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1.
J Leukoc Biol ; 109(1): 77-90, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33617048

RESUMO

B cells play a central role in antiviral and antiparasitic immunity, not only as producers of antibodies, but also as APCs and mediators of inflammation. In this study, we used 16-color flow cytometry analysis to investigate the frequency, differentiation, and activation status of peripheral B cells of patients with SARS-CoV-2 infection or acute Plasmodium falciparum malaria compared with the healthy individuals. As a main result, we observed an increase of the frequency of (CD27-, CD21-) atypical memory B cells and (CD19+, CD27+, CD38+) plasmablasts in malaria and COVID-19 patients. Additionally, CD86, PD-1, CXCR3, and CD39 expression was up-regulated, whereas CD73 was down-regulated on plasmablasts of COVID-19 and malaria patients compared with the bulk B cell population. In particular, there was a more pronounced loss of CD73+ B cells in malaria. The frequency of plasmablasts positively correlated with serum levels of CRP, IL-6, and LDH of COVID-19 patients. In the longitudinal course of COVID-19, a rapid normalization of the frequency of atypical memory B cells was observed. The role and function of plasmablasts and atypical memory B cells in COVID-19 and other acute infections remain to be further investigated. The role of B cells as either "driver or passenger" of hyperinflammation during COVID-19 needs to be clarified.


Assuntos
/imunologia , Memória Imunológica , Malária Falciparum/imunologia , Plasmócitos/imunologia , Plasmodium falciparum/imunologia , /imunologia , Adulto , Idoso , Antígenos CD/imunologia , Feminino , Humanos , Malária Falciparum/patologia , Masculino , Pessoa de Meia-Idade , Plasmócitos/patologia
2.
Medicine (Baltimore) ; 99(39): e22299, 2020 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-32991435

RESUMO

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a condition characterized by a hyperinflammatory state and persistent macrophage activation, resulting in reactive phagocytosis of the hematopoietic elements. In children, it is usually a hereditary disorder, while in adults it is usually acquired secondary to viral infections, collagenoses, or tumors. Although accounting for 10% of hematologic malignancies, HLH is rarely associated with multiple myeloma (MM) and other plasmacytic dyscrasias. PATIENT CONCERNS: A 64-year-old Brazilian man seeked medical care with a 3-month history of intermittent fever, weight loss, night sweats, and progressive anemic symptoms. DIAGNOSIS: Total blood count showed severe bicytopenia (normocytic-normochromic anemia and thrombocytopenia), biochemical exams showed elevation of creatinine, as well as monoclonal peak in serum protein electrophoresis, high IgA dosage, and serum immunofixation with IgA kappa paraprotein. Bone marrow biopsy showed 30% of monoclonal and phenotypically anomalous plasmocytes, confirming the diagnosis of MM. Diagnosis of HLH was established by the presence of clinical and laboratory criteria: fever, splenomegaly, cytopenias, hypofibrinogenemia, hyperferritinemia, elevation of triglycerides, and several figures of erythrophagocytosis in bone marrow aspirate. INTERVENTIONS: The patient experienced pulse therapy with methylprednisolone for hemophagocytic lymphohistiocytosis, followed by initial therapy for multiple myeloma with cyclophosphamide and dexamethasone. OUTCOMES: Once the diagnosis of MM and secondary hemophagocytic syndrome was established, the patient had a rapid clinical deterioration despite the established therapeutic measures, evolving with cardiovascular failure, acute liver failure, acute disseminated intravascular coagulation, worsening renal dysfunction requiring dialysis support, respiratory dysfunction, and lowering of consciousness, characterizing rapid multiple organ dysfunction, ultimately leading to the death of the patient. INNOVATION: Here, we aimed to describe the sixth reported case of HLH associated with MM, according to cases cataloged in the PubMed database, and the first case evaluated by 18-fluordeoxyglucose positron emission tomography (18-FDG-PETCT). CONCLUSION: Our case report seeks to provide support for a better clinical and laboratory characterization of this rare paraneoplastic entity associated with MM, and aims to call the attention of hematologists and intensivists to this condition that falls within the scope of the differential diagnosis of rapid onset multiple organ failure in patients with plasmacytic neoplasms.


Assuntos
Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/etiologia , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Anemia/sangue , Anemia/etiologia , Antineoplásicos Alquilantes/administração & dosagem , Antineoplásicos Alquilantes/uso terapêutico , Medula Óssea/patologia , Brasil/epidemiologia , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Dexametasona/administração & dosagem , Dexametasona/uso terapêutico , Quimioterapia Combinada , Evolução Fatal , Febre/diagnóstico , Febre/etiologia , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Linfo-Histiocitose Hemofagocítica/sangue , Linfo-Histiocitose Hemofagocítica/diagnóstico , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Mieloma Múltiplo/metabolismo , Insuficiência de Múltiplos Órgãos/complicações , Paraproteinemias/sangue , Plasmócitos/patologia , Esplenomegalia/diagnóstico , Esplenomegalia/etiologia , Trombocitopenia/sangue , Trombocitopenia/etiologia , Perda de Peso
3.
Medicine (Baltimore) ; 99(39): e22335, 2020 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-32991445

RESUMO

INTRODUCTION: Plasmablastic lymphoma (PBL) is an uncommon and aggressive large B-cell lymphoma commonly diagnosed in human immunodeficiency viruses -positive patients. Oral cavity is the most commonly PBL affected site. Most oral PBLs presented as asymptomatic swellings, frequently associated with ulcerations and bleeding. Most cases lacked B-symptoms, suggesting a more local involvement of the disease. No standard treatment is yet for oral PBL. Five-year survival rate recorded no more than 33.5%. PATIENT CONCERNS: A 39-year-old male presented to Dental Clinic with 1 month swelling of the oral cavity, in absence of any other symptoms or signs. He followed antibiotic therapy just on suspicion of an oral abscess and later oral surgical treatment on suspicion of bone neoplasm. DIAGNOSIS: Surgical specimen analysis highlighted a diffuse infiltrate of large-sized atypical cells with plasmablastic appearance and plasma cell phenotype. Oral cavity PBL was diagnosed. Blood tests recorded mild lymphopenia and positive human immunodeficiency viruses serology. INTERVENTIONS: Patient underwent chemotherapy including intrathecal methotrexate prophylaxis, in addition to a highly active antiretroviral therapy. OUTCOMES: At 12 months from diagnosis, patient recorded complete hematological remission. CONCLUSIONS: Oral PBL diagnosis requires a high level of suspicion and awareness both by physicians and pathologists. They should be aware of the extent of such disease which is often mistaken as oral abscess or infected tooth, thus leading to delay the most appropriate diagnostic evaluation. As PBL is an aggressive non-Hodgkin lymphoma, a delayed diagnosis might negatively impact on both treatment and survival.


Assuntos
Antineoplásicos/uso terapêutico , Terapia Antirretroviral de Alta Atividade/métodos , Boca/patologia , Linfoma Plasmablástico/tratamento farmacológico , Abscesso/diagnóstico , Abscesso/tratamento farmacológico , Adulto , Assistência ao Convalescente , Antibacterianos/uso terapêutico , Antimetabólitos Antineoplásicos/administração & dosagem , Antimetabólitos Antineoplásicos/uso terapêutico , Quimioterapia Combinada , Edema/etiologia , Infecções por HIV/complicações , Soropositividade para HIV/sangue , HIV-1/imunologia , Humanos , Injeções Espinhais , Linfoma não Hodgkin/patologia , Masculino , Metotrexato/administração & dosagem , Metotrexato/uso terapêutico , Plasmócitos/patologia , Linfoma Plasmablástico/diagnóstico por imagem , Resultado do Tratamento
4.
Ann Hematol ; 99(11): 2599-2609, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32935190

RESUMO

Methods to estimate bone marrow plasma cells (BMPC) basically include histopathology, cytomorphology, and flow cytometry. The present study compares the outcomes of these methods with special focus on the impact of BMPC-specific characteristics on their recovery by either method. Laboratory reports of diagnostic samples from 238 consecutive patients with suspected or known plasma cell disease were retrospectively analyzed. The median (IQR) proportion of BMPC was 30.0% (15.0-70.0%) by histological review (hBMPC), 7.0% (2.0-16.0%) by smear review (sBMPC), and 3.0% (0.8-10.0%) by flow cytometry (fBMPC). The disparity of results between core biopsy and aspirate smear was enhanced in case of poor quality of the smear, increased BM fiber content, higher grade cell atypia, expression of CD56 (all P < 0.0001), the number of cytogenetic aberrations (P = 0.0002), and abnormalities of the MYC gene (P = 0.0002). Conversely, expression of CD19 and a non-clonal plasma cell phenotype were associated with a lower difference between hBMPC and sBMPC (both P < 0.0001). The disparity between the percentages of sBMPC and fBMPC was associated with the quality of the smear (P = 0.0007) and expression of CD56 (P < 0.0001). Our results suggest that the recovery of BMPC in aspirate specimens not only is a matter of sampling quality but also depends on biological cell properties. Aspiration failure due to malignant type features of BMPC may lead to misclassification of plasma cell disorders and represent a bias for the detection of minimal residual disease after therapy.


Assuntos
Antígenos CD19/biossíntese , Células da Medula Óssea , Antígeno CD56/biossíntese , Mieloma Múltiplo , Proteínas de Neoplasias/biossíntese , Plasmócitos , Adulto , Células da Medula Óssea/metabolismo , Células da Medula Óssea/patologia , Feminino , Citometria de Fluxo , Humanos , Imunofenotipagem , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/classificação , Mieloma Múltiplo/metabolismo , Mieloma Múltiplo/patologia , Mieloma Múltiplo/terapia , Neoplasia Residual , Plasmócitos/metabolismo , Plasmócitos/patologia , Estudos Retrospectivos
5.
Medicine (Baltimore) ; 99(34): e21799, 2020 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-32846815

RESUMO

Hepatitis B (HBV) and hepatitis C (HCV) viruses are hepatotropic and lymphotropic viruses that can proliferate either in lymphocytes and monocytes or hepatocytes.The aim of this study was to evaluate the seroprevalence of HBV, HCV, and human immunodeficiency virus (HIV) in patients with plasma cell disorders. We also aimed to compare patients with plasma cell disorders and chronic lymphocytic leukemia (CLL) in terms of HBV, HCV, and HIV seropositivity.This is a retrospective study. The patients who had patient file in the Multiple Myeloma Outpatient Unit of our hospital and were followed in our outpatient unit between January 1, 2012 and September 15, 2019, with diagnoses of either of the plasma cell disorders were included in the study. In addition, 272 CLL patients who were admitted to the Leukemia Outpatient Unit of our hospital were also enrolled in the study. The 2 disease groups were compared in terms of HBV, HCV, and HIV seropositivity.A statistically significant relationship was found between disease groups according to hepatitis B surface antigen (P < .05). Hepatitis B positivity were found to be more common in CLL patients. There was also a statistically significant relationship between the disease groups in terms of hepatitis B e antigen positivity (P = .001).We found that hepatitis B surface antigen positivity rate in CLL patients was higher than in patients with plasma cell disorders. Seroprevalence of HBV, HCV, and HIV was found to be very low in patients with plasma cell disorders.


Assuntos
Soroprevalência de HIV , Antígenos da Hepatite B/sangue , Hepatite B/epidemiologia , Hepatite C/epidemiologia , Leucemia Linfocítica Crônica de Células B/epidemiologia , Paraproteinemias/epidemiologia , Idoso , Comorbidade , Feminino , Infecções por HIV/sangue , Infecções por HIV/imunologia , Hepatite B/sangue , Antígenos da Hepatite B/imunologia , Antígenos do Núcleo do Vírus da Hepatite B/sangue , Antígenos do Núcleo do Vírus da Hepatite B/imunologia , Antígenos de Superfície da Hepatite B/sangue , Antígenos de Superfície da Hepatite B/imunologia , Antígenos E da Hepatite B/sangue , Anticorpos Anti-Hepatite C/sangue , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/epidemiologia , Plasmócitos/patologia , Estudos Retrospectivos , Estudos Soroepidemiológicos
6.
Clin Nucl Med ; 45(9): 681-682, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32657869

RESUMO

A 64-year-old woman who had yellowish orange-colored, sharp-edged, and flat skin plaques and back pain for about a year was referred for F-FDG PET/CT imaging and her bone marrow biopsy revealed 10% to 20% atypical plasma cells suggestive of monoclonal gammopathy. PET/CT showed linear F-FDG uptake on the surface of the skin on the neck, axillary, chest, inframammary, and inguinal regions. Punch biopsy revealed numerous histiocyte infiltrates with large vacuoles, xanthomized cytoplasm in the dermis, and midsection, and there are several Touton-type giant cells supporting the preliminary diagnosis of xanthoma disseminatum.


Assuntos
Fluordesoxiglucose F18 , Histiocitose de Células não Langerhans/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Biópsia , Medula Óssea/patologia , Feminino , Histiócitos/patologia , Histiocitose de Células não Langerhans/patologia , Humanos , Pessoa de Meia-Idade , Plasmócitos/patologia , Baço/patologia
7.
Anticancer Res ; 40(7): 3743-3749, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32620613

RESUMO

BACKGROUND/AIM: The antiproliferative effects of cold atmospheric plasma (CAP) make it a promising application option in oncology. The aim of the present study was to examine whether short-term CAP treatment leads to an initial partial elimination of the treated cells or to long-term impairement and inhibition of cell growth. MATERIALS AND METHODS: Cells were treated with CAP and biostatistical modelling was used to estimate growth rates over the incubation time. Four cell lines (U2-OS and MNNG osteosarcoma cells, 3T3 fibroblasts, HaCaT keratinocytes) and three CAP sources (MiniJet-R, kINPen MED, Maxium) were used. RESULTS: The antiproliferative efficacy of CAP was due to a significant reduction in cell count during treatment and the long-lasting inhibition of growth rate in the remaining cells, detectable in all cell lines and after treatment using all three CAP devices. CONCLUSION: Induction of cell death and inhibition of cell growth are part of a general mechanism of biological CAP efficacy. However, data contradict the hypothesis that cancer cells respond more sensitively to CAP treatment compared to non-malignant cells.


Assuntos
Proliferação de Células/efeitos dos fármacos , Plasmócitos/patologia , Gases em Plasma/farmacologia , Morte Celular/efeitos dos fármacos , Linhagem Celular , Linhagem Celular Tumoral , Fibroblastos/efeitos dos fármacos , Fibroblastos/patologia , Humanos , Cinética , Osteossarcoma/tratamento farmacológico , Osteossarcoma/patologia , Plasmócitos/efeitos dos fármacos
8.
Proc Natl Acad Sci U S A ; 117(25): 14421-14432, 2020 06 23.
Artigo em Inglês | MEDLINE | ID: mdl-32522871

RESUMO

Epstein-Barr virus (EBV) is a B cell transforming virus that causes B cell malignancies under conditions of immune suppression. EBV orchestrates B cell transformation through its latent membrane proteins (LMPs) and Epstein-Barr nuclear antigens (EBNAs). We here identify secondary mutations in mouse B cell lymphomas induced by LMP1, to predict and identify key functions of other EBV genes during transformation. We find aberrant activation of early B cell factor 1 (EBF1) to promote transformation of LMP1-expressing B cells by inhibiting their differentiation to plasma cells. EBV EBNA3A phenocopies EBF1 activities in LMP1-expressing B cells, promoting transformation while inhibiting differentiation. In cells expressing LMP1 together with LMP2A, EBNA3A only promotes lymphomagenesis when the EBNA2 target Myc is also overexpressed. Collectively, our data support a model where proproliferative activities of LMP1, LMP2A, and EBNA2 in combination with EBNA3A-mediated inhibition of terminal plasma cell differentiation critically control EBV-mediated B cell lymphomagenesis.


Assuntos
Transformação Celular Viral , Infecções por Vírus Epstein-Barr/patologia , Herpesvirus Humano 4/patogenicidade , Linfoma de Células B/patologia , Plasmócitos/patologia , Animais , Diferenciação Celular , Linhagem Celular Tumoral , Proteínas de Ligação a DNA/genética , Modelos Animais de Doenças , Infecções por Vírus Epstein-Barr/virologia , Antígenos Nucleares do Vírus Epstein-Barr/metabolismo , Fibroblastos , Herpesvirus Humano 4/metabolismo , Humanos , Linfoma de Células B/virologia , Camundongos , Camundongos Knockout , Plasmócitos/virologia , Cultura Primária de Células , Transativadores/genética , Transativadores/metabolismo , Proteínas da Matriz Viral/metabolismo , Proteínas Virais/metabolismo
9.
APMIS ; 128(9): 531-538, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32578252

RESUMO

Despite the interest of researchers in IgG4-related disease (IgG4-RD), many questions still remain unanswered regarding the thyroid gland. We aimed to clarify the relationship between IgG4-positive plasma cells and the histopathological pattern in the Hashimoto thyroiditis (HT) in a Finnish series. HT specimens (n = 280) were retrieved from the Department of Pathology, Fimlab Laboratories. After re-evaluation, 82 (29%) cases (72 females and 10 males, 52 ± 17 years) with significant fibrosis were selected. CD38, IgG and IgG4 positivity in plasma cells was evaluated by immunohistochemistry. Adjusted IgG4-positive plasma cells per HPF > 20 and IgG4- to IgG-positive plasma cell ratio > 30% were adopted as threshold criteria and related to other morphological features. IgG4-positive HT group included 13 cases (15% from fibrotic HT, 4.6% from all HT, 50 ± 15 years, 11 females) with adjusted HPF count 30 ± 5 (23-40) IgG4-positive cells. IgG4-positivity significantly correlated with the presence of lobulation, oncocytic metaplasia and certain type of fibrosis, fibrosis spread outside the gland, lymphocytes/plasma cells epithelial penetration, the predominance of microfollicles and follicular atrophy in the present study. Despite the persisting uncertainty whether HT is IgG4-RD, HT with IgG4-positive plasma cells is histopathologically distinct entity with some geographic variability.


Assuntos
Doença de Hashimoto/imunologia , Imunoglobulina G/metabolismo , Plasmócitos/metabolismo , ADP-Ribosil Ciclase 1/metabolismo , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Fibrose , Finlândia , Doença de Hashimoto/patologia , Humanos , Inflamação , Contagem de Linfócitos , Masculino , Glicoproteínas de Membrana/metabolismo , Pessoa de Meia-Idade , Plasmócitos/patologia , Glândula Tireoide/imunologia , Glândula Tireoide/patologia , Adulto Jovem
10.
J Clin Pathol ; 73(11): 697-705, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32591351

RESUMO

CONTEXT: Rosai-Dorfman disease is an uncommon histiocytic disorder most frequently presenting as bilateral cervical lymphadenopathy in children and young adults. Extranodal disease occurs in a significant proportion of patients. It has been recently classified as part of the 'R group' of histiocytoses by the Histiocyte Society in 2016. Cutaneous Rosai-Dorfman disease is regarded as a separate disease entity that falls into the 'C group' of histiocytoses according to this classification system. The pathogenesis was previously poorly understood; however, recent evidence demonstrating clonality in a subset of cases raises the possibility of a neoplastic process. A possible association with IgG4-related disease remains controversial. OBJECTIVES: To provide a comprehensive review of Rosai-Dorfman disease, including nodal, extranodal and cutaneous forms, with a particular emphasis on new insights into the possible clonal nature of the disease; to discuss the recently revised classification of the histiocytoses by the Histiocyte Society; and to summarise the findings from the literature regarding the controversial association with IgG4-related disease. DATA SOURCES: This review is based on published peer-reviewed English literature. CONCLUSIONS: Classic Rosai-Dorfman disease, which may be sporadic or familial, is considered a separate entity from cutaneous disease, which is reflected in the revised classification of histiocytoses. An increase in IgG4-positive plasma cells may be seen in Rosai-Dorfman disease. This finding in isolation is of limited significance and should be interpreted with caution. Studies investigating the molecular profile of the disease show that in at least a subset of cases the disease is a clonal process. The classification of Rosai-Dorfman disease is therefore likely to change as our understanding of the aetiopathogenesis evolves.


Assuntos
Histiocitose Sinusal/classificação , Imunoglobulina G/metabolismo , Dermatopatias/classificação , Criança , Feminino , Histiócitos/patologia , Histiocitose Sinusal/patologia , Humanos , Masculino , Plasmócitos/patologia , Pele/patologia , Dermatopatias/patologia , Adulto Jovem
13.
Medicine (Baltimore) ; 99(15): e19728, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32282731

RESUMO

BACKGROUND: The aim of this study was to present several cases of benign and malignant nipple lesions and contribute to diagnosis and differential diagnosis. METHODS: A retrospective study was conducted on 13 patients. All of the patients were evaluated by ultrasonography, and 11 of them had pathological results. We analyzed the clinical and sonographic features. RESULTS: There were 3 malignant lesions, 7 benign lesions, and 3 congenital nipple dysplasia, listed as follows:Malignant lesions (n = 3, 23%): Paget's disease (PD, n = 3, 23%). All of the patients with PD showed unilateral nipple erosion, discharge, and pain. The ultrasound showed abundant blood flow (n = 3, 23%); 2 patients (n = 2, 15%) had microcalcifications.Benign lesions (n = 7, 54%): Adenoma of the nipple (n = 2, 15%). One patient (n = 1, 8%) had nipple erosion and discharge. Two patients (n = 2, 15%) had a palpable nodule in the nipple. The ultrasound of both patients (n = 2, 15%) showed regular-shaped, clear border nodule with abundant blood flow (n = 2, 15%).Leiomyoma of the nipple (n = 1, 8%): This male patient was characterized by unilateral nipple enlargement and pain. The ultrasound showed a regular nodule with absent blood flow.Plasma cell mastitis (n = 2, 15%): Two patients showed unilateral nipple inversion and pain. One patient (n = 1, 8%) showed swollen and redness. The 2 patients showed a lesion in the gland around the nipple present as an irregular shape and unclear boundary hypoechoic mass.Nipple wart (n = 2, 15%): Two patients showed a unilateral soft exogenous neoplasm. Both of the patients showed a hypoechoic wart; the echo was similar to the nipple, the border was clear, and had no blood flow in the wart.Nipple Dysplasia (n = 3, 23%): Accessory nipple (n = 3, 23%). Two patients (n = 2, 15%) had accessory nipples in the subcoastal area, 1 patient (n = 1, 8%) in the areolar. All of the patients' sonographic features were the same as the nipple.The positive predict value (PPV) of the clinical symptoms: Erosion and discharge are both 75% (P < 0.05). The PPV of the US manifestations: irregular shape, indictinct margin, abundant blood flow, microcalcification, thicken skin in diagnosing malignant lesions are 60%,60%,60%,100%,100%, respectively (P < 0.05). CONCLUSIONS: The characteristic sonographic features together with clinical symptoms contribute to the diagnosis of nipple lesions.


Assuntos
Neoplasias da Mama/diagnóstico por imagem , Mamilos/irrigação sanguínea , Mamilos/diagnóstico por imagem , Ultrassonografia/métodos , Adenoma/patologia , Adulto , Neoplasias da Mama/patologia , Calcinose/patologia , Diagnóstico Diferencial , Feminino , Doença da Mama Fibrocística/patologia , Humanos , Leiomioma/patologia , Masculino , Mastite , Pessoa de Meia-Idade , Mamilos/patologia , Doença de Paget Mamária/patologia , Dor/diagnóstico , Plasmócitos/patologia , Valor Preditivo dos Testes , Estudos Retrospectivos , Ultrassonografia/tendências , Verrugas
15.
Presse Med ; 49(1): 104014, 2020 Apr.
Artigo em Francês | MEDLINE | ID: mdl-32234381

RESUMO

IgG4-related disease (IgG4-RD) has been accepted as a distinct entity in various fields. It is being increasingly diagnosed and treated in routine practice. However, difficulties are still associated with the diagnostic process. Serum IgG4 elevations and imaging studies are useful, but not entirely diagnostic for this condition. Therefore, a pathological examination still plays an important role. Three characteristic microscopic changes are dense lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. IgG4 immunostaining reveals many IgG4-positive plasma cells and an IgG4/IgG-positive cell ratio of more than 40%. In addition to the number and ratio of IgG4-positive plasma cells, the diffuse distribution of positive plasma cells needs to be confirmed because IgG4-positive plasma cells may focally aggregate in many other conditions. In small biopsy samples, it is important to recognize not only characteristic findings, but also microscopic changes that are unlikely to occur in IgG4-RD because the identification of the latter findings leads to the exclusion of this condition. Another challenging field regards the diagnosis of long-standing disease. Along with disease progression, inflammatory infiltrate decreases, while storiform fibrosis and obliterative phlebitis are suspected to persistently exist. Therefore, the recognition of the latter two findings will be a diagnostic clue. Given the general suspicion that IgG4-RD has recently been over-diagnosed, precise tissue examinations based on the proposed standards and close clinicopathological correlations are crucial.


Assuntos
Doença Relacionada a Imunoglobulina G4/patologia , Imunoglobulina G/análise , Plasmócitos/patologia , Linfócitos B/patologia , Biópsia , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/patologia , Diagnóstico Diferencial , Fibrose/diagnóstico , Fibrose/patologia , Histiocitose Sinusal/diagnóstico , Histiocitose Sinusal/patologia , Humanos , Imunoglobulina G/sangue , Doença Relacionada a Imunoglobulina G4/diagnóstico , Imuno-Histoquímica , Flebite/diagnóstico , Flebite/patologia , Plasmócitos/imunologia , Linfócitos T/patologia
16.
Virchows Arch ; 477(3): 437-444, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32152665

RESUMO

Hodgkin lymphoma is a B cell neoplasm characterized by Hodgkin and Reed-Sternberg (HRS) cells in an inflammatory background. Classic Hodgkin lymphoma (CHL) accounts for approximately 90% of all cases of HL and four types are recognized in the World Health Organization (WHO) classification: lymphocyte-rich, nodular sclerosis, mixed cellularity, and lymphocyte depleted. Castleman disease (CD) is a designation used for a heterogeneous group of diseases that involve lymph nodes. Histologically, there are hyaline vascular and plasma cell variants, the latter including human herpes virus 8 (HHV8)-positive and HHV8-negative subsets. In this study, we describe three men, 45-57 years of age, one HIV-positive, who had coexistent CHL and CD. All patients had the interfollicular variant of CHL and HHV8-negative plasma cell variant CD. Immunohistochemical analysis supported the diagnosis of CHL; the HRS cells were positive for CD15, CD30, and PAX-5 (dim). In two cases, the HRS cells and the plasma cells of CD expressed interleukin-6 (IL-6). Our review of the literature identified 34 cases of coexistent CHL and CD reported previously. In aggregate, about two-thirds of all cases of CHL have been the interfollicular variant and around 90% of CD cases were plasma cell variant, HHV8-negative in the subset of cases tested. We suggest that interfollicular variant CHL and plasma cell variant CD may be a distinct entity with a common pathogenesis, possibly related to IL-6 dysregulation. The few cases in the literature describing other forms of CHL and hyaline vascular variant CD are different from the entity reported here, with a different pathogenesis, likely similar to focal Castleman-like changes that have been described in association with various types of non-Hodgkin lymphoma.


Assuntos
Hiperplasia do Linfonodo Gigante/patologia , Doença de Hodgkin/patologia , Hiperplasia do Linfonodo Gigante/diagnóstico , Diagnóstico Diferencial , Doença de Hodgkin/diagnóstico , Humanos , Imuno-Histoquímica , Imunofenotipagem , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Plasmócitos/patologia , Células de Reed-Sternberg/metabolismo , Células de Reed-Sternberg/patologia
17.
Am J Surg Pathol ; 44(6): 838-848, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32118627

RESUMO

In patients with multiple myeloma, plasmablastic transformation in the bone marrow is rare and associated with poor outcomes. The significance of discordant extramedullary plasmablastic transformation in patients with small, mature clonal plasma cells in the bone marrow has not been well studied. Here, we report the clinicopathologic, cytogenetic, and molecular features of 10 such patients (male/female: 6/4, median age: 65 y, range: 48 to 76 y) with an established diagnosis of multiple myeloma in the bone marrow composed of small, mature plasma cells in parallel with a concurrent or subsequent extramedullary plasmablastic transformation. Eight patients with available survival data showed an overall aggressive clinical course with a median survival of 4.5 months after the diagnosis of extramedullary plasmablastic transformation, despite aggressive treatment and even in patients with low-level bone marrow involvement. Pathologically, the extramedullary plasmablastic myeloma were clonally related to the corresponding bone marrow plasma cells, showed high levels of CMYC and/or P53 expression with a high Ki-67 proliferation index by immunohistochemistry and harbored more complex genomic aberrations including frequent mutations in the RAS pathway and MYC rearrangements compared with their bone marrow counterparts. In summary, although genetic and immunohistochemical studies were not uniformly performed on all cases due to the retrospective nature of this study, our data suggest that discordant extramedullary plasmablastic transformation of multiple myeloma has an aggressive clinical course and is characterized by frequent mutations in the RAS pathway and more complex genomic abnormalities.


Assuntos
Mieloma Múltiplo/genética , Mieloma Múltiplo/patologia , Plasmócitos/patologia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
18.
Intern Med ; 59(5): 711-714, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32115519

RESUMO

We herein report a 65-year-old man with elevated serum IgG4 levels, enlarged thyroid, and renal dysfunction, mimicking IgG4-related disease (IgG4-RD). The definitive diagnosis of IgG4-RD was not established because a tissue biopsy revealed no IgG4-positive cell infiltration or fibrosis. The presence of an M peak in the ß fraction, Bence Jones protein in urine, and progressive anemia suggested multiple myeloma (MM). The κ/λ ratio was >100, tumor plasma cells were present at >20% in bone marrow, and immunostaining revealed IgG4-positive plasma cells; therefore, he was diagnosed with IgG4-type MM. Patients with elevated IgG4 levels with no significant mass lesions should undergo systemic examinations to exclude malignancy.


Assuntos
Doença Relacionada a Imunoglobulina G4/diagnóstico , Mieloma Múltiplo/diagnóstico , Glândula Tireoide/fisiologia , Idoso , Biópsia , Diagnóstico Diferencial , Humanos , Hipertrofia/sangue , Hipertrofia/diagnóstico , Imunoglobulina G/metabolismo , Doença Relacionada a Imunoglobulina G4/sangue , Masculino , Pessoa de Meia-Idade , Plasmócitos/patologia
19.
Am J Surg Pathol ; 44(5): 617-625, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32187043

RESUMO

Common variable immunodeficiency (CVID) has a heterogenous clinical presentation and can be challenging to diagnose. Distinct histologic changes have been linked with CVID in several organ systems, which can help identify the correct diagnosis. In this study we review a cohort of hepatic CVID biopsies, to better define the spectrum of histologic and biochemical alterations. We reviewed 26 liver biopsies from 24 patients with CVID, obtained at 4 institutions between 2010 and 2019. Histologic slides were examined, and pathologic, biochemical, and clinical features were recorded. A control cohort of 21 patients with nodular regenerative hyperplasia (NRH) but lacking CVID was also examined. Liver function tests were frequently abnormal, especially alkaline phosphatase (median: 193 IU/L) and aspartate transaminase (median: 56 U/L), elevated in 23 and 17 of 25 biopsies, respectively. Fifteen patients had CVID involvement of other organs. Histologic features of primary biliary cholangitis were present in 2 patients, with florid duct lesions and prominent bile duct injury, in association with positive antimitochondrial antibodies. Among the other 24 biopsies, mild to moderate portal and lobular inflammation were present in 18 and 17 of 24 biopsies, respectively. Overall, 22 of 24 biopsies showed NRH-like changes. Plasma cell were absent. A distinct pattern of pericellular fibrosis was present in 23 of 26 biopsies overall. Involvement ranged from focal centrizonal fibrosis to bridging fibrosis and was accompanied by increased intrasinusoidal lymphocytes in 13 of 24 biopsies. Pericellular fibrosis was identified in 1 of 21 biopsies in the control cohort. Additional findings included granulomatous inflammation or nonhepatocellular foreign body-type multinucleate giant cells, identified in 4 biopsies. Three of 6 examined biopsies also demonstrated focal hepatocellular copper deposition. Hepatic disease in CVID is often associated with elevated alkaline phosphatase and aspartate transaminase and is characterized histologically by the mild nonspecific portal and lobular hepatitis, absence of plasma cells, NRH-like changes, and less commonly, typical histologic features of primary biliary cholangitis. We have also identified a distinctive pattern of delicate pericellular fibrosis that is a helpful clue to the diagnosis of hepatic disease in CVID, especially when accompanied by NRH-like changes.


Assuntos
Imunodeficiência de Variável Comum/complicações , Hepatite/etiologia , Hipertensão Portal/etiologia , Cirrose Hepática/etiologia , Fígado/patologia , Adolescente , Adulto , Idoso , Biomarcadores/sangue , Biópsia , Estudos de Casos e Controles , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/imunologia , Feminino , Hepatite/imunologia , Hepatite/patologia , Humanos , Hipertensão Portal/imunologia , Hipertensão Portal/patologia , Fígado/imunologia , Cirrose Hepática/imunologia , Cirrose Hepática/patologia , Testes de Função Hepática , Masculino , Pessoa de Meia-Idade , Plasmócitos/imunologia , Plasmócitos/patologia , Valor Preditivo dos Testes , Turquia , Estados Unidos , Adulto Jovem
20.
Am J Case Rep ; 21: e920489, 2020 Mar 10.
Artigo em Inglês | MEDLINE | ID: mdl-32152261

RESUMO

BACKGROUND Plasma cell myeloma is a neoplastic plasma cell disorder that usually presents after the fifth decade of life; it is rarely described in younger population especially under 30 years of age. However, there are conflicting reports in the literature about the clinical behavior and overall survival in younger age groups. In approximately 2% of plasma cell myeloma, the morphology of the neoplastic cells is highly pleomorphic, quite anaplastic, and may resemble metastatic tumor cells. While this poses a challenge for morphological interpretation during diagnosis, it has been demonstrated that bone marrow morphologic features (including diffuse sheet growth pattern, immature cell morphology and high mitotic index) significantly correlates with high risk disease. Moreover, there is limited description available about the morphology of the neoplastic cells when correlating the age at presentation with the clinical outcome/biological behavior; hence, the need to report and collect such cases. CASE REPORT We report a case of plasma cell myeloma in a 22-year-old male who presented with non-specific clinical features and posed a diagnostic challenge during clinical, radiological, and laboratory examination. The pathology specimens showed anaplastic morphology. Unfortunately, after diagnosis, despite treatment with brotezomib, his disease had an aggressive clinical course and he passed away 4 months after diagnosis. CONCLUSIONS Although plasma cell myeloma is rare in patients younger than 30 years, it must be considered in the differential diagnosis and investigated properly especially in patients with clinical suspicion of a metastatic non-hematological tumor. The anaplastic variant in a young patient is a diagnostic challenge and is associated with bizarre morphology, aggressive presentation, adverse cytogenetics, resistance to chemotherapy, and poor, short-term, survival.


Assuntos
Células da Medula Óssea/patologia , Neoplasias da Medula Óssea/diagnóstico , Mieloma Múltiplo/diagnóstico , Plasmócitos/patologia , Idade de Início , Diagnóstico Diferencial , Evolução Fatal , Humanos , Cariótipo , Masculino , Adulto Jovem
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