Erdheim-Chester disease with chorioretinal and orbital involvement: a case report / Doença de Erdheim-Chester com envolvimento coriorretiniano e orbitário: relato de caso

ABSTRACT A 42-year-old woman presented with bilateral proptosis, chemosis, leg pain, and vision loss. Orbital, chorioretinal, and multi-organ involvement of Erdheim-Chester disease, a rare non-Langerhans histiocytosis, with a negative BRAF mutation was diagnosed based on clinical, radiological, and pathological findings. Interferon-alpha-2a (IFNα-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFNα-2a cessation. The same therapy was administered, and her clinical condition improved. The Erdheim-Chester disease is a rare chronic histiocytic proliferative disease that requires a multidisciplinary approach and can be fatal if left untreated because of multisystemic involvements.

RESUMO Uma mulher de 42 anos apresentou proptose bi-lateral, quemose, dor nas pernas e perda de visão. Com base em achados clínicos, radiológicos e patológicos, foi diag-nosticada doença de Erdheim-Chester com acometimento orbitário, coriorretiniano e multiorgânico. Trata-se de uma rara histiocitose não Langerhans negativa para a mutação BRAF. Foi iniciado tratamento com interferon alfa-2a (IFNα-2a) e o quadro clínico melhorou. No entanto, quatro meses depois, a paciente apresentou perda visual após a cessação do IFNα-2a. A mesma terapia foi administrada novamente e sua condição clínica melhorou novamente. A doença de Erdheim-Chester é uma doença proliferativa histiocítica crônica rara que necessita de uma abordagem multidisciplinar e pode ser fatal se não tratada, devido a envolvimentos multissistêmicos.

The essential role of thoracic duct embolization in management of traumatic iatrogenic chylothorax / O papel fundamental da embolização do ducto torácico no manejo de quilotórax traumático iatrogênico

Abstract This study aims to describe a case series of patients who underwent thoracic duct embolization (TDE) to treat traumatic iatrogenic chylothorax (TIC). Three patients were included: Case #1, a 49-year-old woman with follicular lymphoma developed a TIC following video-assisted thoracoscopic surgery to resect a solid right paravertebral mass and was treated with TDE using microcoils and N-butyl cyanoacrylate (NBCA) glue. Case #2, a 68-year-old man with cardiac amyloidosis developed a TIC following heart transplantation and was treated with TDE using microcoils and ethylene vinyl alcohol copolymer. Case#3: A 6-year-old patient with congenital heart disease developed a TIC following a Fontan procedure and was treated with TDE using NBCA glue. All lesions were identified during lymphangiography and TDE was successfully performed in all cases. TDE is a safe and valuable technique that provides minimally invasive treatment for TCI.

Resumo Este estudo objetiva descrever uma série de casos de pacientes submetidos a embolização do ducto torácico (EDT) para tratamento de quilotórax iatrogênico (QI). Três pacientes foram incluídos. Caso 1: um homem de 49 anos com linfoma folicular apresentou QI após ressecção de uma massa paravertebral por toracoscopia vídeo-assistida e foi submetido a EDT com micromolas e n-butil-cianoacrilato (NBCA). Caso 2: um homem de 68 anos com amiloidose cardíaca apresentou QI após ser submetido a transplante cardíaco e foi submetido a EDT com micromolas e copolímero de etileno e álcool vinílico. Caso 3: um paciente de 6 anos com malformação cardíaca congênita apresentou QI após cirurgia de Fontan e foi submetido a EDT com NBCA. Todas as lesões foram identificadas durante a linfangiografia, e a EDT foi realizada com sucesso. A EDT é uma técnica segura e valiosa, que pode oferecer um tratamento minimamente invasivo em casos de QI.

Malformaciones linfáticas abdominales en una población pediátrica: experiencia en un centro de referencia de Medellín, Colombia / Abdominal lymphatic malformations in a pediatric population: Experience in a referral center in Medellín, Colombia

Introducción. Los linfangiomas son anormalidades benignas del sistema linfático, que corresponden a dilataciones quísticas de estos vasos y se localizan especialmente en el cuello. Solo el 10 % de todas estas malformaciones se encuentran en el abdomen y presentan síntomas variables de acuerdo al tamaño y su ubicación especifica, siendo el dolor abdominal el principal síntoma. Métodos. Se presentan cinco pacientes pediátricos con malformaciones linfáticas abdominales. Se describen su cuadro clínico, localización, tratamiento y la experiencia en el manejo de dicha patología en un hospital de referencia. Resultados. Los métodos más apropiados para hacer una aproximación diagnóstica son la ecografía, la tomografía computarizada y la resonancia nuclear magnética. Dentro de las opciones descritas para el tratamiento están la farmacológica, la escleroterapia y la resección quirúrgica, tanto por vía abierta como por laparoscopia. Conclusión. Existe una variedad de métodos para realizar la resección de los linfangiomas abdominales, pero la cirugía sigue siendo la más efectiva, especialmente cuando se cuenta con la laparoscopia como una herramienta terapéutica.

Introduction. Lymphangiomas are benign abnormalities of the lymphatic system, which correspond to cystic dilations of these vessels and are located especially in the neck. Only 10% of all these malformations are found in the abdomen and present variable symptoms according to size and their specific location, with abdominal pain being the main symptom. Methods. Five pediatric patients with abdominal lymphatic malformations are presented. Their clinical presentation, location, treatment and experience in the management of this pathology in a referral hospital are described. Results. The most appropriate methods to make a diagnostic approach are ultrasound, computed tomography and magnetic resonance imaging. Among the options described for treatment are pharmacological, sclerotherapy and surgical resection, both open and laparoscopic. Conclusion. There are a variety of methods for resecting abdominal lymphangiomas, but surgery remains the most effective, especially when laparoscopy is used as a therapeutic tool.

Manejo de neoplasias oncohematológicas en el contexto de la pandemia de COVID-19: recomendación de expertos / Management of oncohematological neoplasms in the COVID-19 pandemic: experts recommendation

RESUMEN La pandemia por COVID-19 originado por el Coronavirus 2 causante de síndrome respiratorio agudo severo (SARS-CoV-2) es causante de una crisis de salud pública a nivel global. Muchos reportes indican resultados desalentadores en pacientes con cáncer respecto a la población general. Por ello, los expertos en el manejo de neoplasias oncohematológicas del Instituto Nacional de Enfermedades Neoplásicas, hospitales nacionales y una clínica privada de Lima Metropolitana han desarrollado recomendaciones obtenidas por consenso para continuar con el manejo de pacientes con neoplasias oncohematológicas en forma segura ante la coyuntura de pandemia.

ABSTRACT The ongoing COVID-19 pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a global public health crisis. Many reports indicate disappointing results in cancer patients compared to the general population. Therefore, experts in the management of oncohematological malignancies from the National Institute of Neoplastic Diseases, national hospitals and a private clinic in Metropolitan Lima have developed recommendations obtained by consensus to continue with the management of patients with oncohematological neoplasms safely in the face of the pandemic.

Proximal ligation after the side-to-end anastomosis recovery technique for lymphaticovenous anastomosis

BACKGROUND: Lymphaticovenous anastomosis is an important surgical treatment for lymphedema, with lymphaticovenous side-to-end anastomosis (LVSEA) and lymphaticovenous end-to-end anastomosis being the most frequently performed procedures. However, LVSEA can cause lymphatic flow obstruction because of regurgitation and tension in the anastomosis. In this study, we introduce a novel and simple procedure to overcome this problem. METHODS: Thirty-five female patients with lower extremity lymphedema who underwent lymphaticovenous anastomosis at our hospital were included in this study. Eighty-five LVSEA procedures were performed, of which 12 resulted in insufficient venous blood flow. For these 12 anastomoses, the proximal lymphatic vessel underwent clipping after the anastomotic procedure and the venous inflow was monitored. Subsequently, the proximal ligation after side-to-end anastomosis recovery (PLASTER) technique, which involves ligating the proximal side of the lymphatic vessel, was applied. A postoperative evaluation was performed using indocyanine green 6 months after surgery. RESULTS: Despite the clipping procedure, three of the 12 anastomoses still showed poor venous inflow. Therefore, it was not possible to apply the PLASTER technique in those cases. Among the nine remaining anastomoses in which the PLASTER technique was applied, three (33%) were patent. CONCLUSIONS: Our findings show that achieving patent anastomosis is challenging when postoperative venous inflow is poor. We achieved good results by performing proximal ligation after LVSEA. Thus, the PLASTER technique is a particularly useful recovery technique when LVSEA does not result in good run-off.

Repercussões da dor social, gênero e pessoas com doença falciforme: um estudo exploratório / Social pain, gender, and people with sickle cell disease: an exploratory study / Dolor social, género y personas con enfermedad falciforme: estudio exploratorio

OBJETIVO: compreender as repercussões da dor social sobre os papéis de gênero de mulheres e homens com doença falciforme. MÉTODO: estudo qualitativo, exploratório, realizado num Centro de Referência em doença falciforme de um município do interior da Bahia. Os dados obtidos de desenhos estória-tema e entrevistas com 16 pessoas foram submetidos à análise de conteúdo temática. RESULTADOS: emergiram duas categorias: "A dor social nas mulheres com doença falciforme se expressa na impossibilidade de ser dona de casa, mãe e mulher sexuada" e "A dor social de homens com doença falciforme se expressa na dificuldade de manter-se trabalhador, ser provedor e homem sexualmente ativo". DISCUSSÃO: a exclusão do trabalho, as alterações da sexualidade e parentalidade interferem nos projetos de vida e mantém a reprodução de estereótipos de gênero. CONCLUSÃO: a escuta sensível numa abordagem de cuidado multiprofissional deve fazer parte do plano terapêutico de pessoas com doença falciforme.

AIM: Social pain, gender, and people with sickle cell disease: an exploratory study. METHOD: this is a qualitative and exploratory study and it was conducted in a Sickle Cell Disease Reference Center in a municipality in the interior of Bahia. The data obtained from theme-story drawings and interviews with 16 people were submitted to thematic content analysis. RESULTS: two categories have emerged: "Social pain in women with sickle cell disease is expressed in the impossibility of being a housewife, mother and woman sexually" and "The social pain of men with sickle cell disease is expressed in the difficulty of being a worker, a provider and a sexually active man". DISCUSSION: the exclusion of work, changes in sexuality and parenting interfere in life projects and maintain the reproduction of gender stereotypes. CONCLUSION: sensitive listening in a multiprofessional care approach should be part of the therapeutic plan for people with sickle cell disease.

OBJETIVO: compreender las repercusiones del dolor social sobre los papeles de género de mujeres y hombres con enfermedad de las células falciforme. MÉTODO: estudio cualitativo, exploratorio, realizado en un Centro de Referencia en enfermedad falciforme de un municipio del interior de Bahía. Los datos obtenidos de los dibujos historietas-tema y entrevistas con 16 personas se sometieron al análisis de contenido temático. RESULTADOS: emergieron dos categorías: "El dolor social en las mujeres con enfermedad falciforme se expresa en la imposibilidad de ser ama de casa, madre y mujer sexuada" y "El dolor social de hombres con enfermedad falciforme se expresa en la dificultad de mantenerse trabajando, ser proveedor y hombre sexualmente activo". DISCUSIÓN: la exclusión del trabajo, las alteraciones de la sexualidad y la vida parental, interfieren en los proyectos de vida y mantienen la reproducción de estereotipos de género. CONCLUSIÓN: la escucha sensibilizada en el abordaje de cuidado multiprofesional debe hacer parte del plan terapéutico de personas con enfermedad de células falciforme.

Linfangioma cavernoso de la lengua / Cavernous lymphangioma of the tongue

Los linfangiomas son malformaciones congénitas de los vasos linfáticos. Son raros en la cavidad oral y cuando se presentan en la misma pueden provocar trastornos funcionales del sistema estomatognático. El objetivo es presentar un caso de linfangioma cavernoso del dorso de la lengua, revisando los aspectos clínicos y de tratamiento de la enfermedad, con énfasis en la ubicación en la cavidad oral, fundamentalmente en la lengua. Un paciente de 17 años de edad se presenta con una lesión en el dorso de la lengua de aproximadamente 10 años de evolución. La lesión tiene una apariencia pedregosa y una base sésil, y mide aproximadamente 3 cm. El paciente es sometido a escisión quirúrgica de la lesión, sin intercurrencias. Actualmente el paciente se encuentra en seguimiento, sin signos clínicos de recidiva del tumor después de 2 años de tratamiento. La escisión quirúrgica de la lesión parece ser el tratamiento más apropiado para los linfangiomas de la lengua, con bajas tasas de recidiva(AU)

Lymphangiomas are congenital malformations of the lymphatic vessels. They are rare in the oral cavity and when present in this area they may cause functional disturbances of the stomatognathic system. The objective is to report a case of cavernous lymphangioma of the dorsum of the tongue, reviewing the clinical and treatment features of the disease, with emphasis on the oral cavity location, mainly the tongue. A 17-year-old male patient presented with a lesion on dorsum of his tongue of approximately 10 years of evolution. The lesion had a pebbly appearance and a sessile base, and measured about 3 cm. The patient was submitted to surgical excision of the lesion, without any intercurrences. Currently, the patient is under clinical follow-up, without any clinical sign of tumor recurrence after 2 years of treatment. Surgical excision of the lesion seems to be the most adequate treatment for tongue lymphangiomas, with low rates of recurrence(AU)

Clinical and bacteriological analysis of lymphoid tissue neoplasms patients with bacteria bloodstream infections / 中华血液学杂志

Objective@#To investigate the clinical status of lymphoid tissue neoplasms patients with bacteria bloodstream infections, bacteriology and drug susceptibility results, and provide the basis for rational clinical anti-infection option.@*Methods@#A retrospectively analysis of clinical data and bacterial susceptibility test results of patients with bacteria bloodstream infections from September 2010 to December 2014 was conducted.@*Results@#A total of 134 cases including 107 patients with bloodstream infections were enrolled. 84 cases were male, 50 cases were female, the median age was 31 (12-71) years old. 112 cases were agranulocytosis, and 106 cases were severe agranulocytosis (ANC<0.1×109/L) . 27 cases underwent hematopoietic stem cell transplantation, 100 cases received chemotherapy[33 cases with VD (I) CP±L (vincristine+daunorubicin/idarubicin + cyclophosphamide + prednison±asparaginasum) induction chemotherapy, 41 cases with intensive chemotherapy of Hyper-CVAD/MA or MA (mitoxantrone+cytarabine) , 26 cases with other chemotherapy regimens], and 7 cases were infected without chemotherapy. 10 patients discharged from hospital owing to treatment abandoning, 120 cases were cured through anti-infective therapy, 2 patients died of bacteria bloodstream infections, 1 patient died of sudden cardiac, and 1 patient died of GVHD after allogenic hematopoietic stem cell transplantation. A total of 144 strains were isolated, including 108 strains (75.0%) of Gram-negative bacteria and 36 strains (25.0%) of Gram-positive cocci. The susceptibility of Gram-negative bacteria to the carbapenems was 98.00%, and the adjustment treatment rate of carbapenems was 3.0%. The susceptibility of Gram-negative bacteria to the other antibiotics was 60.30%, and the adjustment treatment rate was 90.5%. The susceptibility of Grampositive cocci to the carbapenems was 49.3%, and to glycopeptides and linezolid was 100.0%. Comparing all patients’empirical use of antimicrobial agents with the drugs susceptibility results of blood cultures, 80.1% of the patients’initial drug selection was sensitive.@*Conclusion@#The lymphoid neoplasms patients experienced bacteria bloodstream infections most often after receiving the chemotherapy regimens of treating acute lymphoblastic leukemia. The majority type of bacteria was Gram-negative bacteria. Drug susceptibility test showed that susceptibility of Gram-negative bacteria to the carbapenems was the highest, and the treatment adjustment rate was obviously lower. The susceptibility of Gram-positive cocci to glycopeptides and linezolid was high, and which could be applied to the patients with Gram-positive cocci sepsis on basis of susceptibility results in general.

Parametric imaging of CEUS for blood perfusion in cervical lymphadenopathy / 中国医学影像技术

Objective To explore the diagnostic value of parametric imaging of CEUS in different cervical lymphatic diseases.Methods One hundred eleven patients diagnosed with cervical lymphadenopathy were included.All of the patients underwent parametric imaging of CEUS.According to the pathological results,all patients were divided into reactive hyperplasia group,tuberculosis group,metastasis group and lymphoma group.The features of perfusion phases (perfusion during the artery phase,or perfusion during the artery and parenchymal phase) and perfusion uniformity (homogeneous perfusion or heterogeneous perfusion) were compared among four groups.According to the pathological results,the reactive hyperplasia group and the tuberculosis group were merged into a benign group,and the metastasis group and the lymphoma group were merged into a malignant group.Then,the artery perfusion phase was regarded as benign,and the artery and parenchymal perfusion phase was regarded as malignant.The consistency between parametric imaging of CEUS and the pathological results was compared in two groups.Results The difference of perfusion phase and perfusion uniformity within the four groups had a statistical significance (all P＜0.05).The differences of perfusion phase between the reactive hyperplasia group and the metastasis group,the reactive hyperplasia group and the lymphoma group,the tuberculosis group and the metastasis group were statistical significant respectively (all P＜0.008).The differences of perfusion uniformity between the reactive hyperplasia group and the tuberculosis group,the reactive hyperplasia group and the lymphoma group,the lymphoma group and the tuberculosis group,the lymphoma group and the metastasis group were statistical significant respectively (all P＜0.008).The perfusion phase between the benign group and the malignant group had a statistical significance (P＜0.005),but the perfusion uniformity had no statistical significance in two groups.The consistency was good between the parametric imaging and the pathological results.Conclusion The parametric imaging of CEUS can offer valuable diagnostic information for the cervical lymph nodes underdetermined by conventional ultrasound.

Clinical features of autoimmune liver disease complicated by abdominal lymphadenectasis / 临床肝胆病杂志

Objective To investigate the clinical features of autoimmune liver disease (AILD) complicated by abdominal lymphadenectasis (LA),as well as the clinical significance of LA in AILD.Methods A retrospective analysis was performed for the clinical data of 252 patients who were admitted to The First Affiliated Hospital of Dalian Medical University from January 2005 to April 2016,and among these patients,52 had autoimmune hepatitis (AIH),174 had primary biliary cholangitis (PBC),and 26 had AIH-PBC overlap syndrome (AIH-PBC OS).A total of 78 patients underwent follow-up.According to the presence or absence of LA,these patients were divided into LA group with 70 patients and non-LA (NLA) group with 182 patients.As for general information,laboratory markers,imaging findings,and follow-up results,the normally distributed continuous data were expressed as mean ± SD,and the t-test was used for comparison between groups;the non-normally distributed continuous data were expressed as median,and the Wilcoxon rank sum test was used for comparison between groups.The categorical data were expressed as rates,and the chi-square test,the corrected chi-square test,or the Fisher's exact test was used for comparison of these data between groups.Results There were no significant differences between the two groups in age,sex ratio,proportion of patients who were diagnosed due to abnormal liver function found by physical examination,and incidence rates of other autoimmune diseases.The incidence rate of abdominal LA was 22％ (11/52) in the AIH group,26.4％ (46/174) in the PBC group,and 50％ (13/26) in the AIH-PBC OS group,and the AIH-PBC OS group had a significantly higher incidence rate than the AIH group and the PBC group (x2 =7.693,P =0.021).The LA group had significantly higher levels of alkaline phosphatase and gamma-glutamyl transpeptidase (GGT) than the NLA group (Z =2.944 and 3.169,P =0.003 and 0.002).For the patients with PBC,the LA group had a significantly higher level of GGT than the NLA group (Z =2.136,P =0.033);for the patients with AIH-PBC OS,the LA group had a significantly higher level of total bilirubin thau the NLA group (Z =2.121,P =0.035);for the patients with AIH,there were no significant differences in these indices between the LA group and the NLA group.The LA group had a higher incidence rate of abnormal imaging findings than the NLA group (97.1％ vs 81.9％,x2 =9.863,P =0.002).A total of 78 patients were followed up with a median follow-up time of 18 months.Of all patients in the LA group,6 (23.1％) achieved complete remission,7 (26.9％) achieved incomplete response,1 (3.8％) experienced recurrence,and 12 (46.2％) experienced treatment failure;of all patients in the NLA group,21 (40.4％) achieved complete remission,17 (32.7％) achieved incomplete response,7 (13.5％) experienced recurrence,and 7(13.5％) experienced treatment failure;there was a significant difference in the distribution of treatment outcomes between the LA group and the NLA group (Z =2.406,P =0.016).Conclusion Patients with AIH-PBC OS have a higher incidence rate of abdominal LA than those with AIH and PBC,and patients with AILD complicated by LA may have marked cholestasis and liver impairment and poor treatment response,suggesting that LA might be used to determine disease severity and judge prognosis.

Terapia complexa descongestiva no tratamento intensivo do linfedema: revisão sistemática / Terapia compleja descongestiva en el tratamiento intensivo del linfedema: revisión de literatura / Complex Decongestive Therapy in the intensive care of lymphedema: systematic review

RESUMO O linfedema caracteriza-se por acúmulo de proteínas e fluídos no interstício, com alterações físicas e psicológicas. Entre as técnicas fisioterapêuticas utilizadas para redução do linfedema está a terapia complexa descongestiva. O objetivo desta revisão é identificar evidências para a prática da terapia complexa descongestiva no tratamento intensivo do linfedema. Realizou-se uma busca nas bases de dados PubMed, EMBASE e PEDro. Os artigos selecionados foram estudos randomizados e de coorte, os quais foram avaliados e selecionados de forma independente por dois revisores, que avaliaram a qualidade metodológica dos estudos com a escala PEDro. As buscas resultaram em 414 estudos, depois foram reduzidos a sete estudos elegíveis para análise de qualidade, classificados pela escala PEDro como qualidade alta e eficácia moderada. Os artigos analisados apresentaram boa qualidade metodológica, e seus resultados evidenciaram a efetividade da terapia complexa descongestiva na redução do volume do linfedema no tratamento intensivo.

RESUMEN El linfedema es producido debido a la acumulación de proteínas y fluidos en el intersticio, causando alteraciones físicas y psicológicas. Entre las técnicas fisioterapéuticas empleadas para reducirlo se encuentra la terapia compleja descongestiva. El propósito de este estudio es identificar la práctica de la terapia compleja descongestiva en el tratamiento intensivo del linfedema. Para ello, se hizo una búsqueda en las bases de datos PubMed, EMBASE y PEDro. Los estudios elegidos fueron de tipo aleatorio y de cohorte, en los cuales se hizo una evaluación y elección de forma independiente por dos revisores, quienes trataron de evaluar la calidad metodología en los estudios con la escala PEDro. Se encontraron 414 estudios, después fueron reducidos a siete, elegidos para el análisis de calidad y clasificados en la escala PEDro como de alta calidad y eficacia moderada. Los estudios evaluados presentaron alta calidad metodológica, y sus resultados mostraron la eficacia de la terapia compleja descongestiva para el tratamiento intensivo de reducir el volumen del linfedema.

ABSTRACT Lymphedema is characterized by accumulation of proteins and fluids in the interstice, with physical and psychological changes. Among the physiotherapeutic techniques used to reduce lymphedema we have the complex decongestive therapy. The objective of this review is to identify evidence for the practice of complex decongestive therapy for intensive care of lymphedema. A search was carried out in the PubMed, EMBASE and PEDro databases. The articles chosen were randomized and cohort studies, which have been evaluated and selected independently by two reviewers who evaluated the methodological quality of the studies within the PEDro Scale. The search resulted in 414 studies, reduced to seven eligible studies for quality analysis, classified by PEDro scale as high quality and moderate effectiveness studies. The analyzed articles showed good methodological quality and their results showed the effectiveness of complex decongestive therapy in reducing the volume of lymphedema in intensive care.

Obstructive Jaundice Caused by Tuberculous Lymphadenopathies

Although pulmonary tuberculosis is known to be the most common type in tuberculosis, it actually can affect any organ system. However, abdominal type is very rare among the extra-pulmonary types, and obstructive jaundice caused by lymphadenopathies due to tuberculosis is especially uncommon manifestation even in endemic areas. Tuberculous lymphadenopathies can mimic lymphadenopathies by other metastatic tumors or lymphoma, thus early correct diagnosis is very important for avoiding unnecessary surgical interventions. Here, we reported two cases of obstructive jaundice caused by tuberculous lymphadenopathies. Both were treated with anti-tuberculosis medications and endoscopic retrograde biliary drainage without surgery.

Multi-system Langerhans cell histiocytosis in a neonate:a case report and literature review / 中华围产医学杂志

Objective To investigate the clinical characteristics of neonatal multi-system Langerhans cell histiocytosis.Methods A female neonate with multi-system Langerhans cell histiocytosis in Guangzhou Women and Children's Medical Center in March,2013 was reported and related literature was reviewed.The clinical manifestations,and laboratory test,imaging examination and biopsy results were analyzed.Results The neonate had onset of jaundice and rash,then was found to have hepatomegaly,splenomegaly,lymphadenopathy and abnormal blood test.The lymph node biopsy revealed infiltration of Langerhans cells and eosinophils,which were positive for CDla/S-100 stains.She was diagnosed with multi-system Langerhans cell histiocytosis.Sixty-two cases of neonatal multi-system Langerhans cell histiocytosis were reviewed in the literature,and the most common clinical manifestations included rash,hepatomegaly,respiratory symptoms,imaging changes,abnormal blood test,digestive symptoms and lymphadenopathy.The most frequently involved organs included lung,liver,spleen,bone marrow,bone,gastrointestinal tract,lymph node and mucosa,among which the risky organs (liver,spleen,blood system and lung) accounted for 88.7％(55/62).Conclusions The incidence rate of multi-system Langerhans cell histiocytosis is low in neonates,and it is characterized by multisystem involvement,especially the liver,spleen,blood system,lung and skin.For the neonates diagnosed without intrauterine infections,besides skin biopsy,lymphadenopathy should be dynamically observed and lymph node biopsy should be done earlier so as to establish an early diagnosis.

Enfermedad relacionada a IgG4, el nuevo "gran simulador": caso clínico / IgG4-related disease, the new "great mimicker": Report of one case

Due to its multisystem involvement, IgG4 -related disease should be considered in the differential diagnosis of medical conditions such as lymphadenopathies, aortitis, serositis and retroperitoneal fibrosis. It shares features with other entities historically described as "great mimickers" such as syphilis, tuberculosis, sarcoidosis, and systemic lupus erythematosus. We report a 40 year-old male with recurrent effusive - constrictive pericarditis, lymphadenopathy and aortitis. The study revealed an inactive tuberculosis with negative cultures for acid fast bacilli. The patient had high serum levels of IgG4 and a mediastinal lymph node biopsy was consistent with IgG4 -related disease. The patient was treated with prednisone 40 mg/day with an excellent response.

Perirenal Lymphangiomatosis

Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma may be focal/unilateral or diffuse/bilateral, and in the latter case, it is referred to as lymphangiomatosis. Here, we report a case of a 38-year-old man with perirenal lymphangiomatosis. The patient's chief complaint was left flank pain, and left pleural effusion was found on radiological examination. After radical nephrectomy, the pathological examinations revealed that the kidney was enclosed by a multicystic mass with intrarenal cystic dilatations. We report the case and discuss the management of perirenal lymphangiomatosis with a literature review.

Perirenal Lymphangiomatosis

Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma may be focal/unilateral or diffuse/bilateral, and in the latter case, it is referred to as lymphangiomatosis. Here, we report a case of a 38-year-old man with perirenal lymphangiomatosis. The patient's chief complaint was left flank pain, and left pleural effusion was found on radiological examination. After radical nephrectomy, the pathological examinations revealed that the kidney was enclosed by a multicystic mass with intrarenal cystic dilatations. We report the case and discuss the management of perirenal lymphangiomatosis with a literature review.

Papel da imuno-histoquimica no diagnostico de adenopatias cervicais malignas / Value of immunohistochemistry in the diagnosis of malignant cervical lymph nodes

As adenopatias cervicais constituem importante condição clínica, devido à grande variedade de diagnósticos diferenciais que englobam. O uso da imuno-histoquímica tornou-se importante método auxiliar na avaliação diagnóstica de lesões linfonodais, tanto primárias como secundárias. OBJETIVO: Avaliar o uso da imuno-histoquímica no diagnóstico de malignidade nas biópsias de linfonodos. MÉTODO: Estudo retrospectivo, realizado de 2009 a 2011, com base nos resultados anatomopatológicos arquivados de biópsias de linfonodos. RESULTADOS: A casuística constituiu-se de 32 casos de biópsias de linfonodos, com uso de imuno-histoquímica em 50% (16) casos, dos quais 68,75% foram de linhagem hematogênica e 31,25%, de carcinomas. O método foi utilizado em todos os casos de linfoma. CONCLUSÃO: A imuno-histoquímica foi utilizada em 50% dos casos de biópsias de linfonodos suspeitos de malignidade, sendo em lesões de linhagem epitelial em 31,25% e, para linhagem hematopoiética, em 68,75% dos casos. .

The cervical lymph nodes are relevant due to the diversity of clinical entities. The use of immunohistochemistry is a real method to elucidate the diagnosis of adenopathy, both primary and metastatic neoplasms. OBJECTIVE: To assess the value of immunohistochemistry in the diagnosis of cervical lymph nodes malignancies. METHOD: Retrospective study of the database histopathological specimens from 2009 to 2011. RESULTS: Out of 32 biopsies of cervical lymph nodes, in 16 (50%) the immunohistochemistry was employed, being 68.75% (11) in hematological neoplasms and 31.25% (5) in carcinomas. It was used in all cases of lymphoma. CONCLUSION: The immunohistochemistry was used in 50% of the biopsies of lymph nodes under suspicion of malignancy, being 31.25% in epithelial lesions and 68.75% in lymphoproliferative lesions. .

Isolated Cervical Lymph Node Sarcoidosis Presenting in an Asymptomatic Neck Mass: A Case Report / 결핵및호흡기질환

Sarcoidosis, a systemic granulomatous disease of unknown etiology. The presentation of sarcoidal granuloma in neck nodes without typical manifestations of systemic sarcoidosis is difficult to diagnose. We describe the case of a 37-year-old woman with an increasing mass on the right side of neck. The excisional biopsy from the neck mass showed noncaseating epithelioid cell granuloma of the lymph nodes. No evidence of mycobacterial or fungal infection was noted. Thoracic evaluations did not show enlargement of mediastinal lymph nodes or parenchymal abnormalities. Immunohistochemistry showed abundant expression of tumor necrosis factor-alpha in the granuloma. However, transforming growth factor-beta was not expressed, although interleukin-1beta was focally expressed. These immunohistochemical findings supported characterization of the granuloma and the diagnosis of sarcoidosis. Sarcoidosis can present with cervical lymph node enlargement without mediastinal or lung abnormality. Immunohistochemistry may support the diagnosis of sarcoidosis and characterization of granuloma.

Difficult splenectomy in 126 cases / 中华普通外科杂志

Objective To evaluate the operative safety of difficult splenectomy.Methods The clinical data of 126 splenectomy cases from January 2005 to December 2011 were analyzed retrospectively.Results All patients were cured and discharged.There was no mortality and pancreatic leakage,no gastric and colonic injury.The postoperative complications occurred in 14 patients including intra-abdominal hemorrhage in 5 cases,pulmonary complications in 7 cases (left pleural effusion and left subphrenic abscess),splenic venous thrombophlebitis in 11 cases and massive ascites in 10 patients.After splenectomy splenic and portal vein thrombi rate in HBV-related cirrhosis was 7％ (6/84) and that was 50％ (6/12) in schistosoma-related cirrhosis (t =13.4,P ＜ 0.01).Conclusions Careful evaluation before operation,skillful technique during operation are determinants for the safety of complex splenectomy.

A case of lymphomatoid granulomatosis with fever, pulmonary nodules, joint pain and superficial lymphadenopathy / 中华老年医学杂志

A 69-year-old man with fever,pulmonary nodules,joint pain and superficial lymphadenopathy was admitted to our hospital.The patient had a history of ten year hypertension.She smoked a pack of cigarettes daily for forty years and quitted for fifteen years.Family history of coronary heart disease,diabetes,cancer or other diseases was negative.Chest CT showed a nodule in the left lung lower lobe.Percutaneous lung biopsy revealed a large number of atypical B cell proliferation and infiltration which involved the vessel wall.The atypical B-cell phenotype and genotype was EBERs (+),CD20 (+),CD30 (+),CD15 (-).The patient was diagnosed as pulmonary lymphomatoid granulomatosis (LYG),an angiodestructive and angioinvasive lymphoproliferative disorder which is an Epstein-Barr virus associated B cell disorder with reactive T lymphocytes.The patient received six courses of chemotherapy.In this rare case,misdiagnosis of LYG often occurred due to the complex clinical presentation and non-specific imaging.Percutaneous or open lung biopsy is the main choice in the diagnosis of LYG.