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Plasma cell leukemia (PCL) is a clinically aggressive variant of multiple myeloma, characterized by a high burden of circulating plasma cells, necessitating swift and accurate diagnosis due to its poor prognosis. The conventional diagnostic criteria, including the recent recommendation by the International Myeloma Working Group (IMWG) of > 5% circulating plasma cells as positive, have evolved over time. In this context, we present a detailed case report that underscores the pivotal role of the ADVIA 2120 automated hematology counter in detecting plasma cells through cytogram analysis, along with the significance of routine peripheral blood smear analysis and the utility of a large unstained cells (LUCs) threshold of > 4.5% as an indicator for PCL. The case involves a 64-year-old patient with relapsed multiple myeloma and stable paraprotein levels who experienced sudden renal impairment. In this case report, we highlight how ADVIA analysis and cytochemistry assisted in the diagnosis, and further explore ADVIA's utility in this challenging leukemia.
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AIM: This study aims to investigate demographic data, survival rates, and the relationship of these rates with surgery in a large case series including multiple myeloma (MM) patients. METHOD: MM cases were analyzed retrospectively using the latest version of the SEER database published in April 2020. This version covers January 1975 to December 2017. Patients were classified according to gender, age, and race/ethnicity. Tumors were classified according to their localization, grade, year of diagnosis, and follow-up results. RESULTS: There were 60,239 patients diagnosed with Plasma Cell Myeloma. While 670 patients (1.2%) were operated on, 43,976 patients (76.7%) did not indicate operation, and 12,670 patients (22.1%) could not be operated on despite the recommendation. The mean survival was 62 months in those without an indication for surgery, and 42 months in patients with an indication but could not be operated on, and the difference was significant (p = 0.001). The mean survival was 58 months in the operated patients, and 42 months in the patients who could not be operated on despite the indication, and the difference was significant (p = 0.001). There was no difference between those who did not indicate surgery and those who were operated on with an indication (p = 0.243). CONCLUSION: In multiple myeloma, the best prognosis is in the group of patients who received medical treatments without any indication for operation, while an indication for operation indicates a worse prognosis. A worse prognosis should be expected in patients who do not accept the operation or who cannot be operated on compared to the operated patients.
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Mieloma Múltiplo , Humanos , Mieloma Múltiplo/cirurgia , Estudos Retrospectivos , PrognósticoRESUMO
The use of plasma cell-free DNA (cfDNA) as a useful biomarker in obstetric clinical practice has been delayed due to the lack of reliable quantification protocols. We developed a protocol to quantify plasma cfDNA using an internal standard strategy to overcome difficulties posed by low levels and high fragmentation of cfDNA. cfDNA was isolated from plasma samples of non-pregnant (NP, n = 26) and pregnant (P, n = 26) women using a commercial kit and several elution volumes were evaluated. qPCR parameters were optimized for cfDNA quantification, and several quantities of a recombinant standard were evaluated as internal standard. Absolute quantification was performed using a standard curve and the quality of the complete method was evaluated. cfDNA was eluted in a 50-µl volume, actin-ß (ACTB) was selected as the target gene, and qPCR parameters were optimized. The ACTB standard was constructed and 1000 copies were selected as internal standard. The standard curve showed R2 = 0.993 and E = 109.7%, and the linear dynamic range was defined between 102 and 106 ACTB copies/tube. Repeatability and reproducibility in terms of CV were 19% and up to 49.5% for ACTB copies per milliliter of plasma, respectively. The range of cfDNA levels was 428-18,851 copies/mL in NP women and 4031-2,019,363 copies/mL in P women, showing significant differences between the groups. We recommend the application of internal standard strategy for a reliable plasma cfDNA quantification. This methodology holds great potential for a future application in the obstetric field.
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Ácidos Nucleicos Livres , Gestantes , Humanos , Feminino , Gravidez , Reprodutibilidade dos Testes , Ácidos Nucleicos Livres/genética , BiomarcadoresRESUMO
Plasma cell neoplasms are common, accounting for more than 1% of all malignancies. Its most common form is multiple myeloma, but others, such as extramedullary plasmacytoma (EMP), exist. Spinal cord compression secondary to these pathologies is not uncommon, however, adjacent bone involvement is usually present. Spinal cord compression caused by isolated epidural EMP is extremely rare, with only one case reported to date. We describe the case of a 75-year-old female patient that presented with paraparesis, due to an isolated epidural EMP associated with light chain amyloidosis. She was treated with surgical decompression and neoadjuvant chemotherapy, and is currently with a 15-month disease-free period.
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Introduction: IgG4 related disease (IgG4-RTD) is an infrequent disease with possible multiple organ involvement. It is characteristic to find inflammatory nodules with IgG4 positive plasma cell infiltration, storiform fibrosis and obliterative phlebitis. We present a patient with an inflammatory pseudotumor in the right upper lobe, mimicking a primary lung tumor. Case report: Our patient, a 48-year old heavy smoker (25 pack/year) with no relevant medical background, referred chest pain, non-productive cough and sporadic nightly fever. Image findings revealed a mass in the right upper lobe, with increased SUV in PET-scan, and mediastinal lymphadenopathies. Primary lung tumor was suspected and right upper lobectomy was performed. Due to absence of cellular atypia and the intense plasmacytic activity in the lesion, immunohistochemical analysis was performed: abundant IgG4 plasma cells were identified, with a IgG4/IgG relation of 74%. Diagnosis of IgG4- inflammatory pseudotumor was made. Discussion: After an extensive bibliographic review, we found just one similar case reported with an IgG4-lung pseudotumor without systemic disease. Due to the broad spectrum of clinical features of IgG4-RTD, and the potential multiple organ involvement, it is hard to find a classification and diagnostic criteria with high sensitivity and specificity, nevertheless they can be useful in clinical practice. Conclusion: There are several benign inflammatory diseases which can mimic a primary lung tumor. Although incidence is low, IgG4 pseudotumor should be considered as a differential diagnosis in the absence of malignancy.
Introducción: La enfermedad relacionada con IgG4 (IgG4-RTD) es una enfermedad poco frecuente con posible afectación multiorgánica. La presencia de infiltrados linfoplasmocitarios con células plasmáticas positivas para IgG4, fibrosis y flebitis obliterante. Presentamos el caso de un paciente con un pseudotumor inflamatorio en el lóbulo superior derecho, con presentación clínica compatible con tumor primario de pulmón. Caso clínico: Nuestro paciente de 48 años de edad, tabaquista severo (25 paquetes / año) sin antecedentes médicos relevantes, consulta por dolor torácico, tos no productiva y registros subfebriles aislados. Presenta una masa en el lóbulo superior derecho en estudio por imagen, con aumento de la captación en el PET, asociado a adenopatías mediastínicas. Con diagnóstico inicial de cáncer de pulmón, se realizó lobectomía superior derecha. Debido a la ausencia de atipia celular y la presencia de infiltrados linfoplasmocitarios en la lesión, se realizó análisis inmunohistoquímico: se identificaron abundantes células plasmáticas positivas para IgG4, con una relación IgG4 / IgG del 74%. Se realizó el diagnóstico de pseudotumor inflamatorio por IgG4. Discusión: Tras una extensa revisión bibliográfica, sólo encontramos un caso similar, de una paciente con un pseudotumor pulmonar IgG4 sin enfermedad sistémica. Debido a la variabilidad de la presentación clínica de la enfermedad relacionada a IgG4, y su potencial afectación multiorgánica, es difícil encontrar una clasificación y criterios diagnósticos con alta sensibilidad y especificidad, sin embargo estos suelen ser útiles en la práctica clínica. Conclusión: Múltiples enfermedades inflamatorias son diagnóstico diferencial de tumor primario de pulmón. Si bien la incidencia es baja, el pseudotumor IgG4 debe considerarse como un diagnóstico diferencial cuando no hay evidencia de enfermedad neoplásica.
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Granuloma de Células Plasmáticas , Neoplasias Pulmonares , Humanos , Pessoa de Meia-Idade , Imunoglobulina G/análise , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/patologia , Granuloma de Células Plasmáticas/cirurgia , Inflamação , Diagnóstico DiferencialRESUMO
INTRODUCTION: Numbers of hematopoietic cell transplants continue to increase globally but most of this activity is in resource-rich countries. Limitations to increasing transplant activity in resource-poor countries include lack of sophisticated personnel and infrastructure, complexity in identifying and accessing donors, unavailability of some new drugs and high cost. AREAS COVERED: We searched the biomedical literature for hematopoietic cell transplants and resource-rich and resource-poor countries. Recent advances which potentially make transplants more accessible in resource-poor countries include: (1) outpatient transplants; (2) grafts stored at 4°C; (3) less intensive pretransplant conditioning; (4) use of generic drugs; (5) less complex and costly donor access; and (6) increased collaboration with transplant centers in resource-rich countries. EXPERT OPINION: We reviewed publications on the limitations and solutions discussed above. Paradoxically, most data we analyzed originate from resource-rich countries. We found no convincing epidemiological data to support a recent increased transplant rate in resource-poor countries yet but hope to see increases soon.
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Transplante de Células-Tronco Hematopoéticas , Humanos , Transplante Homólogo , Doadores de TecidosRESUMO
Primary plasma cell leukemia (pPCL) is an infrequent and aggressive plasma cell disorder. The prognosis is still very poor, and the optimal treatment remains to be established. A retrospective, multicentric, international observational study was performed. Patients from 9 countries of Latin America (LATAM) with a diagnosis of pPCL between 2012 and 2020 were included. 72 patients were included. Treatment was based on thalidomide in 15%, proteasome inhibitors (PI)-based triplets in 38% and chemotherapy plus IMIDs and/or PI in 29%. The mortality rate at 3 months was 30%. The median overall survival (OS) was 18 months. In the multivariate analysis, frontline PI-based triplets, chemotherapy plus IMIDs and/or PI therapy, and maintenance were independent factors of better OS. In conclusion, the OS of pPCL is still poor in LATAM, with high early mortality. PI triplets, chemotherapy plus IMIDs, and/or PI and maintenance therapy were associated with improved survival.
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Leucemia Plasmocitária , Humanos , Leucemia Plasmocitária/diagnóstico , Leucemia Plasmocitária/epidemiologia , Leucemia Plasmocitária/terapia , Prognóstico , Bortezomib/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento , América Latina/epidemiologia , Agentes de Imunomodulação , DemografiaRESUMO
BACKGROUND: Cytopathologic analysis is feasible and provides detailed morphological characterisation of head and neck lesions. AIMS: To integrate the available data published on fine-needle aspiration cytology (FNAC) used for the diagnosis of plasma cell neoplasms (PCN) of the head and neck region. MATERIALS AND METHODS: Searches on PubMed, Web of Science, Embase, and Scopus were performed to compile data from case reports/case series published in English. The Joanna Briggs Institute tool was used for the critical appraisal of studies. RESULTS: A total of 82 studies comprising 102 patients were included in this review. There was a predilection for men (68.6%) (male/female ratio: 2.1:1). Individuals in their 50s (29.4%), 60s (22.5%), and 70s (22.5%) were more often affected. The thyroid gland (26.2%) was the main anatomical location, followed by scalp (15.5%), neck/cervical region (15.5%), jaws (13.6%), and major salivary glands (13.6%). For FNAC analysis, a smear was employed in 41 (40.6%) cases and a cell block was used in four (3.9%). In 56 (55.4%) reports, no cytological methods were available. Morphologically, 34 (56.7%) cases had a diagnosis of PCN with agreement between cytopathology and histopathology. The rate of wrong diagnoses when using cytology was 27.5%. Immunophenotyping was performed in 49 (48%) of the cases. The 69-month disease-free survival rate was 60.2%, while the 27-month overall survival rate was 64.1%. CONCLUSION: This study reinforces that FNAC can be an ancillary tool in the first step towards the diagnosis of PCN of the head and neck region, especially when applying a cell block for cytological analysis.
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Neoplasias de Cabeça e Pescoço , Neoplasias de Plasmócitos , Neoplasias , Humanos , Masculino , Feminino , Biópsia por Agulha Fina/métodos , Neoplasias/patologia , Pescoço , Glândula Tireoide/patologia , Neoplasias de Plasmócitos/patologia , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/patologiaRESUMO
Oral melanoacanthoma is an uncommon reactive lesion, characterized by basal and prickle cell keratinocyte proliferation surrounded by pigment-laden dendritic melanocytes. Plasma cell cheilitis (PCC) is an inflammatory disorder of unknown aetiology, microscopically presenting a dense plasma cell infiltrate. Most PCC cases affect the lower lip. Langerhans cell hyperplasia (LCHyp), a non-neoplastic counterpart of the LC proliferations, has been reported in association with chronic inflammatory skin diseases. Here, we present an unusual association of melanoacanthoma, PCC and LCHyp on the lower lip in a 59-old-year male, expanding the clinicopathological spectrum of these uncommon lesions. The dendritic melanocytes were highlighted by Fontana-Masson stain and HMB-45, whereas S100, CD1a and CD207 evidenced numerous LCs. MUM1/IRF4, EMA, and CD138 highlighted sheets of polyclonal plasma cells, with an IgG4+/IgG+ ratio of 24%. FTA-ABS test for syphilis was negative.
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A leucemia de células plasmocitárias (LCP) é uma neoplasia hematológica rara, de caráter agressivo, ainda sem consenso quanto ao melhor esquema terapêutico e prognóstico da doença. Alguns estudos a consideram como uma variante do mieloma múltiplo. O objetivo deste estudo foi realizar um relato de caso e desenvolver uma revisão sistemática da literatura sobre os fatores prognósticos relacionados a essa enfermidade. Esta revisão sistemática seguiu o protocolo PRISMA, utilizou o PubMED como base de dados, buscando estudos que avaliassem fatores prognósticos da LCP. Os estudos selecionados foram posteriormente avaliados quanto a sua qualidade pelo protocolo STROBE. A primeira busca identificou 260 artigos. Após aplicado critério de exclusão, dez foram selecionados. Destes, oito avaliaram fatores prognósticos clínicos e laboratoriais; cinco avaliaram, além disso, fatores citogenéticos; e um avaliou apenas a idade como fator prognóstico. A qualidade dos estudos, avaliada pelo protocolo STROBE, apresentou uma classificação média de 79,1%. Nos estudos analisados, idade > 65 ou > 60 anos, performance status ECOG > 2, hipercalcemia, aumento de lactato desidrogenase (LDH), aumento da creatinina, plaquetopenia e hipoalbuminemia foram encontrados como preditores de prognóstico ruim. Foram também demonstradas alterações citogenéticas consideradas de alto risco, como as translocações e deleções de genes. Diversas condições clínicas, laboratoriais e citogenéticas parecem estar associadas ao pior prognóstico na LCP. O conhecimento desses fatores pode interferir na prática clínica. Entretanto, ainda são necessários estudos mais robustos, multicêntricos e com maior número amostral para aprofundar o conhecimento sobre essa patologia.
Plasma Cell Leukemia (PCL) is rare and aggressive hematologic disorder, with no consensus on the best therapeutic scheme or disease prognosis. Some studies consider it to be a variant of multiple myeloma. This research sought to report a PCL case and to present a systematic literature review on its prognostic factor related. Bibliographic search followed the PRISMA protocol, and was conducted on the PubMED database to identify studies on PCL prognostic factors. Of the 260 articles identified, only ten were included after application of exclusion criteria. Of these, eight evaluated clinical and laboratorial prognostic factors; five assessed cytogenetic factors; and only one investigated age as a prognostic factor. Quality of the selected studies was evaluated by STROBE protocol, presenting a median classification of 79.1%. Age >65 or >60 years old, status performance ECOG>2, hypercalcemia, increased DHL, increased creatinine, thrombocytopenia, and hypoalbuminemia were the factors identified as predictors of a bad prognosis. Studies also showed high-risk cytogenetic abnormalities such as genetic translocation and deletions. Many clinical, laboratorial and cytogenetic conditions seem to be related to a worse PCL prognosis. Since knowledge of these factor can interfere in the clinical practice, more robust studies are needed on this pathology.
La leucemia de células plasmáticas (LCP) es una neoplasia hematológica rara, de carácter agresivo, sin consenso sobre el mejor régimen terapéutico y pronóstico de la enfermedad. Algunos estudios la consideran como una variante del mieloma múltiple. El objetivo de este estudio fue realizar un reporte de caso y desarrollar una revisión sistemática de la literatura sobre los factores pronósticos relacionados con esta enfermedad. Esta revisión sistemática siguió el protocolo PRISMA, utilizó PubMED como base de datos para la búsqueda de estudios que evaluaran los factores pronósticos para LCP. Los estudios seleccionados fueron posteriormente evaluados por su calidad mediante el protocolo STROBE. La primera búsqueda identificó 260 artículos. Después de aplicados los criterios de exclusión, se seleccionaron diez. De estos, ocho evaluaron los factores pronósticos clínicos y de laboratorio, cinco evaluaron también los factores citogenéticos y uno evaluó la edad solo como factor pronóstico. La calidad de los estudios, evaluada por el protocolo STROBE, presentó una calificación promedio del 79,1%. En los estudios analizados, la edad > 65 o > 60 años, el estado funcional ECOG > 2, la hipercalcemia, el aumento de lactato deshidrogenasa (LDH), el aumento de creatinina, la trombocitopenia y la hipoalbuminemia fueron los predictores de mal pronóstico. También se demostraron alteraciones citogenéticas que se consideraron de alto riesgo, como translocaciones y deleciones de genes. Varias condiciones clínicas, de laboratorio y citogenéticas parecen estar asociadas con peor pronóstico en LCP. El conocimiento de estos factores puede interferir en la práctica clínica. Sin embargo, aún se necesitan estudios más robustos, multicéntricos y con mayor tamaño muestral para profundizar en el conocimiento sobre esta patología.
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Prognóstico , Leucemia Plasmocitária , Hipercalcemia , Mieloma MúltiploRESUMO
We propose a mathematical model to study the antibody-dependent enhancement (ADE) phenomenon. Here, we explore the interaction between macrophages, dengue virus and plasma cells, especially the effect of a limitation on plasma cell proliferation, which occurs due to immunological memory. The model has up to three equilibrium points: one virus-free equilibrium and two virus-presence equilibrium, depending on the value of two thresholds. We determine the existence regions for the model equilibrium points and their stability, a sensitivity analysis was performed in the model thresholds. Numerical simulations illustrate that ADE can occur even when the basic reproduction number is less than one.
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Coinfecção , Vírus da Dengue , Dengue , Anticorpos Facilitadores , Clonagem Molecular , Humanos , Modelos Teóricos , PlasmócitosRESUMO
Os plasmocitomas extramedulares são uma proliferação excessiva de células plasmocíticas, que pode acometer nos cães, o sistema cutâneo e mucocutâneo. Classificados como benignos, já que por sua vez possuem um baixo potencial metastático, porém o local onde se infiltra se comporta de forma agressiva. A formação dos nódulos é isolada, mas podem ser múltiplos, apresentam coloração avermelhada e possuem tamanhos médios de 1 a 2 cm. O histopatológico evidencia plasmócitos, em arranjo de cordão e entremeados a uma discreta quantidade de tecido conjuntivo. O objetivo deste relato é identificar e diagnosticar um caso de plasmocitoma extramedular em cão positivo para leishmaniose.(AU)
The extramedullary plasmacytomas are an excessive proliferation of plasmacytic cells, which can affect the cutaneous and mucocutaneous systems in dogs. Classified as benign, as they in turn have a low metastatic potential, but the site where they infiltrate behaves aggressively. The formation of nodules is isolated, but they can be multiple, present with a reddish color and have average sizes of 1 to 2 cm. Histopathology shows plasma cells, in a cord arrangement and intermingled with a small amount of connective tissue. The purpose of this report is to identify and diagnose a case of oral plasmacytoma in a dog.(AU)
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Animais , Plasmocitoma/diagnóstico , Doenças do Cão/diagnóstico , Cães , Leishmaniose/veterináriaRESUMO
The spleen is involved in visceral leishmaniasis immunopathogenesis, and presents alterations in white-pulp microenvironments that are associated with an increased susceptibility to coinfections and patient death. Plasmacytosis in splenic red pulp (RP) is one observed alteration, but the specificity of antibody-secreting cells and the distribution of them has not yet been evaluated. We biotinylated soluble L. infantum membrane antigens (bSLMA) used as probes in modified immunohistochemistry, and detected the presence of anti-L. infantum antibody-secreting cells. Were used spleens from eight dogs from the endemic area for canine visceral leishmaniasis (CanL), and three healthier controls. The spleen sections were cryopreserved, and we performed modified immunohistochemistry. The ratio of plasma cells which were reactive to bSLMA (Anti-Leish-PC) in the spleen RP and periarteriolar lymphatic sheath (PALS) were calculated. Dogs with CanL present hyperglobulinemia and more plasma cells in their RP than the controls. Furthermore, dogs with CanL presented a lower proportion of Anti-Leish-PC in their RP than in PALS. Likewise, dysproteinemia was related to RP and PALS plasmacytosis, and a more severe clinical profile.
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Inflammatory Myofibroblastic Tumor (IMT) is a rare pathologic entity that was first described in 1973. This lesion is most commonly found in the lungs, but other organs' involvement has also been reported. Intracranial location of Inflammatory Myofibroblastic Tumor is rare, and the first case was reported in 1980. An intriguing fact about the intracranial IMT is its resemblance with meningioma on clinical presentation and neuroimaging. We came across a case of intracranial Inflammatory Myofibroblastic Tumor (IIMT) in a 27-year-old male who presented with recurrent episodes of seizures and was diagnosed as meningioma on neuroimaging. The lesion did not subside with medical management and kept on progressing in size. The patient had to undergo surgery, and diagnosis of Inflammatory Myofibroblastic Tumor was ascertained on histopathology. This 'surprise' diagnosis prompted us to review the literature on all cases of IIMTs reported to date to better understand the entity and its implications. In this review article, we present our observations regarding various studied parameters, including patient profile, clinical presentation, site of involvement, focality of the lesion, special associations, and lines of management of the 49 published cases of IIMTs.
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BACKGROUND: There have been reports on the use of hypofractionated stereotactic body radiotherapy (SBRT) for bone plasmacytomas, but no prospective data are available. We present the initial analysis of an ongoing prospective protocol on SBRT addressing the feasibility and safety of this treatment for solitary bone plasmacytomas. PATIENTS AND METHODS: A prospective cohort of SBRT for solitary bone plasmacytoma was developed. Patients could receive different doses depending on the index bone, from single fraction for skull base lesions, 24 Gy in 3 fractions for spine lesions, and 30 Gy in 5 fractions for other bones. Overall survival, bone events, local control, and progression to multiple myeloma (MM) were measured and compared to our retrospective cohort of patients treated with conformal standard-dose radiotherapy. Quality of life was assessed via the EORTC QLQ-C30 questionnaire, and toxicities were assessed by the CTCAE v5.0 criteria. After 1 year or the inclusion of 5-10 patients, a feasibility and safety analysis was programmed. RESULTS: Between April 2018 and April 2019, 5 patients were included. All were male, with a median age of 53.1 years. The median follow-up was 21.8 months. No patient had local progression, bone event, or died. Two patients had progressions to MM. The mean survival free of progression to MM was 18.6 months, compared to 19 months in the retrospective cohort; median values were not reached. There were no grade 3 toxicities. CONCLUSION: SBRT for plasmacytoma is safe and feasible. More robust data are awaited.
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Neoplasias Ósseas/radioterapia , Plasmocitoma/radioterapia , Radiocirurgia , Adulto , Idoso , Neoplasias Ósseas/complicações , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/psicologia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/etiologia , Plasmocitoma/complicações , Plasmocitoma/mortalidade , Plasmocitoma/psicologia , Intervalo Livre de Progressão , Estudos Prospectivos , Qualidade de Vida , Doses de Radiação , Radiocirurgia/efeitos adversos , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Plasma cell neoplasms are characterized by the proliferation of a single clone of plasma cells with production of a monoclonal immunoglobulin. They can manifest as a single lesion (plasmacytoma) or as multiple lesions (multiple myeloma). METHODS: Paraffin-embedded tissue blocks of patients microscopically diagnosed with plasma cell neoplasms in the jaws were retrieved from five pathology files. Data including clinical, radiographic, microscopic and immunohistochemical findings, treatment employed and follow-up status were retrieved from the pathology reports. RESULTS: Fifty-two cases were retrieved (mean age: 59.4 years) without sex predilection. The mandible was the most affected site (67.3%), usually associated with pain and/or paresthesia (53.8%). Lesions in other bones besides the jaws were reported for 24 patients (46.2%). Radiographically, tumours usually presented as poorly defined osteolytic lesions with unilocular or multilocular images, while microscopy revealed diffuse proliferation of neoplastic plasma cells with nuclear displacement and abundant eosinophilic cytoplasm. Two cases were classified as anaplastic, and amyloid deposits were found in two other cases. Immunohistochemistry was positive for plasma cell markers and negative for CD20 and CD3, and monoclonality for kappa light chain predominated. The overall survival rate after 5 years of follow-up was 26.6%. CONCLUSION: Plasma cell neoplasms are aggressive tumours with a poor prognosis and involvement of the jaws may be the first complaint of the patient. Thus, oral pathologists, head and neck surgeons and dentists should be aware of their clinical, radiographic and microscopic manifestations.
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Mieloma Múltiplo , Neoplasias de Plasmócitos , Plasmocitoma , Humanos , Imuno-Histoquímica , Arcada Osseodentária/diagnóstico por imagem , Pessoa de Meia-Idade , Mieloma Múltiplo/diagnóstico por imagem , Neoplasias de Plasmócitos/diagnóstico por imagem , Plasmocitoma/diagnóstico por imagemRESUMO
Patients with rheumatoid arthritis (RA) may be classified as seropositive or seronegative according to the presence of autoantibodies. An abnormal B cell phenotype and function could be one of the main components of the immunopathology of seropositive patients; however, there is little information regarding B cell defects in these patients. This study shows a broad characterization of the B cell phenotype and function in patients with seropositive RA. We focused mainly on the evaluation of subsets, the expression of modulatory molecules of cell activation (CD22, FcÉ£RIIb and FcµR), calcium mobilization, global tyrosine phosphorylation, expression of activation markers, cytokine and immunoglobulin (Ig) production, proliferation and the in-vitro generation of plasma cells. Increased frequency of CD27- IgM- IgD- and CD21- B cells was observed in patients with seropositive RA compared with healthy donors (HD). Decreased expression of CD22 was primarily found in memory B cells of patients with RA regardless of seropositivity. B cells from seropositive patients exhibited normal proliferation, calcium mobilization kinetics and global tyrosine phosphorylation, but showed an increased frequency of CD86+ B cells compared with HD. B cells of seropositive patients secrete less interleukin-10 after in-vitro activation and showed a decreased frequency of plasma cell differentiation and IgM production compared with HD. Our data indicate that patients with seropositive RA have an increased frequency of atypical B cell populations previously associated with chronic activation and antigen exposure. This may result in the observed low responsiveness of these cells in vitro.
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Artrite Reumatoide/imunologia , Linfócitos B/imunologia , Adulto , Idoso , Autoanticorpos/imunologia , Diferenciação Celular/imunologia , Citocinas/imunologia , Feminino , Humanos , Isotipos de Imunoglobulinas/imunologia , Memória Imunológica/imunologia , Ativação Linfocitária/imunologia , Masculino , Pessoa de Meia-Idade , FenótipoRESUMO
Here we described a case of an asymptomatic 73 years-old female patient in geriatric routine consultation, whose laboratory testing showed hyperproteinemia with accompanying hyperglobulinemia. A diagnosis of BGUS was made only after a correlation among SPEP, densitometry tracing and IFE results was established, evidencing a second peak, that was less evident and not reported at first. These biclonal conditions are of very low incidence in the clinical laboratory, requiring the laboratory professional to have particular skills for their identification. As far as is known, clinical findings in BGUS are similar to those found in MGUS. However, they remain not well understood. Therefore, for an accurate diagnosis of BGUS, the clinical laboratory technician must be trained and sensitized to detect a second M - protein as a band or peak; taking in mind the possible different scenarios in heavy and light chain typing.
Se describe el caso de paciente asintomática de 73 años de edad en consulta geriátrica de rutina, cuyos estudios de laboratorios muestran hiperproteinemia acompañada de hiperglobulinemia. Se estableció el diagnóstico de GBSI después de correlacionar entre resultados de electroforesis de proteínas, trazo de densitometría e inmunofijación en suero, los cuales evidenciaron un segundo pico monoclonal menos evidente y no reportado de primera instancia. Este tipo de condiciones biclonales son de muy baja incidencia en laboratorio clínico, lo cual requiere que profesional de laboratorio tenga ciertas habilidades para su identificación. Hasta donde se conoce, los hallazgos clínicos de GBSI son similares a aquellos encontrados en GMSI. Sin embargo, continúan sin ser bien comprendidas. Por tanto, a fin de un diagnóstico más preciso, el técnico de laboratorio debe estar entrenado y sensibilizado para encontrar una segunda proteína M como banda o pico, tomando en cuenta los diferentes posibles escenarios en la tipificación de cadenas pesadas y ligeras.
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BACKGROUND: Solitary extradural plasmacytoma of the skull (SEPS) is an extremely rare entity with only 35 cases reported in the English-language literature. SEPS is a rare presentation of plasma cell dyscrasias, accounting for 4% of plasma cell tumors. The diagnosis of solitary plasmacytoma requires exclusion of multiple myeloma (MM) and prompt diagnosis and treatment. OBSERVATIONS: The authors describe the case of a 52-year-old man with SEPS. He presented with a painless, progressive, soft swelling mass in the left parietal region. Magnetic resonance imaging revealed a left frontotemporal extra-axial lesion that involved the ipsilateral orbital apex and posterior ethmoidal cells. Biological studies did not reveal features suggestive of MM. A diagnosis of SEPS was based on microscopic examination and immunohistochemical analysis after surgery. The patient had an excellent recovery and was discharged the day after surgery without neurological deficit. LESSONS: SEPS is a potentially curable disease, and total resection with or without radiotherapy is associated with a good prognosis and long-term recurrence-free survival. Distinction between SEPS and MM is of paramount importance because the prognosis and treatment differ.
RESUMO
Inflammatory Myofibroblastic Tumor (IMT) is a rare pathologic entity that was first described in 1973. This lesion is most commonly found in the lungs, but other organs' involvement has also been reported. Intracranial location of Inflammatory Myofibroblastic Tumor is rare, and the first case was reported in 1980. An intriguing fact about the intracranial IMT is its resemblance with meningioma on clinical presentation and neuroimaging. We came across a case of intracranial Inflammatory Myofibroblastic Tumor (IIMT) in a 27-year-old male who presented with recurrent episodes of seizures and was diagnosed as meningioma on neuroimaging. The lesion did not subside with medical management and kept on progressing in size. The patient had to undergo surgery, and diagnosis of Inflammatory Myofibroblastic Tumor was ascertained on histopathology. This 'surprise' diagnosis prompted us to review the literature on all cases of IIMTs reported to date to better understand the entity and its implications. In this review article, we present our observations regarding various studied parameters, including patient profile, clinical presentation, site of involvement, focality of the lesion, special associations, and lines of management of the 49 published cases of IIMTs.