Outcome of children and adolescents with lymphoblastic lymphoma.

INTRODUCTION: lymphoblastic lymphoma (LBL) is the second most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course of children and adolescents with LBL treated at a tertiary center. METHODS: this is a retrospective cohort study of 27 patients aged 16 years or less with LBL admitted between January 1981 and December 2013. Patients received intensive chemotherapy regimen derived from acute lymphoblastic leukemia (ALL) therapy. Diagnosis was based on biopsy of tumor and/or cytological examination of pleural effusions. The overall survival was analyzed using the Kaplan-Meier method. RESULTS: the median age at diagnosis was 11.6 years (interquartile range, 4.6-13.8). LBL had T cell origin in 16 patients (59%). The most common primary manifestation in T-cell LBL was mediastinum involvement in 9 patients (56%). Intra-abdominal tumor was the major site of involvement in patients with pB-LBL. Most patients had advanced disease (18 patients - 67%) at diagnosis. Twenty-four patients (89%) achieved complete clinical remission. After a median follow-up of 43 months (interquartile range, 6.4-95), 22 patients (81%) were alive in first complete remission. Five children (18.5%) died, three of them soon after admission and two after relapsing. The probability of survival at five years for 20 patients with de novo LBL was 78% (SD 9.4). CONCLUSION: our findings confirm the favorable prognosis of children with LBL with an intensive chemotherapy regimen derived from ALL therapy.

Outcome of children and adolescents with lymphoblastic lymphoma / Evolução de crianças e adolescentes com linfoma linfoblástico

SUMMARY Introduction: lymphoblastic lymphoma (LBL) is the second most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course of children and adolescents with LBL treated at a tertiary center. Methods: this is a retrospective cohort study of 27 patients aged 16 years or less with LBL admitted between January 1981 and December 2013. Patients received intensive chemotherapy regimen derived from acute lymphoblastic leukemia (ALL) therapy. Diagnosis was based on biopsy of tumor and/or cytological examination of pleural effusions. The overall survival was analyzed using the Kaplan-Meier method. Results: the median age at diagnosis was 11.6 years (interquartile range, 4.6-13.8). LBL had T cell origin in 16 patients (59%). The most common primary manifestation in T-cell LBL was mediastinum involvement in 9 patients (56%). Intra-abdominal tumor was the major site of involvement in patients with pB-LBL. Most patients had advanced disease (18 patients - 67%) at diagnosis. Twenty-four patients (89%) achieved complete clinical remission. After a median follow-up of 43 months (interquartile range, 6.4-95), 22 patients (81%) were alive in first complete remission. Five children (18.5%) died, three of them soon after admission and two after relapsing. The probability of survival at five years for 20 patients with de novo LBL was 78% (SD 9.4). Conclusion: our findings confirm the favorable prognosis of children with LBL with an intensive chemotherapy regimen derived from ALL therapy.

RESUMO Objetivos: linfoma linfoblástico (LL) é o segundo subtipo mais comum de linfoma não Hodgkin em crianças. O objetivo deste estudo foi caracterizar a evolução clínica de crianças e adolescentes com LL em um centro terciário. Métodos: estudo de coorte retrospectivo de 27 pacientes com idade de até 16 anos com LL admitidos entre janeiro de 1981 e dezembro de 2013. Os pacientes foram tratados de acordo com o protocolo de tratamento para leucemia linfoblástica aguda (LLA). O diagnóstico foi baseado em biópsia do tumor e/ou no exame citológico de derrame pleural. A sobrevida global foi analisada pelo método de Kaplan-Meier. Resultados: a média de idade ao diagnóstico foi de 11,6 anos (variação interquartil, 4,6-13,8). LL de células T foi identificado em 16 pacientes (59%) e a manifestação primária mais comum foi o acometimento mediastinal em 9 pacientes (56%). Tumor intra-abdominal foi a manifestação clínica principal nos pacientes com LL de células pré-B. A maioria dos pacientes apresentava doença avançada (18 pacientes - 67%) ao diagnóstico. Vinte e quatro pacientes (89%) alcançaram remissão clínica completa. Após um período de acompanhamento médio de 43 meses (intervalo interquartil, 6,4-95), 22 pacientes (81%) continuam vivos em primeira remissão clínica completa. Cinco crianças (18,5%) morreram, três delas logo após a admissão e duas após recidiva. A probabilidade de sobrevida em cinco anos para 20 pacientes com LL de novo foi de 78% (SD 9.4). Conclusão: nossos resultados confirmam o prognóstico favorável de crianças com LL tratadas com regime de quimioterapia intensiva derivado da terapia de LLA.

Outcome of children and adolescents with lymphoblastic lymphoma.

INTRODUCTION: lymphoblastic lymphoma (LBL) is the second most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course of children and adolescents with LBL treated at a tertiary center. METHODS: this is a retrospective cohort study of 27 patients aged 16 years or younger with LBL admitted between January 1981 and December 2013. Patients were treated according to the therapy protocol used for acute lymphoblastic leucemia. Diagnosis was based on biopsy of tumor and/or cytological examination of pleural effusions. The overall survival was analyzed using the Kaplan-Meier method. RESULTS: the median age at diagnosis was 11.6 years (interquartile range, 4.6- 13.8). LBL had T-cell origin in 16 patients (59%). The most common primary manifestation in T-cell LBL was mediastinal involvement, in 9 patients (56%). Intra-abdominal tumor was the major site of involvement in patients with precursor B-LBL. Most patients had advanced disease (18 patients - 67%) at diagnosis. Twenty-four patients (89%) achieved complete clinical remission. After a median follow-up of 43 months (interquartile range, 6.4-95), 22 patients (81%) were alive in first complete remission. Five children (18.5%) died, three of them soon after admission and two after relapsing. The probability of survival at five years for 20 patients with de novo LBL was 78% (SD 9.4). CONCLUSION: our findings confirm the favorable prognosis of children with LBL with an intensive chemotherapy regimen derived from ALL therapy.

Outcome of children and adolescents with lymphoblastic lymphoma / Evolução de crianças e adolescentes com linfoma linfoblástico

Summary Introduction: lymphoblastic lymphoma (LBL) is the second most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course of children and adolescents with LBL treated at a tertiary center. Methods: this is a retrospective cohort study of 27 patients aged 16 years or younger with LBL admitted between January 1981 and December 2013. Patients were treated according to the therapy protocol used for acute lymphoblastic leucemia. Diagnosis was based on biopsy of tumor and/or cytological examination of pleural effusions. The overall survival was analyzed using the Kaplan-Meier method. Results: the median age at diagnosis was 11.6 years (interquartile range, 4.6- 13.8). LBL had T-cell origin in 16 patients (59%). The most common primary manifestation in T-cell LBL was mediastinal involvement, in 9 patients (56%). Intra-abdominal tumor was the major site of involvement in patients with precursor B-LBL. Most patients had advanced disease (18 patients – 67%) at diagnosis. Twenty-four patients (89%) achieved complete clinical remission. After a median follow-up of 43 months (interquartile range, 6.4-95), 22 patients (81%) were alive in first complete remission. Five children (18.5%) died, three of them soon after admission and two after relapsing. The probability of survival at five years for 20 patients with de novo LBL was 78% (SD 9.4). Conclusion: our findings confirm the favorable prognosis of children with LBL with an intensive chemotherapy regimen derived from ALL therapy.

Resumo Objetivos: linfoma linfoblástico (LL) é o segundo subtipo mais comum de linfoma não Hodgkin em crianças. O objetivo deste estudo foi caracterizar a evolução clínica de crianças e adolescentes com LL em um centro terciário. Métodos: estudo de coorte retrospectivo de 27 pacientes com idade de até 16 anos com LL admitidos entre janeiro de 1981 e dezembro de 2013. Os pacientes foram tratados de acordo com o protocolo de tratamento para leucemia linfoblástica aguda (LLA). O diagnóstico foi baseado em biópsia do tumor e/ou no exame citológico de derrame pleural. A sobrevida global foi analisada pelo método de Kaplan-Meier. Resultados: a média de idade ao diagnóstico foi de 11,6 anos (variação interquartil, 4,6-13,8). Linfoma linfoblástico de células T foi identificado em 16 pacientes (59%) e a manifestação primária mais comum foi o acometimento mediastinal (56%). Tumor intra-abdominal foi a manifestação clínica principal nos pacientes com LL de células pré- -B. A maioria dos pacientes apresentava doença avançada (18 pacientes, 67%) ao diagnóstico. Vinte e quatro pacientes (89%) alcançaram remissão clínica completa. Após um período de acompanhamento médio de 43 meses (intervalo interquartil, 6,4-95), 22 pacientes (81%) continuam vivos em primeira remissão clínica completa. Cinco crianças (18,5%) morreram, três delas logo após a admissão e duas após recidiva. A probabilidade de sobrevida em cinco anos para 20 pacientes com LL de novo foi de 78% (DP 9,4). Conclusão: os resultados confirmam o prognóstico favorável de crianças com LL tratadas com regime de quimioterapia intensiva derivado da terapia de LLA.

[The dermatoscopy in the skin pathergy testing: case series in patients with suspected Behçet's Disease]. / Dermatoscopia no teste cutâneo da patergia: série de casos de pacientes com suspeita de Doença de Behçet.

Behçet's disease is a multisystemic disease consisting of a varying combination of ocular, mucocutaneous, neurologic, cardiovascular, gastrointestinal and other manifestations. Its diagnosis is based on clinical criteria, in which a positive pathergy test scores 1. A case series with 26 suspected patients is presented, and the skin pathergy test was performed in 23. The results were read in 48hours, and they were considered negative when without papule, and positive with a papule or pustule. Positive results were divided by papule size, and dermatoscopy was done to measure and observe its clinical aspects. After the readings, a biopsy was performed, with annotation of histopathological aspects. The test was negative in 2 (8.7%) and positive in 21 (91.3%) patients. The results and the literature review are presented.

Th1 immune response to H. pylori infection varies according to the age of the patients and influences the gastric inflammatory patterns.

To compare children and adults in respect to the effect of H. pylori infection on the gastric concentrations of cytokines linked to innate and Th1 immune response, as well as to investigate the changes in the gastric concentrations of the studied cytokines according to the age. We studied 245 children (142 H. pylori-negative and 103 H. pylori-positive) and 140 adults (40 H. pylori-negative and 100 H. pylori-positive). The gastric concentrations of cytokines representative of the innate and Th1 response were higher in the H. pylori-positive than in the -negative children and adults. The gastric concentrations of IL-1α and TNF-α were significantly higher, while those of IL-2, IL-12p70 and IFN-γ were lower in the infected children than in the infected adults. In the infected children, the gastric concentration of IL-1α, IL-2, IL-12p70 and IFN-γ increased, whereas in adults, the gastric concentrations of IFN-γ and IL-12p70 decreased with the aging. Increased gastric concentration of Th1 associated cytokines correlated with increased degree of gastritis that is the background lesion for the development of the H. pylori associated severe diseases. Concluding, Th1 response to H. pylori infection varies according to the age and seems to have determinant implication in the H. pylori infection outcomes.

Iron status and Helicobacter pylori infection in symptomatic children: an international multi-centered study.

OBJECTIVE: Iron deficiency (ID) and iron deficiency anaemia (IDA) are global major public health problems, particularly in developing countries. Whilst an association between H. pylori infection and ID/IDA has been proposed in the literature, currently there is no consensus. We studied the effects of H. pylori infection on ID/IDA in a cohort of children undergoing upper gastrointestinal endoscopy for upper abdominal pain in two developing and one developed country. METHODS: In total 311 children (mean age 10.7±3.2 years) from Latin America--Belo Horizonte/Brazil (n = 125), Santiago/Chile (n = 105)--and London/UK (n = 81), were studied. Gastric and duodenal biopsies were obtained for evaluation of histology and H. pylori status and blood samples for parameters of ID/IDA. RESULTS: The prevalence of H. pylori infection was 27.7% being significantly higher (p<0.001) in Latin America (35%) than in UK (7%). Multiple linear regression models revealed H. pylori infection as a significant predictor of low ferritin and haemoglobin concentrations in children from Latin-America. A negative correlation was observed between MCV (r = -0.26; p = 0.01) and MCH (r = -0.27; p = 0.01) values and the degree of antral chronic inflammation, and between MCH and the degree of corpus chronic (r = -0.29, p = 0.008) and active (r = -0.27, p = 0.002) inflammation. CONCLUSIONS: This study demonstrates that H. pylori infection in children influences the serum ferritin and haemoglobin concentrations, markers of early depletion of iron stores and anaemia respectively.

Assessment of vascular invasion in gastric cancer: a comparative study.

AIM: To evaluate and compare detection of lymphatic and blood vessel invasion (LVI and BVI) by hematoxylin-eosin (HE) and immunohistochemistry (IHC) in gastric cancer specimens, and to correlate with lymph node status. METHODS: IHC using D2-40 (a lymphatic endothelial marker) and CD34 (a pan-endothelial marker) was performed to study LVI and BVI in surgical specimens from a consecutive series of 95 primary gastric cancer cases. The results of the IHC study were compared with the detection by HE using McNemar test and kappa index. The morphologic features of the tumors and the presence of LVI and BVI were related to the presence of lymph node metastasis. A χ(2) test was performed to obtain associations between LVI and BVI and other prognostic factors for gastric cancer. RESULTS: The detection rate of LVI was considerably higher than that of BVI. The IHC study identified eight false-positive cases and 13 false-negative cases for LVI, and 24 false-positive cases and 10 false-negative cases for BVI. The average Kappa value determined was moderate for LVI (κ = 0.50) and low for BVI (κ = 0.20). Both LVI and BVI were statistically associated with the presence of lymph node metastasis (HE: P = 0.001, P = 0.013, and IHC: P = 0.001, P = 0.019). The morphologic features associated with LVI were location of the tumor in the distal third of the stomach (P = 0.039), Borrmann's macroscopic type (P = 0.001), organ invasion (P = 0.03) and the depth of tumor invasion (P = 0.001). The presence of BVI was related only to the depth of tumor invasion (P = 0.003). CONCLUSION: The immunohistochemical identification of lymphatic and blood vessels is useful for increasing the accuracy of the diagnosis of vessel invasion and for predicting lymph node metastasis.

Increased gastric IL-1ß concentration and iron deficiency parameters in H. pylori infected children.

Association between H. pylori infection, iron deficiency and iron deficiency anaemia has been described, but the mechanisms involved have not been established. We hypothesized that in H. pylori infected children increased gastric concentrations of IL-1ß and/or TNF-α, both potent inhibitors of gastric acid secretion that is essential for iron absorption, are predictors for low blood concentrations of ferritin and haemoglobin, markers of early depletion of iron stores and anaemia, respectively. We evaluated 125 children undergoing endoscopy to clarify the origin of gastrointestinal symptoms. Gastric specimens were obtained for H. pylori status and cytokine evaluation and blood samples for determination of iron deficiency/iron deficiency anaemia parameters and IL1 cluster and TNFA polymorphisms that are associated with increased cytokine secretions. Higher IL-1ß and TNF-α gastric concentrations were observed in H. pylori-positive (n = 47) than in -negative (n = 78) children. Multiple linear regression models revealed gastric IL-1ß, but not TNF-α, as a significant predictor of low ferritin and haemoglobin concentrations; results were reproduced in young children in whom IL1RN polymorphic genotypes associated with higher gastric IL-1ß expression and lower blood ferritin and haemoglobin concentrations. In conclusion, high gastric levels of IL-1ß can be the link between H. pylori infection and iron deficiency/iron deficiency anaemia in childhood.

Clinical course and prognostic factors of children with Burkitt's lymphoma in a developing country: the experience of a single centre in Brazil.

OBJECTIVE: Burkitt's lymphoma is the most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course and prognostic factors of children and adolescents with Burkitt's lymphoma treated in the Hematology Unit of Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG). METHODS: A retrospective cohort study was made of 50 consecutive cases of children and adolescents aged 16 years or less with Burkitt's lymphoma admitted between January 1981 and December 2007. Prognostic factors associated with death were evaluated using the Kaplan-Meier method and compared by the two-tailed log-rank test. RESULTS: The median age at diagnosis was 4.7 years. Most patients had abdominal tumors (66.7%) and advanced disease (68.9%) at diagnosis. Thirty-eight patients (84.4%) achieved complete clinical remission and 33 (73.3%) were alive at the first remission. Twelve children (26.7%) died. The median follow-up was 35 months with the probability of overall survival being 73% (89.2% and 35.7% for patients with uric acid < 7 mg/dL and ≥ 7.0 mg/dL, respectively - p-value < 0.001). Uric acid was the only significant prognostic factor at diagnosis. CONCLUSION: Our findings confirm the favorable prognosis of children with Burkitt's lymphoma even when treated with intermediate doses of methotrexate (500 mg/m2). Survival was significantly lower for individuals with concentrations of uric acid > 7 mg/dL.

A regulatory instead of an IL-17 T response predominates in Helicobacter pylori-associated gastritis in children.

Th17 cells seem to have an important role in the efficacy of vaccines against Helicobacter pylori. Because children are a target group for human vaccination and Th17/T(reg) cells have intrinsically linked and antagonic commitments, we compared the gastric levels of Th17- and T(reg)-associated cytokines of children and adults. IL-6, IL-10 and TGF-ß1 levels and Foxp3(+) cell numbers were higher, but IL-1ß, IL-17A and IL-23 were lower in infected children than in infected adults. In conclusion T(reg) instead of Th17 cell response to H. pylori-infection predominates in children.

Clinical course and prognostic factors of children with Burkitt's lymphoma in a developing country: the experience of a single centre in Brazil

OBJECTIVE: Burkitt's lymphoma is the most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course and prognostic factors of children and adolescents with Burkitt's lymphoma treated in the Hematology Unit of Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG). METHODS: A retrospective cohort study was made of 50 consecutive cases of children and adolescents aged 16 years or less with Burkitt's lymphoma admitted between January 1981 and December 2007. Prognostic factors associated with death were evaluated using the Kaplan-Meier method and compared by the two-tailed log-rank test. RESULTS: The median age at diagnosis was 4.7 years. Most patients had abdominal tumors (66.7%) and advanced disease (68.9%) at diagnosis. Thirty-eight patients (84.4%) achieved complete clinical remission and 33 (73.3%) were alive at the first remission. Twelve children (26.7%) died. The median follow-up was 35 months with the probability of overall survival being 73% (89.2% and 35.7% for patients with uric acid < 7 mg/dL and > 7.0 mg/dL, respectively - p-value < 0.001). Uric acid was the only significant prognostic factor at diagnosis. CONCLUSION: Our findings confirm the favorable prognosis of children with Burkitt's lymphoma even when treated with intermediate doses of methotrexate (500 mg/m2). Survival was significantly lower for individuals with concentrations of uric acid > 7 mg/dL.

The presence of Helicobacter pylori in the liver depends on the Th1, Th17 and Treg cytokine profile of the patient.

The hypothesis that Helicobactermight be a risk factor for human liver diseases has arisen after the detection of Helicobacter DNA in hepatic tissue of patients with hepatobiliary diseases. Nevertheless, no explanation that justifies the presence of the bacterium in the human liver has been proposed. We evaluated the presence of Helicobacterin the liver of patients with hepatic diseases of different aetiologies. We prospectively evaluated 147 patients (106 with primary hepatic diseases and 41 with hepatic metastatic tumours) and 20 liver donors as controls. Helicobacter species were investigated in the liver by culture and specific 16S rDNA nested-polymerase chain reaction followed by sequencing. Serum and hepatic levels of representative cytokines of T regulatory cell, T helper (Th)1 and Th17 cell lineages were determined using enzyme linked immunosorbent assay. The data were evaluated using logistic models. Detection of Helicobacter pylori DNA in the liver was independently associated with hepatitis B virus/hepatitis C virus, pancreatic carcinoma and a cytokine pattern characterised by high interleukin (IL)-10, low/absent interferon-γ and decreased IL-17A concentrations (p < 10(-3)). The bacterial DNA was never detected in the liver of patients with alcoholic cirrhosis and autoimmune hepatitis that are associated with Th1/Th17 polarisation. H. pylori may be observed in the liver of patients with certain hepatic and pancreatic diseases, but this might depend on the patient cytokine profile.

Langerhans cell histiocytosis: 37 cases in a single Brazilian institution

OBJECTIVES: To improve the level of 'definitive' diagnosis of Langerhans cell histiocytosis by immunohistochemical investigation of the CD1a surface antigen and to compare outcomes in respect to age, gender, stage of the disease, treatment response and level of diagnostic accuracy. METHODS: A retrospective study was carried out of 37 children and adolescents with possible Langerhans cell histiocytosis between 1988 and 2008. The diagnoses were revisited using immunohistochemical investigations for CD1a, S-100 and CD68 in an attempt to reach definitive diagnoses for all cases. RESULTS: Before the study, only 13 of 37 patients (35.1 percent) had a 'definitive' diagnosis; by the end of the study, this number rose to 25 patients (67.6 percent). All reviewed cases were positive for the CD1a antigen. Overall survival was 88.5 percent. Multisystem disease (Stage 2; n=19) and absence of response at the 6th week of therapy (n=5) were associated to significantly lower overall survival (p-value = 0.04 and 0.0001, respectively). All deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. Other potential prognostic factors were not significant. Reactivation episodes occurred in 75 percent of the patients with multisystem disease. Diabetes insipidus was the most common sequel (21.6 percent). CONCLUSION: The level of diagnostic accuracy was increased through immunohistochemistry. The overall survival rate was similar to international multicentric studies. Multisystem disease and absence of response at six weeks of treatment were the most important unfavorable prognostic factors. The frequency of reactivation for patients with multisystem disease was higher than described in the literature, probably because maintenance chemotherapy was used only in two cases.

The presence of Helicobacter pylori in the liver depends on the Th1, Th17 and Treg cytokine profile of the patient

The hypothesis that Helicobactermight be a risk factor for human liver diseases has arisen after the detection of Helicobacter DNA in hepatic tissue of patients with hepatobiliary diseases. Nevertheless, no explanation that justifies the presence of the bacterium in the human liver has been proposed. We evaluated the presence of Helicobacterin the liver of patients with hepatic diseases of different aetiologies. We prospectively evaluated 147 patients (106 with primary hepatic diseases and 41 with hepatic metastatic tumours) and 20 liver donors as controls. Helicobacter species were investigated in the liver by culture and specific 16S rDNA nested-polymerase chain reaction followed by sequencing. Serum and hepatic levels of representative cytokines of T regulatory cell, T helper (Th)1 and Th17 cell lineages were determined using enzyme linked immunosorbent assay. The data were evaluated using logistic models. Detection of Helicobacter pylori DNA in the liver was independently associated with hepatitis B virus/hepatitis C virus, pancreatic carcinoma and a cytokine pattern characterised by high interleukin (IL)-10, low/absent interferon-γ and decreased IL-17A concentrations (p < 10-3). The bacterial DNA was never detected in the liver of patients with alcoholic cirrhosis and autoimmune hepatitis that are associated with Th1/Th17 polarisation. H. pylori may be observed in the liver of patients with certain hepatic and pancreatic diseases, but this might depend on the patient cytokine profile.

Langerhans cell histiocytosis: 37 cases in a single brazilian institution.

OBJECTIVES: To improve the level of 'definitive' diagnosis of Langerhans cell histiocytosis by immunohistochemical investigation of the CD1a surface antigen and to compare outcomes in respect to age, gender, stage of the disease, treatment response and level of diagnostic accuracy. METHODS: A retrospective study was carried out of 37 children and adolescents with possible Langerhans cell histiocytosis between 1988 and 2008. The diagnoses were revisited using immunohistochemical investigations for CD1a, S-100 and CD68 in an attempt to reach definitive diagnoses for all cases. RESULTS: Before the study, only 13 of 37 patients (35.1%) had a 'definitive' diagnosis; by the end of the study, this number rose to 25 patients (67.6%). All reviewed cases were positive for the CD1a antigen. Overall survival was 88.5%. Multisystem disease (Stage 2; n=19) and absence of response at the 6th week of therapy (n=5) were associated to significantly lower overall survival (p-value = 0.04 and 0.0001, respectively). All deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. Other potential prognostic factors were not significant. Reactivation episodes occurred in 75% of the patients with multisystem disease. Diabetes insipidus was the most common sequel (21.6%). CONCLUSION: The level of diagnostic accuracy was increased through immunohistochemistry. The overall survival rate was similar to international multicentric studies. Multisystem disease and absence of response at six weeks of treatment were the most important unfavorable prognostic factors. The frequency of reactivation for patients with multisystem disease was higher than described in the literature, probably because maintenance chemotherapy was used only in two cases.

Immune response and gene polymorphism profiles in Crohn's disease and ulcerative colitis.

BACKGROUND: Polymorphisms in genes linked to the innate and adaptive immune response may be involved in inflammatory bowel disease pathogenesis. Our aim was to investigate associations among IL1B-511, IL1B-31, IL1RN, TNFA-307, TLR-2, TLR-4, IL2-330, NOD2 G908R, NOD2 L1007fsinsC polymorphisms and both Crohn's disease (CD) and ulcerative colitis (UC) in a Brazilian population. METHODS: We studied 43 patients with CD, 42 with UC, and 541 blood donors. Polymorphisms were evaluated by PCR, PCR-CTPP, or PCR-RFLP. Data were analyzed in multivariate models adjusting for confounding factors. RESULTS: IL1RN VNTR (P = 0.00, odds ratio [OR] = 2.43, 95% confidence interval [CI] = 1.50-3.90), as well as TNFA-307 polymorphic allele (P = 0.05, OR = 1.70, 95% CI = 1.00-2.94) were associated with UC. Both NOD2 mutations (G908R, P = 0.02, OR = 6.83, 95% CI = 1.62-25.45, and L1007fsinsC, P = 0.00, OR = 20.00, 95% CI = 3.21-124.69) were associated with CD. CONCLUSIONS: Our analyses showed positive associations between proinflammatory polymorphisms at IL1RN and TNFA-307 loci and UC, as well as polymorphisms in the NOD2 gene and CD. These results highlight the importance of different genetic profiles associated with CD and UC.

Esofagite eosinofílica, uma doença emergente: aspectos endoscópicos e proposta de uma classificação histopatológica / Eosinophilic esophagitis, an emerging disease: endoscopic aspects and a proposal of histopathologic classification

A esofagite eosinofílica (EE) é uma doença ainda pouco conhe»cida, cuja prevalência vem aumentando progressivamente, possi»velmente em decorrência de estar sendo mais diagnosticada. E uma condição inflamatória em que há grande infiltrado eosinofílico no epitélio esofágico. Seu quadro clínico inclui: disfagia, impactação de alimentos, dor retroestemal e náuseas/vômitos. Acomete pre»ferencialmente crianças e adultos jovens. Foi realizado estudo re-trospectivo em serviço de histopatologia em Belo Horizonte (MG), no período de janeiro de 2006 a fevereiro de 2007, visando à caracterização de aspectos histopatológicos do esôfago e à pro»posta de uma classificação, acrescido de uma revisão bibliográfica sobre o assunto. Foram identificadas 25 biópsias esofágicas endos»cópicas com contagens elevadas de eosinófilos. Dos 25 casos, 1 7 eram do sexo masculino (68%) e oito do feminino (32%). Os es»pécimes analisados apresentaram diferentes números de eosinófi»los por campo de grande aumento (eosinófilos/hpf) e foram clas»sificados em quatro diferentes graus: grau O - 0%, (N = O), até 14 eosinófilos/hpf; grau I - 48%, (N = 12), de 15 a 30 eosinófilos/ hpf; grau 11 - 28%, (N = 7), de 31 a 45 eosinófilos/hpf e grau 111 »24%, (N = 6), com 46 ou mais eosinófilos/hpf.Os achados en»doscópicos do esôfago também foram analisados em 20 pacien»tes, com os seguintes resultados: em 15% dos casos, (N = 3), de»tectaram-se grumos brancacentos; em 5% dos casos, (N = 1), encontraram-se estrias longitudinais; em 15% dos casos, (N = 3), foram detectados anéis concêntricos (feline aspect); em 25% dos casos, (N = 5), registrou-se espessamento de mucosa; em 45% dos casos, (N = 8), encontrou-se esofagite erosiva e em 10%, (N = 2), encontrou-se estenose parcial. Observamos que a EE é uma entidade clínico-patológica caracterizada pela presença de eosinó»filos intra-epiteliais com contagem acima de 15 eosinófilos / hpf. As queixas clínicas e os aspectos endoscópicos podem direcionar o diagnóstico...

Acuidade da citologia oncótica para o diagnóstico da infecção pelo HPV no colo uterino de mulheres portadoras do HIV / Accuracy of oncotic cytology for HPV infection diagnosis on the cervix uteri of HIV-infected women

OBJETIVO: verificar a acuidade do exame citológico do colo uterino para o diagnóstico do HPV, comparado à reação em cadeia da polimerase (PCR), em amostras de mulheres portadoras do HIV. MÉTODOS: foram incluídas 158 pacientes, sendo realizada uma primeira coleta de material da cérvice uterina com a espátula de Ayre para a PCR. A seguir, foi realizada outra coleta com espátula de Ayre e escova para a citologia oncótica. Foram revisadas 109 lâminas, tendo em vista que 49 foram destruídas, por terem ultrapassado dois anos de arquivo. RESULTADOS: a prevalência de HPV foi de 11 por cento no estudo citológico e 69,7 por cento na PCR. A idade do grupo variou de 20 a 61 anos, com mediana de 35 anos. A forma de contágio pelo HIV foi a heterossexual em 91,8 por cento dos casos e 79,1 por cento dos pacientes tiveram um a cinco parceiros sexuais em toda a vida. A queixa mais freqüente foi massa pélvica (5,1 por cento) e 75,3 por cento procuraram o serviço para consulta de rotina. A comparação de variáveis categóricas foi realizada através de tabelas de contingência sendo utilizado o teste do χ2 com correção de Yates para comparação de proporções. Quando uma das freqüências esperadas foi menor que cinco, foi utilizado o teste de Fisher. Na comparação de testes diagnósticos foram calculados: a sensibilidade, a especificidade e razões de verossimilhança. Das 76 pacientes com HPV detectado pela PCR, somente 12 foram confirmadas pela citologia (sensibilidade=15,8 por cento) que, por outro lado, não apresentou resultados falsos-positivos (especificidade=100 por cento). Comparando-se os dois resultados, encontraram-se valor preditivo positivo de 100 por cento e negativo de 33,3 por cento para a citologia, na predição da presença do HPV. Entre as 12 pacientes com citologia positiva para HPV, quatro (33,3 por cento) apresentaram neoplasias intra-epiteliais cervicais (OR=5,6; razão de verossimilhança positiva=infinidade positiva; razão de verossimilhança...

PURPOSE: to verify the accuracy of uterine cervix cytology for HPV diagnosis, as compared to polymerase chain reaction (PCR) in samples of women with HIV. METHODS: 158 patients who had undergone a first collection of material from the uterine cervix with Ayre's spatula for PCR were included in the study. Then, another collection with Ayre's spatula and brush for oncotic cytology was performed. Only 109 slides were reviewed, as 49 of them had already been destructed for have being filed for over two years. RESULTS: the prevalence of HPV was 11 percent in the cytological exam and 69.7 percent in the PCR. Age varied from 20 to 61 years old, median 35 years. The HIV contagious route was heterosexual in 91.8 percent of the cases, and 79.1 percent of the patients had had from one to five sexual partners along their lives. The most frequent complaint was pelvic mass (5.1 percent), and 75.3 percent of the women had looked for the service for a routine medical appointment. The categorical variable comparison was done through contingency tables, using the χ2 test with Yates's correction to compare the ratios. The Fisher's test was used when one of the expected rates was lower than five. In the comparison of diagnostic tests, sensitivity, specificity and similarity ratios have been calculated. Among the 76 patients with HPV, detected by PCR, only 12 had the diagnosis confirmed by cytology (sensitivity=15.8 percent), which on the other hand did not present any false-positive results (specificity=100 percent). Concerning the HPV presence, the cytological prediction for positive results was 100 percent and 33.3 percent for negative, when both results were compared. Among the 12 patients with HPV positive cytology, four (33.3 percent) presented cervical intraepithelial neoplasia (OR=56; positive similarity ratio=positive infinity; negative similarity ratio=0.83). CONCLUSIONS: As the cytology specificity is quite high, it is possible to rely on...

[Accuracy of oncotic cytology for HPV infection diagnosis on the cervix uteri of HIV-infected women]. / Acuidade da citologia oncótica para o diagnóstico da infecção pelo HPV no colo uterino de mulheres portadoras do HIV.

PURPOSE: to verify the accuracy of uterine cervix cytology for HPV diagnosis, as compared to polymerase chain reaction (PCR) in samples of women with HIV. METHODS: 158 patients who had undergone a first collection of material from the uterine cervix with Ayre's spatula for PCR were included in the study. Then, another collection with Ayre's spatula and brush for oncotic cytology was performed. Only 109 slides were reviewed, as 49 of them had already been destructed for have being filed for over two years. RESULTS: the prevalence of HPV was 11% in the cytological exam and 69.7% in the PCR. Age varied from 20 to 61 years old, median 35 years. The HIV contagious route was heterosexual in 91.8% of the cases, and 79.1% of the patients had had from one to five sexual partners along their lives. The most frequent complaint was pelvic mass (5.1%), and 75.3% of the women had looked for the service for a routine medical appointment. The categorical variable comparison was done through contingency tables, using the chi2 test with Yates's correction to compare the ratios. The Fisher's test was used when one of the expected rates was lower than five. In the comparison of diagnostic tests, sensitivity, specificity and similarity ratios have been calculated. Among the 76 patients with HPV, detected by PCR, only 12 had the diagnosis confirmed by cytology (sensitivity=15.8%), which on the other hand did not present any false-positive results (specificity=100%). Concerning the HPV presence, the cytological prediction for positive results was 100% and 33.3% for negative, when both results were compared. Among the 12 patients with HPV positive cytology, four (33.3%) presented cervical intraepithelial neoplasia (OR=56; positive similarity ratio=positive infinity; negative similarity ratio=0.83). CONCLUSIONS: As the cytology specificity is quite high, it is possible to rely on the positive result, which means that a positive result will surely indicate the presence of HPV. The low sensitivity of cytology does not qualify it as a survey exam for HPV detection in this female group.