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Background Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disease (PTLD) is predominantly of B cell origin. The concept of clonal evolution from poly- to monoclonal lymphoproliferation has been put forward, but T-cell PTLDs are rare with an unknown etiology. Case Presentation In a unique autopsy case of a 53-year-old man with EBV-associated T-cell PTLD, we observed polymorphic T-cell proliferation across several organs and monomorphic T-cell proliferation in the perforated ileum. Interestingly, both manifestations exhibited identical monoclonal peaks in the T-cell receptor rearrangement polymerase chain reaction (PCR) analyses. Conclusion These findings suggest the existence of clonal evolution in EBV-associated T-cell PTLD, leading to the proposal of the novel concept of polymorphic T-cell PTLD.
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[Purpose] This study aimed to identify kinematic gait indicators for a fall risk screening test through quantitative comparisons of gait characteristics measured using mobile inertial sensors between faller and non-faller groups in a population of community-dwelling older people. [Participants and Methods] We enrolled 50 people aged ≥65â years who used long-term care prevention services, interviewed them to determine their fall history during the past year, and divided them into faller and non-faller groups. Gait parameters (velocity, cadence, stride length, foot height, heel strike angle, ankle joint angle, knee joint angle, and hip joint angle) were assessed using the mobile inertial sensors. [Results] Gait velocity and left and right heel strike angles were significantly lower and smaller, respectively, in the faller versus non-faller group. Receiver operating characteristic curve analysis revealed areas under the curve of 0.686, 0.722, and 0.691 for gait velocity, left heel strike angle, and right heel strike angle, respectively. [Conclusion] Gait velocity and heel strike angle during gait assessed using mobile inertial sensors may be important kinematic indicators in a fall risk screening test to estimate the likelihood of falls among community-dwelling older people.
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A 57-year-old man with no family history of amyotrophic lateral sclerosis (ALS) or diabetes was diagnosed with ALS, and placed in long-term care where an artificial respirator with tracheotomy was used. He was fed through a gastric fistula tube. He gradually lost the ability to communicate, and computed tomography revealed advanced atrophy of the frontotemporal lobe. He was abruptly suffered from polyuria 11 years after the onset of ALS, and was diagnosed with hyperosmolar hyperglycemic state (HHS). It recurred six years later with severe ascites and worsening of pleural effusion. He passed away 18 years after the onset of ALS. Pathological examination revealed a reduced numbers of Betz cells in the motor cortex, anterior horn cells in the spinal cord, and hypoglossal neurons. The remaining lower motor neurons and cells in the hippocampal dentate gyrus were positive for phosphorylated TDP-43. These corresponded to Type B on harmonized classification system for frontotemporal lobar degeneration (FTLD)-TDP pathology (Mackenzie, et al. 2011). Furthermore, the lateral, anterior, and anterolateral funiculi of the spinal cord, globus pallidus, thalamus, and brainstem tegmentum showed atrophy. The findings were compatible with ALS pathology in totally locked-in state (TLS). Hyalinized islets of Langerhans were observed scattered throughout the pancreas. The patient's muscles were nearly completely replaced by white, firm fat tissue. We considered that the patient's diabetic conditions contributed to the accumulation of excess fat in internal and external fat tissue as a result of the long-term dependence on enteral nutrition. Moreover, a disturbance in glucose metabolism in skeletal muscles that resulted from severe atrophy could have been another cause.
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Esclerosis Amiotrófica Lateral , Demencia Frontotemporal , Enfermedad de la Neurona Motora , Atrofia , Autopsia , Humanos , Masculino , Persona de Mediana Edad , Neuronas MotorasRESUMEN
Male reproduction encompasses many essential cellular processes and interactions. As a focal point for these events, sperm offer opportunities for advancing our understanding of sexual reproduction at multiple levels during development. Using male sterility genes identified in human, mouse, and fruit fly databases as a starting point, 103 Drosophila melanogaster genes were screened for their association with male sterility by tissue-specific RNAi knockdown and CRISPR/Cas9-mediated mutagenesis. This list included 56 genes associated with male infertility in the human databases, but not found in the Drosophila database, resulting in the discovery of 63 new genes associated with male fertility in Drosophila. The phenotypes identified were categorized into six distinct classes affecting sperm development. Interestingly, the second largest class (Class VI) caused sterility despite apparently normal testis and sperm morphology suggesting that these proteins may have functions in the mature sperm following spermatogenesis. We focused on one such gene, Rack 1, and found that it plays an important role in two developmental periods, in early germline cells or germline stem cells and in spermatogenic cells or sperm. Taken together, many genes are yet to be identified and their role in male reproduction, especially after ejaculation, remains to be elucidated in Drosophila, where a wealth of data from human and other model organisms would be useful.
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Proteínas de Drosophila , Infertilidad Masculina , Animales , Drosophila/genética , Proteínas de Drosophila/genética , Drosophila melanogaster/genética , Infertilidad Masculina/genética , Masculino , Espermatogénesis/genética , TestículoRESUMEN
BACKGROUND: Toxoplasmosis is a rare but life-threatening infection occurring in immunocompromised hosts, including allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients. However, thus far, the clinical features and incidence of toxoplasmosis in autologous HSCT (auto-HSCT) recipients remain unknown. This retrospective survey aimed to analyze 152 patients who received auto-HSCT between 1998 and 2017. METHODS: Serological tests for Toxoplasma gondii-specific IgG were performed on 109 (71.7%) recipients, and 12 pre-HSCT recipients (11%) were Toxoplasma seropositive. Among the 12 recipients, three who did not receive trimethoprim-sulfamethoxazole (TMP/SMX) prophylaxis developed cerebral, pulmonary or disseminated toxoplasmosis due to reactivation after auto-HSCT and died despite treatment. RESULTS: The incidences of toxoplasmosis were 2% and 25% among 152 auto-HSCT recipients (five recipients received auto-HSCT two times) and 12 pre-HSCT Toxoplasma seropositive recipients, respectively. Further, we conducted a literature review and identified 21 cases of toxoplasmosis following auto-HSCT. In these previous cases, the mortality rate was high, especially for pulmonary and disseminated toxoplasmosis. Our findings suggest that, similar to toxoplasmosis after allo-HSCT, toxoplasmosis after auto-HSCT is a fatal complication. CONCLUSIONS: Serial screening of T. gondii-specific IgG before HSCT could contribute to the detection of Toxoplasma reactivation and allow for prompt diagnosis and treatment. The present study is the first to reveal the incidence of toxoplasmosis after auto-HSCT among seropositive patients in Japan.
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Trasplante de Células Madre Hematopoyéticas , Toxoplasma , Toxoplasmosis , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Incidencia , Estudios Retrospectivos , Toxoplasmosis/epidemiología , Trasplante Autólogo/efectos adversosRESUMEN
Objective: Subarachnoid hemorrhage due to ruptured vertebral artery dissecting aneurysm (rVADA) is associated with a high frequency of acute rebleeding and requires early treatment following onset. Parent artery occlusion (PAO) or stent-assisted coiling (SAC) embolization is selected as a treatment option according to the individual patient condition. This report is a retrospective examination evaluating the treatment outcomes for rVADA. Methods: The subjects were 20 rVADA patients (16 men and 4 women) who underwent endovascular treatment at our institution. The mean patient age was 52.9 years. Ten patients each were allocated to the PAO group and SAC group. We evaluated and compared the following parameters: presence of hemorrhagic complications, presence of ischemic complications, requirement of retreatment, and Glasgow Outcome Scale (GOS) after 90 days. Results: The reasons for selecting SAC were contralateral occlusion or a small diameter in three patients, the posterior inferior cerebellar artery (PICA) involvement in three patients, perforating artery from dissected lesion in five patients, and anterior spinal artery in one patient. There was no rebleeding in any patient. Symptomatic ischemic complications were observed in four patients in the PAO group and in one in the SAC group. Hyper-intense lesions in the brainstem on MRI DWI were noted in five patients in PAO group and in one in the SAC group. Retreatment was required for three patients in the PAO group and for four in the SAC group. Favorable outcomes (GOS 4, 5) after 90 days were observed for three patients in the PAO group and for eight patients in the SAC group (p = 0.0257). Conclusion: SAC that can preserve branches is a useful treatment option for rVADA. Further studies on a greater number of subjects are required to establish the optimal dose of antiplatelet agents and anticoagulants, and for stent selection.
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BACKGROUND: Anaplastic thyroid carcinoma is a highly aggressive form of thyroid cancer associated with a very poor prognosis. Anaplastic transformation most commonly occurs in the thyroid itself or within regional lymph nodes. Here we report the case of a patient with papillary thyroid cancer, presenting with colon perforation as a result of anaplastic transformation of metastases in the mesentery tissue. There have been no previous reports of this form of anaplastic transformation. CASE PRESENTATION: A 74-year-old man was admitted to our hospital, presenting with abdominal pain that he had been experiencing for 1 week prior to admission. The patient had a history of papillary thyroid carcinoma, for which he underwent a total thyroidectomy and mediastinal lymph node dissection 6 years earlier, and subsequently received radioactive iodine therapy for postoperative recurrence in the lung 2 years later. During the present reported admission, a computed tomography scan revealed a large intra-abdominal mass infiltrating into the colon and retroperitoneum and also highlighted the pneumoperitoneum. The patient was diagnosed with generalized peritonitis as a result of colon perforation, as such, we conducted an emergency laparotomy. Intraoperative findings showed a mass affecting the ascending colon and kidney, following which, an ileostomy and biopsy were completed. Poorly differentiated spindle cells were identified in the biopsy specimens, and histopathological and immunohistochemical findings revealed the absence of thyroid carcinoma cells. The tumor was therefore believed to be a primary sarcoma. Following surgery, the patient recovered from sepsis that had arisen as a result of colon perforation, however, rapidly developed systemic metastases and died 1 month post-operation. An autopsy was performed, and the patient was diagnosed with anaplastic papillary thyroid cancer at the mesentery site of metastasis. This conclusion was reached owing to the presence of the squamous differentiation of lymph node cells, and because tumor cells were positive results for paired-box gene 8 expressions. CONCLUSIONS: Anaplastic transformation of papillary thyroid carcinoma should be considered in the diagnosis of a large mesentery mass in patients with a history of papillary carcinoma. An appropriate biopsy and paired-box gene 8 immunostaining can be useful in confirming such a diagnosis.
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Endoscopic treatment for chronic subdural hematoma (CSDH) has been reported, but endoscopic findings of CSDH have not been thoroughly investigated. This study aimed to elucidate the relationship between endoscopic findings and CSDH recurrence. Furthermore, it examined the association between Nagahori's histopathological staging of CSDH and outer membrane color. Here, we retrospectively analyzed the operative videos of 70 patients with CSDH. The endoscopic findings were investigated, and their correlations with CSDH recurrence, the reduction ratio of the midline shift, and hematoma thickness on day 30 after the operation were analyzed. The outer membrane was white in 21 cases, yellow in 25 cases, and red in 24 cases. CSDH recurred in three (4.2%) patients, all of whom had a white outer membrane (adjusted odds ratio, 18; 95% confidence interval, 1.6-20.6; P = 0.007). The other endoscopic findings were not significantly related to CSDH recurrence, extent of the reduction ratio of the midline shift, or hematoma thickness. The outer membrane colors of white, red, yellow, and white almost corresponded to the histopathological staging from type I to IV in order. Our findings suggest that a white outer membrane is a risk factor for recurrence; these colors may represent the extent of inflammation related to the evolution of CSDH estimated from the histopathological findings.
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Endoscopía , Hematoma Subdural Crónico/diagnóstico por imagen , Hematoma Subdural Crónico/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Color , Femenino , Hematoma Subdural Crónico/etiología , Humanos , Masculino , Persona de Mediana Edad , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Tomografía Computarizada por Rayos XRESUMEN
A 71-year-old woman was diagnosed with stage â ¢B locally advanced lung squamous cell cancer(cT0N3M0).Programmed death-ligand 1(PD-L1)immunostaining was negative.First -line nedaplatin plus docetaxel and second-line carboplatin plus nab-paclitaxel were followed by sequential thoracic radiation therapy(60 Gy).The patient developed radiation pneumonitis, but her condition improved with corticosteroids.However, chest computed tomography(CT)revealed multiple nodules in both lungs.Third -line carboplatin plus tegafur/gimeracil/oteracil potassium(S-1)was not successful, and fourth- line nivolumab(3mg/kg every 2weeks)was adopted.On day 9 after first administration, she developed fever and radiation recall pneumonitis.Multiple nodules rapidly formed, but they later gradually decreased in number.After 13 courses of nivolumab, the nodules had disappeared completely.Mediastinal lymph nodes decreased in size, but an abdominal lymph node remained enlarged.Nivolumab was continued, and after 24 courses, the abdominal lymph node began to shrink, and the multiple lung metastases continued to disappear.Currently, the best overall response is good partial response to nivolumab.
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Neoplasias Pulmonares , Nivolumab/uso terapéutico , Anciano , Antígeno B7-H1 , Células Epiteliales , Femenino , Humanos , Neoplasias Pulmonares/tratamiento farmacológicoRESUMEN
Histologically, hepatocellular carcinoma (HCC) and atypical pituitary adenoma are similar, posing a potential clinical problem. A 76-year-old woman, whose past medical history was significant for hepatitis C virus (HCV), positivity without liver cirrhosis was presented with abducens nerve (CN VI) palsy. Contrast-enhanced magnetic resonance imaging (MRI) showed a clival tumor with infiltration of the right cavernous sinus. Subtotal resection was performed using a right anterior petrosal approach. Histological diagnosis was pituitary adenoma. Thirty-four days after the operation, MRI imaging showed residual tumor growth without any liver masses on computed tomography (CT). The patient underwent a second operation using an endoscopic transnasal transsphenoidal approach. Histological examination of the specimen gave a positive reaction for α -fetoprotein. Repeat whole-body CT showed an enhanced liver mass. Based on these findings, the lesion was diagnosed as metastatic HCC. The patient died of liver failure 6 months later. In this case, tumor diagnosis was difficult because of the histological similarity of HCC and pituitary adenoma, and the fact that the clival tumor grew prior to presentation of the liver mass on CT. This case emphasizes the importance of comparing the pathological diagnosis with the patient's clinical course.
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An 81-year-old woman with type 2 diabetes mellitus presented to our hospital due to anorexia, leg edema, and respiratory distress. Laboratory results revealed anemia, thrombocytopenia, elevated lactate dehydrogenase, and markedly elevated soluble interleukin-2 receptor levels. Computed tomography showed ground-glass opacities and consolidation in both lung fields, but no lymphadenopathy was noted. Intravascular large B-cell lymphoma (IVLBCL) was considered as a differential diagnosis; therefore, bone marrow and random skin biopsy were performed. Her respiratory condition deteriorated, with the occurrence of acute respiratory distress syndrome, disseminated intravascular coagulation, hemophagocytic syndrome, and further alveolar hemorrhage. Methylprednisolone pulse therapy was performed, but did not improve the patient's condition. On hospital day 6, the acid-fast bacterial smear of the sputum using the Gaffky scale was 2, and on the next day, tuberculosis DNA was detected in the polymerase chain reaction. In the bone marrow biopsy, multiple epithelioid cell granulomas were found; thus, the patient was diagnosed with miliary tuberculosis. Although anti-tuberculosis therapy was started immediately, she died on hospital day 22. The soluble interleukin-2 receptor level increased up to 19,400 U/ml. The differential diagnosis should be cautiously made because miliary tuberculosis can mimic IVLBCL.
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Linfoma de Células B Grandes Difuso , Receptores de Interleucina-2/sangre , Tuberculosis Miliar/diagnóstico , Anciano de 80 o más Años , Diabetes Mellitus Tipo 2/complicaciones , Diagnóstico Diferencial , Resultado Fatal , Femenino , Humanos , Síndrome de Dificultad Respiratoria/complicacionesRESUMEN
We report a case of direct carotid puncture for endovascular thrombectomy in a 96-year-old patient with acute cerebral infarction. Anatomical difficulties of carotid artery access, including an unfavorable aortic arch type and the tortuosity of carotids, could have resulted in a recanalization delay or failure in patients with acute ischemic stroke. A 96-year-old woman developed sudden right hemiparesis. By initiating thrombectomy, we could not access the common carotid artery;therefore, we performed a direct carotid puncture on the neck and achieved successful recanalization with improvement of hemiparesis. We discuss the limitations and technical aspects of this technique. We conclude that in case of stroke, a direct carotid artery puncture is a feasible alternative to an anatomically difficult transfemoral access.
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Infarto Cerebral , Procedimientos Endovasculares , Accidente Cerebrovascular , Trombectomía , Anciano de 80 o más Años , Infarto Cerebral/complicaciones , Femenino , Humanos , PuncionesRESUMEN
A 59-year-old man who complained of abdominal pain was referred to our hospital. Computed tomography (CT) revealed mesenteric lymph node swelling and intestinal perforation. Histopathological study of the resected ileum and lymph node demonstrated diffuse proliferation of medium-sized atypical lymphocytes. Immunohistochemistry results were positive for cluster of differentiation (CD) 3, CD8, and CD56 cells, negative for CD5 and CD4 cells, and negative for Epstein-Barr virus-encoded RNA-fluorescent in situ hybridization (EBER-FISH). It also revealed the expression of γδ T-cell receptors. On the basis of these findings, enteropathy-associated T-cell lymphoma (EATL) was diagnosed. Although the patient received two courses of cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone (CHOP) and dexamethasone, etoposide, ifosfamide, and carboplatin (DeVIC) therapy, facial nerve and lower limb paralysis manifested. Magnetic resonance imaging (MRI) and lumbar puncture revealed central nervous system invasion of the EATL. Despite intrathecal chemotherapy and high-dose cytarabine therapy, the patient's neurological symptoms deteriorated. Fluorodeoxyglucose positron emission tomography (FDG-PET) /CT scan showed the accumulation of FDG along both median and sciatic nerves, and he was diagnosed with neurolymphomatosis (NL). He died on day 120 after admission. Autopsy specimens exhibited infiltration of lymphoma cells in the median and sciatic nerves. Although only one case of suspected NL in a patient with type 2 EATL has been previously reported, we clinically diagnosed NL using FDG-PET/CT and confirmed the diagnosis by autopsy. This case is valuable in terms of the pathological diagnosis of NL.
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Linfoma de Células T Asociado a Enteropatía/complicaciones , Linfoma de Células T Asociado a Enteropatía/diagnóstico por imagen , Neurolinfomatosis/diagnóstico por imagen , Neurolinfomatosis/etiología , Autopsia , Fluorodesoxiglucosa F18 , Humanos , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de PositronesRESUMEN
In Drosophila, mature sperm are transferred from males to females during copulation, stored in the sperm storage organs of females, and then utilized for fertilization. Here, we report a gene named sheepish (shps) of Drosophila melanogaster that is essential for sperm storage in females. shps mutant males, although producing morphologically normal and motile sperm that are effectively transferred to females, produce very few offspring. Direct counts of sperm indicated that the primary defect was correlated to failure of shps sperm to migrate into the female sperm storage organs. Increased sperm motion parameters were seen in the control after transfer to females, whereas sperm from shps males have characteristics of the motion parameters different from the control. The few sperm that occasionally entered the female sperm storage organs showed no obvious defects in fertilization and early embryo development. The female postmating responses after copulation with shps males appeared normal, at least with respect to conformational changes of uterus, mating plug formation, and female remating rates. The shps gene encodes a protein with homology to amine oxidases, including as observed in mammals, with a transmembrane region at the C-terminal end. The shps mutation was characterized by a nonsense replacement in the third exon of CG13611, and shps was rescued by transformants of the wild-type copy of CG13611 Thus, shps may define a new class of gene responsible for sperm storage.
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Proteínas de Drosophila/genética , Drosophila melanogaster/genética , Fertilización/genética , Oxidorreductasas actuantes sobre Donantes de Grupo CH-NH/genética , Espermatozoides/metabolismo , Estructuras Animales/citología , Estructuras Animales/metabolismo , Animales , Tamaño de la Nidada , Copulación , Proteínas de Drosophila/deficiencia , Drosophila melanogaster/crecimiento & desarrollo , Femenino , Fertilidad , Expresión Génica , Prueba de Complementación Genética , Masculino , Oxidorreductasas actuantes sobre Donantes de Grupo CH-NH/deficiencia , Recuento de Espermatozoides , Motilidad Espermática , Espermatozoides/patologíaRESUMEN
Multicentric Castleman's disease (MCD) is a rare, non-malignant lymphoproliferative disorder. We report a case of MCD with multiple liver masses. A 26-year-old woman presented with asymptomatic anemia and hypoalbuminemia. Laboratory tests detected high CRP levels and findings indicative of polyclonal gammopathy. Abdominal CT revealed multiple hepatic large masses (≤10 cm) and partial calcification in the right lobe. Multiple enlarged lymph nodes were also identified in the cardiophrenic angle and porta hepatis. We suspected hepatic malignancy, but pathological examinations of the liver and lymph nodes demonstrated polyclonal plasma cell infiltration and fibrosis. IL-6 staining was positive for plasma cell infiltration of lymph nodes. A few plasma cells were positive for IgG4, and tests for HIV and HHV-8 were negative. Serum IL-6 and plasma VEGF levels were both elevated (45 and 536 pg/ml, respectively). The patient was diagnosed with plasma cell type MCD. We started treatment with PSL 1 mg/kg/day, which led to improvement of anemia, hypoalbuminemia, and high CRP levels. Marginal regression of liver masses was also observed. At the last follow-up, the patient had been progression-free for 18 months. To our knowledge, this is the first report of a plasma cell type MCD with liver masses.
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Enfermedad de Castleman/diagnóstico , Diagnóstico Diferencial , Neoplasias Hepáticas/diagnóstico , Hígado/patología , Adulto , Antiinflamatorios/uso terapéutico , Biopsia , Enfermedad de Castleman/tratamiento farmacológico , Enfermedad de Castleman/patología , Femenino , Humanos , Imagen Multimodal , Prednisolona/uso terapéuticoRESUMEN
The prognosis of pulmonary toxoplasmosis, including disseminated toxoplasmosis involving the lungs, following hematopoietic stem cell transplantation (HSCT) is extremely poor due to the difficulties associated with early diagnosis and the rapidly progressive deterioration of multiorgan function. In our institution, we identified nine cases of toxoplasmosis, representing incidences of 2.2 and 19.6 % among all HSCT recipients and seropositive HSCT recipients, respectively. Of the patients with toxoplasmosis, six had pulmonary toxoplasmosis. Chest computed tomography (CT) findings revealed centrilobular, patchy ground-glass opacities (n = 3), diffuse ground-glass opacities (n = 2), ground-glass opacities with septal thickening (n = 1), and marked pleural effusion (n = 1). All cases died, except for one with suspected pulmonary toxoplasmosis who was diagnosed by a polymerase chain reaction assay 2 days after the onset of symptoms. In pulmonary toxoplasmosis, CT findings are non-specific and may mimic pulmonary congestion, atypical pneumonia, viral pneumonitis, and bronchopneumonia. Early diagnosis and treatment is crucial for overcoming this serious infectious complication. Pulmonary toxoplasmosis should be considered during differential diagnosis in a recipient with otherwise unexplained signs of infection and CT findings with ground-glass opacities, regardless of the distribution.
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Trasplante de Células Madre Hematopoyéticas/efectos adversos , Enfermedades Pulmonares Parasitarias/diagnóstico por imagen , Pulmón/diagnóstico por imagen , Pulmón/parasitología , Toxoplasma/aislamiento & purificación , Toxoplasmosis/diagnóstico por imagen , Adulto , Anciano , Antiparasitarios/uso terapéutico , Femenino , Humanos , Enfermedades Pulmonares Parasitarias/sangre , Enfermedades Pulmonares Parasitarias/tratamiento farmacológico , Enfermedades Pulmonares Parasitarias/etiología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Toxoplasma/efectos de los fármacos , Toxoplasmosis/sangre , Toxoplasmosis/tratamiento farmacológico , Toxoplasmosis/etiología , Adulto JovenRESUMEN
OBJECTIVE: To investigate the prevalence and clinical features of posttransplant CNS symptoms in patients with hereditary ATTR amyloidosis and their Pittsburgh compound B (PiB)-PET imaging correlates. METHODS: We monitored prevalence and type of CNS symptoms in 53 consecutive posttransplant patients with hereditary ATTR amyloidosis. (11)C-PiB-PET was performed in 15 patients with various disease durations. We also analyzed pathologic and biochemical characteristics of ATTR amyloid deposition in the brain of a posttransplant patient. RESULTS: Transient focal neurologic episodes (TFNEs) attributed to ATTR-type cerebral amyloid angiopathy (CAA) were found in 11.3% of posttransplant hereditary ATTR amyloidosis patients. TFNE occurred on average 16.8 years after onset of the disease. Patients with longer duration of illness (≥10 years) showed increased (11)C-PiB retention in the brain. The (11)C-PiB accumulation pattern in hereditary ATTR amyloidosis was unique and different from those in Alzheimer disease or Aß-type CAA. In the autopsy case, ATTR amyloid deposition was mainly localized to leptomeningeal vessels and leptomeninges of the brain. Amyloid fibrils in the brain were almost completely composed of variant transthyretin (TTR). CONCLUSIONS: TFNE due to ATTR-type CAA occurred frequently in posttransplant patients with long disease durations. (11)C-PiB-PET is a useful diagnostic tool for ATTR-type CAA. ATTR amyloid deposition in the CNS, as measured by PiB-PET, was detected approximately 10 years before onset of TFNE.
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Compuestos de Anilina , Enfermedades del Sistema Nervioso Central/etiología , Angiopatía Amiloide Cerebral Familiar/complicaciones , Trasplante de Hígado/métodos , Tomografía de Emisión de Positrones/métodos , Prealbúmina/metabolismo , Tiazoles , Enfermedades del Sistema Nervioso Central/metabolismo , Enfermedades del Sistema Nervioso Central/fisiopatología , Angiopatía Amiloide Cerebral Familiar/metabolismo , Angiopatía Amiloide Cerebral Familiar/cirugía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Prealbúmina/genética , Estudios Retrospectivos , Factores de TiempoRESUMEN
A 79-year-old woman was admitted with a 5-kg weight loss and anorexia. Computed tomography showed diffuse lymphadenopathy, and thickening of the duodenal and ileal walls. The patient then underwent biopsy of these sites. Pathological examination revealed duodenal Epstein-Barr virus (EBV)-positive peripheral T cell lymphoma-not otherwise specified (PTCL-NOS) and EBV-negative ileal diffuse large B-cell lymphoma (DLBCL) to be present simultaneously. Combination chemotherapy including rituximab produced a reduction of the duodenal EBV-positive PTCL-NOS lesion, but had no effect on the EBV-negative ileal DLBCL lesion. Thereafter, new lymphadenopathy, high fever, and lactate dehydrogenase (LD) elevation developed, complicated by pneumonia. The patient died due to rapid deterioration of the lymphoma and pneumonia on day 108 after initiation of treatment. EBV-positive PTCL-NOS is reportedly rare and the prognosis is poor. Moreover, EBV-negative ileal DLBCL was diagnosed simultaneously. This case is considered to have had an extremely rare discordant lymphoma, although the exact etiology of its development remains unknown. We speculate that age-related disorders of the immune system and HCV infection may have been associated with the pathogenic mechanism of lymphomagenesis in this case.
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Infecciones por Virus de Epstein-Barr/complicaciones , Herpesvirus Humano 4 , Neoplasias del Íleon , Linfoma de Células B Grandes Difuso , Linfoma de Células T Periférico , Neoplasias Primarias Múltiples , Anciano , Resultado Fatal , Femenino , Humanos , Neoplasias del Íleon/patología , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/virología , Linfoma de Células T Periférico/tratamiento farmacológico , Linfoma de Células T Periférico/virología , Neoplasias Primarias Múltiples/virologíaRESUMEN
Epstein-Barr virus (EBV)-related post-transplant lymphoproliferative disorder (PTLD) frequently involves the gastrointestinal tract, but the endoscopic characteristics of this condition have not been discussed in detail. We report two cases of EBV-related PTLD involving rapidly forming characteristic lesions. Case 1 was a 60-year-old man with acute myeloid leukemia who underwent cord blood transplantation (CBT) after which he initially achieved complete remission (CR). He developed nausea and vomiting on day 99. Gastrointestinal endoscopy showed no tumor-like lesions in his stomach. However, a second endoscopic evaluation, which was performed 7 days after the first, revealed multiple raised lesions in his stomach, and a histopathological examination of the biopsy specimen resulted in a diagnosis of EBV-related PTLD. Case 2 was a 36-year-old man with acute myeloid leukemia who underwent CBT after achieving his second CR. He suffered nausea and pharyngalgia on day 309. Although the initial gastrointestinal endoscopic examination showed only multiple erosive or small ulcerative lesions, a second endoscopic evaluation, which was performed 10 days after the first, revealed a raised lesion with a central ulcer in the duodenum. Histopathological examination of the biopsy specimen yielded a diagnosis of EBV-related PTLD. Both patients were successfully treated by reducing the dose of immunosuppressive agents and administering rituximab.
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Trasplante de Células Madre de Sangre del Cordón Umbilical/efectos adversos , Sangre Fetal , Enfermedades Gastrointestinales/diagnóstico , Trastornos Linfoproliferativos/diagnóstico , Adulto , Infecciones por Virus de Epstein-Barr/diagnóstico , Enfermedades Gastrointestinales/etiología , Enfermedades Gastrointestinales/virología , Herpesvirus Humano 4 , Humanos , Leucemia Mieloide Aguda/terapia , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/virología , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: Instances of ectopic salivary gland tissue within the pituitary gland are rare, they are mostly asymptomatic, and the underlying pathophysiology of symptomatic cases is unclear. We report a case of intrasellar salivary gland rest that presented clinical symptoms and clearly related to inflammatory changes. CASE DESCRIPTION: In the present case, headache, bitemporal hemianopia, and hormone abnormality led to the detection of ectopic salivary gland tissue within the pituitary gland of a 24-year-old man. Imaging revealed a well-circumscribed intrasellar cystic lesion having a diameter of major axis of 16 mm, for which tumorectomy was performed using the nasal approach. The tumor was cystic with stringy content. Pathologic findings revealed that the lesion was composed principally of secretions lacking cell components, whereas the salivary gland tissue was found in the cyst wall. Dilated ducts due to the leakage of secretions were also observed. Acute and chronic inflammation was present around the salivary gland. CONCLUSIONS: Not only are instances of symptomatic ectopic salivary glands rare, but this was also the first case detected to be caused by the pathophysiology involving the leakage of secretions from an ectopic salivary gland and associated inflammation. We report this case to help elucidate the pathophysiology of the condition.