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1.
Surg Case Rep ; 10(1): 94, 2024 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-38647617

RESUMO

BACKGROUND: Spontaneous clearance of chronic hepatitis C virus (HCV) is rare in adults. A T-lymphocyte response is thought to be involved in HCV-RNA clearance. Splenectomy reportedly has a beneficial effect on T cell immune function in patients with cirrhosis. To the best of our knowledge, the present report is the first to describe spontaneous clearance of serum HCV-RNA within 1 year after splenectomy in a patient with cirrhosis. CASE PRESENTATION: A 55-year-old man with HCV cirrhosis was transferred to our institution with advanced pancytopenia, splenomegaly, and gastric varices. He had a 1-year history of ascites, edema, and general fatigue. The patient had a Child-Pugh score of 8 and serological type 1 HCV; the HCV-RNA level was 4.7 log IU/mL. Contrast-enhanced computed tomography showed gastric varices and marked splenomegaly (estimated spleen volume of 2175 mL). Esophagogastroduodenoscopy revealed enlarged gastric varices with no red color sign, and the varices were larger than those 1 year prior. He was diagnosed with decompensated HCV-related liver cirrhosis and portal hypertension. We considered direct-acting antiviral (DAA) therapy; however, DAA therapy was not approved in Japan for patients with decompensated cirrhosis at that time. Hand-assisted laparoscopic splenectomy was performed to improve the worsening portal hypertension. Further, we planned the initiation of DAA therapy after surgery, when such therapy would become available. DAA therapy was approved 1 year after splenectomy. At that time, we measured the HCV-RNA level before the initiation of DAA therapy; unexpectedly, however, serum HCV-RNA was not detectable, and the virus continued to disappear during the following 4 years. His liver function (total bilirubin, albumin, and prothrombin time) and pancytopenia improved during the 5 years postoperatively. The serum aspartate and alanine aminotransferase levels normalized between 1 and 5 years postoperatively. Esophagogastroduodenoscopy showed no change in the gastric varices during the 5 years after surgery. The patient remained asymptomatic and continued to do well. CONCLUSIONS: We have presented a case of spontaneous clearance of HCV-RNA after splenectomy in a patient with cirrhosis and portal hypertension. Splenectomy may be associated with disappearance of HCV-RNA based on previous reports. More cases should be accumulated and evaluated.

2.
Cytotherapy ; 26(6): 592-598, 2024 06.
Artigo em Inglês | MEDLINE | ID: mdl-38466265

RESUMO

BACKGROUND AIMS: Haploidentical hematopoietic stem cell transplantation (haplo-HCT) is an appropriate option when an HLA-matched related or unrelated donor is not available. Haplo-HCT using post-transplant cyclophosphamide (PTCy) is being increasingly performed worldwide due to its effective suppression of GVHD and its safety. METHODS: We conducted a large nationwide cohort study to retrospectively analyze 366 patients with acute myeloid leukemia undergoing haplo-HCT with PTCy between 2010 and 2019 and to identify prognostic factors. RESULTS: A multivariate Cox analysis revealed that an older recipient age (≥60 years), a male donor to a male recipient, a cytomegalovirus IgG-negative donor to a cytomegalovirus IgG-positive recipient, a poor cytogenetic risk, a noncomplete remission status at the time of transplantation, and a history of HCT were independently associated with worse overall survival (OS). Based on each hazard ratio, these factors were scored (1-2 points) and stratified by their total score into three groups: favorable (0-1 points), intermediate (2-3 points), and poor (4 points or more) groups, and 2-year OS rates were 79.9%, 49.2%, and 25.1%, respectively (P < 0.001). CONCLUSIONS: The present study revealed significant prognostic factors in haplo-HCT with PTCy, and a scoring system based on these factors may be used to predict outcomes.


Assuntos
Ciclofosfamida , Transplante de Células-Tronco Hematopoéticas , Leucemia Mieloide Aguda , Transplante Haploidêntico , Humanos , Masculino , Leucemia Mieloide Aguda/terapia , Leucemia Mieloide Aguda/mortalidade , Feminino , Pessoa de Meia-Idade , Ciclofosfamida/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante Haploidêntico/métodos , Adulto , Prognóstico , Estudos Retrospectivos , Idoso , Adolescente , Doença Enxerto-Hospedeiro/etiologia , Adulto Jovem
3.
Intern Med ; 2024 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-38346738

RESUMO

We herein report a rare case of hypersensitivity pneumonitis (HP) that was initially demonstrated as solitary pure ground-glass opacity (GGO) on chest computed tomography (CT). A 51-year-old woman with a history of breast cancer underwent follow-up CT, which revealed solitary pure GGO. The patient developed exertional dyspnea after two years, and CT revealed diffuse centrilobular nodules in addition to GGO, which had increased in size. An antigen avoidance test was performed to diagnose HP, leading to the resolution of CT abnormalities, including the GGO. Our findings suggested that nonfibrotic HP can present as solitary pure GGO.

4.
Eur Radiol ; 34(1): 374-383, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37535157

RESUMO

OBJECTIVES: To compare the [18F]FDG PET/CT findings of untreated sarcoidosis and malignant lymphoma (ML) and develop convolutional neural network (CNN) models to differentiate between these diseases using maximum intensity projection (MIP) [18F]FDG PET images. METHODS: We retrospectively collected data on consecutive patients newly diagnosed with sarcoidosis and ML who underwent [18F]FDG PET/CT before treatment. Two nuclear radiologists reviewed the images. CNN models were created using MIP PET images and evaluated with k-fold cross-validation. The points of interest were visualized using gradient-weighted class activation mapping (Grad-CAM). RESULTS: A total of 56 patients with sarcoidosis and 62 patients with ML were included. Patients with sarcoidosis had more prominent FDG accumulation in the mediastinal lymph nodes and lung lesions, while those with ML had more prominent accumulation in the cervical lymph nodes (all p < 0.001). For the mediastinal lymph nodes, sarcoidosis patients had significant FDG accumulation in the level 2, 4, 7, and 10 lymph nodes (all p < 0.01). Otherwise, the accumulation in ML patients tended to be in the level 1 lymph nodes (p = 0.08). The CNN model using frontal and lateral MIP images achieved an average accuracy of 0.890 (95% CI: 0.804-0.977), a sensitivity of 0.898 (95% CI: 0.782-1.000), a specificity of 0.907 (95% CI: 0.799-1.000), and an area under the curve of 0.963 (95% CI: 0.899-1.000). Grad-CAM showed that the model focused on the sites of abnormal FDG accumulation. CONCLUSIONS: CNN models based on differences in FDG accumulation sites archive high performance in differentiating between sarcoidosis and ML. CLINICAL RELEVANCE STATEMENT: We developed a CNN model using MIP images of [18F]FDG PET/CT to distinguish between sarcoidosis and malignant lymphoma. It achieved high performance and could be useful in diagnosing diseases with involvement across organs and lymph nodes. KEY POINTS: • There are differences in FDG distribution when comparing whole-body [18F]FDG PET/CT findings in patients with sarcoidosis and malignant lymphoma before treatment. • Convolutional neural networks, a type of deep learning technique, trained with maximum-intensity projection PET images from two angles showed high performance. • A deep learning model that utilizes differences in FDG distribution may be helpful in differentiating between diseases with lesions that are characteristically widespread among organs and lymph nodes.


Assuntos
Linfoma , Sarcoidose , Humanos , Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Estudos Retrospectivos , Linfoma/diagnóstico por imagem , Redes Neurais de Computação , Sarcoidose/diagnóstico por imagem
5.
Respir Med Case Rep ; 46: 101929, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37886215

RESUMO

A 74-year-old man was referred to our hospital with an abnormal chest shadow. Computed tomography (CT) revealed a mass in the left upper lobe and interstitial pneumonia (IP). The patient underwent CT-guided needle biopsy and was diagnosed as lung adenocarcinoma with cT2aN1M1a Stage IVA (PUL). The patient was administered 6 cycles of CBDCA + nab-paclitaxel as first-line, 3 cycles of atezolizumab as second-line, and 8 cycles of S-1 as third-line treatment but finally showed tumor progression. Because comprehensive genome profiling test revealed KRAS G12C mutation, sotorasib was initiated as fourth-line treatment and showed tumor regression without exacerbation of pre-existing IP.

6.
Transplant Cell Ther ; 29(5): 332.e1-332.e11, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36796517

RESUMO

The alkylating agent busulfan is commonly used as conditioning in allogeneic hematopoietic stem cell transplantation for acute myeloid leukemia (AML). However, a consensus has not yet been reached regarding the optimal busulfan dose in cord blood transplantation (CBT). Therefore, we conducted this large nationwide cohort study to retrospectively analyze the outcomes of CBT in patients with AML receiving busulfan at intermediate (6.4 mg/kg i.v.; BU2) or higher (12.8 mg/kg i.v.; BU4) doses within a fludarabine/i.v. busulfan (FLU/BU) regimen. Among 475 patients who underwent their first CBT following FLU/BU conditioning between 2007 and 2018, 162 received BU2 and 313 received BU4. Multivariate analysis identified BU4 as a significant factor for longer disease-free survival (hazard ratio [HR], .85; 95% confidence interval [CI], .75 to .97; P = .014) and a lower relapse rate (HR, .84; 95% CI, .72 to .98; P = .030). No significant differences were observed in non-relapse mortality between BU4 and BU2 (HR, 1.05; 95% CI, .88-1.26; P = .57). Subgroup analyses showed that BU4 provided significant benefits for patients who underwent transplantation while not in complete remission (CR) and those age <60 years. Our present results suggest that higher busulfan doses are preferable in patients undergoing CBT, particularly those not in CR and younger patients.


Assuntos
Transplante de Células-Tronco de Sangue do Cordão Umbilical , Transplante de Células-Tronco Hematopoéticas , Leucemia Mieloide Aguda , Humanos , Pessoa de Meia-Idade , Bussulfano/uso terapêutico , Estudos de Coortes , Estudos Retrospectivos , Transplante de Células-Tronco Hematopoéticas/métodos , Leucemia Mieloide Aguda/terapia , Recidiva
7.
Int J Hematol ; 115(4): 595-599, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35001347

RESUMO

Chronic active Epstein-Barr virus infection (CAEBV) is a systemic T- or NK-lymphoproliferative disorder (LPD) caused by EBV. Allogenic hematopoietic stem cell transplantation (HSCT) is the only curative therapy for CAEBV, but relapse sometimes occurs. Relapse is generally attributed to proliferation of recipient-derived CAEBV cells. We herein report a case of donor-derived CAEBV-like NK-cell post-transplant lymphoproliferative disease (PTLD) in a 41-year-old female after the first allogenic HSCT for CAEBV from an HLA-matched sibling donor. A second HSCT from an HLA-matched unrelated donor successfully controlled the disease, but EBV infection of cells derived from the second donor continued to be detected. Although the mechanisms underlying CAEBV and CAEBV-like NK-cell PTLD have not yet been elucidated in detail, the findings of the present case imply that host genetic factors, including familial factors, may be important in disease development.


Assuntos
Infecções por Vírus Epstein-Barr , Transplante de Células-Tronco Hematopoéticas , Transtornos Linfoproliferativos , Adulto , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/terapia , Feminino , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Herpesvirus Humano 4/genética , Humanos , Células Matadoras Naturais , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/terapia
8.
Int J Hematol ; 115(3): 428-434, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34704233

RESUMO

Shwachman-Diamond syndrome (SDS) is an autosomal recessive inherited disorder characterized by bone marrow failure, exocrine pancreatic dysfunction, and skeletal abnormalities. SDS is typically caused by a pathogenic mutation in the Shwachman-Bodian-Diamond Syndrome (SBDS) gene. Patients with SDS have an increased risk of developing acute myeloid leukemia (AML) and myelodysplastic syndromes. We identified germline biallelic SBDS mutations (p.K62X and p.I167M) in a 50-year-old AML patient who had never experienced the typical symptoms of SDS. The K62X mutation is one of the most common pathogenic mutations, whereas the significance of the I167M mutation was unclear. Based on cellular experiments, we concluded that the I167M mutation contributed to the development of AML, and chemotherapy including topoisomerase inhibitors, which induce DNA double-strand breaks, may have been toxic to this patient. Our experience indicates that some asymptomatic Shwachman-Bodian-Diamond syndrome mutations contribute to the development of leukemia, and that careful treatment selection may be warranted for patients harboring these mutations.


Assuntos
Mutação em Linhagem Germinativa/genética , Leucemia Mieloide Aguda/etiologia , Leucemia Mieloide Aguda/genética , Proteínas/genética , Síndrome de Shwachman-Diamond/genética , DNA/metabolismo , Reparo do DNA/efeitos dos fármacos , Feminino , Genes Recessivos/genética , Humanos , Leucemia Mieloide Aguda/tratamento farmacológico , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/etiologia , Síndrome de Shwachman-Diamond/complicações , Inibidores da Topoisomerase/efeitos adversos
9.
Int J Hematol ; 114(3): 395-400, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34057670

RESUMO

We report a case of acquired hypofibrinogenemia with multiple myeloma presenting λ-type IgG monoclonal protein. The patient had anemia and renal deficiency, and also developed bleeding tendency due to severe coagulopathy. Her fibrinogen level was under the detectable limits in a functional assay. Enzyme-linked immunosorbent assay (ELISA) and immunoblotting analysis results were consistent with functional assay results, and deficiency patterns observed in cross-mixing tests for PT and aPTT confirmed the diagnosis of hypofibrinogenemia. To determine the cause of hypofibrinogenemia, we purified the patient's immunoglobulin via protein A agarose, and confirmed that fibrinogen was included in the bound fraction, strongly indicating paraprotein interference with fibrinogen. As accelerated removal of fibrinogen was indicated, we incubated the patient's plasma up to 48 h, but did not observe significant loss of fibrinogen. In sharp contrast, fibrinogen returned to below the detection level 12 h after infusion of fresh frozen plasma. These findings support leukocyte-mediated fibrinogen removal, rather than paraprotein-triggered fibrinogen instability. Surprisingly, the patient's paraprotein was IgG2, but we speculate the amount of paraprotein (IgG 5346 mg/dL) compensated for lower affinity to Fcγ receptors.


Assuntos
Afibrinogenemia/diagnóstico , Afibrinogenemia/etiologia , Mieloma Múltiplo/complicações , Afibrinogenemia/sangue , Afibrinogenemia/terapia , Coagulação Sanguínea , Testes de Coagulação Sanguínea , Suscetibilidade a Doenças , Feminino , Fibrinogênio/metabolismo , Humanos , Imunoglobulina G , Cadeias lambda de Imunoglobulina , Pessoa de Meia-Idade , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/terapia
10.
Intern Med ; 60(10): 1589-1595, 2021 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-33328401

RESUMO

TAFRO syndrome and POEMS syndrome are lymphoproliferative disorders with elevated interleukin-6 and vascular endothelial growth factor (VEGF) levels; however, their underlying pathogenic mechanisms remain unclear. Similarities have been reported in the pathological findings of the lymph nodes of TAFRO syndrome, Multicentric Castleman disease (MCD), and some cases of POEMS syndrome. However, there is no consensus on the relationship among them. We encountered a case of lymphoproliferative disorder that presented with manifestations of both TAFRO syndrome and POEMS syndrome. This case may be a subtype of idiopathic MCD and will be very important for establishing the disease concept of TAFRO syndrome and POEMS syndrome.


Assuntos
Hiperplasia do Linfonodo Gigante , Síndrome POEMS , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/diagnóstico , Humanos , Linfonodos , Síndrome POEMS/complicações , Síndrome POEMS/diagnóstico , Fator A de Crescimento do Endotélio Vascular
11.
J Immunol ; 206(2): 355-365, 2021 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-33310873

RESUMO

Hypersensitivity pneumonitis (HP) typically presents with interstitial inflammation and granulomas induced by an aberrant immune response to inhaled Ags in sensitized individuals. Although IL-17A is involved in the development of HP, the cellular sources of IL-17A and the mechanisms by which IL-17A contributes to granuloma formation remain unclear. Recent studies report that γδ T cells produce IL-17A and exhibit memory properties in various diseases. Therefore, we focused on IL-17A-secreting memory γδ T cells in the sensitization phase and aimed to elucidate the mechanisms by which IL-17A contributes to granuloma formation in HP. We induced a mouse model of HP using pigeon dropping extract (PDE) in wild-type and IL-17A knockout (IL-17A-/-) mice. IL-17A-/- mice exhibited reduced granulomatous areas, attenuated aggregation of CD11b+ alveolar macrophages, and reduced levels of CCL2, CCL4, and CCL5 in the bronchoalveolar lavage fluid. Among IL-17A+ cells, more γδ T cells than CD4+ cells were detected after intranasal PDE administration. Interestingly, the expansion of IL-17A-secreting Vγ4+ or Vγ1-Vγ4- cells of convalescent mice was enhanced in response to the sensitizing Ag. Additionally, coculture of macrophages with PDE and Vγ4+ cells purified from PDE-exposed convalescent mice produced significantly more IL-17A than coculture with Vγ4+ cells from naive mice. Our findings demonstrate that in the sensitization phase of HP, IL-17A-secreting memory γδ T cells play a pivotal role. Furthermore, we characterized the IL-17A/CCL2, CCL4, CCL5/CD11b+ alveolar macrophage axis, which underlies granuloma formation in HP. These findings may lead to new clinical examinations or therapeutic targets for HP.


Assuntos
Alveolite Alérgica Extrínseca/imunologia , Granuloma/imunologia , Interleucina-17/metabolismo , Macrófagos/imunologia , Linfócitos T/imunologia , Animais , Doenças das Aves/imunologia , Aves , Técnicas de Cocultura , Modelos Animais de Doenças , Humanos , Memória Imunológica , Interleucina-17/genética , Camundongos , Camundongos Endogâmicos C57BL , Receptores de Antígenos de Linfócitos T gama-delta/metabolismo
12.
BMJ Case Rep ; 13(11)2020 Nov 30.
Artigo em Inglês | MEDLINE | ID: mdl-33257362

RESUMO

Heavy chain disease (HCD) is a rare B-cell proliferative neoplasm that is characterised by the production of truncated monoclonal immunoglobulin heavy chains without light chains. Gamma HCD is a subgroup of HCD. A 67-year-old man was admitted to our hospital with dyspnoea and lower leg oedema. Based on the results of heart catheterisation, he was diagnosed with pulmonary hypertension. Laboratory tests revealed an elevated level of IgG, and serum immunoelectrophoresis showed that IgG was a monoclonal gamma heavy chain without light chains. Finally, he was diagnosed with gamma HCD complicated by pulmonary hypertension. Bortezomib and dexamethasone therapy was initiated, but became refractory within 8 months. Therefore, the treatment was switched to lenalidomide and dexamethasone therapy, and the disease has been stably controlled for more than 2 years. To the best of our knowledge, this is the first case of gamma HCD being successfully treated by lenalidomide and dexamethasone therapy.


Assuntos
Doença das Cadeias Pesadas/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Fatores Imunológicos/uso terapêutico , Lenalidomida/uso terapêutico , Idoso , Medula Óssea/patologia , Bortezomib/uso terapêutico , Dexametasona/uso terapêutico , Quimioterapia Combinada , Doença das Cadeias Pesadas/complicações , Humanos , Imunoglobulina G/sangue , Masculino
13.
J Anesth ; 34(5): 694-701, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32524294

RESUMO

PURPOSE: We evaluated the influence of anesthetic management with sevoflurane or propofol on recurrence in patients undergoing breast cancer surgery. METHODS: This single center, retrospective study, included patients who received either sevoflurane or propofol during primary breast cancer surgery between 2008 and 2012. Our primary outcome was recurrence-free survival (RFS) at 1 year. Recurrence was defined as locoregional recurrence and distal metastasis. Propensity scores were calculated using seven variables (age, sex, body mass index, cancer stage, tumor size, intrinsic subtype, and deviation from standard therapy), and Kaplan-Meier survival curves were constructed from the date of diagnosis of recurrence. Hazard ratios (HRs) were estimated using univariable Cox proportional hazard regression analysis. RESULTS: Two-hundred-twelve patients received sevoflurane and 814 patients received total intravenous anesthesia with propofol. The median follow-up was 59 (interquartile range, 44-75) months. Regional anesthetic techniques were not used. Recurrence occurred in 95 patients (9.26%), with 19 (8.96%) and 76 (9.33%) in the sevoflurane and propofol groups, respectively. The HR was 1.167 (95% confidence interval, 0.681-2.000, p = 0.574) for the use of sevoflurane over propofol. After 1:1 propensity-score matching, 318 patients were analyzed. The 1-year RFS rates were similar between the groups (sevoflurane group: 7.5% [n = 12], propofol group: 8.2% [n = 13]), yielding an HR of 1.002 (95% confidence interval 0.457-2.198, p = 0.995) associated with the use of sevoflurane over propofol. CONCLUSION: In patients undergoing primary breast cancer surgery, the use of either sevoflurane or propofol without regional anesthesia did not appear to affect the risk of recurrence after 1 year.


Assuntos
Anestésicos Inalatórios , Neoplasias da Mama , Éteres Metílicos , Propofol , Anestesia Geral , Anestésicos Intravenosos , Neoplasias da Mama/cirurgia , Humanos , Estudos Retrospectivos , Sevoflurano
14.
J Clin Exp Hematop ; 60(2): 37-40, 2020 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-32404568

RESUMO

Methotrexate, hydrocortisone, vincristine, sobuzoxane, and etoposide (MTX-HOPE) chemotherapy was originally reported in 2007 as a salvage regimen for relapsed or refractory non-Hodgkin's lymphoma. To clarify the safety and efficacy of this regimen, we retrospectively analyzed patients at our institute. We analyzed 18 patients, including 16 with diffuse large B-cell lymphoma (DLBCL), one with follicular lymphoma (FL), and one with T-cell lymphoma. The median age at MTX-HOPE therapy was 79 (range: 68-87). Ten patients received more than 3 previous chemotherapy regimens. The median period from the initial treatment to the first MTX-HOPE administration was 53 months. No patient had severe renal dysfunction. The overall response rate was 78%, with 39% achieving CR and 39% achieving PR. The median OS and PFS after the initiation of MTX-HOPE were 10 months (range: 0.5-86 months) and 7 months (range: 0.2-86 months), respectively. The one-year OS rate was 44% and the two-year OS rate was 22%. The median number of treatment cycles was 7 (range: 1-46), and 6 patients received more than 10 cycles. Among eight patients who were over 80 years of age, 7 responded to the therapy, and the median OS and PFS of this subgroup were 19 months and 11 months, respectively. All patients tolerated the treatment well, mostly on an outpatient basis, except for one who died from infection and one who developed intracranial hemorrhage. MTX-HOPE may be a promising treatment option for elderly patients with refractory or relapsed malignant lymphoma.


Assuntos
Linfoma não Hodgkin/terapia , Terapia de Salvação , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Etoposídeo/uso terapêutico , Feminino , Humanos , Hidrocortisona/uso terapêutico , Masculino , Metotrexato/uso terapêutico , Piperazinas/uso terapêutico , Terapia de Salvação/métodos , Resultado do Tratamento , Vincristina/uso terapêutico
15.
Rinsho Ketsueki ; 60(11): 1538-1543, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31839631

RESUMO

A 61-year-old man was admitted to our hospital with fever and massive leukocytosis. A bone marrow smear revealed an increased density of myeloid cells in various stages of maturation as well as dysplasia in the neutrophils. There was no proliferation of blasts, eosinophils, or basophils. Genomic analysis of the bone marrow cells revealed no detectable abnormalities associated with myeloproliferative neoplasms, including BCR-ABL1. Therefore, the patient was diagnosed with atypical chronic myeloid leukemia (aCML). Chromosomal analysis revealed the presence of 1-17 double minute chromosomes (dmin) in 20 of 20 tumor cells examined. Multiple MYC signals were detected via interphase fluorescence in situ hybridization, indicating MYC gene amplification in the dmins. Three months after the oral administration of hydroxyurea, leukocytosis reoccurred. Therefore, induction therapy followed with umbilical cord blood transplantation was performed. However, MYC signals remained detectable in the bone marrow sample obtained immediately after neutrophil engraftment, indicating the presence of residual tumor cells. To the best of our knowledge, this is the first case report of aCML with dmin gene amplification, suggesting that the dmin MYC amplification exacerbated the patient's disease.


Assuntos
Amplificação de Genes , Leucemia Mieloide Crônica Atípica BCR-ABL Negativa , Medula Óssea , Aberrações Cromossômicas , Humanos , Hibridização in Situ Fluorescente , Masculino , Pessoa de Meia-Idade
16.
Rinsho Ketsueki ; 60(12): 1630-1634, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31902812

RESUMO

In 2003, a 60-year-old man presenting with thrombocytosis was referred to our hospital. Laboratory tests revealed normal white blood cell count and hemoglobin level. Bone marrow examination showed an increased number of megakaryocytes with dysplasia. G-banded karyotype analysis revealed del (5q). Initially, the patient was diagnosed with myelodysplastic/myeloproliferative neoplasm (MDS/MPN), and it was treated with aspirin and hydroxyurea. During the treatment course, fluorescence in situ hybridization for CSF1R and EGR1 was performed to detect del (5q), which showed negative results. In 2017, the patient had increased platelet count despite receiving treatment. A comprehensive genomic profiling revealed that the deleted region in this case was present in 5q14-5q23, which was different from the common deleted region of 5q- syndrome (5q32-5q33, where CSF1R was present) and that of high-risk MDS or acute myeloid leukemia (5q31, where EGR1 was present). Moreover, a CALR mutation was also detected. This case met the diagnostic criteria of essential thrombocythemia. The platelet count decreased with the administration of anagrelide. In conclusion, comprehensive genetic profiling is very important, and it leads to accurate diagnosis and therapy.


Assuntos
Síndromes Mielodisplásicas , Trombocitemia Essencial , Deleção Cromossômica , Genômica , Humanos , Hibridização in Situ Fluorescente , Masculino , Pessoa de Meia-Idade
17.
Proc Natl Acad Sci U S A ; 115(51): 13057-13062, 2018 12 18.
Artigo em Inglês | MEDLINE | ID: mdl-30510003

RESUMO

Chronic obstructive pulmonary disease (COPD) is a leading cause of morbidity and mortality worldwide. It has generally been considered a non-Th2-type lung disorder, characterized by progressive airflow limitation with inflammation and emphysema, but its cellular and molecular mechanism remains ill defined, compared with that of asthma characterized by reversible airway obstruction. Here we show a previously unappreciated role for basophils at the initiation phase of emphysema formation in an elastase-induced murine model of COPD in that basophils represent less than 1% of lung-infiltrating cells. Intranasal elastase instillation elicited the recruitment of monocytes to the lung, followed by differentiation into interstitial macrophages (IMs) but rarely alveolar macrophages (AMs). Matrix metalloproteinase-12 (MMP-12) contributing to emphysema formation was highly expressed by IMs rather than AMs, in contrast to the prevailing assumption. Experiments using a series of genetically engineered mice suggested that basophil-derived IL-4, a Th2 cytokine, acted on lung-infiltrating monocytes to promote their differentiation into MMP-12-producing IMs that resulted in the destruction of alveolar walls and led to emphysema development. Indeed, mice deficient for IL-4 only in basophils failed to generate pathogenic MMP-12-producing IMs and hence develop emphysema. Thus, the basophil-derived IL-4/monocyte-derived IM/MMP-12 axis plays a crucial role in emphysema formation and therefore may be a potential target to slow down emphysema progression at the initiation phase of COPD.


Assuntos
Basófilos/patologia , Interleucina-4/metabolismo , Macrófagos Alveolares/patologia , Macrófagos/patologia , Metaloproteinase 12 da Matriz/fisiologia , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Enfisema Pulmonar/etiologia , Animais , Basófilos/metabolismo , Células Cultivadas , Citocinas/metabolismo , Modelos Animais de Doenças , Feminino , Macrófagos/metabolismo , Macrófagos Alveolares/metabolismo , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Endogâmicos C57BL , Neutrófilos , Enfisema Pulmonar/metabolismo , Enfisema Pulmonar/patologia
18.
J Anesth ; 32(1): 82-89, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29214418

RESUMO

PURPOSE: This study aimed to reveal whether the occurrence of periprocedural myocardial damage (PMD) decreases in patients who received volatile anesthetics to maintain general anesthesia compared with those who received propofol during transcatheter aortic valve implantation (TAVI). METHODS: We included one hundred and forty adult patients who underwent transfemoral TAVI under general anesthesia from January 2015 to March 2017 in this single-center retrospective review. We compared the rate of patients who developed PMD between those who received desflurane (Group D, n = 72) and propofol (Group P, n = 68) for anesthetic maintenance. PMD was represented by the peak levels of creatine kinase myocardial band (CK-MB) and troponin I within 72 h following the procedure and defined as an increase >5 times in CK-MB or >15 times in troponin I compared with the institutional upper reference limits. Further analysis was performed to identify the independent predictors of PMD. RESULTS: There was no significant difference in the rate of PMD between groups (Group D 72.2% to Group P 70.6%, P = 0.85) or levels of CK-MB (Group D 7.85 [1.3-72.7] ng/mL to Group P 8.45 [1.8-49.7] ng/mL; P = 0.59) and troponin I (Group D 1.061 [0.050-10.8] ng/mL to Group P 1.214 [0.036-29.0] ng/mL; P = 0.97). The risk of PMD was higher in patients with more intraprocedural blood loss (odds ratio 1.49 per 100 mL, P = 0.048) and lower in those with an implanted permanent pacemaker (odds ratio 0.17; P = 0.02). CONCLUSIONS: Desflurane does not appear to be more cardioprotective than propofol when used for anesthetic maintenance in patients undergoing transfemoral TAVI.


Assuntos
Valva Aórtica/cirurgia , Desflurano/administração & dosagem , Propofol/administração & dosagem , Substituição da Valva Aórtica Transcateter/métodos , Idoso , Idoso de 80 Anos ou mais , Anestesia Geral/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Razão de Chances , Estudos Retrospectivos
19.
Eur J Anaesthesiol ; 34(7): 425-431, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28590308

RESUMO

BACKGROUND: Vocal cord paralysis (VCP) is a rare complication of thoracic cardiovascular surgery. In severe cases, life-threatening airway obstruction may occur. OBJECTIVE: To evaluate the incidence and severity of VCP among patients who underwent thoracic cardiovascular surgery and to identify possible risk factors. DESIGN: Single-centre retrospective review of adult patients. SETTING: Osaka University Hospital, Suita, Japan, from January 2013 to August 2015. PATIENTS: We included 688 patients in the final analysis. Preoperative, intraoperative and postoperative data were collected from medical records. Patients with preoperative VCP or tracheostomy prior to extubation were excluded. The VCP severity in relation to functional recovery was graded using the following categories: absent; mild, remission at 6 months; moderate, partial or persistent VCP at 6 months; or severe, airway obstruction after extubation requiring reintubation. An otolaryngologist diagnosed all VCP cases. MAIN OUTCOME MEASURES: The incidence and severity of VCP after extubation. RESULTS: The incidence (number) of VCP was 4.7% (32), with those of mild, moderate and severe VCP being 1.7% (12), 1.5% (10) and 1.5% (10), respectively. The ICU stay was significantly longer in patients with severe VCP than in patients without VCP [12.5 days (interquartile range 5.5 to 25.5) vs. 3 days (interquartile range 2 to 5), P = 0.0002]. In our multivariable analysis, type 2 diabetes mellitus [odds ratio (OR) 1.853, P = 0.009], intubation period (OR per 24 h 1.136, P = 0.014), ascending aortic arch surgery with brachiocephalic artery reconstruction (OR 8.708, P < 0.001) and ventricular assist device implantation (OR 3.460, P = 0.005) were independent predictors for VCP. CONCLUSION: The identification of these risk factors may facilitate screening for VCP before extubation and possibly help anaesthesia personnel to be prepared to treat VCP-related airway obstruction should it occur.


Assuntos
Procedimentos Cirúrgicos Cardiovasculares/efeitos adversos , Assistência Perioperatória/métodos , Complicações Pós-Operatórias/diagnóstico , Índice de Gravidade de Doença , Paralisia das Pregas Vocais/diagnóstico , Idoso , Procedimentos Cirúrgicos Cardiovasculares/tendências , Feminino , Humanos , Intubação Intratraqueal/efeitos adversos , Intubação Intratraqueal/tendências , Masculino , Pessoa de Meia-Idade , Assistência Perioperatória/tendências , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Risco , Procedimentos Cirúrgicos Torácicos/efeitos adversos , Procedimentos Cirúrgicos Torácicos/tendências , Paralisia das Pregas Vocais/etiologia
20.
JA Clin Rep ; 3(1): 15, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29457059

RESUMO

Acute bioprosthetic valve thrombosis can occur after surgery and sometimes cause hemodynamic instability and cardiogenic shock. Risk factors for bioprosthetic valve thrombosis are hypercoagulability, atrial fibrillation, atrial dilatation, low cardiac function, and lack of anticoagulation therapy. The authors present a case of severe mitral stenosis due to bioprosthetic valve thrombus. The patient was diagnosed with dilated-phase hypertrophic cardiomyopathy and underwent mitral valve replacement. He required venoarterial extracorporeal membrane oxygenation (VA-ECMO) due to extremely low cardiac output and was scheduled for left ventricular assist device (LVAD) implantation. Transesophageal echocardiographic examination before LVAD implantation revealed severe mitral stenosis due to bioprosthetic mitral valve thrombus, which was not detected by transthoracic echocardiography in the intensive care unit and contributed to the low cardiac function. The thrombus was removed through an unscheduled left atriotomy before LVAD implantation. The possibility of bioprosthetic valve thrombosis must be considered when the patient is dependent on VA-ECMO support. Early transesophageal echocardiographic examination of the bioprosthetic valve may be helpful and contribute to surgical decision-making.

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