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Background Allogeneic hematopoietic stem cell transplant (allo-HSCT) is used to treat several hematological diseases, but immunosuppression during allo-HSCT facilitates opportunistic microbial growth in tissues, such as actinomycosis. An effective diagnosis of opportunistic diseases is essential for correct management of the disease and preservation of the immunosuppressed patient’s life. Case description A 57-year-old female patient was diagnosed with extranodal nasal type NK/T cell lymphoma and underwent curative treatment with allo-HSCT. Twenty-one days after the last clinical follow-up, the patient presented a necrotizing lesion in the papilla region between the first and second molars of the second quadrant. Histopathological analysis showed the presence of a bacterial cluster consistent with Actinomyces infection, and a dense lymphoid infiltrate was also observed. Immunohistochemistry for CD20, CD3, and CD56 was performed to exclude the possibility of the recurrence of extranodal NK/T cell lymphoma. Oral microbiota profiling showed a huge increase in the abundance of Actinomyces bacteria in the subgingival region three weeks prior to appearance of the lesion. Conclusions Opportunistic infections with an unusual clinical appearance are confounding factors in therapeutic decision-making. We present for the first time a case of actinomycosis in the gingival papilla region following allo-HSCT. We also highlight how microbiota profiling through next-generation sequencing could be used to anticipate bacterial infection diagnosis.

INTRODUÇÃO: A biópsia cirúrgica (BC) é considerada o método ideal para obtenção de material para o diagnóstico de linfomas, no entanto, técnicas percutâneas menos invasivas têm ganhado espaço, destacando-se a biópsia por agulha grossa (BA). O objetivo do presente estudo foi comparar a capacidade diagnóstica de BCs e BAs, bem como avaliar a aplicabilidade dos critérios da OMS 2017 no diagnóstico de proliferações linfoides benignas e malignas em BAs. CASUÍSTICA E MÉTODOS: Foram selecionados casos de proliferações linfoides, com material de biópsia disponível no Laboratório de Patologia do Hospital Sírio-Libanês no período de outubro de 2013 a agosto de 2017. Os casos foram divididos em grupos de BC e BA e a capacidade diagnóstica dos dois métodos foi medida, levando-se em consideração o percentual de diagnósticos definitivos obtidos. Foi analisado o impacto de fatores como características inerentes a diferentes subgrupos de doenças, obtenção de dados clínicos, avaliação da adequabilidade da amostra por patologista durante o procedimento, número e tamanho de fragmentos, calibre da agulha, bem como realização de imuno-histoquímica (IHQ), imunofenotipagem por citometria de fluxo (IFT CMF) e demais exames complementares. RESULTADOS: Amostras de 476 pacientes foram analisadas, sendo 218 BAs e 258 BCs. A capacidade diagnóstica das BCs foi de 97,3% e das BAs, 91,3% (P=0,010). Em relação à contribuição relativa dos outros fatores analisados, informações clínicas foram imprescindíveis para o diagnóstico em 20,6% das BAs, em comparação com 7,4% das BCs (P>0,001), IHQ em 96,3% das BAs em comparação com 91,5% das BCs (P=0,020), IFT CMF em 12% das BAs em comparação com 6,8% das BCs (P=0,165) e demais exames em 8,9% das BAs em comparação com 17,3% das BCs (P=0,140). Maior porcentagem de BAs não satisfatórias foi observada em casos de linfoma de células T (10%), seguido pelo linfoma de Hodgkin clássico (9,4%). Maior discordância com dados clínicos foi observada na graduação dos linfomas foliculares em amostras de BA (23,1%). Maior porcentagem de amostras insatisfatórias e de resultados discordantes na IFT CMF foi encontrada no Linfoma de Hodgkin clássico (48,5% de discordância) e predominância linfocítica nodular (100% de discordância). Fatores que não influenciaram diretamente no desempenho diagnóstico neste estudo foram a avaliação do material por patologista durante o procedimento, número e tamanho dos fragmentos e calibre da agulha. CONCLUSÃO: A BA demonstrou ser útil no diagnóstico de proliferações linfoides nesta instituição, sendo possível aplicar os critérios da OMS 2017. O seu desempenho dependeu de maior integração entre dados clínicos e exames complementares. Em especial, a IFT CMF mostrou-se útil neste contexto. A principal limitação da BA esteve relacionada ao diagnóstico de algumas entidades como os linfomas de células T e os linfomas de Hodgkin clássico INTRODUCTION: Surgical biopsy (SEB) is considered to be the ideal method to obtain material for lymphoma diagnosis, however, less invasive percutaneous techniques have been gaining space, especially core needle biopsy (CNB). The aim of the present study was to compare the diagnostic yield of SEB and CNB, as well as to assess the applicability of the 2017 WHO criteria in the diagnosis of benign and malignant lymphoid proliferations in CNB. CASUISTRY AND METHODS: Cases of lymphoid proliferation with available material at the Pathology Laboratory of Hospital Sírio-Libanês were selected from October 2013 to August 2017. The cases were divided into SEB and CNB groups and the diagnostic yield of the two methods was measured, taking into account the percentage of definitive diagnoses obtained. We analyzed the impact of factors such as inherent characteristics of different subgroups of diseases, obtainment of clinical data, evaluation of the suitability of the sample by a pathologist during the procedure, number and size of fragments, needle gauge, as well as performing immunohistochemistry (IHC), flow cytometry immunophenotyping (FC) and other complementary tests. RESULTS: Samples from 476 patients were analyzed, 218 CNB and 258 SEB. The diagnostic yield of SEB was 97,3% and of CNB, 91,3% (P=0,010). Regarding the relative contribution of the other factors analyzed, clinical information was essential for diagnosis in 20,6% of CNB, compared to 7,4% of SEB (P

Alagille syndrome (AS) is an autosomal dominant multisystem disorder which can lead to hepatopathy and the development of focal hepatic lesions. The majority of the hepatic lesions are benign, including regenerative nodules, focal hyperplasia, and adenoma. Hepatocellular carcinoma (HCC) is extremely rare in AS, with very few cases reported in the literature. A 38-year-old man complaining of acute right upper quadrant pain with long-standing diagnosis of Alagille syndrome. On imaging, the patient had a large hepatic mass in the right lobe, with arterial hyperenhancement, washout appearance, and areas of internal hemorrhage. The patient underwent a right hepatectomy and histopathology demonstrated HCC. The patient passed away 3 months after the surgery due to infectious complications. HCC is a rare complication of AS, although rare, it should be considered. This case also emphasizes the need of HCC screening in patients with AS in order to allow an early diagnosis and treatment, which can improve patients’ outcome.

Nodal peripheral T-cell lymphoma (PTCL) is an aggressive and heterogeneous malignancy with poor prognosis. We studied the prognostic impact of the expression profile of genes related to cell proliferation (CCNA2, TOP2A, and CHEK1), pro-inflammatory activity (NFkB1 and IKBkB), and angiogenesis (VEGF1) in nodal PTCL outcomes, as well as the ability of this genomic panel to discriminate different histological subtypes. We investigated the relative expression of regulator genes in 63 nodal PTCL patients. CCNA2, TOP2A, CHEK1, and NF-kB1 proteins were also assessed by immunohistochemistry. The median patient age was 47 years, 57.1% were male, 34.9% were diagnosed with PTCL-NOS, 28.6% with ALK-/ALCL, 22.2% with ALK+/ALCL, and 14.3% with AITL. The proliferative genes were associated with worse 3-year OS and PFS in PTCL-NOS and better 3-year PFS in ALK-/ALCL. Expression of CCNA2≥median and overexpression of CHEK1 protein (HR 3.793; p = 0.007) were associated with worse OS for all the cohort of nodal PTCL (HR 1.418; p = 0.001). The genomic expression profile tested in this study was not able to discriminate the different subtypes of nodal PTCL, although it showed a distinct prognostic significance between PTCL-NOS and ALCL-ALK. Overexpression of the CCNA2 gene and CHEK1 protein were associated with poor prognosis in the total nodal PTCL cohort.

Splenic metastases are rare and usually occur in cases of disseminated disease. We report a case of a patient who had isolated splenic metastases with a previous history of left nephrectomy due to a renal cell carcinoma 11 years before. The aim of this report is to describe the case and review the literature of isolated splenic metastases due to renal carcinoma. This case emphasizes the importance of considering splenic metastatic disease even after many years of diagnosis of renal cell carcinoma.

Bone marrow biopsy is recommended for staging of classical Hodgkin lymphoma. The aim of this study was to compare bone marrow evaluation by histology with that obtained by (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET). One hundred and three cases of Classical Hodgkin Lymphoma were reviewed. All patients were submitted to FDG-PET evaluation. Bone marrow biopsy results were compared with clinical data and FDG-PET results. Ninety-one cases had available bone marrow biopsies. Overall, there were 16 positive and one suspect case. In five cases, the FDG-PET scan was positive and biopsy was negative: 1/5 was found to correspond to a bone fracture, 3/5 showed marked reactive bone marrow changes and in 1/5 no explanation for the discrepancy was found. FDG-PET showed high sensitivity, supporting the idea that when it is negative, biopsy could be avoided. Care should be taken in patients with a positive FDG-PET, where confirmation by bone marrow biopsy should be recommended.

Since the 19th century, neuroendocrine neoplasms (NEN) have been identified. Right up to the present day, the nomenclature is still under debate reflecting the heterogeneity of these tumors. Although some of them are slow growing, some can be fearfully aggressive and may develop in almost any organ of the abdomen, thorax, neck, skin, and gonads. The most commonly observed sites of NEN are the lung and the gastroenteropancreatic system (GEP), where more than 50 entities have been observed. In case of a NEN of unknown primary tumor, the histopathological diagnostic workup includes immunohistochemistry for chromogranin A and synaptophysin, followed by specific tissue markers. Clinical presentation is very diverse, depending on the primary site and functionality of the tumor. In the case of the GEP-NEN, the main symptoms are abdominal pain, diarrhea, weight loss, gastrointestinal bleeding, or bowel obstruction. The presence of neuropsychiatric symptoms is not insignificant in this group of tumors. The authors report a case of a 51-year-old man who sought medical attention because of a three-month history of a consumptive disease. The diagnostic workup disclosed a diffuse nodular infiltration of the lungs, hypokalemia, and hypercalcemia in a cachectic patient. The clinical investigation could not proceed because of an infectious intercurrence, which led to the fatal outcome. Autopsy findings showed a diffuse metastatic NEN. The primary tumoral site could not be demonstrated with the available immunohistochemical panel.

Nodal peripheral T-cell lymphomas are a rare group of neoplasms derived from post-thymic and activated T lymphocytes. A review of scientific articles listed in PubMed, Lilacs, and the Cochrane Library databases was performed using the term “peripheral T-cell lymphomas”. According to the World Health Organization classification of hematopoietic tissue tumors, this group of neoplasms consists of peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), angioimmunoblastic T-cell lymphoma (AITL), anaplastic large cell lymphoma-anaplastic lymphoma kinase positive (ALCL-ALK+), and a provisional entity called anaplastic large cell lymphoma-anaplastic lymphoma kinase negative (ALCL-ALK−). Because the treatment and prognoses of these neoplasms involve different principles, it is essential to distinguish each one by its clinical, immunophenotypic, genetic, and molecular features. Except for anaplastic large cell lymphoma-anaplastic lymphoma kinase positive, which has no adverse international prognostic index, the prognosis of nodal peripheral T-cell lymphomas is worse than that of aggressive B-cell lymphomas. Chemotherapy based on anthracyclines provides poor outcomes because these neoplasms frequently have multidrug-resistant phenotypes. Based on this, the current tendency is to use intensified cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) regimens with the addition of new drugs, and autologous hematopoietic stem cell transplantation. This paper describes the clinical features and diagnostic methods, and proposes a therapeutic algorithm for nodal peripheral T-cell lymphoma patients.

Burkitt lymphoma (BL) is a highly aggressive B cell lymphoma, presenting in extranodal sites or as an acute leukemia. Three clinical variants of BL are recognized: endemic BL, sporadic BL and immunodeficiency associated BL. Sporadic BL is seen worldwide, mainly in children and young adults involving the abdominal organs mostly the ileocaecal area. Pancreatic involvement is rare. The authors report a unique case of abdominal Burkitt lymphoma, initially diagnosed and treated as acute pancreatitis. Clinically, the patient presented severe abdominal pain and vomiting. Imaging findings were suggestive of inflammatory involvement of the pancreas, heading treatment towards this hypothesis. Unfortunately, the patient died during the diagnostic work up, and the autopsy findings demonstrated advanced Burkitt lymphoma with extensive involvement of pancreatic parenchyma and other organs within the abdominal cavity. Once Burkitt lymphoma is a potentially curable disease, early diagnosis is crucial for better outcomes.

O objetivo do estudo foi caracterizar a leishmaniose tegumentar em pacientes do Distrito Federal, investigar infecção subclínica nos moradores das localidades dos pacientes e identificar as espécies de flebotomíneos e leishmanias. Foram selecionados pacientes atendidos no Hospital Universitário de Brasília de agosto de 2006 a junho de 2007. Parentes e vizinhos dos mesmos foram submetidos à intradermorreação de Montenegro e imunofluorescência indireta. Foram capturados flebotomíneos nas localidades de origem dos pacientes e identificados quanto às espécies, bem como foram identificadas as espécies de leishmanias encontradas nos pacientes. Foram registrados 10 casos autóctones de leishmaniose tegumentar. Em 32 moradores, foi realizada intradermorreação, com positividade de 71,8%. Trinta e sete imunofluorescências realizadas foram negativas. Foram capturadas Lutzomyia whitmani, inclusive no domicílio/peridomicílio e Lutzomyia flaviscutellata. O percentual de positividade das intradermorreações de Montenegro sugere infecção subclínica dos moradores. A captura do vetor Lutzomyia whitmani no peri/intradomicílio sugere transmissão peri/intradomicíliar. _______________________________________________________________________________________ ABSTRACT The objectives of this study were to characterize cutaneous leishmaniasis in patients from the Federal District, investigate subclinical infection in people living in the same localities as patients and identify the species of Phlebotomus and Leishmania. Patients attended at the University Hospital of Brasília between August 2006 and June 2007 were selected. Relatives and neighbors of the patients underwent the Montenegro intradermal test and indirect immunofluorescence. Phlebotomines were caught at the localities where the patients came from and their species were identified. The species of Leishmania in the patients were also identified. Ten autochthonous cases of cutaneous leishmaniasis were recorded. The intradermal test was done on 32 local residents and 71.8% were positive. Immunofluorescence was performed on 37 individuals and all of them were negative. Lutzomyia whitmani was caught, including in domestic/peridomestic areas, along with Lutzomyia flaviscutellata. The percentage of positive Montenegro intradermal tests suggests that the local residents had subclinical infection. Capture of the vector Lutzomyia whitmani in domestic/peridomestic areas suggests that domestic/peridomestic transmission was occurring.

The objectives of this study were to characterize cutaneous leishmaniasis in patients from the Federal District, investigate subclinical infection in people living in the same localities as patients and identify the species of Phlebotomus and Leishmania. Patients attended at the University Hospital of Brasília between August 2006 and June 2007 were selected. Relatives and neighbors of the patients underwent the Montenegro intradermal test and indirect immunofluorescence. Phlebotomines were caught at the localities where the patients came from and their species were identified. The species of Leishmania in the patients were also identified. Ten autochthonous cases of cutaneous leishmaniasis were recorded. The intradermal test was done on 32 local residents and 71.8% were positive. Immunofluorescence was performed on 37 individuals and all of them were negative. Lutzomyia whitmani was caught, including in domestic/peridomestic areas, along with Lutzomyia flaviscutellata. The percentage of positive Montenegro intradermal tests suggests that the local residents had subclinical infection. Capture of the vector Lutzomyia whitmani in domestic/peridomestic areas suggests that domestic/peridomestic transmission was occurring.

Background: Peripheral T-cell lymphoma (PTCL) are a biologically and clinically heterogeneous group of rare diseases arising from mature or activated post-thymic T lymphocytes. Correspond to 10% to 15% of lymphoid malignancies with marked geographical variation in incidence. According to the WHO classification they are divided into nodal, extranodal, primary cutaneous and leukemic or disseminated and encompass 18 distinct entities. The nodal group involves the peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), angioimmunoblastic lymphoma (AITL), anaplastic large cell lymphoma ALK positive (ALCL-ALK+) and anaplastic large cell lymphoma ALK negative (ALCL-ALK-). The literature of PTCL is scarce, especially in our country where data of epidemiology, clinical features and outcomes are usually rarely available. So, to better understand PTCL we performed a retrospective study with patients treated in a reference service for cancer treatment in Brazil. Methods: Eight-seven nodal PTCL patients treated with anthracyclne-based regimen (CHOP or, CHOEP) from January 2000 to June 2014 were evaluated retrospectively at the Medicine School of Sao Paulo University, Brazil. All patients lower than 60 years were consolidated with autologous hematopoietic stem cell transplantation (ASCT) in first CR or PR except that with ALCL-ALK+ diagnosis. Refractory and relapsed patients were salvaged with 3-4 cycles of IVAC (Ifosphamide 1.5 g/m2 i.v D1-D5, etoposide 100mg/m2 i.v D1-D5, aracytin 2g/m2 i.v twice a day D1-D2) regimen and submitted to ASCT. It was performed a central histopathological review and clinical and epidemiological data were obtained from medical records. Patients were evaluated for overall response (OR) including complete response (CR) and partial response (PR), overall survival (OS) and progression free survival (PFS). Statistical analysis was performed using the STATA-3 program using and a p-value ≤ 0.05 was considered statistically significant. Results: Of the 87 patients, 34 (39.08%) cases were classified as ALCL-ALK-, 27 (31.03%) as PTCL-NOS, 16 (18.39%) as ALCL-ALK+, 6 (6.89%) as AITL and in 4 (4.1%) cases the diagnosis could not be performed and an expansion of the immunohistochemical is ongoing. Thirty-six (45.38%) cases were female and 51(54.62%) were male, 59(67.81%) patients were lower than 60 years. Seventy-six (87.35%) patients presented in advanced stage (III or IV) at diagnosis but 73(83.90%) patients presented an ECOG < 2 and 14(16.10%) ≥ 2. Eighteen (20.70%) patients were of low-risk, 26 (29.88%) of low-intermediate risk and 43(49.42%) of high-intermediate and high-risk of international prognostic index (IPI). The CR and PR was obtained for 44(50.57%) and 8(9.19%), respectively with 59.76% OR. Thirty (34.48%) patients were primary refractory and five remain under treatment. In a median of follow of 30 months, ALCL-ALK+ show higher OS (median 140.98 months) than ALCL-ALK- (44.20 months), PTCL-NOS (median 20.62 months) and AITL (median 7.24 months) (p=0.41) (Figure 1A). The median of PFS was 3.84 months for AITL, 23.44 months for ALCL-ALK+, 40.03 months for PTCL-NOS and was not yet reached for ALCL-ALK- (p=0.0006) (Figure 1B). Figure 1: Overall survival (1A) and Progression Free Survival (1B) of nodal PTCL Figure 1:. Overall survival (1A) and Progression Free Survival (1B) of nodal PTCL Figure 2 Figure 2. Conclusion: In this study we showed that ALCL-ALK+ as well as found in the literature presented a better OS in comparison to others nodal T-cell lymphoma as AITL, PTCL-NOS and ALCL-ALK-. Surprisingly the PFS of ALCL-ALK+ was statistically significant lower than of ALCL-ALK-. We thought that this result may be explained because in our service until to perform this analysis we did not indicate ASCT in first CR for ALCL-ALK+, but for all ALCL-ALK-. This hypothesis may be reinforced as the most of our cases presented high-intermediate and high-risk of IPI and that could equalize the favorable effect of ALK expression. In addition, we changed our approach and we are also indicating ASCT in first line for patients with ALCL-ALK+ with intermediate-high and high-risk of IPI . Disclosures No relevant conflicts of interest to declare.