RESUMEN
The classification of clonal plasmacytoid dendritic cell (pDC) proliferation associated with myeloid neoplasms remains a topic of ongoing debate. Although the fifth edition of the World Health Organization classification classifies clonal pDC proliferation into two categories, it is unclear whether this classification adequately captures the complexities of clonal pDC pathogenesis. We present a clinical case featuring myeloid sarcoma with pDC-like cells in cervical lymph nodes and bone marrow (BM). Analysis of biopsy specimens and BM aspirate revealed two distinct cellular populations expressing myeloid and pDC markers. One population exhibited myeloid leukemia and monocyte markers, including MPO, CD13, CD33, CD11b, and CD14, while the other manifested an immunophenotype reminiscent of pDCs, characterized by expression of CD56 and CD123. Additionally, whole exome sequencing and RNA sequencing of BM mononuclear cells were conducted to explore the pathophysiology of this rare malignancy, and unveiled pDC-like cell proliferation driven by IKZF1 and ETV6 mutations originating from clonal hematopoiesis initiated by a DNMT3A mutation. Notably, venetoclax-based therapy exhibited efficacy for achieving and sustaining complete remission. This case provides pivotal insights into the mechanistic aspects of pDC/pDC-like cell proliferation in myeloid sarcoma, offering valuable perspectives on therapeutic strategies.
Asunto(s)
Proliferación Celular , ADN (Citosina-5-)-Metiltransferasas , ADN Metiltransferasa 3A , Células Dendríticas , Factor de Transcripción Ikaros , Mutación , Proteínas Represoras , Sarcoma Mieloide , Humanos , Sarcoma Mieloide/genética , Sarcoma Mieloide/diagnóstico , Sarcoma Mieloide/patología , Factor de Transcripción Ikaros/genética , ADN Metiltransferasa 3A/genética , ADN (Citosina-5-)-Metiltransferasas/genética , Células Dendríticas/patología , Proteínas Represoras/genética , Masculino , Persona de Mediana Edad , FemeninoRESUMEN
High-grade B-cell lymphoma with 11q aberrations (HGBL-11q) has been classified for the first time as a high-grade mature B-cell neoplasm according to the 5th edition of the World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues. HGBL-11q is morphologically and immunohistochemically similar to Burkitt lymphoma (BL) or HGBL; it is characterized by gain in the 11q23.2-11q23.3 region and loss in the 11q24.1-qter region but it lacks MYC translocation. HGBL-11q is a rare tumor, and its exact frequency in Japan remains unclear. In this study, we classified 113 Germinal center B-cell (GCB) type aggressive B-cell lymphomas (BCLs), which were divided into BL, high-grade (HG), and large cell (LC) morphologies. We performed fluorescence in situ hybridization (FISH) to identify 11q aberrations. Nine patients had 11q aberrations (7.96%, 9/113), including six HGBL-11q. The age range was from 8 to 87 years, and all were male. Six out of 14 patients with HG morphology were diagnosed with HGBL-11q (6/14, 42.9%). HGBL-11q has been found to occur primarily in children and young adults but also in middle-aged and older adults. Patients with HG morphology without MYC translocation should undergo FISH for 11q aberrations regardless of age. However, the pathogenesis, clinical findings, and prognosis of HGBL-11q remain unclear. The accumulation of cases with an accurate HGBL-11q diagnosis in daily practice and accurate and detailed data on HGBL-11q will contribute to further understanding of 11q aberrations.
Asunto(s)
Linfoma de Burkitt , Aberraciones Cromosómicas , Masculino , Humanos , Femenino , Hibridación Fluorescente in Situ , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/genética , Translocación Genética , JapónRESUMEN
Endoscopic resection is a treatment of choice for a metachronous early-stage esophageal squamous cell carcinoma (ESCC) appearing after a radical cure of esophageal cancer by chemoradiotherapy (CRT). However, non-curative resection, and procedural complications including perforation due to radiation-induced submucosal fibrosis, are a concern. This study aimed to evaluate the association between submucosal fibrosis and the usefulness and safety of endoscopic submucosal dissection (ESD) in ESCC after CRT. This study retrospectively analyzed 13 lesions in 11 patients in our institute. Submucosal fibrosis under the lesion (F score) was classified into three levels (F0: none or mild, F1: moderate, and F2: severe) based on endoscopic and histopathologic findings. All lesions were F1 or greater (F1: 8 lesions and F2: 5 lesions). En bloc and R0 resection rates were both 100%. The procedural speed was slower in F2 than in F1 (F1 vs. F2; 15.1 mm2/min vs. 7.1 mm2/min, p = 0.019), without procedure-related adverse events. At a median follow-up of 42 months (range: 14-117 months) after ESD, 7 of 11 (63.6%) patients were alive without recurrence, and without ESCC-related death. ESCC after CRT reliably and safely resected en bloc by ESD but was more difficult in lesions with strong submucosal fibrosis.
RESUMEN
C-reactive protein (CRP) is an important biomarker of infection and inflammation, as CRP is one of the most prominent acute-phase proteins. CRP is usually detected using anti-CRP antibodies (Abs), where the intermolecular interactions between CRP and the anti-CRP Ab are largely affected by the pH and ionic strength of environmental solutions. Therefore, it is important to understand the environmental effects of CRP-anti-CRP Ab interactions when designing highly sensitive biosensors. Here, we investigated the efficiency of fluorescently labeled CRP-anti-CRP monoclonal antibody (mAb) interactions at different pHs and ionic strengths. Our results indicate that the affinity was insensitive to pH changes in the range of 5.9 to 8.1, while it was significantly sensitive to ionic strength changes. The binding affinity decreased by 55% at an ionic strength of 1.6 mM, when compared to that under a physiological condition (~150 mM). Based on the isoelectric focusing results, both the labeled CRP and anti-CRP mAb were negatively charged in the studied pH range, which rendered the system insensitive to pH changes, but sensitive to ionic strength changes. The decreased ionic strength led to a significant enhancement of the repulsive force between CRP and the anti-CRP mAb. Although the versality of the findings is not fully studied yet, the results provide insights into designing highly sensitive CRP sensors, especially field-effect transistor-based sensors.
RESUMEN
The diagnosis of plasmablastic lymphoma (PBL), plasmablastic myeloma (PBM), and plasmablastic neoplasm (PBN) may be arbitrary in some cases because these entities can be indistinct. We conducted this scoping review to investigate heterogeneity in diagnostic criteria used in previous studies and validate the diagnostic results of previous diagnostic algorithms and the algorithm we developed, which also includes diagnosis of PBN. Using the PRISMA Extension for Scoping Reviews, we analyzed literature published between September 2017 and April 2020. We identified a total of 163 cases (128 PBL, 32 PBM, and 3 PBN) from 77 case reports and 8 case series. We found that diagnostic criteria in the literature varied for PBL but were consistent for PBM. Our algorithm was the first attempt to include PBN in a complete structure. The results of the three diagnostic algorithms varied significantly. Hematologists and pathologists should pay more attention to the differential diagnosis of PBL, PBM, and PBN.
Asunto(s)
Mieloma Múltiple/diagnóstico , Neoplasias de Células Plasmáticas/diagnóstico , Linfoma Plasmablástico/diagnóstico , Algoritmos , Biomarcadores de Tumor , Toma de Decisiones Clínicas , Diagnóstico Diferencial , Manejo de la Enfermedad , Susceptibilidad a Enfermedades , Humanos , Mieloma Múltiple/etiología , Clasificación del Tumor , Estadificación de Neoplasias , Neoplasias de Células Plasmáticas/etiología , Linfoma Plasmablástico/etiología , Evaluación de SíntomasRESUMEN
BACKGROUND: If the depth of gallbladder malignant tumor (GBMT) invasion is deeper than the subserosa (ss), cholecystectomy is insufficient. In past reports that used endoscopic ultrasonography (EUS) to diagnose the depth of tumor invasion, it was difficult to diagnose GMBT invasion in the ss without a narrow or disrupted lateral hyperechoic layer (LHEL). Therefore, we developed a simple preoperative method to diagnose GBMTs with ss invasion. METHODS: Forty-nine GBMT patients who underwent both EUS and surgery were enrolled: 15 patients whose tumors invaded the mucosa (m) or muscularis propria (mp) were classified as the "shallow group", and 34 patients whose tumors invaded the ss were classified as the "deep group". The EUS findings were compared between the two groups. RESULTS: An irregular (narrow or thickened) LHEL was significantly more frequently observed on EUS in the deep group than in the shallow group. The diagnosis of ss invasion based on an irregular LHEL had the highest sensitivity and accuracy among the EUS imaging parameters (sensitivity 97.1% (33/34), specificity 86.7% (13/15), accuracy 93.8% (46/49)). When the deep group was limited to patients with a tumor depth of ss, the results were similar. When an irregular LHEL was used, the diagnostic accuracy of GBMTs with ss invasion was not significantly different between EUS specialists and beginners. CONCLUSIONS: The observation of an irregular (thickened or narrow) LHEL observed on EUS could be a reliable and simple method of diagnosing GBMTs with ss invasion and could contribute to choosing an appropriate surgical method.
Asunto(s)
Endosonografía , Neoplasias de la Vesícula Biliar/diagnóstico , Vesícula Biliar/diagnóstico por imagen , Anciano , Anciano de 80 o más Años , Colecistectomía , Femenino , Vesícula Biliar/patología , Vesícula Biliar/cirugía , Neoplasias de la Vesícula Biliar/patología , Neoplasias de la Vesícula Biliar/cirugía , Humanos , Masculino , Persona de Mediana Edad , Invasividad Neoplásica , Estadificación de Neoplasias , Reproducibilidad de los Resultados , Estudios RetrospectivosRESUMEN
Gastric adenocarcinoma with enteroblastic differentiation (GAED) is rare, highly malignant, and has higher vascular invasion and metastasis rates than conventional differentiated gastric cancer (CDGC). We report two cases of GAED that underwent curative resection by endoscopic submucosal dissection (ESD). Case 1 was an 82-year-old man with an elevated lesion in the gastric cardia. Biopsy revealed well-differentiated tubular adenocarcinoma. Pathological diagnosis of the ESD specimen revealed intramucosal gastric cancer without lymphovascular invasion (LVI). Although the surface layer of the lesion showed well and moderately differentiated tubular adenocarcinoma, clear cytoplasmic cancer cells positive for Sal-like protein-4 (SALL4) and Glypican-3 were found in a part of the deep layer. Therefore, GAED was diagnosed as present in a part of the whole lesion and covered with CDGC. Case 2 was an 83-year-old man with an elevated lesion in the gastric angulus. Biopsy revealed papillary and well-differentiated tubular adenocarcinomas. Pathological diagnosis of the ESD specimen revealed intramucosal gastric cancer without LVI. The entire lesion was occupied by papillary and tubular cancer cells, and had clear vesicles. Pure GAED was diagnosed, because the cells were SALL4 positive. In both cases, resection was curative despite the difference in pathological features.
Asunto(s)
Adenocarcinoma , Resección Endoscópica de la Mucosa , Neoplasias Gástricas , Adenocarcinoma/cirugía , Anciano de 80 o más Años , Biopsia , Diferenciación Celular , Mucosa Gástrica , Humanos , Masculino , Neoplasias Gástricas/cirugíaRESUMEN
RATIONALE: Osteosarcoma is the most common primary malignant bone tumor in children. The prognosis of osteosarcoma has improved with the use of aggressive systemic chemotherapy in addition to surgery. The relapse of osteosarcomas is usually as lung metastasis observed within 2 to 3 years after the initial treatment. A relapse is rarely observed at >10 years. PATIENT CONCERNS: We report the case of a 51-year-old Japanese man who was treated for high-grade osteosarcoma of the femur at 13 years old. He was referred to our hospital with a suspicion of primary lung cancer based on back pain, respiratory distress, and an abnormal mass on chest radiograph. DIAGNOSES: Computed tomography-guided biopsy confirmed the lung lesion as a metastatic recurrence of high-grade osteosarcoma without local recurrence. INTERVENTIONS: Chemotherapy was planned, but the patient's general condition rapidly deteriorated and thus palliative therapy was provided. OUTCOMES: The patient died 2 months after the initial consultation. LESSONS: The survival durations of osteosarcoma patients have been prolonged by recent progress in multimodality therapy, and thus clinicians as well as osteosarcoma patients should always keep in mind the possibility of very late relapse.
Asunto(s)
Neoplasias Femorales/diagnóstico , Neoplasias Pulmonares/diagnóstico , Osteosarcoma/diagnóstico , Resultado Fatal , Neoplasias Femorales/diagnóstico por imagen , Neoplasias Femorales/patología , Humanos , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/secundario , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia , Osteosarcoma/diagnóstico por imagen , Osteosarcoma/secundario , Cuidados Paliativos , Recurrencia , Sobrevivientes , Tomografía Computarizada por Rayos XRESUMEN
RATIONALE: Novel treatment strategies such as immunotherapy are being evaluated to further improve the outcomes of colorectal cancer patients. To our knowledge, this is the first report to show both the successful treatment of pulmonary squamous cell carcinoma (SCC) with pembrolizumab alongside histological and immunohistochemical findings of resected colon cancer under immunotherapy for lung cancer. PATIENT CONCERNS: This patient was a 70-year-old man who presented with a right lung tumor and simultaneous adenocarcinoma of the sigmoid colon. DIAGNOSES: Biopsy examination revealed squamous cell carcinoma in the right lung and adenocarcinoma of the sigmoid colon. INTERVENTIONS: The patient underwent successful pembrolizumab treatment as first-line immunotherapy for lung cancer, as demonstrated by computed tomography, and the sigmoid colon tumor was excised during an immunotherapy-free window. OUTCOMES: No unusual tumor growth in the right lung or abnormal abdominal signs was observed during the 9-month follow-up. LESSONS: Microscopically, the resected colon cancer specimen was characterized by numerous lymphoid cells in the partial stroma, with a large number of infiltrating lymphocytes consisting of CD3+, CD8+ T cells. In summary, this case demonstrates how immunotherapy affects PD-L1-negative colon cancer and indicates future treatment prospects.
Asunto(s)
Adenocarcinoma/cirugía , Anticuerpos Monoclonales Humanizados/uso terapéutico , Antineoplásicos Inmunológicos/uso terapéutico , Carcinoma de Células Escamosas/tratamiento farmacológico , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Primarias Múltiples/tratamiento farmacológico , Neoplasias Primarias Múltiples/cirugía , Neoplasias del Colon Sigmoide/cirugía , Adenocarcinoma/metabolismo , Adenocarcinoma/patología , Anciano , Anticuerpos Monoclonales Humanizados/efectos adversos , Antineoplásicos Inmunológicos/efectos adversos , Carcinoma de Células Escamosas/patología , Humanos , Inmunohistoquímica , Neoplasias Pulmonares/patología , Masculino , Neoplasias Primarias Múltiples/patología , Neoplasias del Colon Sigmoide/metabolismo , Neoplasias del Colon Sigmoide/patologíaRESUMEN
We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20+), T lymphocytes (CD3+, CD8+), and plasma cells (CD138+). Immunohistochemical analysis revealed increased expression of transforming growth factor-ß (TGF-ß) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect. However, after treatment with rituximab, the patient's pancytopenia showed improvement, allowing us to rapidly reduce the PSL dosage. The present case suggests the possibility that increased expression of TGF-ß in infiltrating lymphocytes and macrophages of bone marrow may contribute to the pathogenesis of AIMF. Prednisolone combined with rituximab may thus be an effective option for steroid-refractory cases.
Asunto(s)
Anemia Hemolítica Autoinmune , Resistencia a Medicamentos/efectos de los fármacos , Hepatitis Autoinmune , Prednisolona/administración & dosificación , Mielofibrosis Primaria , Rituximab/administración & dosificación , Trombocitopenia , Factor de Crecimiento Transformador beta/biosíntesis , Anemia Hemolítica Autoinmune/sangre , Anemia Hemolítica Autoinmune/complicaciones , Anemia Hemolítica Autoinmune/tratamiento farmacológico , Anemia Hemolítica Autoinmune/patología , Médula Ósea/metabolismo , Médula Ósea/patología , Femenino , Hepatitis Autoinmune/sangre , Hepatitis Autoinmune/complicaciones , Hepatitis Autoinmune/tratamiento farmacológico , Hepatitis Autoinmune/patología , Humanos , Persona de Mediana Edad , Mielofibrosis Primaria/sangre , Mielofibrosis Primaria/complicaciones , Mielofibrosis Primaria/tratamiento farmacológico , Mielofibrosis Primaria/patología , Trombocitopenia/sangre , Trombocitopenia/complicaciones , Trombocitopenia/tratamiento farmacológico , Trombocitopenia/patologíaRESUMEN
A survey of nitrate-ion concentrations in plant-factory-cultured leafy vegetables was conducted. 344 samples of twenty-one varieties of raw leafy vegetables were examined using HPLC. The nitrate-ion concentrations in plant-factory-cultured leafy vegetables were found to be LOD-6,800 mg/kg. Furthermore, the average concentration values varied among different leafy vegetables. The average values for plant-factory-cultured leafy vegetables were higher than those of open-cultured leafy vegetables reported in previous studies, such as the values listed in the Standard Tables of Food Composition in Japan- 2015 - (Seventh revised edition). For some plant-factory-cultured leafy vegetables, such as salad spinach, the average values were above the maximum permissible levels of nitrate concentration in EC No 1258/2011; however, even when these plant-factory-cultured vegetables were routinely eaten, the intake of nitrate ions in humans did not exceed the ADI.