1791-1793, 2018 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-30692355

RESUMO

AIM: The significance of radiofrequency ablation(RFA)for colorectal liver metastases(CRLM)remains to be elucidated. Therefore, this retrospective study aimed to evaluate the therapeutic efficacy of RFA for local recurrence of CRLM. SUBJECTS: Between June 2005 and June 2017, we retrospectively examined 63 patients(137 nodules)with CRLM who underwent RFA. RESULTS: The local recurrence rate was 36.5%, and the median local recurrence free survival(LRFS)was 26.3 months. We compared treatment background between the 2 groups with(50 nodules)and without(87 nodules)local recurrence. In the multivariate analysis, tumor size of the ablated lesion and method for ablation(direct tumor puncture)were independent risk factors for local recurrence. Receiver operating characteristic curve for tumor size of the ablated lesion showed an optimal cutoff value for tumor size of 1.8 cm(AUC=0.734, 95%CI: 0.612-0.855, p<0.0001). CONCLUSIONS: RFA for effective control of local recurrence of CRLM might be suitable for selected patients with tumor size of ablated lesion ofC1.8 cm and no touch ablation method.


Assuntos
Ablação por Cateter , Neoplasias Colorretais , Neoplasias Hepáticas , Ablação por Radiofrequência , Neoplasias Colorretais/patologia , Humanos , Neoplasias Hepáticas/secundário , Neoplasias Hepáticas/terapia , Recidiva Local de Neoplasia , Estudos Retrospectivos , Resultado do Tratamento
18.
Gan To Kagaku Ryoho ; 45(13): 1815-1817, 2018 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-30692363

RESUMO

A 74-year-old man was admitted to a local hospital with liver dysfunction. Imaging modalities revealed bile duct stenosis at the bifurcation of the anterior and posterior trunk. Exfoliative cytology of the bile and brushing cytology of the bile duct both revealed Class Ⅴ, and biopsy from the stenotic bile duct showed well differentiated adenocarcinoma. We diagnosed the patient with hilar cholangiocarcinoma and performed extended right bisectionectomy and biliary reconstruction after percuta- neous transhepatic right portal vein embolization(PTPE). Preoperatively, he was administered S-1(80mg/body weight/day) orally for 19 days. Histopathological assessment of the resected specimen revealed hemosiderin-laden macrophages without viable cancer cells, confirmingpatholog ical complete response(pCR).


Assuntos
Neoplasias dos Ductos Biliares , Colangiocarcinoma , Tumor de Klatskin , Idoso , Neoplasias dos Ductos Biliares/tratamento farmacológico , Neoplasias dos Ductos Biliares/cirurgia , Ductos Biliares Intra-Hepáticos , Colangiocarcinoma/tratamento farmacológico , Colangiocarcinoma/cirurgia , Hepatectomia , Humanos , Tumor de Klatskin/tratamento farmacológico , Tumor de Klatskin/cirurgia , Masculino , Terapia Neoadjuvante
19.
Ann Surg Oncol ; 24(1): 219-226, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27699610

RESUMO

BACKGROUND: Short-course radiotherapy with delayed surgery (SRT-delay) is still under clinical investigation for its efficacy in treating low rectal cancer (≤5 cm from the anal verge). This study was designed to assess the pattern of local recurrence and oncologic outcomes in T3 low rectal cancer treated with SRT-delay. METHODS: This study enrolled T3 low rectal cancer patients without distant metastasis between 2003 and 2015. All patients received total mesorectal excision following SRT-delay (25 Gy/10 fractions/5 days + S-1 radiosensitizer with a 4-week delay of surgery). The median follow-up period was 69 (range 1-149) months. RESULTS: A total 119 consecutive patients had low rectal cancer; 104 (87.4 %) underwent intersphincteric resection (ISR), and 15 (12.6 %) underwent abdominoperineal resection (APR). Fifty-six patients (47.1 %) were ypT-downstaged, 86 (72.2 %) were ypN0, and 10 (8.4 %) had circumferential resection margin involvement. The 5-year local recurrence-free survival, recurrence-free survival, and overall survival were 93.0, 76.2, and 80.5 %, respectively. Nine patients experienced local recurrence: lateral pelvic recurrence in six patients (5.0 %) and central pelvic recurrence in three (2.5 %). CONCLUSIONS: A total of 87.4 % of sphincter-preserving surgeries were performed for T3 low rectal cancer following SRT-delay. Pathological tumor downstaging, circumferential resection margin involvement, local recurrence, and oncologic outcomes were acceptable; therefore, the SRT-delay regimen may be an option for treating T3 low rectal cancer.


Assuntos
Neoplasias Retais/radioterapia , Neoplasias Retais/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Procedimentos Cirúrgicos do Sistema Digestório , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Taxa de Sobrevida , Resultado do Tratamento
20.
Clin Orthop Relat Res ; 475(8): 2074-2080, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28512691

RESUMO

BACKGROUND: Complete circumferential osseous extension in the acetabular rim has been reported to occur in the deep hip with pincer impingement. However, this phenomenon occasionally is observed in dysplastic hips without pincer impingement, and the degree to which this finding might or might not be associated with hip pain, and how often it occurs bilaterally among patients, are not well characterized. QUESTIONS/PURPOSES: (1) To determine the proportion of patients with complete circumferential osseous extension in the acetabular rim using three-dimensional (3-D) CT in patients with and without hip pain who had CT scans obtained for various reasons. (2) To elucidate how often this complete circumferential osseous extension occurred bilaterally among those patients. (3) To investigate the relationship between the proportions of patients with complete circumferential osseous extension observed on CT scans among three different acetabular coverage groups: dysplasia, normal, and overcoverage. (4) To determine how often the finding of hip pain was associated with complete circumferential osseous extension. PATIENTS AND METHODS: Between September 2011 to July 2016, we evaluated 3788 patients with pelvic complaints such as hip, groin, thigh, buttock, or sacroiliac joint pain. We obtained consent from 26% (992 of 3788) of them, and obtained 3-D CT scans as part of that evaluation. For the current retrospective study, we excluded patients younger than 20 years or 80 years or older (181 patients), patients who had previous hip surgery (185 patients), patients with severe osteoarthritis with Tönnis Grades 2 or 3 (301 patients), and patients who could not have an accurate lateral center-edge (LCE) angle measured owing to poor-quality radiographs (24 patients), leaving 301 patients (602 hips) for this analysis. In this study population, patients reported pain in 131 hips (22%), defined as all types of hip pain except for trauma, including activity pain, pain with sports, pain on motion, and impingement pain; the others did not report hip pain. The mean age of the patients was 56 ± 16 years, and the mean LCE angle was 26° ± 8° (range, -9° to 47°). We first determined the proportion of patients with complete circumferential osseous extension in the acetabular rim using 3-D CT for those with and without hip pain who had CT obtained for various reasons. We next elucidated how often this complete circumferential osseous extension occurred bilaterally among the patients, and finally we investigated the relationship between the proportion of patients with complete circumferential osseous extension observed on CT scans among the three groups: dysplasia (defined as LCE angles of 22° or smaller), normal, and overcoverage (defined as LCE angles of 34° or larger) groups. We finally determined how often the finding was associated with hip pain attributable to complete circumferential osseous extension. RESULTS: The proportion of patients with complete circumferential osseous extension was 6% (18 of 301 patients). Eighty-nine percent (16 of 18) of the patients had bilateral complete circumferential osseous extension. There were no differences in the proportions of patients with complete circumferential osseous extension among the three groups: 5.3% (odds ratio [OR], 1.02; 95% CI, 0.45-2.31; p = 0.97), 5.3%, and 7.4% (OR, 0.70; 95% CI, 0.28-1.73; p = 0.44) in the dysplasia, normal, and overcoverage groups, respectively, with the numbers available. Eighteen percent (six of 34) of the hips with complete circumferential osseous extension had pain. CONCLUSIONS: Complete circumferential osseous extension in the acetabular rim is relatively uncommon. When it occurs, it usually is bilateral, it occurs regardless of acetabular coverage, and it is associated with pain in a minority of patients. LEVEL OF EVIDENCE: Level III, prognostic study.


Assuntos
Acetábulo/patologia , Artralgia/patologia , Luxação do Quadril/patologia , Articulação do Quadril/patologia , Acetábulo/diagnóstico por imagem , Adulto , Idoso , Artralgia/diagnóstico por imagem , Artralgia/etiologia , Feminino , Luxação do Quadril/complicações , Luxação do Quadril/diagnóstico por imagem , Articulação do Quadril/diagnóstico por imagem , Humanos , Imageamento Tridimensional/métodos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Adulto Jovem
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