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Resumen Se presenta el caso clínico de una paciente del sexo femenino, de 30 años, con adenopatías supraclaviculares y axilares ipsilaterales, quien fue sometida a biopsia excisional con posterior estudio. El análisis histopatológico de la muestra de tejido resecado reveló una serie de características distintivas asociadas con la enfermedad de Castleman variante hialinovascular. La presentación de este caso no solo proporciona información detallada sobre la evolución clínica de la paciente, sino que también sirve como base para ilustrar los aspectos clave del diagnóstico histopatológico y las implicaciones inmunohistoquímicas en la enfermedad de Castleman. Además de hacer una revisión de tema respecto a esta patología poco común, en la cual los informes de casos son fundamentales para aumentar la comprensión de su variabilidad clínica y su abordaje diagnóstico, ilustrando los desafíos en el diagnóstico diferencial y como deben abordarse los mismos.
Abstract The clinical case of a 30-year-old female patient with supraclavicular and ipsilateral axillary lymphadenopathy who underwent excisional biopsy with subsequent study is presented. Histopathological analysis of the resected tissue sample revealed a series of distinctive features associated with hyalinevascular variant Castleman disease. The presentation of this case not only provides detailed information about the clinical evolution of the patient, but also serves as a basis to illustrate Key aspects of histopathological diagnosis and immunohistochemical implications in Castleman disease. In addition to making a review of the topic regarding this rare pathology in which case reports are essential to increase the understanding of its clinical variability and its diagnostic approach, illustrating the challenges in differential diagnosis and how they should be addressed.
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A Dengue induz uma resposta exacerbada e transitória das células secretoras de anticorpos (ASCs) no sangue de pacientes cerca de sete dias após o início dos sintomas. A frequência dessas ASCs chega a representar mais de 50% de todas as células B circulantes neste período. No entanto, ainda é desconhecido se a magnitude dessa resposta tem relação com a gravidade da Dengue. Nosso grupo de pesquisa já mostrou que a cultura de células nucleares do sangue periférico (PBMCs) de indivíduos saudáveis com partículas do vírus da Dengue (DENV), por 7 dias, levava a diferenciação de células B em ASCs em magnitude similar àquelas estimuladas por mitógenos. Essas culturas apresentavam um consumo significativamente maior de triptofano (TRP), associado à maior expressão das enzimas IDO1 e IDO2, e, consequentemente, maior síntese de quinurenina (KYN) em relação ao estímulo por mitógenos. Considerando que as concentrações de TRP e KYN detectadas nos sobrenadantes dessas culturas eram diretamente proporcionais ao aumento de ASCs, decidimos investigar o papel desse metabolismo do TRP e de seus respectivos metabólitos na diferenciação das ASCs. Para isso, análises do transcriptoma público de células únicas do sangue periférico de pacientes com Dengue (estudo E-MTAB- 9467) foram realizadas para inferir a real participação do metabolismo do TRP na geração de ASCs. Com o programa R foram executadas análises de Downstream. Identificamos um aumento massivo das ASCs nas amostras dos pacientes infectados com Dengue. No entanto, os principais genes desencadeadores da ativação do metabolismo do TRP (IDO1 e IDO2) não foram expressos nas subpopulações de células B, mas sim em células dendríticas e monócitos CD14+ respectivamente. Isso sugeriria que esta via não seria ativada nos linfócitos B. Por outro lado, genes codificadores de outros participantes da via do TRP (HSD17B10, ECHS1 e SIRT3) foram detectados em células B e podem estar relacionados com a proliferação das ASCs. Além disso, a análise de enriquecimento mostrou uma aumentada expressão de genes associados com moléculas de MHC de classe II em plasmablastos e plasmócitos de pacientes com Dengue. Porém, com a expressão aumentada de ENTPD1 nessas células durante a fase sintomatológica, nossos dados sugerem também que um eventual papel de plasmablastos e plasmócitos como apresentadoras de antígenos na Dengue poderia induzir uma resposta supressora de células T
Dengue can cause an exacerbated and transient antibody-secreting cell (ASC) response in the blood of patients nearly 7 days of symptomatology. The ASC frequency reaches more than 50% of all circulating B cells during that period. However, it is still unknown whether the magnitude of this response may be directly related to the severity of Dengue. Our research group has already shown that the culture of peripheral blood mononuclear cells (PBMCs) from healthy individuals with Dengue virus (DENV) particles for 7 days led to a differentiation of B cells into ASCs to a magnitude similar to those stimulated by mitogens. These cultures showed significantly higher consumption of tryptophan (TRP), associated with higher expression of enzymes IDO1 and IDO2, and consequently, higher synthesis of quinurenine (KYN) compared to mitogen stimulation. The concentrations of TRP and KYN detected in the supernatants of these cultures were directly proportional to ASC frequency increase. Thus, we have decided to investigate the role of TRP metabolism and its respective metabolites in ASC differentiation. For this, we performed an analysis of single-cell transcriptome with peripheral blood from Dengue patients (dataset from E-MTAB-9467 study). Downstream analyses were performed with R software. Corroborating with literature, we identified a massive increase in ASC frequency of Dengue infected patients. However, the main genes triggering TRP metabolism activation (IDO1 and IDO2) were not expressed in B-cell subsets, but in dendritic cells and CD14+ monocytes, respectively. This would suggest that this pathway would not be activated in B lymphocytes. Nevertheless, genes encoding other participants in the TRP pathway (HSD17B10, ECHS1, and SIRT3) were detected in B cells and may be related to ASC proliferation. Futhermore, a Gene Ontology analysis showed an increased expression of genes associated with MHC class II molecules in plasmablasts and plasma cells of Dengue patients. As these cells also presented an increased expression of ENTPD1 during the symptomatic phase, our data suggest a potential role of plasmablasts and plasma cells as antigen-presenting cells associated with a suppressive T cell response in Dengue
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Patients/classification , Plasma Cells/classification , Histocompatibility Antigens Class II/pharmacology , Dengue/pathology , Single-Cell Gene Expression Analysis/instrumentation , Tryptophan/analogs & derivativesРеферат
SUMMARY: In solid and malignant tumors, innate and adaptive immunity are combined in antitumor responses. This study aimed to analyze the activation of plasma cells and the correlation between the infiltration of B and T lymphocytes with the degree of malignancy or Gleason grade in human prostate biopsies diagnosed with cancer. Prostate cancer biopsies were obtained from the Clinical Hospital of Universidad de Chile (n=70), according to the bioethical norms of the institution. Histological sections of 5µm thickness were processed for immunohistochemistry with primary antibodies against BL and total TL (HRP/DAB). Recognition and quantification were performed under a Leica DM750 optical microscope. Microsoft Excel and GraphPad software were used for the statistical study. Correlation coefficient (Pearson) and mean comparison tests (Kruskal-Wallis and Dunn) and p≤ 0.05 were developed. B and T lymphocyte populations were inversely interregulated in prostate cancer (Gleason) (r= -0.46). Their relationship with Gleason grade is variable according to lymphocyte type (LB vs. Gleason r= -0.0.47 and LT vs. Gleason r= -0.21). Histological diagnosis of prostate cancer correlates with a predominance of LT. The malignancy of the pathology correlates with a predominance of LTs, according to the Gleason grade. The increased knowledge of B and T lymphocyte infiltration and plasma cell activation could be used to better target clinical trials on treatments based on immune system responses. Immunotherapy could be a new paradigm to apply better antitumor therapy strategies.
En tumores sólidos y malignos, la inmunidad innata y adaptativa se combinan en respuestas antitumorales. Este estudio tuvo como objetivo analizar la activación de células plasmáticas y la correlación entre la infiltración de linfocitos B y T con el grado de malignidad o grado de Gleason en biopsias de próstata humana diagnosticadas con cáncer. Las biopsias de cáncer de próstata se obtuvieron del Hospital Clínico de la Universidad de Chile (n=70), de acuerdo con las normas bioéticas de la institución. Secciones histológicas de 5 µm de espesor fueron procesadas para inmunohistoquímica con anticuerpos primarios contra LB y LT total (HRP/DAB). El reconocimiento y las cuantificaciones se realizaron bajo un microscopio óptico Leica DM750. Para el estudio estadístico se utilizaron los programas Microsoft Excel y GraphPad. Se desarrollaron pruebas de coeficiente de correlación (Pearson) y comparación de medias (Kruskal-Wallis y Dunn) y p≤ 0.05. Los resultados muestran que las poblaciones de linfocitos B y T están inversamente interreguladas en el cáncer de próstata (r= -0,4578). Su relación con el grado de Gleason es variable según el tipo de linfocito (LB vs Gleason r= -0,47* y LT vs Gleason r= -0,21). Se concluye que la malignidad del cáncer de próstata se correlaciona con un predominio de LT, versus el grado de Gleason. El mayor conocimiento de la infiltración de linfocitos B y T y la activación de células plasmáticas podría aprovecharse para una mejor orientación de ensayos clínicos en tratamientos basados en las respuestas del sistema inmunitario. La inmunoterapia podría ser un nuevo paradigma para aplicar mejores estrategias de terapias antitumorales.
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Humans , Male , Adult , Middle Aged , Aged , Plasma Cells , Prostatic Neoplasms/immunology , Prostatic Neoplasms/pathology , T-Lymphocytes , Biopsy , Immunohistochemistry , B-Lymphocytes , Immunomodulation , Neoplasm Grading , MicroscopyРеферат
Background: Amyloid light chain (AL) amyloidosis is characterized by amyloid fibril deposition derived from monoclonal immunoglobulin light chains, resulting in multiorgan dysfunction. Limited data exist on the clinical features of AL amyloidosis. Objective: This study aims to describe the clinical characteristics, treatments, and outcomes in Colombian patients with AL amyloidosis. Methods: A retrospective descriptive study was conducted at three high-complexity centers in Medellín, Colombia. Adults with AL amyloidosis diagnosed between 2012 and 2022 were included. Clinical, laboratory, histological, treatment, and survival data were analyzed. Results: The study included 63 patients. Renal involvement was most prevalent (66%), followed by cardiac involvement (61%). Multiorgan involvement occurred in 61% of patients. Amyloid deposition was most commonly detected in renal biopsy (40%). Bortezomib-based therapy was used in 68%, and 23.8% received high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HDCT-ASCT). Hematological response was observed in 95% of patients with available data. Cardiac and renal organ responses were 15% and 14%, respectively. Median overall survival was 45.1 months (95% CI: 22.2-63.8). In multivariate analysis, cardiac involvement was significantly associated with inferior overall survival (HR 3.27; 95% CI: 1.23-8.73; p=0.018), HDCT-ASCT had a non-significant trend towards improved overall survival (HR 0.25; 95% CI: 0.06-1.09; p=0.065). Conclusions: In this study of Colombian patients with AL amyloidosis, renal involvement was more frequent than cardiac involvement. Overall survival and multiorgan involvement were consistent with data from other regions of the world. Multivariate analysis identified cardiac involvement and HDCT-AHCT as possible prognostic factors.
Antecedentes: La amiloidosis por amiloide de cadenas ligeras (AL) se caracteriza por el depósito de fibrillas amiloides derivadas de cadenas ligeras de inmunoglobulinas monoclonales, lo que resulta en disfunción multiorgánica. Existen datos limitados sobre las características clínicas de la amiloidosis AL. Objetivo: Este estudio tiene como objetivo describir las características clínicas, tratamientos y desenlaces en pacientes colombianos con amiloidosis AL. Métodos: Se llevó a cabo un estudio descriptivo retrospectivo en tres centros de alta complejidad en Medellín, Colombia. Se incluyeron adultos con diagnóstico de amiloidosis AL entre 2012 y 2022. Se analizaron datos clínicos, de laboratorio, histológicos, de tratamiento y de supervivencia. Resultados: El estudio incluyó 63 pacientes. La afectación renal fue más prevalente (66%), seguida de la afectación cardíaca (61%). El 61% de los pacientes presentaron afectación multiorgánica. El depósito amiloide se detectó con mayor frecuencia en la biopsia renal (40%). El tratamiento basado en bortezomib se utilizó en el 68%, y el 23.8% recibió altas dosis de quimioterapia con trasplante autólogo de progenitores hematopoyéticos (ADQT-TAPH). Se observó respuesta hematológica en el 95% de los pacientes con datos disponibles. La respuesta de órgano cardíaca y renal fue del 15% y 14%, respectivamente. La mediana de la supervivencia global fue de 45.1 meses (IC del 95%: 22.2-63.8). En el análisis multivariado, la afectación cardíaca se asoció significativamente con una supervivencia global inferior (HR 3.27; IC del 95%: 1.23-8.73; p=0.018), ADQT-TAPH mostró una tendencia no significativa hacia una mejora en la supervivencia global (HR 0.25; IC 95%: 0.06-1.09; p=0.065). Conclusiones: En este estudio de pacientes colombianos con amiloidosis AL, la afectación renal fue más frecuente que la afectación cardíaca. La supervivencia global y la afectación multiorgánica fueron consistentes con datos de otras regiones del mundo. El análisis multivariado identificó la afectación cardíaca y ADQT-TAPH como posibles factores pronósticos.
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Presentamos el caso de un paciente de 49 años, de sexo masculino, que consulta en el servicio de urgencias por un cuadro de dos meses de evolución, caracterizado por compromiso del estado general, baja de peso, dolor abdominal, sensación febril y elevación de los parámetros inflamatorios. Al estudio imagenológico se observa una voluminosa lesión hepática, asociada a dilatación de la vía biliar y adenopatías en hilio hepático, espacio porto-cavo y retroperitoneales (inter-cavo-aórticos), que plantea dentro de los diagnósticos diferencias un colangiocarcinoma intrahepático. Basados en esta sospecha se realiza una segmentectomía y linfadenectomía regional. El estudio histopatológico e inmunohistoquímico de la pieza quirúrgica, evidencia un proceso inflamatorio linfoplasmocitario, con la presencia de células plasmáticas IgG4 positivas, compatible con una enfermedad asociada a IgG4. Posterior a la resección se decide manejo expectante, evolucionando el paciente de forma favorable, asintomático y sin signos de recurrencia. Presentamos un caso y una breve revisión de la literatura de un pseudotumor inflamatorio hepático, entidad poco frecuente y de comportamiento benigno.
We report the case of a 49-year-old man who attended the emergency department for a two-month history of compromised general condition, weight loss, abdominal pain, fever, and elevated inflammatory parameters. An imaging study demonstrates a bulky liver tumor associated with dilation of the bile duct and retroperitoneal adenopathies (hepatic hilum, intermediate, and right lumbar groups). These findings raise intrahepatic cholangiocarcinoma within the differential diagnoses, reason why segmental hepatectomy and regional lymphadenectomy were performed. Histopathology and immunochemistry revealed a lymphoplasmacytic inflammatory process with IgG4-positive plasma cells compatible with IgG4-associated disease. After the resection, expectant management was decided, with the patient evolving favorably, asymptomatic, and without signs of recurrence. We present a case and a brief literature review of an hepatic inflammatory pseudotumor, a rare entity with a benign behavior.
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Background: Identification of plasma cells into abnormal (APC) and normal (NPC) compartments is of utmost importance in flow cytometric (FC) analysis of multiple myeloma (MM) and related plasma cell dyscrasias for diagnosis, prognosis, and follow-up. No single phenotypic marker is sufficient to distinguish NPC from APC. Materials and Methods: 43 newly diagnosed cases of MM and 13 controls were included in the study. Bone marrow (BM) samples from the 2nd pass were processed on the same day with antibodies against CD38, CD138, CD19, CD81, CD45, CD117, CD200, CD56, cytoKappa, and cytoLambda in a 4-color experiment with CD38 and CD138 as gating antibodies. Results: Mean APC% in cases was 96.5%. The expected Immunophenotype (IP) of APC which is CD19-/56+/45-/81-/117+/200+ was found in only 13/43 MM cases. In 30/43 cases, APC revealed deviation from expected IP either for single or a combination of markers. Sensitivity for APC detection was highest for CD19 (95.2%) followed by CD56 (90.4%) and CD81 (83.7%). Specificity was highest for CD19 (100%), CD56 (100%), and CD81 (100%) followed by CD117 (92.3%). Combination of markers with maximum sensitivity to detect APC (97.6%) was CD81- or CD19- and CD200+ or CD56+ (two markers); and for NPC (92.3%) was CD81+ and CD19+ and CD56- (three markers). Conclusion: Plasma cell IP can be highly variable with multiple minor subpopulations in both cases and normal controls. CD 19 and CD56 are highly informative markers for a 4-color experiment. Assessment of multiple markers in an 8–10 color experiment is more informative but the lack of advanced flow cytometers should not limit the use of FC in a 4-color approach. Our results emphasize that even basic equipment with limited fluorochrome can provide meaningful information if used appropriately.
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Objective:To investigate the value of bone marrow plasma cell morphology in the diagnosis and prognosis of plasma cell myeloma (PCM).Method:Observational study.Collect the bone marrow morphology image reports and corresponding monoclonal protein (M protein) identification results of 1071 patients [629 males and 442 females, Median age 62 (29, 93) years] diagnosed with PCM in the outpatient and inpatient departments of Beijing Chaoyang Hospital affiliated to Capital Medical University from January 1, 2017 to February 28, 2022. Combined with Durie‐Salmon(DS) and International Staging System (ISS) of 427 patients diagnosed with PCM and overall survival time (OS) of 436, summarize the relevant plasma cell morphological characteristics. Statistical methods include chi-square test, Kruskal-Walls test, Spearman correlation analysis and Kaplan-Meier survival analysis.Result:The bone marrow morphology reports showed that the typical morphological features of peripheral blood in 573 patients with PCM included plasma cells (40.84%), immature granulocytes (30.89%), rouleaux formation in erythrocytes (68.94%) and nucleated red blood cells (8.55%). The types of bone marrow plasma cells in 1 071 patients diagnosed with PCM included 372 (34.73%) plasmablasts, 674 (62.93%)immature plasma cells, and 25 (2.34%) mature plasma cells. There is a significant positive correlation between the number of bone marrow plasma cells (proportion of nuclear cells) and the concentration of IgG and IgA type, from M protein identification( r=0.55, r=0.60, P<0.01). The proportions of M protein types in 1 071 patients with PCM from high to low were IgG (45.75%), IgA (23.53%), light chain (19.61%), IgD (4.76%), non-secretory (4.3%), biclonal (1.78%), IgE (0.19%), IgM (0.08%). The typical characteristics of the bone marrow plasma cells in various M protein types included clustered distribution, different cell body sizes, inclusions in the cytoplasm, binuclear, polynuclear, and abnormal nuclear. The proportion of plasmablasts in DSⅢ stage was 44.81% (164/366), higher than 21.57% (11/51) in DSⅡstage, and the difference was statistically significant(χ 2=10.2, P<0.05). There was a significant positive correlation between the number of bone marrow plasma cells and DS and ISS stages( r=0. 0.23, r=0.30, P<0.01). The median OS of the PCM patients in the plasmablasts group was significantly shorter than that in the immature plasma cells group [56.0 (23.0, 101.8) months vs 75.9(31.6, 121.5) months, HR=1.42,95% CI 1.05-1.91, P=0.02]. The median OS of the PCM patients in the group of tumor plasma cells burden≥37.5% was shorter than that of the tumor plasma cells burden<37.5% [75.9 (21.4, 122.6)months vs 81.3 (36.6, 108) months, HR=1.54,95% CI 1.14-2.07, P<0.05]. Conclusion:The morphology and tumor burden of bone marrow plasma cells provide an important basis for the diagnosis of PCM and can be used as a prognostic indicator for patients with PCM.
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OBJECTIVE@#to analyze the effect of circulating plasma cells(CPC) on the prognosis of patients with multiple myeloma(MM) in the era of new drugs, and to explore the new definition standard of primary plasma cell leukemia(pPCL).@*METHODS@#The clinical data of 321 patients with newly diagnosed MM and 21 patients with pPCL admitted to our hospital from January 2014 to May 2022 were retrospectively analyzed. According to the proportion of CPC in peripheral blood smears, all patients were divided into 4 groups: CPC 0% group(211 cases), CPC 1%-4% group(69 cases), CPC 5%-19% group(41 cases) and CPC≥20% group(21 cases). The clinical features of patients in each group were compared and the prognosis fators was analyzed.@*RESULTS@#The median OS of the four groups were 44.5,21.3,24.6 and 12.8 months, respectively. Among them, 295 patients(86.3%) were treated with new drugs, and the median OS of the four groups were not reached, 26.7, 24.6 and 14.9 months, respectively. As the survival curves of CPC 5%-19% group and CPC≥20% group were similar, the patients were divided into CPC<5% group and CPC≥5% group, the median OS of CPC<5% group was better than that in CPC≥5% (43.5 vs 22.3 months, P<0.001). In addition, the median OS of patients in the CPC 1%-4% group was also significantly lower than that in the CPC 0% group and similar to that in the CPC≥5% group. Multivariate analysis showed that 1%-4% CPC was an independent risk factor for the OS of patients with CPC<5%. The patients with CPC<5% were stratified by R-ISS staging, and the OS of R-ISS stage Ⅰ or stage Ⅱ with 1%-4% CPC was similar to that of R-ISS stage Ⅲ. The newly defined pPCL patients showed increased tumor load and obvious invasive characteristics. Multivariate analysis showed no independent prognostic factors for pPCL, and high-risk cytogenetic abnormalities(HRCA) had no significant effect on the prognosis.@*CONCLUSION@#The validity of IMWG's new pPCL definition standard was verified, and it was found that the survival of MM with 1%-4% CPC also is poor and the prognosis is very close to pPCL. In addition, the newly defined pPCL has unique clinical and biological characteristics.
Тема - темы
Humans , Multiple Myeloma/pathology , Plasma Cells/pathology , Retrospective Studies , Prognosis , Leukemia, Plasma Cell/diagnosisРеферат
Introducción: El mieloma múltiple con expresión de inmunoglobulina M de superficie constituye una enfermedad rara cuya causa es desconocida y se caracteriza por una alta tasa de anormalidades genéticas en las células plasmáticas o sus precursores. Objetivo: Determinar las características clínicas y sus asociaciones con la expresión inmunofenotípica de inmunoglobulina M de superficie e inmunohistoquímica de CD20 en una paciente afectada de mieloma múltiple precedido por síndrome mielodisplásico. Presentación del caso: Paciente femenina, 68 años de edad. Admitida en el Servicio de Hematología Clínica. Al momento del diagnóstico presentó palidez, trombocitopenia, hipercalcemia y lesiones óseas. Inicialmente, mediante citometría de flujo se detectaron patrones aberrantes para granulocitos, neutrófilos, monocitos y serie eritroide, sugerentes de síndrome mielodisplásico. Posteriormente se observó aumento de las células plasmáticas del 18 % en el frotis de médula ósea, exhibiendo una morfología similar a linfocitos. Se reportó una población patológica de 6 % de la celularidad total, mostrando positividad para CD38, CD117 e inmunoglobulina M de superficie, negatividad para CD19 y CD45, fenotipo coherente con células plasmáticas anormales. Adicionalmente resultados de inmunohistoquímica relataron tinción difusa de CD20 en biopsia de médula ósea. La paciente logró recuperarse luego de un trasplante autólogo de células progenitoras hematopoyéticas. Conclusión: Los resultados resaltan la importancia de diagnosticar y monitorear casos únicos que permitan un tratamiento oportuno del paciente.
Introduction: Multiple Myeloma with expression of surface immunoglobulin M is a rare entity, whose cause is unknown, and is characterized by a high rate of genetic abnormalities in plasma cells or their precursors. Objective: To determining the clinical characteristics and their associations with the immunophenotypic expression of surface immunoglobulin M and CD20 immunohistochemistry in a patient affected by Multiple Myeloma preceded by Myelodysplastic syndrome. Case presentation: A 68-year-old female patient is admitted to the Clinical Hematology Service. At the time of diagnosis, she presented pallor, thrombocytopenia, hypercalcemia, and bone lesions. Initially, flow cytometry detected aberrant patterns for neutrophilic granulocytes, monocytes, and the erythroid series suggestive of myelodysplastic syndrome. Subsequently, an 18% increase in plasma cells was observed in the bone marrow smear, exhibiting a lymphocyte-like morphology. A pathological population of 6% of the total cellularity was reported, showing positivity for CD38, CD117 and surface immunoglobulin M, negativity for CD19 and CD45, a phenotype consistent with abnormal plasma cells. Additionally, immunohistochemical results reported diffuse CD20 staining in bone marrow biopsy. The patient managed to recover after an autologous hematopoietic stem cell transplant. Conclusion: The results found highlight the importance of diagnosing and monitoring single cases that allow timely treatment of the patient.
Тема - темы
Female , AgedРеферат
Non-immunoglobulin intracytoplasmic inclusions in plasma cells of multiple myeloma are very rare presentation. These are morphologically similar to Auer rods but chemically different from them. We studied two cases of multiple myeloma in a 60-year-old woman and 45-year-old man. In both cases, plasma cells of bone marrow aspirate revealed multiple Auer rod-like inclusions (ARLI). Sudan black B (SBB) and myeloperoxidase (MPO) cytochemistry were negative. Serum protein electrophoresis in both of them showed M spike, one with raised IgA-kappa levels, while the other with raised IgG-kappa levels. Very few case reports have been published in the literature and its prognostic implications are still unknown. Due to its rarity, it is important to distinguish such cases from acute myeloid leukemia and its exact incidence with its therapeutic and prognostic implications
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Objetivo: Describir la presentación histopatológica atípica en médula ósea de mieloma múltiple. Reporte de Caso: Presentamos el caso de un varón de 75 años, quien manifiesta lumbalgia de intensidad variable asociada a disminución del flujo urinario, por lo que acude a emergencia. Al examen físico se encuentran funciones vitales dentro de parámetros normales, disminución generalizada del tejido adiposo y palidez terrosa, el resto del examen no contributorio. En estudios de médula ósea se reporta 13% de células plasmáticas, observándose células neoplásicas tetranucleadas con 1 y 2 nucleolos, citoplasma basófilo con presencia de vacuolas; es corroborado en citometría de flujo con fenotipo patológico en relación a neoplasia de células plasmáticas. Paciente recibe esquema terapéutico con ciclofosfamida, dexametasona y talidomida. Conclusión: La presentación tetranucleada es una variante histopatológica del mieloma múltiple poco frecuente; por lo que este hallazgo peculiar, debe ser valorado en el diagnóstico microscópico de la entidad clínica.
Objetive: To describe the atypical histopathologic presentation in bone marrow of multiple myeloma. Case Report: We present the case of a 75-year- old male who manifested low back pain of variable intensity associated with decreased urinary flow, for which he went to the emergency room. On physical examination, vital functions were within normal parameters with generalized decrease in adipose tissue and earthy pallor, the rest of the examination being non-contributory. Bone marrow studies showed 13% of plasma cells with tetranucleated neoplastic cells with 1 and 2 nucleoli, basophilic cytoplasm with presence of vacuoles, which was corroborated in flow cytometry with pathological phenotype in relation to plasma cell neoplasia. The patient receives a therapeutic regimen with cyclophosphamide, dexamethasone and thalidomide. Conclusion: Tetranucleated presentation is a rare histopathologic variant of multiple myeloma, so this peculiar finding should be appreciated in the microscopic diagnosis of this clinical entity.
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Objective@# To summarize the clinical manifestations of IgG4-related diseases in the head and neck, explore treatment methods.@*Methods @#The clinical data of 21 patients diagnosed with IgG4-related diseases were retrospectively analyzed. The clinical data and the results of glucocorticoid and immunosuppressive therapy were studied retrospectively.@* Results@#All patients had swollen sclerotic masses, and CT showed irregular high-density masses with uniform enhancement in the enlarged glands. Some patients had mucosal thickening and mass-like changes in theoral cavity, nose, sinuses, throat and other tissues, and most of the patients had cervical lymphadenopathy and elevated serum IgG4 levels (≥ 1.35 g/L). Histopathological examination of affected exosine glands and affected mucosa and lymph nodes in all patients showed infiltration of lymphocytes, plasma cells and IgG4+ plasma cells. In 21 patients, the mass in the affected glands and mucosa (including head, neck and other tissues) disappeared, and the clinical symptoms were relieved after the application of glucocorticoids. However, with a reduction in glucocorticoids, the mass recurred or even worsened.@*Conclusion @#For patients with a single mass in the submandibular gland, parotid gland and other salivary glands, as well as lymph node enlargement, CT is the first choice to identify the nature of gland neoplasms. Combined with pathological examination, related auxiliary examination and peripheral blood examination are also needed to obtain a definitive diagnosis. Glucocorticoid therapy is used to achieve a good prognosis, and long-term follow-up and timely adjustment of medication regimens are required.
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Abstract Background: The treatment of cutaneous leishmaniasis is a challenge. A better understanding of the in situ mechanisms involved in the evolution and cure of the disease is essential for the development of new therapies. Objective: Correlate histopathological and immunological characteristics of cutaneous leishmaniasis lesions with clinical outcome after different treatment regimens. Methods: The authors analyzed cellular infiltration and immunohistochemistry staining for CD4, CD8 and IL-17 in biopsy samples from 33 patients with cutaneous leishmaniasis before treatment. All patients were recruited in a randomized clinical trial at Corte de Pedra (Bahia-Brazil) and assigned to receive Glucantime®, Glucantime® + Oral Tamoxifen or Glucantime® + Topical Tamoxifen. Patients were followed for 2 to 6 months to define disease outcome. Results: A similar expression of CD4, CD8 and IL-17 was observed in lesion samples regardless of clinical outcome. In general, a higher amount of CD8 cells were observed compared with CD4 cells. An important observation was that all patients whose cellular infiltrate did not contain plasma cells were cured after treatment. Study limitations: Isolated quantification of TCD8 and IL-17 using immunohistochemistry is insufficient to analyze the role of these molecules in the immunopathogenesis of cutaneous leishmaniasis. In addition, the expansion of the immunohistochemistry panel would allow a more complete analysis of the immune response in situ. Conclusions: The absence of plasma cells in cutaneous leishmaniasis lesions was related to a favorable therapeutic outcome.
Тема - темы
Humans , Leishmaniasis, Cutaneous/drug therapy , CD4-Positive T-Lymphocytes , Treatment Outcome , CD8-Positive T-Lymphocytes , Meglumine AntimoniateРеферат
Objetivo: En los granulomas periapicales, los plasmocitos (PL) participan activamente mediante la liberación de inmunoglobulinas. El propósito de este ensayo fue identificar y contar el número de PL en diferentes períodos de tiempo en lesiones periapicales experimentales en ratas. Materiales y métodos: Mediante la exposición al medio oral de la pulpa de los primeros molares inferiores izquierdos, se indujeron granulomas periapicales en ratas a las que previamente se les suministró anestesia. La pulpa de los primeros molares inferiores derechos no fue expuesta, y estos dientes se utilizaron como control. Los animales fueron eutanasiados a los 10, 30 y 60 días de la exposición. Los maxilares inferiores fueron removidos, y los primeros molares, junto con los tejidos circundantes, se procesaron para su estudio histológico. Se obtuvieron secciones semiseriadas, posteriormente coloreadas con verde de metilo-pironina (VMP). Cada tres secciones, las tres siguientes fueron coloreadas con hematoxilina y eosina (H-E). Los controles también fueron coloreados con H-E. Resultados: Todos los especímenes experimentales coloreados con H-E revelaron la presencia de granulomas periapicales. Luego de la exposición pulpar, el número de PL que reaccionó positivamente al VMP se incrementó de manera progresiva desde el día 10 hasta los días 30 y 60. A pesar de que a los 60 días el número de PL fue ligeramente menor que a los 30 días, no hubo diferencias estadísticamente significativas entre estos dos períodos. Los especímenes del grupo control coloreados con H-E mostraron que los tejidos periapicales se encontraban dentro de los parámetros normales en todos los períodos de observación. Conclusiones: Los resultados de este estudio revelaron que el número de plasmocitos VMP positivos se incrementa progresivamente en función del tiempo transcurrido pero se estabiliza al finalizar el experimento. También sugieren que el empleo de la coloración de VMP es un procedimiento adecuado para la identificación y la cuantificación de plasmocitos en los granulomas periapicales inducidos experimentalmente en ratas (AU)
Aim: Plasma cells (PL) release immunoglobulin in periapical lesions. The purpose of this assay was to identify and count the number of plasmocytes observed in periapical lesions in rats. Materials and methods: By exposing the pulp of the lower left first molars to the oral environment, periapical granulomas were induced in rats previously anesthetized. The pulp of right mandibular first molars was not exposed and these teeth were used as negative controls. The animals were euthanized at 10, 30 and 60 days after pup exposure. The mandibles were removed and specimens of the molar teeth along with the surrounding tissues were prepared for histology. Semi serial sections of the left first molar were stained with methyl green pyronine (MGP). Every three sections, the following three sections were stained with hematoxilyn and eosin (H-E). Negative control samples were stained with H-E. Results: All the H-E stained experimental samples revealed the presence of periapical granulomas. After pulp exposure, the number of PL increased from day 10 to 30 and 60. In the 60-day samples the number of PL was slightly less than that of the 30-day samples, with no statistically significant difference. The H-E stained control samples showed normal periapical tissues in all observation periods. Conclusions: The results of this study revealed that the number of VMP positive PL, increased progressively with time but it was stabilized at the end of the experiment. In addition, the results suggest that the use of VMP stain is a suitable procedure for the identification and counting of PL in experimentally induced periapical granulomas in rats (AU)
Тема - темы
Animals , Rats , Periapical Granuloma/immunology , Plasma Cells/immunology , Inflammation/physiopathology , Periapical Tissue , Immunoglobulins , Photomicrography , Histological Techniques , Radiography, Dental, DigitalРеферат
SUMMARY: Inflammatory infiltrates are frequently present in melanocytic lesions, with different distribution and composition. Much attention has been devoted to tumor-infiltrating lymphocytes (TIL) in the tumor microenvironment, establishing their prognostic and predictive value in many malignancies, including melanoma. However, lymphocytes, albeit the most numerous and consistent presence, constitute only part of the immune microenvironment. Other inflammatory cells, including neutrophils, plasma cells, eosinophils and mast cells, are found in melanoma and other melanocytic lesions.Few studies offer a detailed count of these inflammatory infiltrates across the spectrum of melanocytic lesions. By using whole slide image analysis and open source software, in the present study we report the enumeration of different inflammatory infiltrates in benign melanocytic nevi, dysplastic nevi, melanoma in situ and invasive malignant melanomas. Significant higher numbers of plasma cells and neutrophils were observed in melanoma. These results indicate that composition of the inflammatory infiltrate may contribute to the diagnostic algorithm of melanocytic lesions.
RESUMEN: Los infiltrados inflamatorios están presentes con frecuencia en las lesiones melanocíticas, con diferente distribución y composición. Se ha prestado mucha atención a los linfocitos infiltrantes de tumores (TIL) en el microambiente tumoral, estableciendo su valor pronóstico y predictivo en muchas neoplasias malignas, incluido el melanoma. Sin embargo, los linfocitos de presencia más numerosa y constante, constituyen solo una parte del microambiente inmunológico. Otras células inflamatorias, incluidos neutrófilos, células plasmáticas, eosinófilos y mastocitos, se encuentran en el melanoma y otras lesiones melanocíticas. Pocos estudios ofrecen un recuento detallado de estos infiltrados inflamatorios en todo el espectro de lesiones melanocíticas. Mediante el uso de análisis de imágenes de diapositivas completas y software de código abierto, en el presente estudio informamos la enumeración de diferentes infiltrados inflamatorios en nevos melanocíticos benignos, nevos displásicos, melanoma in situ y melanomas malignos invasivos. Se observaron números significativamente más altos de células plasmáticas y neutrófilos en el melanoma. Estos resultados indican que la composición del infiltrado inflamatorio puede contribuir al algoritmo diagnóstico de las lesiones melanocíticas.
Тема - темы
Humans , Skin Neoplasms/immunology , Skin Neoplasms/pathology , Melanocytes/immunology , Melanocytes/pathology , Melanoma/immunology , Melanoma/pathology , Plasma Cells , Lymphocytes, Tumor-Infiltrating , Inflammation , Neutrophils/immunology , Neutrophils/pathologyРеферат
Plasmacytoma is a neoplastic proliferation of monoclonal plasma cells, which can present clinically as solitary bone neoplasm, extramedullary plasmacytoma, and multiple myeloma. The biological behavior of these tumors is variable from periods of clinical latency to rapid growth and progression from localized forms to more disseminated multiple myeloma. We present the case of solitary plasmacytoma of the mandible with rare bilateral involvement in a 65-year-old female patient. This paper highlights the importance of understanding the maxillofacial manifestations of the disease by the dentist for early diagnosis and thus better prognosis.
Тема - темы
Humans , Female , Aged , Plasmacytoma/pathology , Bone Neoplasms/pathology , Mandible/abnormalities , Plasma Cells/pathology , Early Diagnosis , Multiple MyelomaРеферат
@#Cutaneous multiple myeloma (MM) is a rare disease. It can be primary or secondary in origin. The secondary type is further classified into specific and nonspecific types. The specific type is uncommon and is known as a secondary cutaneous plasmacytoma. We report a case of secondary cutaneous plasmacytoma in a 58-year-old man who had a history of plasma cell tumour of the lung and multiple myeloma. He achieved complete remission after the completion of chemotherapy and autologous stem cell transplant (ASCT). However, five months later, he developed multiple erythematous nodules on the whole body. Skin biopsy revealed diffuse neoplastic cells infiltrate in the reticular dermis with sparing of the upper papillary dermis and epidermis. The neoplastic cells were monotonous and homogenous with variable degrees of cytological atypia. Occasional cells showed distinctive plasma cell features. Plasma cell lineage was confirmed with CD138. The cells were immunoreactive to Kappa. Ki-67 was greater than 90%. They were non-immunoreactive to CD45, CD3, CD20, CD79 alpha and CK AE1/AE3. The findings were consistent with secondary cutaneous plasmacytoma. Our case illustrates that MM may present with nonspecific dermatological manifestations. As specific cutaneous involvement of MM is very uncommon; a high degree of clinical suspicion, detailed medical history and histopathological examination are required to arrive at an early diagnosis.
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Objective:To observe the therapeutic efficacy and prognosis of daratumumab combined with chemotherapy bridging to allogeneic hematopoietic stem cell transplantation (allo-HSCT) followed by daratumumab and lenalidomide maintenance treatment for primary plasma cell leukemia (PCL).Methods:The clinical data of a patient with primary PCL admitted to the First People's Hospital of Yunnan Province in January 2020 were retrospectively analyzed, and relevant literatures were reviewed.Results:The patient was diagnosed with primary PCL and treated with daratumumab combined with BD (bortezomib + dexamethasone) for 1 course and BCDD (bortezomib + cyclophosphamide + liposomaldoxorubicin + dexamethasone) for two courses. The patient was treated with daratumumab combined with allo-HSCT after complete remission. The donor cells were successfully implanted and the chimerism rate of donor cells was 94.36% without acute graft-versus-host disease reaction. And then the patient received intermittent maintenance therapy of daratumumab combined with low dose lenalidomide after transplantation, and the current remission period after transplantation reached 4 months.Conclusions:Daratumumab combined with chemotherapy bridging to allo-HSCT followed by daratumumab and lenalidomide may improve the prognosis of primary PCL and prolong survival time.
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Abstract Introduction Isolated amyloidosis involving the head and neck is a rare entity. The pathophysiology of the localized disease appears to be distinct from that of the systemic counterpart. Systemic progression of the localized disease is unusual, and the prognosis of the localized form is excellent. Objective To describe the demographic and clinicopathological characteristics of patients presenting with localized head and neck subsite amyloidosis. Methods A retrospective chart review of the patients with head and neck amyloidosis identified by the electronic search of the electronic database of the Departments of Pathology and Otorhinolaryngology was performed. The various demographic and clinical data were tabulated. Results In total, seven patients (four females, three males) with localized head and neck amyloidosis (three supraglottic, three lingual and one sinonasal) were identified. Six patients had AL-amyloid deposits, and one patient had AA-amyloid deposits. Supraglottic involvement and that of the base of the tongue were treated surgically using CO2 laser, and these patients were disease-free at the last follow-up. The patient with sinonasal amyloidosis experienced symptom recurrence after six months of the functional endoscopic sinus surgery. All of the patients were screened for systemic amyloidosis with abdominal fat pad biopsy, and were found to be free of systemic spread. Conclusion Isolated head and neck amyloidosis, as opposed to systemic amyloidosis, has an excellent prognosis in terms of survival. Therefore, systemic amyloidosis should be excluded in all cases. The treatment of choice remains surgical excision; however, watchful waiting may be a suitable strategy for mild symptoms or for cases in which the disease was discovered incidentally.
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RESUMEN La plasmocitosis cutánea es una enfermedad poco frecuente, de curso crónico y benigno, que predomina en hombres entre los 20 y 62 años, particularmente en poblaciones asiáticas. Presentamos un caso de un hombre colombianoquien presentabamáculas y placas pardo-violáceas de bordes definidos, ligeramente infiltradas en tórax posterior y dorso de pies, sin otroshallazgos. Debido a que es una enfermedad que puede tener manifestaciones extracutáneaso transformación maligna por infiltración de células plasmáticas en otros órganos, se realizaron estudios de extensión que determinaron en este paciente que el compromiso era exclusivamente cutáneo. No existe un tratamiento estándar para esta enfermedad, se han usado antibióticos, corticosteroides tópicos y sistémicos, tacrolimus tópico, quimioterapia, talidomida, fototerapia UVB de banda estrecha y azatioprina, con resultados variables.
SUMMARY Cutaneous plasmacytosis is a rare disease of chronic and benign course, which occurs more frequently in men between 20 and 62 years, particularly in Asian populations. We present the case of a Colombian man who presents macules and violet-brown patches with defined edges, slightly infiltrated in the posterior thorax and feet, without any other manifestation. Because it is a disease that can have extracutaneous manifestations or malignant transformation due to the infiltration of plasma cells in other organs, extension studies were carried out, which determinedwhich determined exclusive cutaneous involvement. There is no standard treatment for this disease, antibiotics, topical and systemic corticosteroids, topical tacrolimus, chemotherapy, thalidomide, narrow-band UVB treatment and azathioprine have been used with variable results.