Clinicopathological features of aggressive B-cell lymphomas including B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell and Burkitt lymphomas: a study of 44 patients from Argentina.

Aggressive B-cell lymphomas incorporate a wide spectrum of lymphomas that pose challenges in diagnosis as well as treatment. We evaluated the clinicopathological features of 44 patients with aggressive B-cell lymphomas which were classified into 3 groups based on the World Health Organization 2008 classification as follows: including 30 cases of diffuse large B-cell lymphoma (DLBCL), 8 cases of Burkitt lymphoma (BL) and 6 cases of B-cell lymphoma, unclassifiable, with features intermediate between Burkitt lymphoma and diffuse large B-cell lymphoma (BCLU). Male predominance was observed in BL and BCLU groups and the mean age varied from 29 years in BL, 61 years in DLBCL and 70 years in BCLU. Patients with BCLU presented at more advanced stages and had a higher international prognostic index. By immunohistochemistry, they shared characteristics of both BL (including more frequent expression of SOX11) and DLBCL. FISH analyses showed three cases with more than one rearrangement: one MYC/BCL2 and two BCL2/BCL6, in addition to which one case with BCL2/IGH translocation and another with MYC rearrangement were also detected. The mean follow-up survival time of BCLU was 6.6 months, which was significantly shorter in comparison to DLBCL (31 months) and BL (30 months), respectively. The importance of recognizing this BCLU group relies on its different clinical course, poor prognosis and shorter survival than DLBCL and BL. An accurate diagnosis is critical for risk stratification and to improve therapeutic approaches and outcomes.

The contribution of HGAL/GCET2 in immunohistological algorithms: a comparative study in 424 cases of nodal diffuse large B-cell lymphoma.

Diffuse large B-cell lymphoma can be subclassified into at least two molecular subgroups by gene expression profiling: germinal center B-cell like and activated B-cell like diffuse large B-cell lymphoma. Several immunohistological algorithms have been proposed as surrogates to gene expression profiling at the level of protein expression, but their reliability has been an issue of controversy. Furthermore, the proportion of misclassified cases of germinal center B-cell subgroup by immunohistochemistry, in all reported algorithms, is higher compared with germinal center B-cell cases defined by gene expression profiling. We analyzed 424 cases of nodal diffuse large B-cell lymphoma with the panel of markers included in the three previously described algorithms: Hans, Choi, and Tally. To test whether the sensitivity of detecting germinal center B-cell cases could be improved, the germinal center B-cell marker HGAL/GCET2 was also added to all three algorithms. Our results show that the inclusion of HGAL/GCET2 significantly increased the detection of germinal center B-cell cases in all three algorithms (P<0.001). The proportions of germinal center B-cell cases in the original algorithms were 27%, 34%, and 19% for Hans, Choi, and Tally, respectively. In the modified algorithms, with the inclusion of HGAL/GCET2, the frequencies of germinal center B-cell cases were increased to 38%, 48%, and 35%, respectively. Therefore, HGAL/GCET2 protein expression may function as a marker for germinal center B-cell type diffuse large B-cell lymphoma. Consideration should be given to the inclusion of HGAL/GCET2 analysis in algorithms to better predict the cell of origin. These findings bear further validation, from comparison to gene expression profiles and from clinical/therapeutic data.

The spectrum of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a description of 10 cases.

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma, is a diagnostic provisional category in the World Health Organization (WHO) 2008 classification of lymphomas. This category was designed as a measure to accommodate borderline cases that cannot be reliably classified into a single distinct disease entity after all available morphological, immunophenotypical and molecular studies have been performed. Typically, these cases share features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma, or include characteristics of both lymphomas. The rarity of such cases poses a tremendous challenge to both pathologists and oncologists because its differential diagnosis has direct implications for management strategies. In this study, we present 10 cases of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma and have organized the criteria described by the WHO into four patterns along with detailed clinical, morphological and immunophenotypic characterization and outcome data. Our findings show a male preponderance, median age of 37 years and a mediastinal presentation in 80% of cases. All cases expressed at least two markers associated with B-cell lineage and good response to combination chemotherapy currently employed for non-Hodgkin lymphomas.

Clinicopathologic and molecular features of 122 Brazilian cases of nodal and extranodal NK/T-cell lymphoma, nasal type, with EBV subtyping analysis.

Extranodal natural killer/T-cell lymphoma, nasal type (NK/TCL) is more prevalent in Asia and in some areas of South and Central America, but it is rarely seen in the United States and Europe. In this study, a series of 122 cases of NK/TCL from Brazil was analyzed with respect to clinicopathologic features. Clinical characteristics and geographic distribution were evaluated in 97 cases of nasal/nasopharyngeal region and 23 cases in extranasal sites including 6 nodal cases. Clinical staging and follow-up information was available in a subset of 21 patients. All cases harbored Epstein-Barr virus (EBV), 95% and 85% expressed cytoplasmic CD3 and CD56, respectively, and all cases were positive for at least 1 marker for cytotoxic granules. The global distribution of EBV subtypes showed predominance of strain subtype A, 89%, and subtype B, 11%. No dual infections were detected. TCR-γ TCR-gene rearrangement was observed in 7 cases; all of them extranodal. Three of TCR-γ(+) cases showed EBV subtype A. Two TCR-γ(+)/CD56(+) cases showed EBV subtype B. Geographic distribution of NK/TCL showed higher frequency in the southeast and northeast regions of Brazil. Striking differences among geographic regions were seen with the vast majority of EBV subtype B (86%) occurring in the south and southeast regions.

Diffuse large B-cell lymphoma involving the central nervous system.

Lymphomas involving the central nervous system are recognized increasingly in immunocompetent as well as immunosuppressed individuals, and the majority of the cases are diffuse large B-cell lymphoma (DLBCL). The aim of this study was to compare the immunophenotype, clinicopathological features, and association with Epstein-Barr virus (EBV) of DLBCL of the central nervous system (CNS) in 3 different clinical situations: primary, in immunocompetent patients; "primary," in immunosuppressed patients; and in patients with secondary involvement by systemic lymphoma. The authors reviewed the clinicopathological features, morphology, immunophenotype (according to germinal-center B-cell-like and nongerminal B-cell-like subtypes), and association with EBV in 36 cases of DLBCL of the CNS, including 25 primary cases, 5 associated with immunosuppression, and 6 cases with secondary involvement. Survival was evaluated in 15 cases of primary CNS lymphomas. Of the 36 patients, 19 were male and 18 female. Only 2 cases of lymphomas were EBV-positive; both occurred in immunosuppressed patients. Separation into germinal-center and non-germinal center subtypes by an immunohistochemistry panel showed that 68% of primary, 80% of secondary, and 83% of the cases associated with immunosuppression were of non-germinal-center subtype, respectively. Patients with non-germinal-center immunophenotype showed significantly worse survival than those with CNS lymphomas of the germinal-center subtype.

Hodgkin's lymphoma presenting as dominant gastric lesion in immunocompetent patients: report of 5 cases with EBV analysis.

Primary Hodgkin's lymphoma (HL) of the stomach is an extremely rare entity. Most cases of gastric involvement by HL are observed in the setting of disseminated disease. The nonspecific nature of the symptoms and endoscopic findings, which include a large malignant-looking ulcer and mass or wall thickening, together with the considerable histological overlap between HLs and some non-HLs or undifferentiated carcinoma, make the surgical resection diagnosis extremely difficult. An accurate diagnosis is important as treatment and outcome differ significantly for these neoplasms. In small endoscopic gastric biopsies and even in postoperative specimens, the precise histological diagnosis of HL is particularly challenging. Here, the authors report 5 cases of 2 women and 3 men aged 22 to 68, with gastric involvement by classic HLs-3 primary gastric HLs and 2 as part of widespread disease. All 5 patients presented with digestive symptoms. At endoscopy, the lesions presented as ulcerated and elevated lesions, with or without mucosal thickening. Four patients were misdiagnosed in the preoperative biopsy or in the gastrectomy specimen. Association with Epstein-Barr virus (EBV) was detected in 4 cases, with a predominance of subtype A EBV. These cases illustrate the significant difficulties, both clinical and pathological, in achieving the diagnosis of HL involving the stomach in immunocompetent patients.

Primary central nervous system peripheral T-cell lymphoma in a child.

A 10-year-old Caucasian boy was admitted to the hospital with a 3-month history of headache, vomiting, ataxia, and right amaurosis. A magnetic resonance imaging (MRI) showed a solid, expansive, parasagittal mass in the right parietal hemisphere that extended sagitally to include the optical chiasm. The lesion was considered unresectable. Histology and immunophenotyping of biopsy tissue revealed characteristics of peripheral T-cell lymphoma. No other anatomical region, including bone marrow, was compromised. Primary T-cell lymphomas of the central nervous system are rare, especially in childhood. Here, we describe the rapidly deteriorating and fatal clinical course of a boy with a primary T-cell lymphoma in the central nervous system.

T-cell leukemia 1 expression in nodal Epstein-Barr virus-negative diffuse large B-cell lymphoma and primary mediastinal B-cell lymphoma.

The physiologic expression of the product of the proto-oncogene TCL1 (T-cell leukemia 1) is primarily restricted to early embryonic cells. In nonneoplastic B cells, the expression of TCL1 is determined by the differentiation step with silencing at the germinal center stage. TCL1 protein is overexpressed in a wide variety of human diseases. It has been shown that TCL1 is a powerful B-cell oncogene, which has been implicated in the pathogenesis of various types of mature B-cell lymphomas. There is no comparative information in the literature addressing the expression of TCL1 in pediatric and adult nodal diffuse large B-cell lymphoma or primary mediastinal large B-cell lymphoma. We studied 55 cases of adult and pediatric diffuse large B-cell lymphoma and primary mediastinal large B-cell lymphoma to analyze the phenotypic profile of these lymphomas, including TCL1 expression, and its relationship with clinical outcome in different age groups. The cases were analyzed by immunohistochemistry for the expression of TCL1, CD10, BCL-2, BCL-6, and MUM1. We also evaluated c-MYC translocation by fluorescence in situ hybridization. TCL1 was observed in 11 cases, 5 pediatric and 6 adult cases, all but one diffuse large B-cell lymphoma. Pediatric cases showed a significant association between TCL1 expression, high proliferative index, and presence of c-MYC translocation. TCL1 positivity was predominantly found in germinal center phenotype diffuse large B-cell lymphoma. Overall survival was worse in adult TCL1-positive cases than pediatric ones. Primary mediastinal large B-cell lymphomas infrequently expressed TCL1 in both age groups.

MUM1/IRF4: A Review.

MUM1/IRF4 protein is a member of the interferon regulatory factor (IRF) family of transcriptional factors initially described as downstream regulators of interferon signaling. The quantity of this factor varies within the hematopoietic system in a lineage and stage-specific way. It is considered to be a key regulator of several steps in lymphoid, myeloid, and dendritic cell differentiation and maturation. MUM1/IRF4 expression is observed in many lymphoid and myeloid malignancies, and may be a promising target for the treatment of some of these neoplasms. We reviewed the literature on MUM1/IRF4, with emphasis on the pathologic aspects of this marker in reactive and malignant hematologic and nonhematologic conditions.

Postsplenectomy sclerosing extramedullary hematopoietic tumor with unexpected good clinical evolution: morphologic, immunohistochemical, and molecular analysis of one case and review of the literature.

Sclerosing extramedullary hematopoietic tumor has been described as a rare manifestation of chronic myeloproliferative neoplasm. The lack of knowledge about this entity has caused it to be mistaken for many types of nonhematopoietic and hematopoietic tumors. We present the case of a 71-year-old lady with a long history of primary myelofibrosis, which developed multiple abdominal sclerosing extramedullary hematopoietic tumors with good clinical evolution. Nonchronic myeloid leukemia myeloproliferative neoplasm included a JAK2 mutation as part of the diagnosis algorithm. Particularly, idiopathic myelofibrosis is related with a JAK2 mutation in 50% of the cases with a pejorative prognosis. The absence of JAK2 demonstrated in the paraffin samples of the tumors may be related to the unusual evolution in this particular case. Morphologically differential diagnoses considered in the evaluation of this entity and in our case included sarcomas mainly liposarcoma, anaplastic carcinoma, and Hodgkin lymphoma.

Pediatric lymphomas in Brazil.

OBJECTIVE: This study provides the clinical pathological characteristics of 1301 cases of pediatric/adolescent lymphomas in patients from different geographic regions of Brazil. METHODS: A retrospective analyses of diagnosed pediatric lymphoma cases in a 10-year period was performed. We believe that it represents the largest series of pediatric lymphomas presented from Brazil. RESULTS: Non-Hodgkin lymphomas represented 68% of the cases, including those of precursor (36%) and mature (64%) cell origin. Mature cell lymphomas comprised 81% of the B-cell phenotype and 19% of the T-cell phenotype. Hodgkin lymphomas represented 32% of all cases, including 87% of the classical type and 13% of nodular lymphocyte predominant type. The geographic distribution showed 38.4% of the cases in the Southeast region, 28.7% in the Northeast, 16.1% in the South, 8.8% in the North, and 8% in the Central-west region. The distribution by age groups was 15-18 years old, 33%; 11-14 years old, 26%; 6-10 years old, 24%; and 6 years old or younger, 17%. Among mature B-cell lymphomas, most of the cases were Burkitt lymphomas (65%), followed by diffuse large B-cell lymphomas (24%). In the mature T-cell group, anaplastic large cell lymphoma, ALK-positive was the most prevalent (57%), followed by peripheral T-cell lymphoma, then not otherwise specified (25%). In the group of classic Hodgkin lymphomas, the main histological subtype was nodular sclerosis (76%). Nodular lymphocyte predominance occurred more frequently than in other series. CONCLUSION: Some of the results found in this study may reflect the heterogeneous socioeconomical status and environmental factors of the Brazilian population in different regions.

BCL6, MUM1, and CD10 expression in mantle cell lymphoma.

Mantle cell lymphoma (MCL) characteristically express CD20, CD5, and cyclin-D1, carries the translocation t(11;14) (q13;q32) and typically has no expression of germinal center cell markers. So-called aberrant phenotypes such as CD5 negative and cyclin-D1-negative MCL have been described. Also few cases with CD10 and/or BCL-6 protein expression have been reported. We analyzed 127 MCL looking for the frequency of aberrant immunophenotype, CD10, BCL-6, and MUM1 expression. All cases were CD20 and cyclin-D1 positive, 96% expressed CD5, and 98% showed the t(11;14). BCL-6 expression was observed in 12% of the cases and MUM1 in 35%. No one case showed CD10 positivity in 30% or more neoplastic cells. Only 3 cases showed 10% to 20% of tumoral cells positive for CD10. MUM1 expression was observed in 67% of the BCL-6 positive cases. Thirty-two percent of the cases showed a MUM1+/BCL-6-/CD10- phenotype and 56% had a triple-negative-pattern. Aberrant phenotype is infrequent but not rare, and does not rule out a diagnosis of MCL in an otherwise typical case.

Pediatric lymphomas in Brazil

OBJECTIVE: This study provides the clinical pathological characteristics of 1301 cases of pediatric/adolescent lymphomas in patients from different geographic regions of Brazil. METHODS: A retrospective analyses of diagnosed pediatric lymphoma cases in a 10-year period was performed. We believe that it represents the largest series of pediatric lymphomas presented from Brazil. RESULTS: Non-Hodgkin lymphomas represented 68 percent of the cases, including those of precursor (36 percent) and mature (64 percent) cell origin. Mature cell lymphomas comprised 81 percent of the B-cell phenotype and 19 percent of the T-cell phenotype. Hodgkin lymphomas represented 32 percent of all cases, including 87 percent of the classical type and 13 percent of nodular lymphocyte predominant type. The geographic distribution showed 38.4 percent of the cases in the Southeast region, 28.7 percent in the Northeast, 16.1 percent in the South, 8.8 percent in the North, and 8 percent in the Central-west region. The distribution by age groups was 15-18 years old, 33 percent; 11-14 years old, 26 percent; 6-10 years old, 24 percent; and 6 years old or younger, 17 percent. Among mature B-cell lymphomas, most of the cases were Burkitt lymphomas (65 percent), followed by diffuse large B-cell lymphomas (24 percent). In the mature T-cell group, anaplastic large cell lymphoma, ALK-positive was the most prevalent (57 percent), followed by peripheral T-cell lymphoma, then not otherwise specified (25 percent). In the group of classic Hodgkin lymphomas, the main histological subtype was nodular sclerosis (76 percent). Nodular lymphocyte predominance occurred more frequently than in other series. CONCLUSION: Some of the results found in this study may reflect the heterogeneous socioeconomical status and environmental factors of the Brazilian population in different regions.

Analysis of human T-cell lymphotropic virus in CD25+ anaplastic large cell lymphoma in children.

Anaplastic large cell lymphoma (ALCL) is recognized as 2 distinct diseases: anaplastic lymphoma kinase (ALK)+ ALCL and ALK- ALCL. ALK+ ALCL occurs in younger patients and has a better prognosis. Human T-cell lymphotropic virus (HTLV-1) is linked to the development of adult T-cell leukemia/lymphoma (ATLL), which frequently expresses CD25. CD25 is significantly expressed in childhood ALCL. In Brazil, HTLV-1 infection is endemic, and vertical transmission is responsible for spread to children. Of HTLV-1 carriers, 90% or more remain asymptomatic. Some cases of adult HTLV-1-related lymphomas have characteristics of ALCL but are considered CD30+ ATLL subtypes. No similar cases have been described in children. We analyzed 33 cases of pediatric ALCL, CD25+ and CD25-, for proviral HTLV-1 DNA. All cases corresponded to the common histologic ALCL type and were CD30+ in virtually all neoplastic cells. ALK expression was observed in 31 (94%) of 33 cases; CD25 was positive in 27 (82%), including 1 ALK- ALCL case. There was a strong positive correlation between ALK and CD25 expression. None of the cases showed proviral HTLV-1 DNA. ALCL in children has no relationship with HTLV-1; the frequent CD25 expression must be explained by a mechanism different from that in ATLL.

Nodal diffuse large B-cell lymphomas in children and adolescents: immunohistochemical expression patterns and c-MYC translocation in relation to clinical outcome.

Diffuse large B-cell lymphoma (DLBCL) is a very infrequent neoplasm in the pediatric age group; therefore there are very few studies on the immunophenotype or genetics of these cases. We studied a series of 16 patients with nodal DLBCL occurring in patients between 10 and 18 years of age. The cases were classified according to the 2008 World Health Organization classification criteria, with application of immunohistochemistry for the detection of CD10, BCL-6, and MUM1 proteins to divide the lymphomas into germinal center and nongerminal center types. In addition, TCL1, BCL-2 expression, and the Ki-67 proliferation index were evaluated by immunohistochemistry, and c-MYC and BCL2 translocations were evaluated by fluorescence in situ hybridization. All these parameters were correlated with clinical features and outcome. Our study revealed that centroblastic morphology and the germinal center type of DLBCL are more prevalent in these young patients (63%), with 37% containing a c-MYC translocation. Only 1 case showed a BCL2 translocation, reflecting a double-hit case with features intermediate between DLBCL and Burkitt lymphoma. We found a higher frequency of BCL-2 expression than previously reported, with no direct influence on the outcome of the disease in univariate or multivariate analysis. The expression of TCL1 has not been specifically studied in nodal pediatric DLBCL before; we found a 31% incidence of TCL1 expression. MUM1 expression was observed in 44% of the cases and these positive cases showed a significant negative impact on clinical outcome. TCL1 is directly and significantly associated with the presence of c-MYC and a high proliferative index. The germinal center and nongerminal center subtypes showed significant differences for both overall survival and disease-free interval. c-MYC translocation was found in 37% of patients, and had a favorable impact on clinical outcome. We conclude that nodal pediatric and adolescent DLBCL are mainly of the germinal center type, with a generally good outcome despite the frequent expression of BCL-2 and the presence of c-MYC translocation. TCL1 expression seems to be associated with a good clinical outcome, whereas MUM1 expression predicts a poor clinical outcome.

Validación de la punción aspiración con aguja fina (PAAF) en el diagnóstico de linfadenitis tuberculosa en pacientes con infección por el virus de la inmunodeficiencia humana (VIH) / Assessment of fine needle puncture-aspiration assessment (PAAF) for the diagnosis of Tuberculous lymphadenitis in patients with human immunedeficiency virus (HIV)

Objetivo: el objetivo del estudio fue validar la PAAF en el diagnóstico de linfadenitis tuberculosa (TB) en pacientes infectados por el virus de la inmunodeficiencia humana (VIH), utilizando como gold standard el cultivo de Mycobacterium tuberculosis en biopsia ganglionar o la histopatología más la respuesta al tratamiento específico. Material y método: estudio clínico-patológico prospectivo. Se incluyeron pacientes VIH infectados, previo consentimiento informado, mayores a 18 años, con adenomegalias superficiales mayores a 2 cm, asistidos en la Cátedra de Enfermedades Infecciosas entre octubre de 2004 y diciembre de 2006. Se realizó PAAF y biopsia del mismo ganglio. En las muestras citológicas e histopatológicas se realizaron tinciones habituales, Ziehl-Neelsen y cultivos para micobacterias. Se definió como citología sugestiva de linfadenitis TB: 1) linfadenitis reactiva con necrosis; 2) linfadenitis reactiva con células epitelioides y/o macrófagos y/o células gigantes multinucleadas; con o sin bacilos cido-alcohol resistentes en el directo con tinción de Ziehl-Neelsen. De 30 pacientes incluidos, 23 correspondieron a una TB ganglionar. En los 7 pacientes restantes la PAAF descartó el diagnóstico de TB en 6, evitando tratamientos innecesarios, e indicando estudio histológico para diagnóstico definitivo. La sensibilidad, especificidad, valor predictivo positivo y valor predictivo negativo de la PAAF para el diagnóstico de linfadenitis TB fue de 95%, 86%, 95% y 86%. El coeficiente de concordancia de Kappa entre ambos procedimientos fue de 90%. Conclusiones: la PAAF de ganglio linfático en pacientes VIH tiene un elevado rendimiento como técnica diagnóstica de TB ganglionar. A su vez permite seleccionar qué pacientes requieren biopsia ganglionar para diagnóstico definitivo, evitando la realización sistemática de la misma en todos los pacientes con adenomegalias.(AU)

Objective: the objective of the study was to validate PAAF for the diagnosis of tuberculous lymphadenitis in patients with HIV, by using culture for Mycobacodeterium tuberculosis in node biopsy or histopathology as ôgoldstandardö, together to the response to the specific treatment. Method: prospective clinical-pathological study. Patients included in our study: were infected with HIV, provided their informed consent, were older than 18 years of age, had superficial adenomagaly larger than 2 cm, and were seen at the Infectious Diseases Service from October 2004 through December 2006. Fine needle puncture aspiration (PAAF ) and biopsy of the same node were performed. Usual stain and Ziehl-Neelsen stain were use din cytological and histopatological samples, and microbacteria cultures were made. We defined the following citology suggested TB lymphadenitis:1) reactive lymphadenitis with necrosis; 2) reactive lymphadenitis with epithelial cells and/or macrophagos and/or multinuclear giant cells; with or without acid-alcohol resistant bacilli in the direct (sample?) with Ziehl-Neelsen stain. Results: out of 30 patients included in the study, 23 corresponded to node TB. In the remaining seven patients, diagnosis was negative in six of them, according to PAAF, and thus unnecessary treatment was avoided, and histological study was determined for final diagnosis. Sensitivity, specificity, positive and negative predictive value of PAAF for the diagnosis of TB lymphadenitis was 95%, 86%, 95% and 86% respectively. Kappa concordance coefficient between both procedures was 90%. Conclusions: lymph node PAAF in patients with HIV is a high performance diagnostic technique for the diagnosisof node TB. Likewise, it enables selecting patients to undergo node biopsy for final diagnose, avoiding its systematic conduction in all patients with adenomegaly.(AU)

Objetivo: o objetivo deste trabalho foi validar a PAAF no diagnóstico de linfadenite tuberculosa (TB) em pacientes infectados pelo vírus da imunodeficiência humana (VIH), utilizando como ôgold standardö o cultivo de Mycobacterium tuberculosis em biopsia ganglionar ou a histopatologia com resposta ao tratamento específico. Material e método: estudo clínico-patológico prospectivo. Foram incluídos pacientes portadores de VIH, com consentimento informado prévio, maiores de 18 anos, com adenomegalias superficiais maiores que 2 cm, atendidos na Cátedra de Doenças Infecciosas entre outubro de 2004 e dezembro de 2006. Em cada gânglio foi realizada PAAF e biopsia. Nas amostras citológicas e histopatológicas foram realizadas as colorações habituais, Ziehl-Neelsen e cultivos para micobacterias. Foi definida como citologia sugestiva de linfadenite TB: 1) linfadenite reativa com necrose; 2) linfadenite reativa com células epitelioides e/ou macrófagos e/ou células gigantes multinucleadas; com ou sem bacilos ácido-álcool resistentes no exame direto com coloração de Ziehl-Neelsen. Resultados: dos 30 pacientes incluídos, 23 apresentaram TB ganglionar. Nos sete pacientes restantes a PAAF descartou o diagnóstico de TB em seis, evitando tratamentos desnecessários, e indicando exame histológico para diagnóstico definitivo. A sensibilidade, especificidade, valor preditivo positivo e valor preditivo negativo da PAAF para o diagnóstico de linfadenite TB foram respectivamente 95%, 86%, 95% e 86%. O coeficiente de concordância de Kappa entre ambos procedimentos foi de 90%. Conclusães: a PAAF de gânglio linfático em pacientes VIH tem alto rendimento como técnica diagnóstica de TB ganglionar. Permite também selecionar os pacientes que necessitam biopsia ganglionar para diagnóstico definitivo, evitando sua realização sistemática em todos os pacientes com adenomegalias.(AU)

Validación de la punción aspiración con aguja fina (PAAF) en el diagnóstico de linfadenitis tuberculosa en pacientes con infección por el virus de la inmunodeficiencia humana (VIH) / Assessment of fine needle puncture-aspiration assessment (PAAF) for the diagnosis of Tuberculous lymphadenitis in patients with human immunedeficiency virus (HIV)

Objetivo: el objetivo del estudio fue validar la PAAF en el diagnóstico de linfadenitis tuberculosa (TB) en pacientes infectados por el virus de la inmunodeficiencia humana (VIH), utilizando como "gold standard" el cultivo de Mycobacterium tuberculosis en biopsia ganglionar o la histopatolog¡a más la respuesta al tratamiento espec¡fico. Material y método: estudio clínico-patológico prospectivo. Se incluyeron pacientes VIH infectados, previo consentimiento informado, mayores a 18 años, con adenomegalias superficiales mayores a 2 cm, asistidos en la Cátedra de Enfermedades Infecciosas entre octubre de 2004 y diciembre de 2006. Se realizó PAAF y biopsia del mismo ganglio. En las muestras citológicas e histopatológicas se realizaron tinciones habituales, Ziehl-Neelsen y cultivos para micobacterias. Se definió como citolog¡a sugestiva de linfadenitis TB: 1) linfadenitis reactiva con necrosis; 2) linfadenitis reactiva con células epitelioides y/o macrófagos y/o células gigantes multinucleadas; con o sin bacilos cido-alcohol resistentes en el directo con tinción de Ziehl-Neelsen. De 30 pacientes incluidos, 23 correspondieron a una TB ganglionar. En los 7 pacientes restantes la PAAF descartó el diagnóstico de TB en 6, evitando tratamientos innecesarios, e indicando estudio histológico para diagnóstico definitivo. La sensibilidad, especificidad, valor predictivo positivo y valor predictivo negativo de la PAAF para el diagnóstico de linfadenitis TB fue de 95%, 86%, 95% y 86%. El coeficiente de concordancia de Kappa entre ambos procedimientos fue de 90%. Conclusiones: la PAAF de ganglio linfático en pacientes VIH tiene un elevado rendimiento como técnica diagnóstica de TB ganglionar. A su vez permite seleccionar qué pacientes requieren biopsia ganglionar para diagnóstico definitivo, evitando la realización sistemática de la misma en todos los pacientes con adenomegalias.

Objective: the objective of the study was to validate PAAF for the diagnosis of tuberculous lymphadenitis in patients with HIV, by using culture for Mycobacodeterium tuberculosis in node biopsy or histopathology as “goldstandard”, together to the response to the specific treatment. Method: prospective clinical-pathological study. Patients included in our study: were infected with HIV, provided their informed consent, were older than 18 years of age, had superficial adenomagaly larger than 2 cm, and were seen at the Infectious Diseases Service from October 2004 through December 2006. Fine needle puncture aspiration (PAAF ) and biopsy of the same node were performed. Usual stain and Ziehl-Neelsen stain were use din cytological and histopatological samples, and microbacteria cultures were made. We defined the following citology suggested TB lymphadenitis:1) reactive lymphadenitis with necrosis; 2) reactive lymphadenitis with epithelial cells and/or macrophagos and/or multinuclear giant cells; with or without acid-alcohol resistant bacilli in the direct (sample?) with Ziehl-Neelsen stain. Results: out of 30 patients included in the study, 23 corresponded to node TB. In the remaining seven patients, diagnosis was negative in six of them, according to PAAF, and thus unnecessary treatment was avoided, and histological study was determined for final diagnosis. Sensitivity, specificity, positive and negative predictive value of PAAF for the diagnosis of TB lymphadenitis was 95%, 86%, 95% and 86% respectively. Kappa concordance coefficient between both procedures was 90%. Conclusions: lymph node PAAF in patients with HIV is a high performance diagnostic technique for the diagnosisof node TB. Likewise, it enables selecting patients to undergo node biopsy for final diagnose, avoiding its systematic conduction in all patients with adenomegaly.

Objetivo: o objetivo deste trabalho foi validar a PAAF no diagnóstico de linfadenite tuberculosa (TB) em pacientes infectados pelo v¡rus da imunodeficiência humana (VIH), utilizando como “gold standard” o cultivo de Mycobacterium tuberculosis em biopsia ganglionar ou a histopatologia com resposta ao tratamento espec¡fico. Material e método: estudo clínico-patológico prospectivo. Foram inclu¡dos pacientes portadores de VIH, com consentimento informado prévio, maiores de 18 anos, com adenomegalias superficiais maiores que 2 cm, atendidos na Cátedra de Doenças Infecciosas entre outubro de 2004 e dezembro de 2006. Em cada gânglio foi realizada PAAF e biopsia. Nas amostras citológicas e histopatológicas foram realizadas as colorações habituais, Ziehl-Neelsen e cultivos para micobacterias. Foi definida como citologia sugestiva de linfadenite TB: 1) linfadenite reativa com necrose; 2) linfadenite reativa com células epitelioides e/ou macrófagos e/ou células gigantes multinucleadas; com ou sem bacilos  cido- lcool resistentes no exame direto com coloração de Ziehl-Neelsen. Resultados: dos 30 pacientes inclu¡dos, 23 apresentaram TB ganglionar. Nos sete pacientes restantes a PAAF descartou o diagnóstico de TB em seis, evitando tratamentos desnecessários, e indicando exame histológico para diagnóstico definitivo. A sensibilidade, especificidade, valor preditivo positivo e valor preditivo negativo da PAAF para o diagnóstico de linfadenite TB foram respectivamente 95%, 86%, 95% e 86%. O coeficiente de concordância de Kappa entre ambos procedimentos foi de 90%. Conclusães: a PAAF de gânglio linfático em pacientes VIH tem alto rendimento como técnica diagnóstica de TB ganglionar. Permite também selecionar os pacientes que necessitam biopsia ganglionar para diagnóstico definitivo, evitando sua realização sistemática em todos os pacientes com adenomegalias.

Primary and secondary T-cell lymphomas of the breast: clinico-pathologic features of 11 cases.

Breast involvement by non-Hodgkin lymphomas is rare, and exceptional for T-cell lymphomas; we studied the morphologic, immunophenotypic, and clinical features of 11 patients with T-cell non-Hodgkin lymphomas involving the breast. Four cases fulfilled the definition criteria for primary breast lymphomas, 3 females and 1 male, with a median age of 51 years. One primary breast lymphomas was T-cell lymphoma unspecified, other was subcutaneous panniculitis-like T-cell lymphoma, and 2 cases were anaplastic large cell lymphomas. One of the anaplastic large cell lymphoma cases was found surrounding a silicone breast implant and presented as clinically as mastitis; whereas the other case occurred in a man. T-cell lymphoma secondarily involved the breast in 7 patients, all women and 1 bilateral, with a median age of 29 years. These secondary breast lymphomas occurred as part of widespread nodal or leukemic disease. Three patients had adult T-cell leukemia/lymphoma, including the patient with bilateral lesions, 3 others had precursor T-lymphoblastic lymphoma/leukemia, and the other presented with a peripheral-T-cell lymphoma non otherwise specified type. Breast T-cell lymphomas are very infrequent and are morphologically and clinically heterogeneous.