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1.
Article in Spanish | LILACS, COLNAL | ID: biblio-1253868

ABSTRACT

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.


Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.


Subject(s)
Humans , Female , Adult , Lymphoma, Non-Hodgkin/complications , Orbital Neoplasms/complications , Exophthalmos/etiology , Vision, Low/etiology , Lymphoproliferative Disorders/complications , Lymphoma, Non-Hodgkin/surgery , Lymphoma, Non-Hodgkin/diagnosis , Orbital Neoplasms/surgery , Orbital Neoplasms/diagnosis , Exophthalmos/surgery , Exophthalmos/diagnosis , Vision, Low/surgery , Vision, Low/diagnosis , Diagnosis, Differential , Lymphoproliferative Disorders/surgery , Lymphoproliferative Disorders/diagnosis
2.
Autops. Case Rep ; 10(2): e2020147, Apr.-June 2020. graf
Article in English | LILACS | ID: biblio-1131811

ABSTRACT

In adults, B-lymphocytes comprise approximately 10% of circulating lymphocytes. The majority of peripheral B cells are B2 cells ("Mature" B-cells), which function as part of the humoral adaptive immune system. B1 cells ("Innate-like" B cells) are another sub-class of B lymphocytes, considered as innate immune cells with a characteristic phenotype (CD20+, CD27+, CD43+, CD70-, CD11b+, sIgM++, sIgD+) which can be divided into two subtypes; B1a (CD5+): spontaneously produce broadly reactive natural IgM, and B1b (CD5-): can generate T-cell independent, long-lasting IgM. There is very limited data available, indicating a correlation between allogeneic bone marrow transplantation and an increase in B1a cells. Here we present a case of a 17-year-old female with homozygous sickle cell disease (HbSS disease) who underwent hematopoietic stem cell transplant (HSCT). Approximately seven months post-transplant, she was found to have 16% immature mononuclear cells on complete blood count (CBC)-differential report. A follow-up peripheral blood flow cytometry showed that these cells were polyclonal CD5+/CD20+ B-cells, and comprised 66% of lymphocytes. Further workup and follow up failed to reveal any lymphoproliferative disorders. It is important not to misdiagnose these cells as an atypical CD5+ lymphoproliferative disorder. The presence of B1a cells has not been widely reported in non-neoplastic post-stem cell transplanted patients. This case also adds to and expands our knowledge regarding the presence of increased circulating B1a cells after stem cell transplant in a patient with no history of hematological malignancy.


Subject(s)
Humans , Female , Adolescent , Stem Cell Transplantation/adverse effects , Blood Cell Count , Hematopoietic Stem Cells , B-Lymphocytes/cytology , B-Lymphocyte Subsets/pathology , Flow Cytometry , Anemia, Sickle Cell , Lymphoproliferative Disorders/diagnosis
3.
Gastroenterol. latinoam ; 29(supl.1): S21-S23, 2018.
Article in Spanish | LILACS | ID: biblio-1117633

ABSTRACT

The gastrointestinal lymphoid system plays a relevant role. The daily and continuous interaction between gastrointestinal lymphocytes with food and intestinal microbes requires precise functioning. The pathologic spectrum of lymphocyte malfunction results in lymphomas. MALT lymphoma is the most frequently diagnosed lymphoma, but there are other lymphoproliferative diseases such as diffuse large B cell lymphoma, mantle cell lymphoma and T associated lymphoma. The gastroenterologist and the endoscopist need to know these diseases in detail to achieve early diagnosis and treatment.


El sistema linfoide de defensa abdominal tiene un relevante rol en el buen funcionamiento sistémico. La interacción diaria y continua con patógenos alimentarios y microbios comensales intestinales precisa un estrecho funcionamiento. Las alteraciones linfoides clonales favorecen el desarrollo de linfomas de diversos tipos. Si bien, el linfoma asociado a tejido linfoide de mucosas (MALT) es el más conocido en contexto de su asociación con Helicobacter pylori, el tracto gastrointestinal se puede ver afectado por otros linfomas como el linfoma difuso de células grandes B y linfomas indolentes como el linfoma folicular, el linfoma del manto y el linfoma T asociado a enteropatía. El gastroenterólogo y endoscopista precisan conocer en detalle estas entidades para un oportuno diagnóstico y adecuado tratamiento.


Subject(s)
Humans , Gastrointestinal Diseases/diagnosis , Gastrointestinal Diseases/therapy , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/therapy , Helicobacter pylori , Lymphoma, Large B-Cell, Diffuse/diagnosis , Lymphoma, Large B-Cell, Diffuse/therapy , Lymphoma, B-Cell, Marginal Zone/diagnosis , Lymphoma, B-Cell, Marginal Zone/therapy , Lymphoma, Mantle-Cell/therapy , Enteropathy-Associated T-Cell Lymphoma/diagnosis , Enteropathy-Associated T-Cell Lymphoma/therapy
4.
Article in English | WPRIM | ID: wpr-220403

ABSTRACT

Epstein-Barr virus (EBV) is a ubiquitous herpesvirus, affecting >90% of the adult population. EBV targets B-lymphocytes and achieves latent infection in a circular episomal form. Different latency patterns are recognized based on latent gene expression pattern. Latent membrane protein-1 (LMP-1) mimics CD40 and, when self-aggregated, provides a proliferation signal via activating the nuclear factor-kappa B, Janus kinase/signal transducer and activator of transcription, phosphoinositide 3-kinase/Akt (PI3K/Akt) and mitogen-activated protein kinase pathways to promote cellular proliferation. LMP-1 also induces BCL-2 to escape from apoptosis and gives a signal for cell cycle progression by enhancing cyclin-dependent kinase 2 and phosphorylation of retinoblastoma (Rb) protein and by inhibiting p16 and p27. LMP-2A blocks the surface immunoglobulin-mediated lytic cycle reactivation. It also activates the Ras/PI3K/Akt pathway and induces Bcl-xL expression to promote B-cell survival. Recent studies have shown that ebv-microRNAs can provide extra signals for cellular proliferation, cell cycle progression and anti-apoptosis. EBV is well known for association with various types of B-lymphocyte, T-lymphocyte, epithelial cell and mesenchymal cell neoplasms. B-cell lymphoproliferative disorders encompass a broad spectrum of diseases, from benign to malignant. Here we review our current understanding of EBV-induced lymphomagenesis and focus on biology, diagnosis and management of EBV-associated B-cell lymphoproliferative disorders.


Subject(s)
B-Lymphocytes/pathology , Diagnosis, Differential , Disease Management , Epstein-Barr Virus Infections/complications , Herpesvirus 4, Human/physiology , Humans , Lymphoproliferative Disorders/diagnosis
5.
Article in English | WPRIM | ID: wpr-61564

ABSTRACT

Epstein Barr virus (EBV)-associated lymphoproliferative diseases (LPDs) express all EBV latent antigens (type III latency) in immunodeficient patients and limited antigens (type I and II latencies) in immunocompetent patients. Post-transplantation lymphoproliferative disease (PTLD) is the prototype exhibiting type III EBV latency. Although EBV antigens are highly immunogenic, PTLD cell proliferation remains unchecked because of the underlying immunosuppression. The restoration of anti-EBV immunity by EBV-specific T cells of either autologous or allogeneic origin has been shown to be safe and effective in PTLDs. Cellular therapy can be improved by establishing a bank of human leukocyte antigen-characterized allogeneic EBV-specific T cells. In EBV+ LPDs exhibiting type I and II latencies, the use of EBV-specific T cells is more limited, although the safety and efficacy of this therapy have also been demonstrated. The therapeutic role of EBV-specific T cells in EBV+ LPDs needs to be critically reappraised with the advent of monoclonal antibodies and other targeted therapy. Another strategy involves the use of epigenetic approaches to induce EBV to undergo lytic proliferation when expression of the viral thymidine kinase renders host tumor cells susceptible to the cytotoxic effects of ganciclovir. Finally, the prophylactic use of antiviral drugs to prevent EBV reactivation may decrease the occurrence of EBV+ LPDs.


Subject(s)
Antiviral Agents/therapeutic use , Cell- and Tissue-Based Therapy , DNA Methylation , Epstein-Barr Virus Infections/complications , Genome, Viral , Hematopoietic Stem Cell Transplantation , Herpesvirus 4, Human/physiology , Humans , Immunotherapy, Adoptive , Lymphoproliferative Disorders/diagnosis , Organ Transplantation/adverse effects , T-Lymphocytes/immunology , Transplantation, Homologous , Virus Latency
6.
Article in English | WPRIM | ID: wpr-61562

ABSTRACT

Epstein-Barr virus, a ubiquitous human herpesvirus, can induce both lytic and latent infections that result in a variety of human diseases, including lymphoproliferative disorders. The oncogenic potential of Epstein-Barr virus is related to its ability to infect and transform B lymphocytes into continuously proliferating lymphoblastoid cells. However, Epstein-Barr virus has also been implicated in the development of T/natural killer cell lymphoproliferative diseases. Epstein-Barr virus encodes a series of products that mimic several growth, transcription and anti-apoptotic factors, thus usurping control of pathways that regulate diverse homeostatic cellular functions and the microenvironment. However, the exact mechanism by which Epstein-Barr virus promotes oncogenesis and inflammatory lesion development remains unclear. Epstein-Barr virus-associated T/natural killer cell lymphoproliferative diseases often have overlapping clinical symptoms as well as histologic and immunophenotypic features because both lymphoid cell types derive from a common precursor. Accurate classification of Epstein-Barr virus-associated T/natural killer cell lymphoproliferative diseases is a prerequisite for appropriate clinical management. Currently, the treatment of most T/natural killer cell lymphoproliferative diseases is less than satisfactory. Novel and targeted therapies are strongly required to satisfy clinical demands. This review describes our current knowledge of the genetics, oncogenesis, biology, diagnosis and treatment of Epstein-Barr virus-associated T/natural killer cell lymphoproliferative diseases.


Subject(s)
Cell Transformation, Viral , Epstein-Barr Virus Infections/complications , Herpesvirus 4, Human/physiology , Humans , Killer Cells, Natural/immunology , Lymphoproliferative Disorders/diagnosis , T-Lymphocytes/immunology
7.
Rev. Soc. Bras. Clín. Méd ; 12(2)abr.-jun. 2014. ilus
Article in Portuguese | LILACS | ID: lil-712266

ABSTRACT

Os pseudolinfomas cutâneos representam um grupo heterogêneo de reações linfoproliferativas benignos que podem simular clinica e histologicamente linfomas cutâneos. O objetivo deste estudo foi relatar a apresentação anatomoclínica de um caso de pseudolinfoma cutâneo para o aperfeiçoamento do seu diagnóstico diferencial com linfomas cutâneos. Paciente do gênero masculino, 66 anos procurou atendimento médico relatando história de nódulos em região superior do dorso há seis anos. Ao exame físico foram observados sete nódulos com aproximadamente 0,5 cm de diâmetro e sem sinais de linfonodomegalias. A biópsia de pele mostrava um denso infiltrado de linfócitos e histiócitos na derme. A análise imuno-histoquímica revelou uma população mista de linfócitos B (CD 20) e linfócitos T (CD 3). O diagnóstico diferencial entre as lesões benignas e malignas é a principal prioridade nesses casos. Tanto o pseudolinfoma, quanto o linfoma cutâneo se manifestam como nódulos solitários, pápulas e placas decorrentes de infiltração linfocitária. Para o diagnóstico diferencial, estão indicadas a avaliação imuno-histoquímica e técnicas de biologia molecular...


Cutaneous pseudolymphoma represents heterogeneous groups of benign lymphoproliferative reactions that may simulate clinically and histologically cutaneous lymphomas. The aim of this study was to report a case of cutaneous pseudolymphoma. Male patient, 66-year-old, came to us reporting a 6-year history of nodules on the upper back. On physical examination we noticed 7 nodules with 0.5 cm of diameter and no sign of adenopathy. The skin biopsy showed a dense infiltrate of lymphocytes and histiocytes in the dermis. The immunohistochemical analysis revealed a mixed population of B lymphocytes (CD 20) and T lymphocytes (CD 3). Differential diagnosis between benign and malignant lesions is the main concern in these cases. In some cases, pseudolymphoma manifests as solitary nodules, papules and plaques that are clinically indistinguishable from cutaneous lymphomas. The differentiation process can be further facilitated by immunohistochemical and molecular biological techniques...


Subject(s)
Humans , Male , Aged , Pseudolymphoma/diagnosis , Lymphoproliferative Disorders/diagnosis , Diagnosis, Differential
8.
Braz. j. infect. dis ; 18(3): 271-280, May-June/2014. tab, graf
Article in English | LILACS | ID: lil-712960

ABSTRACT

INTRODUCTION: The quantification of circulating Epstein-Barr virus (EBV) DNA is used to monitor transplant patients as an early marker of Post-Transplant Lymphoproliferative Disorders (PTLD). So far no standardized methodology exists for such determination. OBJECTIVE: Our purpose was to develop and validate a real-time PCR assay to quantify EBV DNA in clinical samples from transplant recipients. METHODS: A duplex real-time PCR method was developed to amplify DNA from EBV and from a human gene. The EBV load was determined in peripheral blood mononuclear cells (PBMC), plasma and oropharyngeal tissue from 64 non-transplanted patients with lymphoid-hypertrophy (Non-Tx), 47 transplant recipients without PTLD (Tx), 54 recipients with PTLD (Tx-PTLD), and 66 blood donors (BD). WinPEPI, version 11.14 software was used for statistical analysis. RESULTS: Analytical validation: the intra and inter-assays variation coefficients were less than 4.5% (EBV-reaction) and 3% (glyceraldehyde 3-phosphate dehydrogenase - GAPDH reaction). Linear ranges comprised 107-10 EBV genome equivalents (gEq) (EBV-reaction) and 500,000-32 human gEq (GAPDH-reaction). The detection limit was 2.9 EBV gEq (EBV-reaction). Both reactions showed specificity. Application to clinical samples: higher levels of EBV were found in oropharyngeal tissue from transplanted groups with and without PTLD, compared to Non-Tx (p < 0.05). The EBV load in PBMC from the groups of BD, Non-Tx, Tx and Tx-PTLD exhibited increasing levels (p < 0.05). In BD, PBMC and plasma, EBV loads were undetectable. CONCLUSIONS: The performance of the assay was suitable for the required clinical application. The assay may be useful to monitor EBV infection in transplant patients, in particular in laboratories from low-income regions that cannot afford to use commercial assays. .


Subject(s)
Adolescent , Adult , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Young Adult , DNA, Viral/blood , Epstein-Barr Virus Infections/diagnosis , Heart Transplantation/adverse effects , /genetics , Kidney Transplantation/adverse effects , Liver Transplantation/adverse effects , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/virology , Real-Time Polymerase Chain Reaction , Sensitivity and Specificity , Viral Load
9.
Yonsei Medical Journal ; : 672-678, 2013.
Article in English | WPRIM | ID: wpr-193935

ABSTRACT

PURPOSE: The purpose of this study was to evaluate the usefulness of convex probe endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for detecting malignancy in parenchymal pulmonary lesions located adjacent to the central airways. MATERIALS AND METHODS: We retrospectively reviewed the diagnostic performance of EBUS-TBNA in consecutive patients with high clinical suspicion of a centrally located primary lung cancer who had undergone EBUS-TBNA at the Samsung Medical Center between May 2009 and June 2011. RESULTS: Thirty-seven patients underwent EBUS-TBNA for intrapulmonary lesions adjacent to the central airways. Seven lesions were located adjacent to the trachea and 30 lesions were located adjacent to the bronchi. Cytologic and histologic samples obtained via EBUS-TBNA were diagnostic in 32 of 37 (86.4%) of patients. The final diagnosis was lung cancer in 30 patients (7 small cell lung cancer, 23 non-small cell lung cancer), lymphoma in one and malignant fibrous histiocytoma in one patient. The diagnostic sensitivity of EBUS-TBNA in detecting malignancy and detecting both malignancy and benignity was 91.4% and 86.5%, respectively. Two patients experienced minor complications. CONCLUSION: EBUS-TBNA is an effective and safe method for tissue diagnosis of parenchymal lesions that lie centrally close to the airways. EBUS-TBNA should be considered the procedure of choice for patients with centrally located lesions without endobronchial involvement.


Subject(s)
Adult , Aged , Aged, 80 and over , Carcinoma, Non-Small-Cell Lung/diagnosis , Diagnosis, Differential , Endoscopic Ultrasound-Guided Fine Needle Aspiration/methods , Female , Histiocytoma, Malignant Fibrous/diagnosis , Humans , Lung Neoplasms/diagnosis , Lymphoma/diagnosis , Lymphoproliferative Disorders/diagnosis , Male , Middle Aged , Retrospective Studies , Sensitivity and Specificity , Small Cell Lung Carcinoma/diagnosis , Tomography, X-Ray Computed
11.
Indian J Pathol Microbiol ; 2011 Apr-Jun 54(2): 330-334
Article in English | IMSEAR | ID: sea-141994

ABSTRACT

Background: Lymphoid malignancies are a heterogeneous group of disorders which may be difficult to differentiate from reactive proliferations even after immunohistochemistry. Polymerase chain reaction (PCR) is believed to be a good adjunct tool for diagnosis. Materials and Methods: We examined 24 cases of neoplastic and non-neoplastic lymphoproliferative lesions in this study and evaluated the PCR as an additional tool in the confirmation of the diagnosis. Two different PCR methodologies were evaluated. Results: In the evaluation of the T-cell PCR, it was seen that the correlation using both the commercial kits and the custom-synthesized primers was highly significant at a P value of <0.05. In the evaluation of the B-cell PCR, it was seen that the correlation using both the commercial kits and the custom-synthesized primers was not significant using either method (P > 0.05). Conclusions: Both the methods showed an excellent concordance for T-cell γ gene rearrangements, However, the same was not seen in the B-cell receptor rearrangements. This may be because of the small sample size or the inability of consensus V primers to recognize complementary DNA sequences in all of the V segments.


Subject(s)
Clone Cells , DNA Primers/genetics , Humans , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/genetics , Lymphoproliferative Disorders/pathology , Pathology, Molecular/methods , Polymerase Chain Reaction/methods , Reagent Kits, Diagnostic , T-Lymphocytes/cytology
12.
Article in English | WPRIM | ID: wpr-100568

ABSTRACT

The World Health Organization (WHO) recently defined systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorders (LPD) of childhood as a life-threatening illness. However, this rare disease has not been extensively studied. Here we report a case of systemic EBV-positive T-cell LPD in a previously healthy middle-aged man with a chief complaint of chronic diarrhea. The initial colon biopsy showed focal infiltration of EBV-positive small lymphocytes without any atypia. However, the disease rapidly progressed and the patient required a total colectomy due to severe gastrointestinal bleeding. Three and half months after admission, the patient died from a complication of disseminated intravascular coagulation. The resected colon showed diffuse infiltration of EBV-positive atypical lymphocytes with ischemic change. Most atypical lymphocytes were CD3+ or CD5+. The monoclonality of EBV was demonstrated by sequence variation analysis of the latent membrane protein 1 (LMP1) gene in the colectomy specimen as well as in the initial biopsy.


Subject(s)
Chronic Disease , Colonoscopy , Diarrhea/diagnosis , Disseminated Intravascular Coagulation/diagnosis , Epstein-Barr Virus Infections/complications , Feces/virology , Gastrointestinal Hemorrhage , Herpesvirus 4, Human/genetics , Humans , Lymphoproliferative Disorders/diagnosis , Male , Middle Aged , RNA, Viral/analysis , T-Lymphocytes/immunology
13.
Med. infant ; 16(2): 134-138, jun. 2009. tab, graf
Article in Spanish | LILACS | ID: lil-538115

ABSTRACT

La enfermedad linfoproliferativa post trasplante (PTLD) es la neoplasia más frecuente observada en receptores pediátricos de órganos sólidos. Analizamos 883 pacientes trasplantados: 547 trasplantes renales (TxR) y 336 trasplantes hepáticos (TxH). 44 de ellos desarrollaron un PTLD luego de estos procedimientos. La incidencia en TxR fue del 4.02 por ciento (22 de 547) y en TxH de 5.75 por ciento (22 de 336). El tiempo medio de la aparición del PTLD fue de 37.47 más menos 35 meses. Localización: cavun 50 por ciento, adenopatías cervicales 14.4 por ciento, masa abdominal 11.4 por ciento, adenopatías axilares e inguinales 9 por ciento, compromiso del SNC 4.5 por ciento, piso de la boca 4.5 por ciento , higado ortótopico 2.3 por ciento, páncreas 2.3 por ciento, injerto renal 2.3 por ciento. Los diagnósticos histopatológicos obtenidos fueron: hiperplasia plasmocitica 52.3 por ciento, lesiones polimorfas 20.5 por ciento, y lesiones monomorgas (linforma B o Linfoma de células grandes) 27.2 por ciento de los pacientes. El tratamiento indicado incluyó reducción de la inmunosupresión de mantenimiento en todos los pacientes, excéresis en 22 pacientes, anticuerpos antCD20 en 17 de ellos y quimioterapia (régimen de CHOP) en 6 pacientes. La sobrevida fue del 79 por ciento (IC 64 por ciento-88 por ciento) a 5 años de seguimiento. Conclusión: En nuestra serie la incidencia de PTLAD es similar a la de otros autores. La respuesta al tratamiento con disminución de la inmunosupresón, empleo de monoclonales, ha sido satisfactoria. El seguimiento cercano, el alto índice de sospecha para el diagnóstico precóz y la individualización de la inmunosupresión para cada paciente, fueron conductas eficaces.


Subject(s)
Infant , Child, Preschool , Liver Transplantation , Kidney Transplantation , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/therapy , Statistical Analysis , Epidemiology, Descriptive , Retrospective Studies , Observational Studies as Topic
15.
Article in English | WPRIM | ID: wpr-113720

ABSTRACT

This study is to identify the spectrum of Epstein-Barr virus (EBV)-positive lymphoproliferative diseases (LPD) and relationships between these diseases in Korea. The EBV status and clinicopathology of 764 patients, including acute EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH), chronic active EBV (CAEBV) infections, B-LPD arising in chronic latent EBV infection, T & natural killer (NK) cell non-Hodgkin's lymphomas (NHL), B-NHLs, and Hodgkin's lymphomas (HD), were analyzed. T or NK cell NHLs were the most common forms of EBV-positive NHLs (107/167, 64%); among these, nasal-type NK/T cell lymphomas were the most common (89/107, 83%). According to the age, Burkitt's lymphoma was the most common in early childhood; in teenagers, chronic (active) EBV infection-associated LPD was the most common type. The incidence of NK/T cell lymphoma began to increase from the twenties and formed the major type of EBV-associated tumor throughout life. Diffuse large B cell lymphoma formed the major type in the sixties and seventies. In conclusion, primary infections in early childhood are complicated by the development of CAEBV infections that are main predisposing factors for EBV-associated T or NK cell malignancies in young adults. In old patients, decreased immunity associated with old age and environmental cofactors may provoke the development of peripheral T cell lymphoma, unspecified, and diffuse large B cell lymphoma.


Subject(s)
Adolescent , Adult , Age Factors , Aged , Aged, 80 and over , Child , Child, Preschool , Epstein-Barr Virus Infections/diagnosis , Herpesvirus 4, Human/metabolism , Humans , Infant , Korea , Lymphoproliferative Disorders/diagnosis , Middle Aged , Treatment Outcome
16.
Rev. chil. cir ; 59(6): 463-466, dic. 2007. ilus
Article in Spanish | LILACS | ID: lil-482842

ABSTRACT

El 70 por ciento de los casos de la Enfermedad de Castleman (EC) se presentan como masas ganglionares que afectan el mediastino. El compromiso del tubo digestivo de la EC es muy raro. Se presenta un caso excepcional de EC ubicado en el espesor de la pared rectal que fue sometida a una resección anterior baja con la sospecha clínica de una endometriosis. El estudio histopatológico demostró una EC del tipo hialino-vascular y la paciente está asintomática luego de 2 años de seguimiento. Se reconoce una variedad hialino-vascular de la EC, que es generalmente asintomática y que se presenta como lesiones solitarias. La variedad de células plasmáticas de la EC, en cambio, tiende a ser multicéntrica, con compromiso sistémico y en el 18 por ciento de los casos puede ocurrir la transformación a un linfoma maligno o un sarcoma de Kaposi. El diagnóstico preoperatorio de la EC localizada en el abdomen o en la pelvis es muy difícil. Como el estudio por imágenes no permite descartar un tumor maligno, habitualmente se requiere una exploración quirúrgica con el fin de realizar una resección completa o, al menos, una biopsia incisional diagnóstica. Las lesiones solitarias que son resecadas en forma completa tienen un pronóstico excelente y no hay casos de recidiva publicados. La variedad multicéntrica de la EC, en cambio, tiene un pronóstico reservado y requiere de un manejo multidisciplinario.


Catleman disease usually presents as mediastinal lymph nodes and gastrointestinal involvement is exceptional. We report a 49 years old female with a vascularized mass located in the sigmoid colon. The mass was excised and the pathological study showed a Castleman disease, hyaline vascular variety. The patient is free of symptoms after two years of follow up. This variety of Castleman disease usually presents as solitary asymptomatic masses and if it is completely excised, the prognosis is good.


Subject(s)
Humans , Female , Middle Aged , Castleman Disease/surgery , Castleman Disease/diagnosis , Rectal Neoplasms/surgery , Rectal Neoplasms/diagnosis , Treatment Outcome , Lymphoproliferative Disorders/surgery , Lymphoproliferative Disorders/diagnosis
17.
J. bras. pneumol ; 33(6): 625-634, nov.-dez. 2007. ilus, tab
Article in English, Portuguese | LILACS | ID: lil-471283

ABSTRACT

OBJETIVO: Determinar a utilidade, na prática rotineira, da análise da clonalidade dos linfócitos T e B nos tecidos pulmonares por reação em cadeia da polimerase no diagnóstico das doenças linfoproliferativas pulmonares. MÉTODOS: Avaliaram-se, mediante análise imunohistoquímica e rearranjo molecular dos genes, 8 casos de pneumonia intersticial linfocítica (PIL) e 7 casos de doenças linfoproliferativas pulmonares. RESULTADOS: Todos os 8 casos de PIL expressaram imunocoloração moderada a forte para CD3, em contraste com apenas 2 casos de linfoma e 1 caso de pseudolinfoma. Rearranjo gênico foi detectado em 4 de 8 casos de PIL, o que mudou o diagnóstico de PIL para linfoma, indicando, assim, a importância da detecção de rearranjo gênico em casos de PIL. Nesta situação, rearranjo gênico usando-se os pares de primers VH/JH e Vgama11/Jgama12 foi detectado em 3 e 1 casos de PIL, respectivamente, e não foram detectadas anormalidades gênicas usando-se as pares Dbeta1/Jbeta2 e Vgama101/Jgama12. Uma associação positiva foi detectada entre a intensidade de imunoexpressão CD20 e CD68 e rearranjo gênico usando-se o par de primers VH/JH. Antes do rearranjo gênico, 4 pacientes com PIL morreram rapidamente, enquanto que, após o rearranjo gênico, apenas 1 paciente com PIL morreu. CONCLUSÕES: A detecção de células B e T monoclonais por imunofenotipagem e reação em cadeia da polimerase mostrou impacto no diagnóstico de linfomas pulmonares em pacientes previamente diagnosticados com PIL. Portanto, imunofenotipagem e reação em cadeia da polimerase devem ser incluídas como métodos de 'padrão ouro' na rotina diagnóstica.


OBJECTIVE: To determine the usefulness, in routine practice, of using polymerase chain reaction to analyze B and T lymphocyte clonality in pulmonary tissue as a tool for the diagnosis of pulmonary lymphoproliferative disorders. METHODS: Immunohistochemistry and molecular gene rearrangement analysis were performed in order to assess 8 cases of lymphoid interstitial pneumonia (LIP) and 7 cases of pulmonary lymphoproliferative disorders. RESULTS: All 8 cases of LIP presented moderate to strong immunostaining for CD3, compared with only 2 cases of lymphoma and 1 case of pseudolymphoma (p = 0.02). Gene rearrangement was detected in 4 of the 8 cases, which changed the diagnosis from LIP to lymphoma, showing the importance of gene rearrangement detection in cases of LIP. In this situation, gene rearrangement using the VH/JH and Vgamma11/Jgamma12 primer pairs was detected in 3 cases and 1 case, respectively, and no gene abnormalities were found using the Dbeta1/Jbeta2 and Vgamma101/Jgamma12 primer pairs in any of the cases. A significant positive association was found between the intensity of CD20 and CD68 expression and gene rearrangement using the VH/JH primer pair. Prior to the gene rearrangement, 4 patients with LIP died quickly, whereas only one patient with LIP died after the gene rearrangement. CONCLUSIONS: Detection of monoclonal B and T cells by immunophenotyping and polymerase chain reaction had an impact on the diagnosis of pulmonary lymphomas in patients previously diagnosed with LIP. Therefore, immunophenotyping and polymerase chain reaction should be used as 'gold standard' techniques in routine practice.


Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Child, Preschool , Female , Humans , Male , Middle Aged , Gene Rearrangement , Immunophenotyping , Lung Diseases, Interstitial/immunology , Lung Neoplasms/immunology , Lymphoma/immunology , Antigens, CD/analysis , Case-Control Studies , Diagnosis, Differential , DNA Primers , Feasibility Studies , Gene Rearrangement, B-Lymphocyte, Heavy Chain/genetics , Gene Rearrangement, B-Lymphocyte, Heavy Chain/immunology , Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor/genetics , Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor/immunology , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/genetics , Lung Neoplasms/diagnosis , Lung Neoplasms/genetics , Lymphoid Tissue/pathology , Lymphoma/diagnosis , Lymphoma/genetics , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/genetics , Lymphoproliferative Disorders/immunology , Polymerase Chain Reaction , Pseudolymphoma/diagnosis , Pseudolymphoma/genetics , Pseudolymphoma/immunology , Retrospective Studies
18.
Rev. chil. tecnol. méd ; 26(2): 1287-1296, dic. 2006. ilus, tab
Article in Spanish | LILACS | ID: lil-464946

ABSTRACT

Sin duda para quienes se desempeñan en investigación científica y en histopatología, el diagnóstico y clasificación de linfomas y leucemias representa el campo mas desafiante dentro de la anatomía patológica, ya que es en esta área donde la inmunidad y los procesos neoplásicos, así como la creación de nuevos conceptos y técnicas son puestos constantemente a prueba. En el presente trabajo se ha seleccionado al linfoma no hodkin (LNH) como objeto de estudio, debido a que es el de aparición mas frecuente tanto en Chile como en el mundo. El objetivo es lograr un diagnóstico diferencial mediante anticuerpos monoclonales, entre los linfomas no hodkin de células B y los síndromes linfoproliferativos benignos (SLB), patologías que a nivel morfológico resultan –en muchas oportunidades- difíciles de distinguir. Se utilizó un panel básico de inmunohistoquímica, específico para la identificación de cadenas livianas kappa y lambda, se determinó la clonalidad de 10 casos diagnosticados por biopsia diferida como LNH de células B y 10 casos diagnosticados por biopsia diferida como SLB. Los resultados obtenidos han permitido corroborar que una alteración en la proporción esperada kappa/lambda (2:1) es indicador de proceso monoclonal y en consecuencia se está ante la presencia de una neoplasia de células linfoides. Esta información permite efectuar el correcto diagnóstico de una patología, confirmando o descartando la presencia de un proceso neoplásico, lo que implica que un paciente pueda comenzar con prontitud su tratamiento en el sistema público de salud.


Subject(s)
Humans , Antibodies, Monoclonal , Lymphoma, Non-Hodgkin/diagnosis , Immunoglobulin kappa-Chains , B-Lymphocytes/pathology , Lymphoproliferative Disorders/diagnosis
19.
Arq. bras. cardiol ; 87(4): e108-e111, out. 2006. ilus
Article in Portuguese, English | LILACS | ID: lil-438248

ABSTRACT

Terapias de imunossupressão, a que pacientes transplantados devem ser submetidos, os expõe a um alto risco de desenvolver desordens linfoproliferativas pós-transplante (PTLD). Descrevemos o caso de uma criança submetida a transplante cardíaco aos sete meses de idade e que acabou desenvolvendo PTLD, aos nove anos, diagnosticada por meio de retirada de nódulo pulmonar.


Immunosuppressive therapy for transplanted patients exposes them to a high risk of developing posttransplantation lymphoproliferative disorders (PTLD). We report the case of a child undergoing heart transplantation at seven months of age who developed PTLD at nine years of age, diagnosed by resection of a pulmonary nodule.


Subject(s)
Humans , Male , Child , Heart Transplantation/adverse effects , Immunosuppressive Agents/therapeutic use , Lymphoproliferative Disorders/etiology , Cardiomyopathy, Dilated/surgery , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/drug therapy , Lymphoproliferative Disorders/pathology , Treatment Outcome
20.
Indian J Pathol Microbiol ; 2005 Apr; 48(2): 173-6
Article in English | IMSEAR | ID: sea-74905

ABSTRACT

The bone marrow examination is invaluable in the diagnosis of certain haematological and non haematological conditions. The diagnosis of haematological disorders is achieved mainly by the examination of peripheral blood and bone marrow aspirate smears. Although bone marrow histology can provide additional information, for many technical reasons, trephines are not so popular and their diagnostic value is overlooked. Our experiences in the role of trephine biopsies in the diagnosis are presented in this article. All the bone marrow biopsies performed at the St. John's Medical College Hospital over the last 12 years (1990-2002) were reviewed The bone marrow biopsies were grouped into 3 categories for analysis. The trephine biopsy is invaluable in cases where the aspirate fails or is a dry tap as in the case of myelofibrosis, focal marrow involvement as in granulomatous lesions, metastatic tumour and lymphomas.


Subject(s)
Biopsy/statistics & numerical data , Bone Marrow/pathology , Bone Marrow Examination/methods , Hematologic Diseases/diagnosis , Humans , Leukemia/diagnosis , Lymphoproliferative Disorders/diagnosis , Neoplasm Metastasis/drug therapy , Primary Myelofibrosis/diagnosis
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