RESUMO
Tuberculosis. a disease of great public concern, is spread through inhalation of micro-droplets from an infected person. Despite lungs being the primary site, there may be multisystemic involvement, very rarely involving bone marrow, a dreaded manifestation of disseminated tuberculosis, associated with high mortality and morbidity. We report a case of tuberculosis of bone marrow with concomitant secondary hemophagocytic lympho-histiocytosis, bringing into light the importance of clinical suspicion and evaluation of bone marrow being a primary site of involvement in patients of disseminated tuberculosis.
Assuntos
Icterícia Obstrutiva , Linfo-Histiocitose Hemofagocítica , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/complicações , Icterícia Obstrutiva/etiologia , Icterícia Obstrutiva/diagnóstico , Masculino , Medula Óssea/patologia , Diagnóstico Diferencial , Doenças da Medula Óssea/complicações , Doenças da Medula Óssea/diagnóstico , Tuberculose/diagnóstico , Tuberculose/complicações , Antituberculosos/uso terapêutico , AdultoRESUMO
Non-neoplastic bone marrow disorders are main causes of non-regenerative anemia in dogs. Despite the high incidence of the diseases, their molecular pathophysiology has not been elucidated. We previously reported that Miniature Dachshund (MD) was a predisposed breed to be diagnosed with non-neoplastic bone marrow disorders in Japan, and immunosuppressive treatment-resistant MDs showed higher number of platelets and morphological abnormalities in peripheral blood cells. These data implied that treatment-resistant MDs might possess distinct pathophysiological features from treatment-responsive MDs. Therefore, we conducted transcriptomic analysis of bone marrow specimens to investigate the pathophysiology of treatment-resistant MDs. Transcriptomic analysis comparing treatment-resistant MDs and healthy control dogs identified 179 differentially expressed genes (DEGs). Pathway analysis using these DEGs showed that "Wnt signaling pathway" was a significantly enriched pathway. We further examined the expression levels of DEGs associated with Wnt signaling pathway and confirmed the upregulation of AXIN2 and CCND2 and the downregulation of SFRP2 in treatment-resistant MDs compared with treatment-responsive MDs and healthy control dogs. This alteration implied the activation of Wnt signaling pathway in treatment-resistant MDs. The activation of Wnt signaling pathway has been reported in human patients with myelodysplastic syndrome (MDS), which is characterized by dysplastic features of blood cells. Therefore, the results of this study implied that treatment-resistant MDs have distinct molecular pathological features from treatment-responsive MDs and the pathophysiology of treatment-resistant MDs might be similar to that of human MDS patients.
Assuntos
Doenças do Cão , Perfilação da Expressão Gênica , Cães , Animais , Doenças do Cão/genética , Doenças do Cão/patologia , Perfilação da Expressão Gênica/veterinária , Medula Óssea/patologia , Síndromes Mielodisplásicas/veterinária , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/patologia , Transcriptoma , Masculino , Feminino , Via de Sinalização Wnt , Doenças da Medula Óssea/veterinária , Doenças da Medula Óssea/genética , Doenças da Medula Óssea/patologiaAssuntos
Leucemia Mieloide Aguda , Linfoma Difuso de Grandes Células B , Síndrome de Shwachman-Diamond , Humanos , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/complicações , Leucemia Mieloide Aguda/patologia , Leucemia Mieloide Aguda/complicações , Masculino , Adulto Jovem , Lipomatose/patologia , Lipomatose/complicações , Insuficiência Pancreática Exócrina/complicações , Insuficiência Pancreática Exócrina/genética , Insuficiência Pancreática Exócrina/patologia , Doenças da Medula Óssea/patologia , AdultoRESUMO
BACKGROUND: It is beneficial for society to discover the risk factors associated with surgery and to carry out some early interventions for patients with these risk factors. Few studies specifically explored the relationship between bone marrow lesions (BMLs) and long-term incident joint surgery. OBJECTIVE: To investigate the association between BML severity observed in knee osteoarthritis (OA) patients' first MRI examination and incident knee surgery within 5 years. Additionally, to assess the predictive value of BMLs for the incident knee surgery. DESIGN: Retrospective cohort study. METHODS: We identified patients diagnosed with knee OA and treated at our institution between January 2015 and January 2018, and retrieved their baseline clinical data and first MRI examination films from the information system. Next, we proceeded to determine the Max BML grades, BML burden grades and Presence BML grades for the medial, lateral, patellofemoral, and total compartments, respectively. Multi-variable logistic regression models examined the association of the BML grades with 5-year incident knee surgery. Positive and negative predictive values (PPVs and NPVs) were determined for BML grades referring to 5-year incident knee surgery. RESULTS: Totally, 1011 participants (knees) were found eligible to form the study population. Within the 5 years, surgery was performed on 74 knees. Max BML grade 2 and grade 3 of medial, patellofemoral and total compartments were strongly and significantly associated with incident surgery. None of the BML grades from lateral compartment was associated with incident surgery. The PPV was low and NPV was high for BMLs. CONCLUSIONS: BMLs found in the first MRI examination were associated with 5-year incident joint surgery, except for those allocated in lateral compartments. The high NPVs imply that patients without BMLs have a low risk of requiring surgery within 5 years.
Assuntos
Medula Óssea , Imageamento por Ressonância Magnética , Osteoartrite do Joelho , Humanos , Osteoartrite do Joelho/cirurgia , Osteoartrite do Joelho/diagnóstico por imagem , Osteoartrite do Joelho/patologia , Estudos Retrospectivos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Medula Óssea/diagnóstico por imagem , Medula Óssea/patologia , Estudos de Coortes , Fatores de Tempo , Fatores de Risco , Articulação do Joelho/diagnóstico por imagem , Articulação do Joelho/cirurgia , Articulação do Joelho/patologia , Doenças da Medula Óssea/diagnóstico por imagem , Doenças da Medula Óssea/etiologia , Doenças da Medula Óssea/patologia , Artroplastia do Joelho/métodos , Índice de Gravidade de DoençaRESUMO
Bone marrow failure (BMF) has become one of the most studied autoimmune disorders, particularly due to its prevalence both as an inherited disease, but also as a result of chemotherapies. BMF is associated with severe symptoms such as bleeding episodes and susceptibility to infections, and often has underlying characteristics, such as anemia, thrombocytopenia, and neutropenia. The current treatment landscape for BMF requires stem cell transplantation or chemotherapies to induce immune suppression. However, there is limited donor cell availability or dose related toxicity associated with these treatments. Optimizing these treatments has become a necessity. Polymer-based materials have become increasingly popular, as current research efforts are focused on synthesizing novel cell matrices for stem cell expansion to solve limited donor cell availability, as well as applying polymer delivery vehicles to intracellularly deliver cargo that can aid in immunosuppression. Here, we discuss the importance and impact of polymer materials to enhance therapeutics in the context of BMF.
Assuntos
Polímeros , Humanos , Polímeros/química , Animais , Doenças da Medula Óssea/induzido quimicamente , Doenças da Medula Óssea/terapia , Transtornos da Insuficiência da Medula Óssea/terapia , Materiais BiocompatíveisRESUMO
Recent advances in in-depth data-independent acquisition proteomic analysis have enabled comprehensive quantitative analysis of >10,000 proteins. Herein, an integrated proteogenomic analysis for inherited bone marrow failure syndrome (IBMFS) was performed to reveal their biological features and to develop a proteomic-based diagnostic assay in the discovery cohort; dyskeratosis congenita (n = 12), Fanconi anemia (n = 11), Diamond-Blackfan anemia (DBA, n = 9), Shwachman-Diamond syndrome (SDS, n = 6), ADH5/ALDH2 deficiency (n = 4), and other IBMFS (n = 18). Unsupervised proteomic clustering identified eight independent clusters (C1-C8), with the ribosomal pathway specifically downregulated in C1 and C2, enriched for DBA and SDS, respectively. Six patients with SDS had significantly decreased SBDS protein expression, with two of these not diagnosed by DNA sequencing alone. Four patients with ADH5/ALDH2 deficiency showed significantly reduced ADH5 protein expression. To perform a large-scale rapid IBMFS screening, targeted proteomic analysis was performed on 417 samples from patients with IBMFS-related hematological disorders (n = 390) and healthy controls (n = 27). SBDS and ADH5 protein expressions were significantly reduced in SDS and ADH5/ALDH2 deficiency, respectively. The clinical application of this first integrated proteogenomic analysis would be useful for the diagnosis and screening of IBMFS, where appropriate clinical screening tests are lacking.
Assuntos
Doenças da Medula Óssea , Transtornos da Insuficiência da Medula Óssea , Proteogenômica , Humanos , Transtornos da Insuficiência da Medula Óssea/genética , Transtornos da Insuficiência da Medula Óssea/patologia , Proteogenômica/métodos , Masculino , Feminino , Doenças da Medula Óssea/genética , Doenças da Medula Óssea/patologia , Criança , Adulto , Adolescente , Pré-Escolar , Anemia de Diamond-Blackfan/genética , Anemia de Diamond-Blackfan/diagnóstico , Adulto Jovem , Anemia de Fanconi/genética , Anemia de Fanconi/diagnóstico , Proteômica/métodos , Lactente , Síndrome de Shwachman-Diamond/genética , Disceratose Congênita/genética , Disceratose Congênita/diagnóstico , Disceratose Congênita/patologiaRESUMO
Dual-energy computed tomography (CT) is an excellent substitute for identifying bone marrow edema in magnetic resonance imaging. However, it is rarely used in practice owing to its low contrast. To overcome this problem, we constructed a framework based on deep learning techniques to screen for diseases using axial bone images and to identify the local positions of bone lesions. To address the limited availability of labeled samples, we developed a new generative adversarial network (GAN) that extends expressions beyond conventional augmentation (CA) methods based on geometric transformations. We theoretically and experimentally determined that combining the concepts of data augmentation optimized for GAN training (DAG) and Wasserstein GAN yields a considerably stable generation of synthetic images and effectively aligns their distribution with that of real images, thereby achieving a high degree of similarity. The classification model was trained using real and synthetic samples. Consequently, the GAN technique used in the diagnostic test had an improved F1 score of approximately 7.8% compared with CA. The final F1 score was 80.24%, and the recall and precision were 84.3% and 88.7%, respectively. The results obtained using the augmented samples outperformed those obtained using pure real samples without augmentation. In addition, we adopted explainable AI techniques that leverage a class activation map (CAM) and principal component analysis to facilitate visual analysis of the network's results. The framework was designed to suggest an attention map and scattering plot to visually explain the disease predictions of the network.
Assuntos
Aprendizado Profundo , Edema , Tomografia Computadorizada por Raios X , Humanos , Tomografia Computadorizada por Raios X/métodos , Edema/diagnóstico por imagem , Imagem Radiográfica a Partir de Emissão de Duplo Fóton/métodos , Redes Neurais de Computação , Doenças da Medula Óssea/diagnóstico por imagem , Medula Óssea/diagnóstico por imagem , AlgoritmosRESUMO
Cytogenetic studies are essential in the diagnosis and follow up of patients with bone marrow failure syndromes (BMFSs), but obtaining good quality results is often challenging due to hypocellularity. Optical Genome Mapping (OGM), a novel technology capable of detecting most types chromosomal structural variants (SVs) at high resolution, is being increasingly used in many settings, including hematologic malignancies. Herein, we compared conventional cytogenetic techniques to OGM in 20 patients with diverse BMFSs. Twenty metaphases for the karyotype were only obtained in three subjects (15%), and no SVs were found in any of the samples. One patient with culture failure showed a gain in chromosome 1q by fluorescence in situ hybridization, which was confirmed by OGM. In contrast, OGM provided good quality results in all subjects, and SVs were detected in 14 of them (70%), mostly corresponding to cryptic submicroscopic alterations not observed by standard techniques. Therefore, OGM emerges as a powerful tool that provides complete and evaluable results in hypocellular BMFSs, reducing multiple tests into a single assay and overcoming some of the main limitations of conventional techniques. Furthermore, in addition to confirming the abnormalities detected by conventional techniques, OGM found new alterations beyond their detection limits.
Assuntos
Hibridização in Situ Fluorescente , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Idoso , Hibridização in Situ Fluorescente/métodos , Mapeamento Cromossômico/métodos , Transtornos da Insuficiência da Medula Óssea/genética , Aberrações Cromossômicas , Adolescente , Análise Citogenética/métodos , Doenças da Medula Óssea/genética , Cariotipagem/métodos , Adulto JovemRESUMO
OBJECTIVE: To determine whether the presence of preoperative subchondral bone marrow oedema (SBME) is associated with inferior outcomes after lateral unicompartmental knee arthroplasty (LUKA). STUDY DESIGN: Descriptive study. Place and Duration of the Study: Department of Orthopaedic Surgery, Chongqing Orthopaedic Hospital of Traditional Chinese Medicine, Chongqing, China, from January 2019 to June 2022. METHODOLOGY: Data on patients treated with LUKA were obtained from the Medical Registry Database. Two groups were made based on the presence and absence of SBME on preoperative magnetic resonance imaging (MRI). The visual analogue scale (VAS), American Knee Society Scores (AKSS), and rate of patient satisfaction were compared between the two groups. RESULTS: A total of 20 patients treated with LUKA were reviewed. The SBME was present in 9 cases and absent in 11 cases. Patients with SBME had inferior scores at preoperative evaluation and at 1, 3, and 6 months postoperatively. However, there was no significant difference between the groups at the 12-month follow-up. Eight (88.9%) patients with SBME were satisfied with the LUKA surgery versus 9 (81.8%) patients without SBME, showing no significant differences between groups. CONCLUSION: Presence of preoperative SBME is associated with inferior functional outcomes after LUKA within six months of follow-up. KEY WORDS: Bone marrow, Oedema, Knee, Arthroplasty, Outcome, Patient satisfaction.
Assuntos
Artroplastia do Joelho , Doenças da Medula Óssea , Edema , Humanos , Artroplastia do Joelho/métodos , Masculino , Feminino , Pessoa de Meia-Idade , Edema/etiologia , Idoso , Doenças da Medula Óssea/cirurgia , Resultado do Tratamento , Imageamento por Ressonância Magnética , Satisfação do Paciente , Osteoartrite do Joelho/cirurgia , Estudos Retrospectivos , Articulação do Joelho/cirurgia , Período Pré-Operatório , Medula Óssea/patologia , China/epidemiologiaRESUMO
BACKGROUND: An inflammatory bone marrow microenvironment contributes to acquired bone marrow failure syndromes. CK0801, an allogeneic T regulatory (Treg) cell therapy product, can potentially interrupt this continuous loop of inflammation and restore hematopoiesis. METHODS: In this phase 1 dose-escalation study of CK0801 Treg cells, we enrolled patients with bone marrow failure syndromes with suboptimal response to their prior therapy to determine the safety and efficacy of this treatment for bone marrow failure syndromes. RESULTS: We enrolled nine patients with a median age of 57 years (range, 19 to 74) with an underlying diagnosis of aplastic anemia (n=4), myelofibrosis (n=4), or hypoplastic myelodysplasia (n=1). Patients had a median of three prior therapies for a bone marrow failure syndrome. Starting dose levels of CK0801 were 1 × 106 (n=3), 3 × 106 (n=3), and 10 × 106 (n=3) cells per kg of ideal body weight. No lymphodepletion was administered. CK0801 was administered in the outpatient setting with no infusion reactions, no grade 3 or 4 severe adverse reactions, and no dose-limiting toxicity. At 12 months, CK0801 induced objective responses in three of four patients with myelofibrosis (two had symptom response, one had anemia response, and one had stable disease) and three of four patients with aplastic anemia (three had partial response). Three of four transfusion-dependent patients at baseline achieved transfusion independence. Although the duration of observation was limited at 0.9 to 12 months, there were no observed increases in infections, no transformations to leukemia, and no deaths. CONCLUSIONS: In previously treated patients, CK0801 demonstrated no dose-limiting toxicity and showed evidence of efficacy, providing proof of concept for targeting inflammation as a therapy for bone marrow failure. (Funded by Cellenkos Inc.; Clinicaltrials.gov number, NCT03773393.).
Assuntos
Anemia Aplástica , Transtornos da Insuficiência da Medula Óssea , Humanos , Pessoa de Meia-Idade , Idoso , Masculino , Adulto , Feminino , Transtornos da Insuficiência da Medula Óssea/terapia , Anemia Aplástica/terapia , Doenças da Medula Óssea/terapia , Adulto Jovem , Mielofibrose Primária/terapia , Linfócitos T Reguladores/imunologiaRESUMO
BACKGROUND: Bone marrow lesions (BMLs) in knee osteoarthritis (OA) have been assessed histopathologically and by dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI); however, a direct comparison of the results has not been reported. PURPOSE: To evaluate and compare the findings by DCE-MRI and histopathology of subchondral BMLs in knee OA. MATERIAL AND METHODS: In total, 19 patients with medial tibiofemoral knee OA undergoing total knee arthroplasty were analyzed. Preoperative MRI, including a DCE sequence, was performed, and bone biopsies were obtained from the resected specimens corresponding to BML areas. The contrast enhancement by DCE-MRI was analyzed using semi-quantitative (area under the curve [AUC]), peak enhancement [PE]), and quantitative (Ktrans, Kep) methods. Enhancement in the medial OA compartment was compared with similar areas in a normal lateral compartment, and the DCE characteristics of BMLs were correlated with semi-quantitatively graded histopathological features. RESULTS: AUC and PE were significantly higher in medial tibial and femoral BMLs compared with the values in the lateral condyles; Ktrans and Kep were only significantly higher in the tibial plateau. In the tibia, AUC and PE were significantly correlated with the grade of vascular proliferation, and PE also with the degree of marrow fibrosis. There was no significant correlation between AUC/PE and histopathological findings in the femur and no correlation between quantitative DCE parameters and histopathological findings. CONCLUSION: BML characteristics by semi-quantitative DCE in the form of AUC and PE may be used as parameters for the degree of histopathological vascularization in the bone marrow whereas quantitative DCE data were less conclusive.
Assuntos
Medula Óssea , Meios de Contraste , Imageamento por Ressonância Magnética , Osteoartrite do Joelho , Humanos , Osteoartrite do Joelho/diagnóstico por imagem , Osteoartrite do Joelho/patologia , Feminino , Masculino , Imageamento por Ressonância Magnética/métodos , Idoso , Pessoa de Meia-Idade , Medula Óssea/diagnóstico por imagem , Medula Óssea/patologia , Doenças da Medula Óssea/diagnóstico por imagem , Doenças da Medula Óssea/patologia , Articulação do Joelho/diagnóstico por imagem , Articulação do Joelho/patologia , Idoso de 80 Anos ou mais , Gadolínio DTPAAssuntos
Transtornos da Insuficiência da Medula Óssea , Humanos , Gravidez , Feminino , Adulto , Resultado da Gravidez , Complicações Hematológicas na Gravidez/terapia , Doenças da Medula Óssea , Anemia Aplástica/terapia , Recém-Nascido , Hemoglobinúria Paroxística/terapia , Hemoglobinúria Paroxística/diagnósticoRESUMO
Bone marrow edema (BME) is a frequent MRI finding in patients with knee pain. According to the etiology, BME of the knee can be classified into three main categories: ischemic, mechanic, and reactive. The diagnosis may be difficult, because of the specificity of symptoms and the poor radiographic findings. MRI is the gold standard, showing an area of altered signal of the bone with an high signal intensity on fat-suppressed, T2 weighted images, usually in combination with an intermediate or low signal intensity on T1 weighted images. Bone marrow edema tends to be self-limiting and, in most cases, resolves without any consequences in a varying amount of time. However, since it may evolve to complete joint destruction, early diagnosis and correct treatment are crucial to prevent the articular degeneration. Conservative therapy is the first step, with no weight-bearing for 3 to 6 weeks on the affected side, in combination with the administration of anti-inflammatory drugs or painkillers to manage symptoms. In non-responding forms and more advanced stages, minimally invasive preservative surgery can provide significant results, with subchondroplasty and core decompression being the two main procedures available. Knee arthroplasty, both total (TKA) or unicompartmental (UKA), is the only effective option when the degradation of cartilage is diffuse and in patients with subchondral bone collapse.
Assuntos
Doenças da Medula Óssea , Edema , Articulação do Joelho , Imageamento por Ressonância Magnética , Humanos , Edema/etiologia , Doenças da Medula Óssea/terapia , Doenças da Medula Óssea/diagnóstico por imagem , Doenças da Medula Óssea/etiologia , Articulação do Joelho/diagnóstico por imagemRESUMO
This study aimed to investigate the clinical predictors, including traditional Chinese medicine tongue characteristics and other clinical parameters for chemotherapy-induced myelosuppression (CIM), and then to develop a clinical prediction model and construct a nomogram. A total of 103 patients with lung cancer were prospectively enrolled in this study. All of them were scheduled to receive first-line chemotherapy regimens. Participants were randomly assigned to either the training group (nâ =â 52) or the test group (nâ =â 51). Tongue characteristics and clinical parameters were collected before the start of chemotherapy, and then the incidence of myelosuppression was assessed after treatment. We used univariate logistic regression analysis to identify the risk predictors for assessing the incidence of CIM. Moreover, we developed a predictive model and a nomogram using multivariate logistic regression analysis. Finally, we evaluated the predictive performance of the model by examining the area under the curve value of the receiver operating characteristic, calibration curve, and decision curve analysis. As a result, a total of 3 independent predictors were found to be associated with the CIM in multivariate regression analysis: the fat tongue (ORâ =â 3.67), Karnofsky performance status score (ORâ =â 0.11), and the number of high-toxic drugs in chemotherapy regimens (ORâ =â 4.78). Then a model was constructed using these 3 predictors and it exhibited a robust predictive performance with an area under the curve of 0.82 and the consistent calibration curves. Besides, the decision curve analysis results suggested that applying this predictive model can result in more net clinical benefit for patients. We established a traditional Chinese medicine prediction model based on the tongue characteristics and clinical parameters, which could serve as a useful tool for assessing the risk of CIM.
Assuntos
Antineoplásicos , Doenças da Medula Óssea , Neoplasias Pulmonares , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Modelos Estatísticos , Prognóstico , LínguaRESUMO
We report 5 children with bone marrow failure (BMF) after primary varicella zoster virus (VZV) infection or VZV vaccination, highlighting the highly variable course. Two patients were treated with intravenous immunoglobulins; one had a slow hematologic recovery, and the other was rescued by allogeneic hematopoietic stem cell transplantation (HSCT). Of the 2 patients treated with immunosuppressive therapy with antithymocyte globulin and cyclosporine, one had a complete response, and the other was transplanted for nonresponse. One patient underwent a primary allograft. All patients are alive. This study demonstrated that VZV-associated BMF is a life-threatening disorder that often requires HSCT.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Infecção pelo Vírus da Varicela-Zoster , Humanos , Masculino , Feminino , Criança , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Pré-Escolar , Herpesvirus Humano 3 , Transtornos da Insuficiência da Medula Óssea/etiologia , Vacinação/efeitos adversos , Doenças da Medula Óssea/etiologia , Vacina contra Varicela/efeitos adversos , Adolescente , Imunoglobulinas Intravenosas/uso terapêutico , LactenteRESUMO
BACKGROUND: Fat quantification methods in magnetic resonance imaging (MRI) have been studied to differentiate bone marrow pathologies in adult patients; however, scarce literature is available in pediatric patients. PURPOSE: To evaluate the efficacy of the T1 signal intensity value (T1-SIV), out-of-phase/in-phase signal ratio (OP/IP SR), and fat fraction (FF) to differentiate between normal, benign, and malignant pathological processes. MATERIAL AND METHODS: A total of 48 pediatric patients with lumbar and pelvic MRI were classified into three groups according to bone marrow pathology (group 1, normal; group 2, benign pathology/reconversion; group 3, malignant). The efficacy of T1-SIV, OP/IP SR, and FF values in differentiating these pathologies was evaluated using Kruskal-Wallis or analysis of variance and followed by Bonferroni or Dunn-Bonferroni tests. Cutoff values for malignant infiltration were defined using ROC analysis. RESULTS: Although these values were significantly different in all three groups (P = 0.001-0.008), this difference was not sufficient to discriminate between all groups. Subgroup analyses showed significant differences in T1-SIV between groups 1-3, in OP/IP SR between groups 1-3, 2-3, and 1-2, in FF between groups 1-2 and 1-3 in various regions (P = 0.001-0.049). Cutoff values had a sensitivity and specificity of 90%-100% for OP/IP SR and FF. CONCLUSION: T1-SIV, OP/IP SR, and FF may potentially distinguish normal from pathological bone marrow. OP/IP SR and FF values detected malignant infiltration with high sensitivity and specificity in this study. However, only OP/IP SR may significantly differentiate benign and malignant bone marrow pathologies which needs to be confirmed in the future study with a larger patient population.
Assuntos
Tecido Adiposo , Doenças da Medula Óssea , Medula Óssea , Imageamento por Ressonância Magnética , Humanos , Criança , Imageamento por Ressonância Magnética/métodos , Feminino , Masculino , Diagnóstico Diferencial , Adolescente , Tecido Adiposo/diagnóstico por imagem , Tecido Adiposo/patologia , Pré-Escolar , Doenças da Medula Óssea/diagnóstico por imagem , Medula Óssea/diagnóstico por imagem , Medula Óssea/patologia , Neoplasias da Medula Óssea/diagnóstico por imagem , Sensibilidade e Especificidade , Estudos Retrospectivos , LactenteRESUMO
PURPOSE: Invasive lobular carcinoma (ILC) exhibits a low affinity for 18F-FDG. The estrogen receptor (ER) is commonly expressed in ILCs, suggesting a potential benefit of targeting with the ER probe 18F-FES in this patient population. The objective of this study was to evaluate the diagnostic performance of 18F-FES imaging in patients with metastatic ILC and compare it with that of 18F-FDG. METHODS: We conducted a retrospective analysis of 20 ILC patients who underwent concurrent 18F-FES and 18F-FDG PET/CT examinations in our center. 18F-FES and 18F-FDG imaging were analyzed to determine the total count of tracer-avid lesions in nonbone sites and their corresponding organ systems, assess the extent of anatomical regions involved in bone metastases, and measure the SUVmax values for both tracers. RESULTS: Among 20 ILC patients, 65 nonbone lesions were found to be distributed in 13 patients, and 16 patients were diagnosed with bone metastasis, which was distributed in 54 skeletal anatomical regions. The detection rate of 18F-FDG in nonbone lesions was higher than that of 18F-FES (57 vs 37, P < 0.001). 18F-FES demonstrated a superior ability to detect nonbone lesions in 4 patients, whereas 18F-FDG was superior in 5 patients (P > 0.05). Among 9/16 patients with bone metastasis, 18F-FES demonstrated a significant advantage in the detection of bone lesions compared with 18F-FDG (P = 0.05). Furthermore, patients with only 18F-FES-positive lesions (12/12) were administered endocrine regimens, whereas patients lacking 18F-FES uptake (2/3) predominantly received chemotherapy. CONCLUSIONS: 18F-FES is more effective than 18F-FDG in detecting bone metastasis in ILC, but it does not demonstrate a significant advantage in nonbone lesions. Additionally, the results of examination with 18F-FES have the potential to guide patient treatment plans.
Assuntos
Doenças da Medula Óssea , Neoplasias Ósseas , Neoplasias da Mama , Carcinoma Lobular , Humanos , Feminino , Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Carcinoma Lobular/diagnóstico por imagem , Carcinoma Lobular/patologia , Estudos Retrospectivos , Receptores de Estrogênio , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/tratamento farmacológico , Neoplasias da Mama/diagnóstico por imagem , Neoplasias da Mama/tratamento farmacológicoRESUMO
OBJECTIVES: The study aimed to explore the value of texture analysis of radiomics based on the short tau inversion recovery (STIR) sequence to evaluate the activity of bone marrow oedema of sacroiliac joints in early AS. METHODS: 43 patients with early AS whose data were randomly divided into the training cohort (n=116) and verification cohort (n=56) according to the ratio of 7:3. The optimal feature subsets were obtained by Mann-Whitney U-test, the minimum-Redundancy Maximum-Relevancy (mRMR), and then least absolute shrinkage and selection operator (LASSO) using these texture feature parameters, which were used to construct the final prediction model and obtained the Radscore. The ROC curve was performed to evaluate the performance of the model. The Spearman correlation test was used to analyse the correlation of various indicators. RESULTS: In the training cohort, to differentiate early AS sacroiliac joint bone marrow oedema between the active and stable groups, the AUCs of the Radscore, SPARCC and ADC were 0.81, 0.91, 0.78, respectively. In the validation cohort, the AUCs were 0.87, 0.89, 0.85. In the two cohorts, there were no significant differences in AUCs between values of the Radscore and SPARCC, ADC (p>0.05). There was a significant difference in AUC between SPARCC and ADC in the training cohort (p<0.05), with no statistical significance in the validation cohort (p>0.05). The correlations were all low between the Radscore values and the values of ESR, CRP, tI, ASDAS-ESR and ASDAS-CRP (p<0.05). CONCLUSIONS: Radiomics analysis based on STIR texture analysis has a good prediction for the evaluation of bone marrow oedema activity of sacroiliac joints in AS. It can be a new non-invasive and objective evaluation method for AS activity.
Assuntos
Edema , Imageamento por Ressonância Magnética , Valor Preditivo dos Testes , Articulação Sacroilíaca , Espondilite Anquilosante , Humanos , Masculino , Feminino , Espondilite Anquilosante/diagnóstico por imagem , Articulação Sacroilíaca/diagnóstico por imagem , Adulto , Edema/diagnóstico por imagem , Edema/etiologia , Reprodutibilidade dos Testes , Pessoa de Meia-Idade , Doenças da Medula Óssea/diagnóstico por imagem , Curva ROC , Medula Óssea/diagnóstico por imagem , Adulto Jovem , Diagnóstico Precoce , Estudos Retrospectivos , Índice de Gravidade de Doença , RadiômicaRESUMO
Pure red cell aplasia (PRCA) is a rare bone marrow (BM) disorder characterized by ineffective erythropoiesis, reduced reticulocyte count, normocytic anemia, and the absence of erythroid precursors. Here, we present a rare instance of PRCA occurring after ABO-matched allo-HSCT in a refractory/relapsed acute myeloid leukemia (R/R AML) patient. In this case, the patient received a combination treatment of Gilteritinib, Venetoclax, and Azacitidine. Remarkably, this treatment not only reduced myeloblasts but also facilitated the restoration of erythroid hematopoiesis.
Assuntos
Compostos de Anilina , Doenças da Medula Óssea , Compostos Bicíclicos Heterocíclicos com Pontes , Transplante de Células-Tronco Hematopoéticas , Leucemia Mieloide Aguda , Pirazinas , Aplasia Pura de Série Vermelha , Sulfonamidas , Humanos , Compostos de Anilina/uso terapêutico , Azacitidina/uso terapêutico , Doenças da Medula Óssea/complicações , Compostos Bicíclicos Heterocíclicos com Pontes/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Leucemia Mieloide Aguda/terapia , Leucemia Mieloide Aguda/complicações , Pirazinas/uso terapêutico , Aplasia Pura de Série Vermelha/etiologia , Sulfonamidas/uso terapêuticoRESUMO
Haploidentical stem cell transplantation (haplo-SCT) represents the main alternative for children with inherited bone marrow failure syndrome (I-BMF) lacking a matched donor. This retrospective study, conducted on behalf of the EBMT SAAWP and PDWP, aims to report the current outcomes of haplo-SCT in I-BMFs, comparing the different in vivo and ex vivo T-cell depletion approaches. One hundred and sixty-two I-BMF patients who underwent haplo-SCT (median age 7.4 years) have been registered. Fanconi Anemia was the most represented diagnosis (70.1%). Based on different T-cell depletion (TCD) approaches, four categories were identified: (1) TCRαß+/CD19+-depletion (43.8%); (2) T-repleted with post-transplant Cyclophosphamide (PTCy, 34.0%); (3) In-vivo T-depletion with ATG/alemtuzumab (14.8%); (4) CD34+ positive selection (7.4%). The cumulative incidences (CI) of neutrophil and platelet engraftment were 84% and 76% respectively, while that of primary and secondary graft failure was 10% and 8% respectively. The 100-day CI of acute GvHD grade III-IV(95% CI) was 13%, while the 24-month CI of extensive chronic GvHD was 4%. After a median follow-up of 43.4 months, the 2-year overall survival(OS) and GvHD/Rejection-free Survival (GRFS) probabilities are 67% and 53%, respectively. The TCR CD3+αß+/CD19+ depletion group showed a significantly lower incidence of both acute and chronic GvHD and higher OS (79%; p0.013) and GRFS (71%; p < .001), while no significant differences in outcomes have been observed by different diagnosis and conditioning regimens. This large retrospective study supports the safety and feasibility of haplo-SCT in I-BMF patients. TCRαß+/CD19+ depletion offers higher chances of patients' survival, with a significantly lower risk of severe a- and c-GvHD in I-BMFs compared to other platforms.