A pediatric case of Bartonella henselae and Epstein Barr virus disease with bone and hepatosplenic involvement / Un caso pediátrico de enfermedad por Bartonella henselae y virus de Epstein Barr con afectación ósea y hepatoesplénica

Abstract Background: Cat scratch disease (CSD) is an infectious disorder caused by Bartonella henselae. The infection usually presents as local lymphadenopathy, fever, and mild constitutional symptoms. Systemic or severe disease is reported in 5-20% of patients with CSD. We report a case of disseminated CSD with osteomyelitis and hepatosplenic disease and a review of the literature. Case report: A previously healthy 5-year-old male presented with prolonged fever and abdominal pain, followed by low back pain. The serologic test showed positive IgG for B. henselae and IgM and IgG for Epstein Barr virus (EBV). The abdominal ultrasound showed hepatic and splenic hypoechoic lesions, and the magnetic resonance imaging (MRI) revealed spondylitis of the D6 vertebra. He received treatment with azithromycin for 4 weeks and rifampicin for 6 weeks. The symptoms disappeared, and the abdominal ultrasound was normal nine months later. Conclusions: Disseminated CSD is infrequent. The diagnosis requires a high rate of suspicion. Laboratory findings of Bartonella infection are often non-specific. Serologic test, polymerase chain reaction of B. henselae in blood or biopsied material of the site of involvement and imaging test can be performed to confirm the diagnosis. The diagnosis of disseminated B. henselae was based on significantly elevated blood titers, radiologic findings, and epidemiologic history. Treatment of CSD depends on the disease presentation. Azithromycin is used as a first-line agent for lymphadenopathy. The optimum treatment and its duration have not been established in atypical or complicated CSD, including patients with osteomyelitis and hepatosplenic disease.

Resumen Introducción: La enfermedad por arañazo de gato (EAG) es una patología infecciosa originada por Bartonella henselae. Habitualmente se presenta como linfadenopatía local, fiebre y síntomas constitucionales leves. El 5-20% de los pacientes con EAG manifiestan una afectación sistémica. Se presenta un caso de EAG diseminada, con osteomielitis y enfermedad hepatoesplénica, y se hace una revisión de la literatura sobre la EAG. Caso clínico: Paciente de sexo masculino de 5 años, previamente sano, que presentó fiebre prolongada y dolor abdominal, seguidos de dolor en la parte baja de la espalda. En la serología, presencia de IgG frente a B. henselae y de IgM e IgG frente al virus de Epstein-Barr. En la ecografía abdominal se observaron lesiones hipoecoicas en el hígado y el bazo, y la resonancia magnética mostró espondilitis de D6. Recibió tratamiento con azitromicina 4 semanas y rifampicina 6 semanas. Los síntomas desaparecieron y la ecografía abdominal a los 9 meses fue normal. Conclusiones: La EAG diseminada es infrecuente. El diagnóstico requiere un alto grado de sospecha. Los hallazgos de laboratorio en la infección por Bartonella suelen ser poco específicos. Para confirmar el diagnóstico pueden hacerse serología, reacción en cadena de la polimerasa para B. henselae en sangre o en biopsia de tejidos afectados, y estudios de imagen. El diagnóstico de EAG diseminada se basa en títulos elevados en la sangre, hallazgos radiológicos e historia epidemiológica. El tratamiento depende de la forma de presentación. En los casos de linfadenopatía se utiliza azitromicina. En la EAG atípica o complicada, que incluye osteomielitis y afectación hepatoesplénica, no están bien establecidos el tratamiento ni su duración.

Chidamide monotherapy for the treatment of hepatosplenic T-cell lymphoma: A case report / 中国输血杂志

【Objective】 To generate an efficacious therapeutic plan for patients suffered from Hepatosplenic T-cell lymphoma (HSTCL). 【Methods】 A patient diagnosed as HSTCL in January 2016 was observed, and chidamide monotherapy instead of traditional chemotherapy was applied due to the poor performance and infection complications of the patient. 【Results】 The patient has achieved amazing improvements after chidamide monotherapy. As we followed and evaluated recently, the patient has received partial remission and can keep durable remission for 4 years. 【Conclusion】 The study suggests that chidamide is a novel therapeuticchoice in patients with HSTCL.

Variations in the configuration of the celiac trunk among South Africans: clinical relevance / Variaciones en la configuración del tronco celíaco en sudafricanos: relevancia clínica

SUMMARY: The celiac trunk is the first major unpaired branch of the abdominal aorta found at the twelfth vertebral level (T12). It gives off branches supplying the spleen, liver and the stomach. However, the branching patterns of the celiac trunk tend to vary by population throughout the world. We sought to investigate the branching patterns of the celiac trunk in a South African Caucasian sample. The celiac trunk was assessed by visual observation in 66 dissected bodies comprised of both males (n= 30) and females (n=36). These samples were obtained at the School of Anatomical Sciences, University of the Witwatersrand, Johannesburg. The celiac trunk arose directly from the abdominal aorta in all cases, with none connected to the superior mesenteric artery. We observed celiac trunk trifurcation in 84.84 % of the sample, although a celiac trunk with four branches was observed in 10.61 %. Bifurcation into the common hepatic and splenic arteries forming a hepatosplenic trunk (2 females) or into the left gastric artery and splenic artery forming a splenogastric trunk (1 male) was also observed. The results are largely comparable with other studies in Caucasians, showing a high rate of celiac trunk trifurcation (above 75 %). Our sample exhibited fewer variations than reported in previous studies worldwide. Therefore, a larger study with more samples may be required in the future to ascertain all the existing celiac trunk branching patterns in the South African Caucasian population.

RESUMEN: El tronco celíaco es la primera rama principal de la parte abdominal de la aorta en el nivel de la duodécima vértebra torácica (T12), con ramas que irrigan el bazo, el hígado y el estómago. Sin embargo a nivel mundial, las ramificaciones del tronco celíaco tienden a variar según la población. En este estudio se investigaron los patrones de ramificación del tronco celíaco en una muestra caucásica sudafricana. El tronco celíaco se analizó mediante observación visual en 66 cuerpos disecados compuestos por hombres (n = 30) y mujeres (n = 36). Estas muestras se obtuvieron en la Facultad de Ciencias Anatómicas de la Universidad de Witwatersrand, Johannesburgo. El tronco celíaco surgió directamente de la parte abdominal de la aorta en todos los casos, sin que ninguno estuviera unido a la arteria mesentérica superior. Se observó trifurcación del tronco celíaco en el 84,84 % de la muestra, aunque en el 10,61 % se observó un tronco celíaco con cuatro ramas. También se observó bifurcación en las arterias hepática y esplénica común formando un tronco hepatoesplénico (2 mujeres) o en la arteria gástrica izquierda y la arteria esplénica formando un tronco esplenogástrico (1 hombre). Los resultados son comparables con otros estudios en caucásicos que muestran una alta tasa de trifurcación del tronco celíaco (mayor al 75%). Nuestra muestra presentó menos variaciones que las reportadas en estudios previos. Por lo tanto, es posible que se requieran estudios más amplios con más muestras en el futuro, para determinar todos los patrones de ramificación del tronco celíaco en la población caucásica sudafricana.

Expect the unexpected – Loss of surface CD3 on flow cytometry in hepatosplenic T-cell lymphoma: An eye opener

Hepatosplenic T-cell lymphoma (HSTCL) is a rare extranodal T-cell lymphoma that shows preferential sinusoidal infiltration of spleen and liver. It usually shows bright expression of surface CD3 (sCD3) with restriction for γδ-T cell receptors (TCR). We present a case of a 34-year-old male who presented with hepatosplenomegaly and B symptoms. His peripheral blood and bone marrow (BM) was involved by atypical lymphoid cells that were CD2+, CD7+, CD56+, cytoplasmic CD3+, and sCD3− on immunophenotyping by flow cytometry. As sCD3 is a lineage marker for T-cell lymphomas, the loss of sCD3 posed a diagnostic dilemma. However, typical pattern of sinusoidal BM and liver involvement by CD3+ cells and TCR gene rearrangement positivity led to final diagnosis of HSTCL. The differential diagnosis, workup, and clinical course of the case are discussed. To the best of our knowledge, only one case of de novo HSTCL with negative sCD3 has been reported before in the literature.

Morbidity of schistosomiasis mansoni in a low endemic setting in Ouro Preto, Minas Gerais, Brazil

Abstract INTRODUCTION: Despite the advances of disease control programs, severe forms of schistosomiasis are prevalent. The prevalence of the disease in areas frequented by tourists urges for permanent prevention and control. The aim of this study was to describe the morbidity of schistosomiasis in the district of Antônio Pereira, Ouro Preto, Minas Gerais, Brazil. METHODS: The proportion of positives was defined by Kato-Katz coproscopy and urinary POC-CCA rapid test. Hepatosplenic form was diagnosed using abdominal ultrasound. RESULTS: Out of 180 participants,97 were examined by Kato-Katz, with 4 (4.1%) being positive. Thirty-four (22.1%) out of 154 were positive by POC-CCA. Five (2.8%) of 177 examined by ultrasound had hepatosplenic form. One of them had undergone splenectomy. One (0.6%)participant had myeloradiculopathy. CONCLUSIONS: Severe forms of schistosomiasis are still prevalent in low endemic areas and should be thoroughly investigated.

Sensitivity and specificity of the circulating cathodic antigen rapid urine test in the diagnosis of Schistosomiasis mansoni infection and evaluation of morbidity in a low- endemic area in Brazil

Abstract INTRODUCTION: The Kato-Katz technique is the standard diagnostic test for Schistosoma mansoni infection in rural areas. However, the utility of this method is severely limited by the day-to-day variability in host egg excretion in the stool. In high-transmission areas, the point-of-care circulating cathodic antigen (POC-CCA) urine assay has proven to be a reliable test. However, investigations of the reliability of the POC-CCA assay in low-transmission regions are under way. This study aimed to evaluate the sensitivity and specificity of the POC-CCA assay and the morbidity of schistosomiasis in a low-endemic area in Brazil. METHODS: Pains City is a low-transmission zone for schistosomiasis. A total of 300 subjects aged 7-76 years were randomly selected for the POC-CCA cassette test. For S. mansoni diagnosis, three stool samples on six slides were compared with one urine sample for each subject. The sensitivity and specificity in the absence of a gold standard were calculated using latent class analysis. Clinical examinations and abdominal ultrasounds were performed in 181 volunteers to evaluate morbidity associated with schistosomiasis. RESULTS: The sensitivity and specificity of the Kato-Katz technique were 25.6% and 94.6%, respectively. By contrast, the sensitivity and specificity of the POC-CCA assay were 68.1% and 72.8%, respectively. Hepatosplenic schistosomiasis was diagnosed in two patients (1.1%). CONCLUSIONS: Overall, the POC-CCA urine assay proved to be a useful test for diagnosing S. mansoni in a low-endemic area in Brazil. Severe clinical forms of schistosomiasis can be present even in such low-endemic areas.

Alterações duodenais na hipertensão portal da esquistossomose mansônica / Duodenal changes in portal hypertension schistosomiasis

A Hipertensão Portal (HP) na Esquistossomose Mansônica (EM) tem, como repercussão, varizes esofagogástricas, gastropatia, colopatia, sendo pouco estudadas as repercussões duodenais já evidenciadas em cirróticos. As lesões duodenais observadas na esquistossomose podem ser secundárias à HP (Duodenopatia da Hipertensão Portal - DHP) ou à agressão parasitária, duodenite, sendo importante saber a causa das lesões duodenais em pacientes esquistossomóticos a fim de realizar uma abordagem terapêutica com maior segurança e especificidade nos pacientes. Objetivos: avaliar a ocorrência e as características de alterações duodenais em pacientes com esquistossomose mansônica na forma hepatoesplênica; a frequência da DHP endoscópica e histológica; verificar a associação da DHP endoscópica com DHP histológica; presença de GHP; presença e grau das VE; presença de HDA; presença de circulação colateral; diâmetro longitudinal do baço e padrão ultrassonográfico da Fibrose Periportal (FPP). Métodos: avaliados 65 pacientes portadores de EM, estudo tipo série de casos. Preencheram critérios de inclusão 50 pacientes, com a forma hepatoesplênica, com ou sem história de HDA, exames realizados (parasitológico de fezes, marcadores virais para VHB e VHC, bioquímica, função e testes hepáticos, hemograma, ultrassonografia e endoscopia digestiva alta - EDA com biopsia de estômago e duodeno). Resultados: a média de idade de 50, 58 anos, sendo 29 (58%) do sexo feminino: apenas 8/50 (16%) tinham história de HDA. Lesões duodenais foram observadas à EDA em 47/50 (94%) dos pacientes, sendo, as mais frequentes, o eritema em 16/50 (32%), a congestão em 9/50 (18%) e associados (eritema e congestão) em 16/50 (32%). DHP à EDA foi observada em 56% (28/50) dos pacientes, 53,6% (15/28) com intensidade acentuada. DHP à histologia foi evidenciada em 62% (31) dos pacientes. Houve associação do diagnóstico endoscópico e histológico da DHP em 23/28 (82,1%) pacientes (p=0,001). Houve associação entre DHP endoscópica com diâmetro longitudinal do baço (P=0,045) e com padrão da FPP (p=0,038). Não houve associação entre DHP endoscópica com a GHP (p=0,569), com presença e grau das VE (p=0,444; p=0,350), nem com história de HDA (p=0,116). Conclusão: na EM forma hepatoesplênica, as lesões duodenais são bastante frequentes, sendo as mais encontradas o eritema, eritema e congestão, a erosão e a congestão. A DHP foi evidenciada à EDA em 56%, histologia em 62% dos casos. Houve concordância do diagnóstico endoscópico com histológico em 82,1%. Não houve significância estatística entre DHP endoscópica com GHP, VE e HDA. Houve significância entre o padrão da FPP com a presença de DHP à endoscopia e relação inversa entre o diâmetro longitudinal do baço e a presença de DHP à endoscopia.

Despite of the fact that Portal Hypertension (PH) in Mansoni Schistosomiasis can cause esophagogastric varices, gastropathy and colopathy, its duodenal repercussions, already evidenced in cirrhotic patients, have been little studied. The duodenal lesions seen in schistomiasis may be secondary to PH (Portal Hypertensive Duodenopathy -PHD) or parasitic aggression, duodenitis. It is particularly important to understand the cause of duodenal lesions in schistosomotic patients in order to approach their treatment with greater security and specificity. Aims: The present study has the following aims: to evaluate the occurrence of alterations and the type of duodenal lesions in patients suffering from the hepatosplenic form of mansoni schistosomiasis; to determine the frequency of PHD by histological and endoscopic means; and to associate the presence of PHD on endoscopy with the following: PHD on histology; the presence of portal hypertensive gastropathy (PHG); the presence and degree of esophageal varices, the presence of upper gastrointestinal bleeding; the longitudinal diameter of the spleen, and the pattern of periportal fibrosis. Patients and methods: Sixty- -five patients with mansoni schistomiasis were studied in a case series study. Fifty patients met the inclusion criteria; all of them had the hepatosplenic form, with or without a history of upper gastrointestinal bleeding, and underwent a series of investigations, namely parasitological feces examination, viral markers for HVB and HCV, ultrasound and endoscopy of the upper gastrointestinal tract with biopsy of the stomach and duodenum. Results: The mean age was 50.58 yr (range, 26-70 yr), 29 (58%) being females, and only eight of the 50 (16%) had a history of bleeding of the digestive tract. Duodenal lesions were observed on endoscopy of the upper gastrointestinal tract in 47 (94%) patients, the most frequent ones being erythema in 16 (32%), congestion in 9 (18%) and both erythema and congestion in 16 (32%). Portal hypertensive duodenopathy on endoscopy was observed in 28 (56%), and in 15 of these (53.6%) it was particularly intense. Portal hypertensive duodenopathy was evidenced on histology in 31 (62%) patients. In 23 of 28 (82.1%) patients the histological diagnosis of PHD was associated with the endoscopic diagnosis (p=0.001). Portal hypertensive duodenopathy was associated with the longitudinal diameter of the spleen (p=0.045) and with the pattern of the periportal fibrosis (p=0.038). There was no association between PHD on endoscopy with PHG (p=0.569), the presence and degree of esophageal varices (p=0.444; p=0.350) or a history of upper gastrointestinal bleeding (p=0.116). Conclusion: on the basis of the results of the present study, it is concluded that in the hepatosplenic form of mansoni schistosomiasis duodenal lesions are extremely frequent, the most common ones being erythema, erythema and congestion, erosion and congestion. Portal hypertensive duodenopathy was evidenced endoscopically in 56% and histologically in 62% of the cases, with an 82.1% agreement between the endoscopic and histological diagnoses. There was no statistically significant association between PHD on endoscopy and PHG, esophageal varices or upper gastrointestinal bleeding. There was a significant association between the pattern of periportal fibrosis in the presence of PHD on endoscopy and the inverse relationship between the longitudinal diameter of the spleen and the presence of PHD on endoscopy.

Participação da via do Hedgehog na fibrose hepática da esquistossomose mansoni humana e murina experimental / Participation via the Hedgehog in liver fibrosis of human schistosomiasis and experimental murine

INTRODUÇÃO/OBJETIVO: A esquistossomose mansonica é causa importante de fibrose hepática e hipertensão porta em regiões tropicais, e a patogênese da fibrose não está bem esclarecida. Como a via do hedgehog e um dos seus genes alvos, a osteopontina, estão envolvidos em fibroses hepáticas de outras etiologias o objetivo foi investigar a ativação destas vias na esquIsitossomose humana e murina experimental, no intuito de verificar o seu envolvimento no desenvolvimento da forma hepatoesplênica da esquistossomose mansonica (FHE). MATERIAL E MÉTODOS: 87 biópsias em cunha de fígados de pacientes com FHE submetidos a cirurgia e fragmentos de fígado de camundongos suiços infectados com Schistosoma mansoni foram submetidos a métodos imunohistoquímicos e de biologia molecular para avaliar a expressão de ligantes hedgehog (Ihh, Shh), receptor Patched, fatores de transcrição Gli 1 e 2...

inglês: BACKGROUND AND AIMS: Schistosomiasis is a major cause of liver fibrosis and portal hypertension in tropical regions, and the pathogenesis of fibrosis is not well established. As hedgehog pathway and one of its target genes, osteopontin, are involved in liver fibrosis of other etiologies our aims were to investigate the activation of these pathways in human and experimental murine schistosomiasis, in an attempt to verify their involvement in the development of hepatosplenic schistosomiasis mansoni (HS). METHODS: 87 wedge liver biopsies of patients with HS submitted to surgery and liver fragments Swiss mice infected with Schistosoma mansoni were submitted to immunohistochemistry and molecular biology methods to evaluate the expression of hedgehog ligands (Ihh, Shh), patched receptor , Gli transcription factors and osteopontin...

Participação da via do Hedgehog na fibrose hepática da esquistossomose mansoni humana e murina experimental / Participation via the Hedgehog in liver fibrosis of human schistosomiasis and experimental murine

INTRODUÇÃO/OBJETIVO: A esquistossomose mansonica é causa importante de fibrose hepática e hipertensão porta em regiões tropicais, e a patogênese da fibrose não está bem esclarecida. Como a via do hedgehog e um dos seus genes alvos,a osteopontina, estão envolvidos em fibroses hepáticas de outras etiologias o objetivo foi investigar a ativação destas vias na esquIsitossomose humana e murina experimental, no intuito de verificar o seu envolvimento no desenvolvimento da forma hepatoesplênica da esquistossomose mansonica (FHE). MATERIAL E MÉTODOS: 87 biópsias em cunha de fígados de pacientes com FHE submetidos a cirurgia e fragmentos de fígado de camundongos suiços infectados com Schistosoma mansoni foram submetidos a métodos imunohistoquímicos e de biologia molecular para avaliar a expressão de ligantes hedgehog (Ihh, Shh), receptor Patched, fatores de transcrição Gli 1 e 2 e osteopontina. Osteopontina sérica e ligante Shh do hedgehog foram avaliados em camundongos suíços infectados e os de osteopontina em camundongos CBA/J infectados e em pacientes com FHE e forma hepatointestinal da esquistossomose. In vitro foi avaliado o efeito de antígeno solúvel do ovo (SEA) em células de Kuppfer, células estreladas, macrófagos, colangiócitos e células endoteliais sinusoidais hepáticas. A relação com a via da IL-13 foi avaliada em camundongos geneticamente deficientes ou hiperexpressando a citocina. Foi avaliado in vitro se a IL-13 induz ligantes hedghog ou ativação da via em células de Kuppfer. RESULTADOS: Os resultados mostraram: (a) aumento expressão de ligantes Ihh, de fatores de transcrição Gli2 e de osteopontina no fígado de camundongos suíços infectados com Schistosoma mansoni, aumento de shh e osteopontina no plasma de camundongos suíços e de osteopontina no plasma de camundongos CBA/J infectados com S. mansoni; (b) aumento na expressão de Ihh, Shh, Gli1 e 2, receptor Patched e de osteopontina no fígado de pacientes com esquistossomose e aumento da osteopontina sérica em pacientes com a FHE; (c) A expressão de ligantes hedgehog e de Gli2 foi observada em macrófagos, células estreladas, ductos biliares e células endoteliais, e a de osteoponina em ductos biliares,macrófagos e células estreladas/miofibroblastos; (d) correlação positiva entre ativação do hedgehog (Gli2 e osteopontina) e fibrose, no modelo murino experimental e nos pacientes; nestes a correlação também foi observada com o grau de fibrose classificada pelo ultrassom e com a hipertensão porta; (e) Inibição in vitro do hedgehog com ciclopamina e vismodegib ou por nocauteamento condicional de receptor Smoothened bloqueou a ativação alternativa de macrófagos e inibiu a angiogênese a partir de células endoteliais sinusoidais hepáticas; (f) que o bloqueio da via da IL-13 reduziu e a hiperexpressão aumentou a ativação da via do hedgehog e IL-13 diretamente induziu, in vitro,produção de ihh em células de Kupffer de camundongos e de humanos, demonstrando a inter-relação das duas vias...

BACKGROUND AND AIMS: Schistosomiasis is a major cause of liver fibrosis and portal hypertension in tropical regions, and the pathogenesis of fibrosis is not well established. As hedgehog pathway and one of its target genes, osteopontin, are involved in liver fibrosis of other etiologies our aims were to investigate the activation of these pathways in human and experimental murine schistosomiasis, in an attempt to verify their involvement in the development of hepatosplenic schistosomiasis mansoni (HS). METHODS: 87 wedge liver biopsies of patients with HS submitted to surgery and liver fragments Swiss mice infected with Schistosoma mansoni were submitted to immunohistochemistry and molecular biology methods to evaluate the expression of hedgehog ligands (Ihh, Shh), patched receptor , Gli transcription factors and osteopontin. Serum osteopontin and Shh were evaluated in infected Swiss mice and osteopontin was evaluated in serum of infected CBA/J mice and plasma from patients with hepatointestinal and HS forms of schistosomiasis. The effect of soluble egg antigen (SEA) on Kuppfer cells, stellate cells, macrophages, cholangiocytes and liver sinusoidal endothelial cells was evaluated in vitro. Relationship with IL-13 pathway was evaluated in mice genetically deficient or with hyperexpression of this cytokine. Whether IL-13 induces production of ligands and/or activation of the hedgehog pathway in Kuppfer cells was evaluated in vitro. RESULTS: Results demonstrated: (a) increased expression of Ihh, transcription factor Gli2 and osteopontin in the livers of Swiss mice infected with S. mansoni, increased plasma levels of shh and osteopontin in infected Swiss mice and increased osteopontin in plasma of S. mansoni infected CBA/J mice; (b) increased expression of ihh, shh, Gli1 and 2, patched and osteopontin receptor in the liver of patients with schistosomiasis and increased serum osteopontin in patients with HS; (c) expression of hedgehog ligands and Gli2 was observed in macrophages, stellate cells, endothelial cells and bile duct and expression of osteopontin was detected in macrophages and stellate/myofibroblast cells; (d) positive correlation between activation of the hedgehog (Gli2 and osteopontin) and fibrosis in experimental murine model and in patients; these correlation was also observed with the degree of fibrosis classified by ultrasound and with portal hypertension; (e) in vitro inhibition of hedgehog pathway with cyclopamine or vismogedib or by conditional knockout of Smoothened co-receptor blocked the alternative activation of macrophage and inhibited angiogenesis in liver sinusoidal endothelial cells; (f) reduction of IL-13 pathway or IL-13 over-expression respectively reduced or increased the activation of the hedgehog pathway and IL-13 directly induced in vitro ihh production in Kupffer cells from mice and human, demonstrating a cross-talk between the two pathways...

A case report of childhood hepatosplenic γδT cell lymphoma and literature review / 中华实用儿科临床杂志

Objective To investigate the clinic characteristics of hepatosplenic γδT cell lymphoma (γδ HSTCL) and analyze the important role of morphology,immunology,cytogenetics and molecular(MICM) in the early diagnosis of γδHSTCL.Methods The clinical features of MICM of a rare case of childhood γδHSTCL had been analyzed,and the related literatures were reviewed.Results Bone marrow aspiration showed approximately 0.365 abnormal cells infiltrated.The immune phenotype analysis displayed 0.245 of the nuclear cells were abnormal ones,which possessed CD2/CD3/CD7/CD19/CD3s/cCD3 and TCRγδ expression.The immunohistochemical staining of spleen tissues showed clusters of differentiation (CD) 3/CD45 RO/CD8/Cytotoxic-granule associated protein (TIA-1)/leukocyte common antigen (LCA)/Granzyme B positive,CD4/CD20/T cell receptor (TCR) β/CD79 α/CD30/terminal deoxynucleotidy transferase (TdT) /CD10/ myeloperoxidase (MPO)/anaplasticlymphoma kinase (ALK) negative and 80.00％ of tumor cells displayed Ki-67 positive.The detection of spleen tissues by flow cytometry displayed that 72.26％ of the karyocytes were lymphocytes,with 88.90％ T lymphocytes.And 92.09％ of the T lymphocytes showed Ki-67/CD7dim,TCRγδ/CD2/CD8/CD34 positive,and TCRαβ/CD5/CD4/CD56 negative.And about 59.16％ of these karyocytes were abnormally matured T lymphocytes.The cytogenetic karyotype of bone marrow showed 46,XY.The detection of T cell receptor gene rearrangement showed TCRβ,TCRγ and TCRδ positive.The patient had been given prednisone of 60 mg/m2 when the diagnosis of γδHSTCL affirmed.But his parents discontinued chemotherapy for him and died.Conclusions MICM has shown a significant value in the diagnosis of γδHSTCL,a rare lymphoma in childhood.

Revisiting the concept of hepatosplenic schistosomiasis and its challenges using traditional and new tools

Different aspects of hepatosplenic schistosomiasis are revisited here. Manson's schistosomiasis causes periportal fibrosis and portal hypertension in approximately 6% of infected subjects, usually with preservation of their hepatic function. The assessment of liver involvement is of major importance in determining the prognosis and risk of complications from schistosomiasis, such as upper digestive bleeding secondary to variceal rupture. For many years, the diagnosis of hepatosplenic schistosomiasis and liver fibrosis was made by abdominal palpation and the finding of liver and/or spleen enlargement. However, there is no consensus regarding the clinical parameters of the liver and spleen to be considered in this physical evaluation. For the last three decades, abdominal ultrasound (US) has become the best imaging technique to evaluate liver fibrosis caused by schistosomiasis mansoni. However, US is a subjective procedure and is therefore examiner-dependent. Magnetic resonance imaging (MRI) findings have provided valuable information in addition to ultrasound and clinical examination. The combination of a comprehensive history and physical examination, basic laboratory tests (a stool examination for Schistosoma mansoni eggs and a blood cell count), biomarkers for liver fibrosis/portal hypertension and imaging methods seem to offer the best approach for evaluating patients with this disease. In situations where research is involved or in patients with severe disease, MRI may be considered.

Thrombocytopenia as a surrogate marker of hepatosplenic schistosomiasis in endemic areas for Schistosomiasis mansoni

Introduction This study aimed to evaluate whether a low platelet count is a good surrogate marker of hepatosplenic schistosomiasis (HSS) in a rural area of Brazil. A small district in southeastern Brazil, with a population of 1,543 individuals and a 23% prevalence of schistosomiasis, was selected for this investigation. Methods In July 2012, 384 volunteers were subjected to clinical, ultrasonography (US), and laboratory examinations, including stool sample analysis. The HSS patients were classified into four groups: Group 1 consisted of patients with a spleen >13cm and liver fibrosis; Group 2 consisted of patients with a palpable spleen and spleen>13cm measured by US; Group 3 consisted of patients with a spleen >13cm measured by US; and Group 4 consisted of patients with a palpable spleen. Results Eight patients were in Group 1 (2.1%), twenty-one were in Group 2 (5.5%), eight were in Group 3 (2.1%), and eighteen were in Group 4 (4.7%). A significant difference in the mean platelet counts was observed between the patients with and without HSS (p<0.01). Based on the receiver operating characteristic (ROC) curve (platelet count <143,000/mm3), the sensitivity was greater than 92% in all groups, and the specificity varied from 44.4% to 75%. Conclusions We concluded that in endemic areas, thrombocytopenia demonstrates good sensitivity for detecting HSS and may be used as a screening tool to identify patients with HSS. .

Métodos de imagem no diagnóstico de esquistossomose hepatoesplânica / Imaging techniques in the diagnosis of hepatosplenic schistosomiasis

A esquistossomose constitui grave problema de saúde pública, com mais de 200 milhões de infectados no mundo. Deste total, cerca de 10% desenvolvem a forma hepatoesplênica da doença caracterizada por fibrose periportal e hipertensão porta. No passado, o diagnóstico da esquistossomose hepatoesplênica (EHE) era realizado por métodos invasivos como esplenoportografia e biópsia hepática. O estudo das alterações no sistema porta e da morfologia hepática e esplênica com métodos de imagem representou um avanço significativo no diagnóstico da doença. Os métodos mais utilizados atualmente são a ultrassonografia abdominal, a ressonância nuclear magnética e a tomografia computadorizada de abdomen. O objetivo do presente artigo é abordar as principais contribuições de cada método no diagnóstico da EHE.

Schistosomiasis is a serious public health problem with over 200 million infected worldwide. Nearly 10% of infected individuals develop the hepatosplenic form of the disease characterized by periportal fi brosis and portal hypertension. In the past, the diagnosis of hepatosplenic schistosomiasis (HHS) was performed by invasive methods such as liver biopsy and splenoportography. The study of changes in portal system and morphological aspects of liver and spleen with imaging techniques represented a significant advance in the diagnosis of the disease. The most widely used techniques are abdominal ultrasonography, magnetic resonance imaging and computed tomography of the abdomen. The aim of this article is to discuss the main contribution of each technique in the diagnosis of HHS.

A Case of Hepatosplenic T-cell Lymphoma Diagnosed by Bone Marrow Examination

Hepatosplenic T-cell lymphoma (HSTL) is a condition in which lymphoma cells infiltrate the sinusoids of the liver, spleen, and bone marrow, without lymph node involvement. We encountered a case of hepatosplenic T-cell lymphoma in a Vietnamese woman. The patient was hospitalized with epigastric pain and nausea. Splenomegaly and multiple poorly defined, low-attenuating nodular lesions in the liver were visualized on computed tomography (CT), and thrombocytopenia was noted. The lymph nodes were not significantly enlarged. Splenic biopsy could not be performed because of severe thrombocytopenia. Neoplastic lymphoid cells were present in bone marrow aspirates. Bone marrow sections revealed infiltration of CD3(+) and CD20(-) neoplastic lymphoid cells in the sinusoids. A clonality assay (IdentiClone T-Cell Receptor Delta Gene Clonality Assay; Invivoscribe Technologies, USA) showed gene rearrangements in the T-cell receptor delta gene. Thus, we made a confirmatory diagnosis of HSTL. When splenic biopsy is not available, bone marrow aspirates and clonality assessment may become useful diagnostic tools.

Variant Anatomy of the Celiac Trunk and its Branches / Variación Anatómica del Tronco Celíaco y sus Ramas

The knowledge of abdominal vascular anatomy is very important for surgeons and radiologists to perform many of the clinical diagnostic evaluations. The celiac trunk, one of the branches of the abdominal aorta shows numerous variations in its branching pattern and its branches. The present study is to demonstrate the rare branching pattern of celiac trunk, into hepatogastric and hepatosplenic trunks. The hepatogastric trunk divided into left gastric and accessory left hepatic arteries and the hepatosplenic into common hepatic and splenic artery. The inferior phrenic artery was arising from left gastric artery. The gastroduodenal artery divided into right gastroepiploic artery and a common trunk for right gastric and anterior superior pancreaticoduodenal artery. The posterior superior pancreatico-duodenal artery was arising directly from the gastroduodenal artery and supraduodenal from proper hepatic artery. The variant anatomy of the celiac trunk as found in the present case may be clinically significant during invasive procedures like angiography, chemotherapy, chemoembolization and other surgical conditions of the abdomen.

El conocimiento de la anatomía vascular abdominal es importante para los cirujanos y radiólogos para realizar las evaluaciones de diagnóstico clínico. El tronco celíaco, una de las ramas de la parte abdominal de la aorta presenta numerosas variaciones en su patrón de ramificación y sus ramas colaterales. El presente estudio muestra un raro patrón de ramificación del tronco celíaco, en los troncos hepatogástrico y hepatoesplénico. El tronco hepatogástrico se dividió en las arterias gástrica izquierda y accesoria hepática izquierda, mientras que el hepatoesplénico en las arterias hepática común y esplénica. La arteria frénica inferior se originó desde la arteria gástrica izquierda. La arteria gastroduodenal se dividió en la arteria gastroepiploica derecha y en un tronco común para las arterias gástrica y pancreaticoduodenal anterosuperior. La arteria pancreaticoduodenal posterosuperior se originó directamente de la arteria gastroduodenal y la arteria supraduodenal de la arteria hepática. La variaciónanatómica del tronco celíaco presente en este caso puede ser clínicamente significativa durante procedimientos invasivos como la angiografía, quimioterapia, quimioembolización y afecciones quirúrgicas del abdomen.

A Case of Hepatosplenic T-cell Lymphoma with Colonic Involvement

Hepatosplenic T-cell lymphoma is a rare histologic type of peripheral T-cell lymphoma that usually presents with hepatosplenomegaly, B-symptoms, and often with rapid and aggressive progression. There have been no previous domestic reports of a hepatosplenic T-cell lymphoma involving the colon. We report a case of a 61-year old man with colonic invasion of a hepatosplenic T-cell lymphoma. He presented the sudden onset of fever accompanied by severe pancytopenia and rapid liver enzyme elevation without evidence of lymphadenopathy. Multiple tiny erosions were noted during colonoscopy. In addition, many immature lymphocytes with vague nucleoli, abundant eosinophilic cytosol, and nuclei of small-to-medium size were seen within the sinusoids on liver biopsy. A similar pattern existed on colonic mucosa obtained from colonoscopic biopsy. Thus, with the aid of such a specific clinical presentation and the results of immunohistochemical staining, we made a definitive diagnosis of hepatosplenic T-cell lymphoma with colonic involvement.

A Case of Hepatosplenic T-cell Lymphoma with Colonic Involvement

Hepatosplenic T-cell lymphoma is a rare histologic type of peripheral T-cell lymphoma that usually presents with hepatosplenomegaly, B-symptoms, and often with rapid and aggressive progression. There have been no previous domestic reports of a hepatosplenic T-cell lymphoma involving the colon. We report a case of a 61-year old man with colonic invasion of a hepatosplenic T-cell lymphoma. He presented the sudden onset of fever accompanied by severe pancytopenia and rapid liver enzyme elevation without evidence of lymphadenopathy. Multiple tiny erosions were noted during colonoscopy. In addition, many immature lymphocytes with vague nucleoli, abundant eosinophilic cytosol, and nuclei of small-to-medium size were seen within the sinusoids on liver biopsy. A similar pattern existed on colonic mucosa obtained from colonoscopic biopsy. Thus, with the aid of such a specific clinical presentation and the results of immunohistochemical staining, we made a definitive diagnosis of hepatosplenic T-cell lymphoma with colonic involvement.

Serum hyaluronan and collagen IV as non-invasive markers of liver fibrosis in patients from an endemic area for schistosomiasis mansoni: a field-based study in Brazil

Non-invasive markers of fibrosis have been used to diagnose liver fibrosis in a variety of diseases. Hyaluronic acid (HA) and collagen IV (C-IV) levels were measured in the sera of patients from an endemic area for schistosomiasis in Brazil to diagnose and to rank the intensity of liver fibrosis. Seventy-nine adult patients with schistosomiasis, in the age range of 21-82 years (49 ± 13.4) were submitted to clinical and ultrasonographic examinations. Ultrasound was employed to diagnose and categorise liver fibrosis according to World Health Organization patterns. Serum HA and C-IV levels were measured using commercial ELISA kits. Ultrasound revealed six patients with intense liver fibrosis, 21 with moderate, 23 with light and 29 without. Serum HA was able to separate individuals with fibrosis from those without (p < 0.001) and light from intense fibrosis (p = 0.029), but C-IV was not (p = 0.692). The HA diagnostic accuracy for fibrosis was 0.89. The 115.4 ng/mL cut-off level diagnosed patients with fibrosis (sensitivity 0.98, specificity 0.64). HA correlated positively with portal hypertension. Periportal fibrosis (subjective evaluation), age and collateral circulation predicted HA increase. In conclusion, we propose that serum HA can be used to identify patients with liver fibrosis in an endemic area for schistosomiasis mansoni in Brazil.

Ultrasound in schistosomiasis mansoni

We reviewed ultrasound features in patients with schistosomiasis mansoni. The alterations that we observed in acute and hepatosplenic schistosomiasis are described. The advantages and disadvantages of using ultrasound patterns in the evaluation of liver fibrosis are discussed. Other diseases that are important in the differential diagnosis of schistosomal liver fibrosis are presented. Ultrasound is an effective and flexible diagnostic tool in the evaluation of a variety of diseases. It presents no harmful effects to patients, allowing non-invasive studies in hospitalized patients and in other facilities.

Schistosomiasis control program in the state of Minas Gerais in Brazil

The Schistosomiasis Control Program (PCE) was implemented in Minas Gerais (MG) in 1984. In 1999, the state started the investigation and control of schistosomiasis in 470 municipalities. The aim of the present paper is to report the evolution of this Program from 1984-2007. The program included a coproscopic survey carried out in the municipalities of known endemic areas using a quantitative method. Positives were treated with praziquantel and given a program of health education. The information for this study was obtained from data collected and stored by the Health State Department. From 2003-2007, 2,643,564 stool examinations resulted in 141,284 positive tests for Schistosoma mansoni (5.3 percent). In the first evaluation after treatment, a decrease in the number of municipalities with prevalence over 10 percent was documented. In one village, selected for a more detailed evaluation, the percentage of positive tests decreased from 14.9 percent in the baseline survey to 5.3 percent after treatment. A reference centre for patients with severe schistosomiasis was created in Belo Horizonte, MG. Based on our findings, we believe that the implementation of PCE in MG is on the right path and in due time these new initiatives will provide desirable results.