RESUMO
A ten-year-old girl was admitted to our hospital with a mass in the left breast after a local recurrence following a resection done in a district hospital three months earlier. A modified radical mastectomy with axillary lymph node dissection was performed in our department. Histological examination revealed a lipid secreting carcinoma (lipid-rich carcinoma) of the breast. To our knowledge she is the first case in childhood in the literature and is therefore reported.
Assuntos
Neoplasias da Mama/metabolismo , Carcinoma/metabolismo , Metabolismo dos Lipídeos , Mama/patologia , Neoplasias da Mama/patologia , Neoplasias da Mama/terapia , Carcinoma/patologia , Carcinoma/terapia , Quimioterapia Adjuvante , Criança , Terapia Combinada , Citoplasma/ultraestrutura , Feminino , Humanos , Mastectomia Radical Modificada , Dosagem Radioterapêutica , Vacúolos/ultraestruturaRESUMO
The "histiocytes" are a group of proliferative disorders of the mononuclear phagocyte system whose etiologies are basically unknown. The majority of childhood histiocytoses are expressions of excessive numbers of Langerhans cells, representing so-called Langerhans cell histiocytosis. Fifteen patients who were diagnosed with histiocytosis syndrome at the Pediatric Hematology and Oncology Department of Ege University Hospital between October 1986 and January 1995 were included in this study. The majority of the patients had Langerhans cell histiocytosis (LCH), and skeletal involvement was the most common manifestation. A good response to radiotherapy and chemotherapy was obtained by our patients with unifocal and multifocal involvement of LCH. Two patients with disseminated LCH died with progressive disease. In the patient with Rosal-Dorfman disease, a partial response was obtained with prednisone. The patient with malignant histiocytosis died during a relapse at the end of one year. Organ dysfunction and the patient's age are important factors affecting the outcome of the disease.
Assuntos
Histiocitose , Adolescente , Idade de Início , Doenças Ósseas/patologia , Doenças Ósseas/terapia , Criança , Pré-Escolar , Feminino , Histiocitose/patologia , Histiocitose/terapia , Histiocitose de Células de Langerhans , Humanos , Lactente , Masculino , Prognóstico , Estudos RetrospectivosRESUMO
Mild and marginal malnutrition must be identified to prevent the development of severe protein-energy malnutrition in pediatric cancer patients. We aimed to evaluate nutritional status and determine daily energy, protein and micronutrient intake to identify mild or marginal malnutrition in pediatric cancer patients. Daily energy, protein and micronutrient intake, anthropometric measurements and biochemical indices were studied in 45 patients (25 in remission, 20 newly diagnosed or relapsed) who consumed energy, protein, vitamins and minerals below the recommended quantities. According to the weight-for-height index, 23 children (51.1%) were determined to be malnourished. Absolute and relative prealbumin values were 19.4 +/- 7.2 mg/dl and 74.3 +/- 29.1 mg/dl in the remission group, and 14.8 +/- 5.1 mg/dl and 58.1 +/- 23.3 in the active disease group, respectively (p < 0.05). Relative prealbumin values were found to be low in 63.6 percent of nonmalnourished children, and 80 percent of children with mild malnutrition. We conclude that malnutrition is common in pediatric cancer patients, and prealbumin is a reliable and sensitive indicator of mild and marginal malnutrition. Determining prealbumin values and assessing the deficiency of micro- and macronutrients before malnutrition is detected by anthropometric measures may provide a warming that nutritional problems may occur.
Assuntos
Neoplasias/complicações , Distúrbios Nutricionais/diagnóstico , Adolescente , Antropometria , Biomarcadores , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Distúrbios Nutricionais/complicações , Pré-Albumina/metabolismoRESUMO
Ten pediatric patients with solid tumors and chemotherapy-induced neutropenia were given recombinant human granulocyte-macrophage colony-stimulating factor (rHuGM CSF). The duration of the neutropenic phase was then compared with the results obtained from eight patients also with solid tumors, but not treated with rHuGM-CSF. It was found that rHuGM-CSF treatment significantly decreased the duration of the neutropenic phase. Endogenous plasma GM-CSF, IL-3, and IL-4 levels were also measured in the study group and in healthy children. No significant correlation has been found between plasma GM-CSF concentrations and absolute neutrophil counts. However, IL-3 levels of the neutropenic patients positively correlated with platelet counts. Furthermore, IL-4 concentrations were positively correlated with the GM-CSF level in the same individual. Plasma GM-CSF, IL-3, and IL-4 levels in the neutropenic solid tumor group were found to be significantly higher than those in healthy children. Plasma IL-4 levels were significantly elevated in patients with osteosarcoma as compared to patients with other solid tumors. Although rHuGM-CSF has a half-life of only two to three hours, one day after rHuGM-CSF therapy, plasma GM-CSF levels were found to be higher than initial values. In contrast, plasma IL-4 values decreased significantly after administration of rHuGM-CSF. The probable mechanisms for the changes in cytokine levels are discussed.
Assuntos
Fator Estimulador de Colônias de Granulócitos e Macrófagos/uso terapêutico , Neoplasias/tratamento farmacológico , Neutropenia/tratamento farmacológico , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Fator Estimulador de Colônias de Granulócitos e Macrófagos/efeitos adversos , Fator Estimulador de Colônias de Granulócitos e Macrófagos/sangue , Humanos , Interleucina-3/sangue , Interleucina-4/sangue , Masculino , Neoplasias/sangue , Proteínas Recombinantes , Análise de Regressão , Estatísticas não ParamétricasRESUMO
Serum IgG, IgM, IgA, IgG subclasses (IgG1, G2, G3, G4), isohemagglutinins and complement-3 concentrations were measured in 23 beta-thalassemic patients suffering from recurrent infections. No significant abnormalities were found in these humoral immunity investigations, both in splenectomized and non-splenectomized patients. On the other hand, iron overload or repeated blood transfusions were not found to down-regulate the humoral immune system of thalassemic patients.
Assuntos
Complemento C3/metabolismo , Hemaglutininas/sangue , Imunoglobulina G/classificação , Imunoglobulinas/sangue , Talassemia beta/imunologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Tolerância Imunológica/imunologia , Imunoglobulina G/sangue , Masculino , Esplenectomia , Talassemia beta/diagnóstico , Talassemia beta/cirurgiaAssuntos
Vacinas contra Hepatite B/administração & dosagem , Hepatite B/prevenção & controle , Hospedeiro Imunocomprometido , Vacinação , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Avaliação como Assunto , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/imunologia , Hemofilia A/complicações , Hemofilia A/imunologia , Humanos , Imunidade , Talassemia beta/complicações , Talassemia beta/imunologiaAssuntos
Neoplasias/epidemiologia , Neoplasias/genética , Adolescente , Estudos de Casos e Controles , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Índia/epidemiologia , Lactente , Masculino , Neoplasias/diagnóstico , Vigilância da População , Fatores de RiscoRESUMO
Although the beta (beta) thalassaemia carrier frequency in Turkey was stated to be 2 per cent, the prevalence rate varies widely in different regions and there is limited data confirming the disorder in Aegean region. This prevalence study was planned to determine frequency of beta thalassaemia trait in the Aegean region among 1124 high school students, between 13 and 18 years old, who were selected as target population. Sensitivity of mean corpuscular volume (MCV) and mean corpuscular haemoglobin (MCH) in prediction of beta thalassaemia trait were evaluated. Venous blood samples were obtained for haemoglobin electrophoresis, HbA2 and HbF, serum iron and total iron binding capacity from students in whom the levels of haemoglobin (Hb), haemotocrite (Hct), MCV, or MCH, were low compared to normal values. The prevalence of beta thalassaemia trait in Aegean region was 3 per cent. Sensitivity of MCV and MCH for determining beta thalassaemia trait were 100 and 96 per cent, respectively.
Assuntos
Talassemia beta/epidemiologia , Adolescente , Análise Química do Sangue , Feminino , Humanos , Incidência , Masculino , Prevalência , Turquia/epidemiologiaRESUMO
The levels of soluble interleukin-2 receptors (sIL-2R) and tumor necrosis factor (TNF) were determined in the serum of 61 children with hematological malignancy, including 20 patients with leukemia and 16 with lymphoma in active state of malignancy, and 20 patients with leukemia and 5 with lymphoma in complete remission. In addition, serum samples from 15 healthy children were used as controls. It was found that the mean serum levels of sIL-2R and TNF were significantly increased in active malignancy (207.0 +/- 17.1 pmol/L for sIL-2R; 209.7 +/- 35.0 pg/mL for TNF) compared to the remission status and normal controls (P < 0.001). No such difference was observed between leukemia and lymphoma groups. It was concluded that serum sIL-2R and TNF are of potential value in the diagnosis and follow up of patients with neoplastic diseases.
Assuntos
Leucemia/sangue , Linfoma/sangue , Receptores de Interleucina-2/análise , Fator de Necrose Tumoral alfa/análise , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Leucemia/terapia , Linfoma/terapia , Masculino , Valores de Referência , Indução de Remissão , SolubilidadeRESUMO
Myelofibrosis with myeloid metaplasia, or agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder characterized by fibrosis of the bone marrow accompanied by aniso- and poikilocytosis, leukoerythroblastosis and hepatosplenomegaly with extramedullary hematopoiesis. Agnogenic myeloid metaplasia is very rare in children. In this report, two cases of AMM in whom the onset of the illness were at 3 and 12 months of age, are presented. Both had severe anemia, hepatosplenomegaly and bone marrow fibrosis. Lymph node biopsy of the first patient and liver biopsy of the second revealed extramedullary hematopoiesis. They were treated with an intravenous high dose of methylprednisolone (daily 30 mg/kg for 3 days, 20 mg/kg for 4 days, 10 mg/kg for 1 week, 5 mg/kg for 1 week). A complete improvement of hematological and clinical findings was observed.
Assuntos
Relação Dose-Resposta a Droga , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Mielofibrose Primária/tratamento farmacológico , Anemia/complicações , Medula Óssea/diagnóstico por imagem , Medula Óssea/patologia , Doença Crônica , Hematopoese , Hepatomegalia/complicações , Humanos , Lactente , Injeções Intravenosas , Masculino , Mielofibrose Primária/complicações , Mielofibrose Primária/patologia , Esplenomegalia/complicações , Resultado do Tratamento , UltrassonografiaRESUMO
Neutrophil chemotaxis and random migration were evaluated in 21 patients with thalassemia major and 21 healthy controls by a filter technique (Boyden chamber). Chemotactic and random migrations in patient group were found to be defective, which may partially account for the increased susceptibility to infection occasionally observed in these patients. The effects of serum ferritin levels, transferrin saturations that show iron overload, total count of blood transfusions for chronic immunostimulation, desferrioxamine therapy, and splenectomy on these neutrophil functions were examined in thalassemic patients in order to determine whether they are responsible for these defective functions because the mechanism of abnormal neutrophil chemotaxis and random mobility in thalassemic patients is not still clear.
Assuntos
Quimiotaxia de Leucócito , Neutrófilos/fisiologia , Talassemia beta/sangue , Transfusão de Sangue , Movimento Celular , Criança , Pré-Escolar , Desferroxamina/uso terapêutico , Suscetibilidade a Doenças , Feminino , Ferritinas/sangue , Humanos , Ferro/sangue , Masculino , Valores de Referência , Sideróforos/uso terapêutico , Esplenectomia , Transferrina/análise , Talassemia beta/terapiaRESUMO
Prophylaxis has been practiced for many years in Europe and is gaining acceptance worldwide with current viral inactivation procedures. Unfortunately, the high cost of prophylaxis is currently the major obstacle to its implementation in developing countries such as Turkey. The aim of this controlled preliminary study is to evaluate the efficacy, safety, and feasibility of prophylaxis. Seven boys aged 1.5-7 years (5.0 +/- 1.8), who had severe hemophilia (six A, one B) received 20-50 IU/kg factor twice weekly and were followed up for 6-24 months (14.5 +/- 6.6). Intermediate concentrates have been used in hemophilia A and ultrapure product for hemophilia B. The data obtained for the same group of patients before prophylaxis were used as a control group. Another control group was selected in another group of 10 hemophiliacs, mean age 12.5, and received treatment on demand. During prophylactic treatment, the episodes of bleeding were decreased (from 10.5 +/- 3.2 to 4.5 +/- 3.6). Orthopedic and radiologic joint scores were stable (from 0 to 1 and from 1.1 +/- 1.2 to 1.0 +/- 1.5). The patients spent significantly fewer days in the hospital (from 18 +/- 12 to 0.7 +/- 0.6). None of the patients was infected with hepatitis A, hepatitis B, or human immunodeficiency virus. One patient was seroconverted with anti-hepatitis C virus in the third month of prophylaxis. Mean consumption of concentrates for prophylaxis was 3489 +/- 960 IU/kg per year compared with 2073 +/- 1302 in conventional therapy. Prophylaxis was superior to treatment on demand even when given in a twice-weekly period with intermediate concentrates. In Third World countries, prophylaxis should be tried at least in selected severely hemophilic children in order to prevent disabilities.
Assuntos
Fator IX/administração & dosagem , Fator VIII/administração & dosagem , Hemofilia A/prevenção & controle , Hemofilia B/prevenção & controle , Criança , Pré-Escolar , Fator IX/efeitos adversos , Fator VIII/efeitos adversos , Hemofilia A/epidemiologia , Hemofilia B/epidemiologia , Humanos , Lactente , Infusões Intravenosas , Masculino , Turquia/epidemiologiaRESUMO
Serum erythropoietin (EPO) levels were determined by radioimmunoassay in 37 beta-thalassemia patients, the phenotype being thalassemia major (TM) in 30 and thalassemia intermedia (TI) in 7. The control group consisted of 37 healthy children. The mean serum EPO levels were significantly higher in patients with both TM (215.1 +/- 144.5) and TI (53.8 +/- 40.2) compared with the control group (9.3 +/- 4.6). Although the mean hemoglobin (Hb) concentrations in the patients with TM and TI were similar (8.6 +/- 0.9 and 8.7 +/- 1.1, respectively), the mean serum EPO level was significantly higher in TM patients than the patients with TI (P < .01). This finding may indicate that some other factors contributing to the metabolic adaptation to low oxygen concentration or improvement of the tissue oxygenation are as effective as the Hb concentration in EPO production. It is also suggestive of the fact that some amount of tissue hypoxia cannot be prevented in spite of polytransfusion regimens in TM patients. Serum EPO levels of TM patients were not found to be age related or correlated with the mean pretransfusional Hb levels. In the TM patients, the serum EPO concentration was not consistently correlated with clinical signs of erythropoietic activity. This may be indicative of personal differences with respect to the sensitivities of erythroid precursors to the increasing EPO levels in TM patients.
Assuntos
Eritropoetina/sangue , Talassemia beta/sangue , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Radioimunoensaio , Talassemia beta/fisiopatologiaRESUMO
Beta (beta) globin gene analysis was performed in 54 homozygous beta-thalassemia patients followed up in the Pediatric Hematology Department of Medical School of Ege University. The spectrum of beta-thalassemia alleles and their effect on clinical severity of disease were investigated. Twelve different mutations were determined in our patients. The six most frequent alleles, IVSI-110 (G-A), IVSI-6 (T-C), IVSI-I (G-A), IVSII-745 (C-G), Cd39 (C-T), and FSC8, account for 80.6% of all the disease genes. Eleven percent of the chromosomes could not be identified with the probes used in this study. In 38 patients both of whose beta-thalassemia alleles were identified, the beta-thalassemia alleles were found to be the major determinant of the clinical severity of disease. The clinical progress of disease was also closely related to the degree of iron overload.
Assuntos
Heterogeneidade Genética , Globinas/genética , Talassemia beta/genética , Adolescente , Alelos , Criança , Pré-Escolar , Feminino , Genótipo , Homozigoto , Humanos , Masculino , Puberdade/genética , Puberdade Tardia/genética , TurquiaRESUMO
It has been shown that high doses of human recombinant erythropoietin (r epo) increase haemoglobin levels by augmentation of F-cells, and Hb-F production in animal models and in human trials. In this study, r epo was used in patients with beta thalassemia intermedia. Our purpose was to improve haemoglobin levels by at least 2 g and maintain an average level between 10 and 12 g/dl. Ten patients aged 6-29 years (mean 14 +/- 7.6 years) with thalassemia intermedia were treated with r epo. It was given subcutaneously in rising doses from 500 to 1000 U/kg three times weekly for 3 months. During r epo therapy eight cases (80 per cent) showed an increase in haemoglobin, haematocrit, and reticulocyte levels, and an increase of at least 2 g of haemoglobin was obtained. Blood transfusion was not needed during the study except in one case. Five cases (50 per cent) improved life quality with therapy. Hb levels of all patients returned to baseline values over 1 or 2 months after r epo was discontinued. There was no significant change in absolute Hb-F, F-cells, and ferritin levels during treatment. Generally, the drug was well tolerated. No patient had hypertension. Recombinant erythropoietin seems to be an effective treatment for anaemia of beta-thalassemia intermedia, but longer term randomized trials are needed especially in patients with beta thalassemia major.
Assuntos
Eritropoetina/uso terapêutico , Hemoglobinas/metabolismo , Talassemia beta/tratamento farmacológico , Adolescente , Adulto , Criança , Eritropoetina/administração & dosagem , Eritropoetina/sangue , Feminino , Hematócrito , Humanos , Masculino , Estudos Prospectivos , Radioimunoensaio , Proteínas RecombinantesRESUMO
Plasma levels of IL-3 and IL-7 were studied in 23 patients with homozygous beta-thalassemia in order to determine whether these cytokines are involved in abnormalities in erythropoiesis and immune responses as observed in thalassemic patients. No significant difference was found in plasma IL-7 concentrations between thalassemic patients and healthy controls and it was suggested that IL-7 is not a cytokine involved in cellular immunological alterations in beta-thalassemia. However, the number of thalassemic patients with detectable IL-3 concentrations was significantly higher. It was concluded that IL-3 production has to be studied in detail in order to learn more about the involvement of this cytokine in erythropoiesis of thalassemic patients.
Assuntos
Interleucina-3/sangue , Interleucina-7/sangue , Talassemia beta/imunologia , Adolescente , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Ensaio de Imunoadsorção Enzimática , Eritropoese/imunologia , Feminino , Homozigoto , Humanos , Imunidade Celular/fisiologia , Interleucina-3/biossíntese , Interleucina-7/biossíntese , Masculino , Sensibilidade e Especificidade , Talassemia beta/genéticaRESUMO
Augmentation of gamma-gene synthesis by using recombinant human erythropoietin (r-Hu-EPO) represents a new approach to the therapy of beta-thalassemia. A prospective study was conducted in 26 transfusion-dependent beta-thalassemia major patients. r-Hu-EPO (Eprex/Cilag, Switzerland) was given to the patients at an initial dose of 500 IU/kg s.c. 3 times a week for at least 2 months during which no transfusion was applied. A sustained hemoglobin (Hb) level greater than 8 g/dl was considered as a response to EPO treatment. In the patients whose Hb levels remained under 8 g/dl or did not increase in comparison to pretreatment levels within 4 weeks, the dose of r-Hu-EPO was increased to 1,000 IU/kg 3 times a week and applied for another 4 weeks. Only 16 cases also received oral iron supplementation. The whole blood and reticulocyte counts, the biochemical tests including BUN, creatinine, AST, ALT, alkaline phosphatase and ferritin were done and the percentages of HbF and F cells were analyzed regularly. At the end of the 2nd month, 6 cases qualified to continue with the trial. At the end of the 6th month, r-Hu-EPO therapy was ceased in 3 cases of the 6 since their Hb levels had decreased below 7 g/dl. Only 3 cases (11.5%) continued with the r-Hu-EPO therapy without transfusion for up to 12 months. In conclusion, r-Hu-EPO may be useful in some selected transfusion-dependent patients with beta-thalassemia major. Selection criteria should include a mild beta-genotype of coinheritance of alpha-thalassemia, splenectomy and pretreatment reticulocyte response of the patients as well as the patients' compliance.
Assuntos
Eritropoetina/administração & dosagem , Talassemia beta/genética , Talassemia beta/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Hemoglobinas/efeitos dos fármacos , Humanos , Masculino , Estudos Prospectivos , Proteínas RecombinantesRESUMO
We have evaluated the efficacy of treatment with recombinant Interferon-2b (IFN-2b) in 12 children with cancer who developed chronic hepatitis-B infection. Seven of them had lymphoblastic leukaemia and others had solid tumours. Seven cases were male. Mean age was 10.5 years with a range of 5-16 years. Chronic Hepatitis B was diagnosed biochemically, serologically and histopathologically. They were HBsAg(+), HBV-DNA(+), and HCV(-), HIV(-). Seven cases were HBeAg(+) and two of them were anti-Delta IgG(+). Liver biopsy revealed chronic active hepatitis in six cases and persistent hepatitis in three cases. IFN was given at the dose of 5 MU/m2 three times a week, subcutaneously for 6 months. It was well tolerated. After IFN therapy, ALT levels returned to normal in seven cases. All cases were still HBsAg(+). Four of them seroconverted to anti-HBe antibody. Loss of serum HBV-DNA in three cases, but 11 cases showed a marked decrease after IFN. The control liver biopsies showed that histopathological activity index was diminished in five cases. Other 16 patients, serving as control, received no therapy. Five of them were leukaemia and others were solid tumours. Twelve cases were male. Mean age was 9.3 years with a range of 4-19 years. After 6 months, only one patient lost HBV-DNA and three of them seroconverted to anti-HBe with normalization of ALT values. In our study, IFN treatment favourably influenced the progress of chronic hepatitis B in children with cancer.
Assuntos
Hepatite B/tratamento farmacológico , Interferon Tipo I/uso terapêutico , Neoplasias/imunologia , Adolescente , Criança , Pré-Escolar , Doença Crônica , Estudos de Avaliação como Assunto , Feminino , Hepatite B/complicações , Humanos , Interferon Tipo I/efeitos adversos , Masculino , Neoplasias/complicações , Estudos Prospectivos , Proteínas Recombinantes , Resultado do TratamentoRESUMO
WT syndrome, an autosomal dominant condition, combines hematological abnormalities with mild lib defects. Anemia, pancytopenia, leukemia and lymphoma can occur at varying ages from childhood to middle age. Limb defects include ulnar and radial defects, bifid or hypoplastic thumbs and cutaneous syndactyly. Castleman disease is characterized by tumorous masses of lymphoid tissue showing plasma cell or hyaline vascular type changes in histological specimens. A 13 year old boy, diagnosed as WT syndrome with ulnar and radial deviation and 5th finger clinodactyly also had neutropenia, cervical and mediastinal lymphadenopathy. Histology of the cervical lymph node showed angiofollicular hyperplasia of the hyaline-vascular type (Castleman disease). This interesting patient is reported because Castleman disease, together with WT syndrome has not been previously described.