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Primary immune deficiencies are a group of heterogenous genetic disorders characterized by frequent infections, autoimmunity and malignancy. In this study, we aimed to evaluate clinical characteristics, outcomes of children with malignancy developed on background of primary immunodeficiency and compare survival rates of patients between malignant lymphoma with primary immunodeficiency and without immunodeficiency from tertiary oncology center in a developing country. A total 23 patients with primary immunodeficiency and malignancy were evaluated retrospectively. A total of 26 malignancies (first or second) in 23 patients were determined. The median age at the time of the first malignancy was 8 years (ranges 2-18 years) with increased male ratio (M/F:14/9). Non-Hodgkin lymphoma (n = 17; 65%) was the most common malignancy, followed by Hodgkin lymphoma (n = 5), anaplastic ependymoma (n = 1), spinal glioblastoma multiforme (n = 1), retinoblastoma (n = 1) and intracranial hemangiopericytoma (n = 1). The median follow-up time of patients was 25 months (ranges between 1 and 189 months). The 5-year overall survival rate of patients with malignant lymphoma associated with primary immunodeficiency (41%) were lower than immunocompetent patients with malignant lymphoma (80%) (p = 0.000). The 5-year overall survival of patients was diagnosed between 2021 and 2013 years (62%) was higher than previous years (22%) (p = 0.03). In conclusion, non-Hodgkin lymphomas were the most common histopathologic type in patients with malignancy associated with primary immunodeficiency in the present study. The survival of patients with malignant lymphoma associated with primary immunodeficiency has improved in recent years, yet it is still lower than immunocompetent patients with lymphoma and new targeted drugs are required for better survival rates.
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Linfoma no Hodgkin , Linfoma , Neoplasias , Adolescente , Niño , Preescolar , Países en Desarrollo , Humanos , Linfoma/epidemiología , Linfoma/terapia , Linfoma no Hodgkin/epidemiología , Linfoma no Hodgkin/patología , Linfoma no Hodgkin/terapia , Masculino , Neoplasias/epidemiología , Neoplasias/terapia , Estudios RetrospectivosAsunto(s)
Mutación de Línea Germinal , Síndrome de Li-Fraumeni , Neoplasias Peritoneales , Proteína p53 Supresora de Tumor , Humanos , Síndrome de Li-Fraumeni/genética , Neoplasias Peritoneales/genética , Neoplasias Peritoneales/patología , Proteína p53 Supresora de Tumor/genética , Mesotelioma/genética , Mesotelioma/patología , Femenino , Masculino , NiñoRESUMEN
BACKGROUND: Oxidative stress has a potential role in carcinogenesis. Anti-oxidant enzymes have a neutralizing effect on both cancer initiation and progression. We aimed to assess the oxidant and anti-oxidant levels of pediatric cancer patients and to compare the levels in healthy controls. MATERIALS AND METHODS: The study involved 105 pediatric cancer patients (40 undergoing chemotherapy, 65 survivors) and 40 healthy children. The serum total oxidant status (TOS) and total anti-oxidant status (TAS) were measured. RESULTS: The oxidative stress index was significantly lower in pediatric cancer patients compared to the levels in the controls (0.20 ± 0.07 vs. 0.26 ± 0.10; P = 0.001). The mean serum TAS level was significantly higher in patient groups compared to the level in the control (1.87 ± 0.48 vs. 1.63 ± 0.32 mmol/L, P = 0.001). The TAS level of children with cancer in survivors was also found to be significantly higher compared to the levels in the control group (1.85 ± 0.45 vs. 1.63 ± 0.32 mmol/L, P = 0.005). Radiotherapy, surgery, relapsed disease, presence of metastases, and receiving enteral nutritional support caused no change in the TAS/TOS level. CONCLUSION: It has been revealed for the first time that the serum total anti-oxidant level was high in children undergoing chemotherapy and the survivor group as well. Moreover, the oxidative stress index was low in children with cancer. Longitudinal prospective studies are needed to reveal the alterations in oxidant status among children with cancer.
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Antioxidantes , Neoplasias , Niño , Humanos , Antioxidantes/metabolismo , Oxidantes , Estrés Oxidativo , Neoplasias/terapia , Estudios de Casos y ControlesRESUMEN
Background: Non-Hodgkin lymphoma (NHL) includes pathologies of different clinical courses, treatments, outcomes. Our study aims to investigate the late effects of NHL survivors (NHLS). Materials and Methods: Among 59 NHL cases, 50 survivors completed their NHL treatment between 2003 and 2019. Out of 59 patients, the cumulative survival rates and event-free survival rates after 10 years since diagnosis were 82.9% ±5.2% and 84.1% ±5.2%, respectively. In addition, we compared the data related to chronic health and psychosocial conditions with their siblings (n = 61). Results: The age and gender ratios were similar in the NHLS (n = 50) and the control group (n = 61). The rate of nephrotoxicity (P = 0.02) and the frequency of admission to the hospital (P < 0.01) were significantly higher in the survivors than in the control group. Cardiotoxicity is detected in 3 (6%) of NHLS with cumulative anthracycline dose <300 mg/m2. The social status (being married [P < 0.01], having children [P = 0.003]) is impaired in NHLS. The alcohol and smoking habits, education status, and health conditions (endocrinologic, cardiac, neurological, and pulmonary) were similar in both groups. One patient had acute myeloid leukemia as a secondary malignancy. Twenty NHLS took rituximab, two of them took brentuximab vedotin plus chemotherapy. NHLS have impairment in health status, social life. Conclusion: Nephrotoxicity is a statistically more common late effect than the others in the survivors. We observe cardiotoxicity in low cumulative doses of anthracycline. A more significant number of patients is required to reveal late side effects on novel drugs.
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Cardiotoxicidad , Linfoma no Hodgkin , Adolescente , Antraciclinas/uso terapéutico , Antibióticos Antineoplásicos/uso terapéutico , Brentuximab Vedotina , Niño , Humanos , Linfoma no Hodgkin/diagnóstico , Linfoma no Hodgkin/tratamiento farmacológico , Rituximab/uso terapéuticoRESUMEN
INTRODUCTION: Parvovirus B19 infection generally presents as a transient viral illness in children but rarely shows systemic lupus erythematosus-like symptoms. CASE REPORT: Here we present a 7-year-old girl with parvovirus B19 infection who had prolonged fever, rash, pancytopenia and hypocomplementemia making it difficult do differentiate from the first episode of systemic lupus erythematosus. Because she had severe progressive pancytopenia she was administered intravenous immune globulin treatment and her clinical course was dramatically improved. DISCUSSION: Parvovirus B19 infection can mimic systemic lupus erythematosus and it may be difficult to differentiate a recent parvovirus B19 infection and the first presentation of systemic lupus erythematosus. Absence of discoid lesions, alopecia, Raynaud phenomenon and autoimmune hemolytic anemia may help to distinguish parvovirus B19 infection from systemic lupus erythematosus. CONCLUSIONS: Parvovirus B19 infection may cause a severe clinical picture resembling systemic lupus erythematosus even in otherwise healthy children. Intravenous immune globulin treatment might be considered in cases resistant to supportive management.
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Balkaya E, Bozkurt C, Aksu AE, Özmen S, Incesoy-Özdemir S, Sahin G. Ewing`s sarcoma of the mandible misdiagnosed as periodontal inflammation: Report of three cases. Turk J Pediatr 2017; 59: 704-707. Ewing`s sarcoma (ES) is the second most common childhood primary malignant tumor of the bone. The most popular locations of ES are long bones and pelvis. The involvement of the mandible is very rare in childhood. In last 10 years, we met with three cases of ES of the mandible in our department. Initially the patients had symptoms similar to periodontal inflammation. The involvement of the mandible might be considered with periodontal inflammation in its initial stages, what frequently leads to delayed treatment. Although this tumor has an aggressive clinical behavior and rapid growth, early diagnosis can reduce patient`s morbidity and mortality and thus it is important to distinguish from periodontal inflammation.
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OBJECTIVE: The role of cord milking as an alternative to delayed cord clamping is an area that requires more research. Purpose of this clinical trial was to investigate the impact of umbilical cord milking on the absolute neutrophil counts (ANCs) and the neutropenia frequency of preterm infants. METHODS: Fifty-eight pregnant women were randomly assigned to one of the umbilical cord milking and control groups. A total of 54 preterm infants (gestational age ≤ 32 weeks) were enrolled into the study. The umbilical cords of 25 infants were clamped immediately after birth, and in 29 infants, umbilical cord milking was performed first. RESULTS: The ANCs were statistically significantly lower in the cord milking group compared with the control group on days 1, 3 and 7. The frequency of neutropenia was higher in the cord milking group compared with the control group. CONCLUSION: In our study, ANCs were lower in the cord milking group and the frequency of neutropenia was higher. Umbilical cord milking plays a role on the ANCs of preterm infants.
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Recien Nacido Prematuro , Atención Posnatal , Cordón Umbilical , Recuento de Células , Constricción , Femenino , Humanos , Recién Nacido , Masculino , Neutropenia/etiología , Neutrófilos/citología , Recuento de Plaquetas , EmbarazoRESUMEN
In this study, clinical characteristics, treatment modalities and outcome of patients diagnosed with bladder/prostate rhabdomyosarcoma (BP RMS) were evaluated retrospectively. Files of 8 children diagnosed with BP RMS and treated between 2004-2014 were reviewed for clinical characteristics, treatment modalities and outcome. Seven males and one female were diagnosed with BP RMS between 2004-2014. Median age was 33.5 months (range, 2 to 176 months). At presentation the main clinical symptoms were hematuria in 5 patients, and constipation, oliguria and prolonged jaundice in 1 patient each. All patients were non-metastatic and only one had an embryonal histology. Primary resection before chemotherapy was performed on only one patient. Six patients were treated initially with VAC chemotherapy for 12 weeks, two patients were treated PIAV (ifosfamide, cisplatin, doxorubicin, vincristine). Local relapse or progressive disease occurred in 5 of 8 patients, and two of these patients underwent primary or secondary tumor resection without radiotherapy. Three patients developed a local relapse after combination of radiochemotherapy and tumor resection. Radical surgical treatment was performed in 3 patients with local relapse. Only one patient underwent partial cystectomy. Six of 8 patients were alive and under follow-up without disease at a median survival of 53 months (range, 13 to 78 months). BP RMS requires a multidisciplinary treatment approach. There is a general consensus that chemotherapy is the mainstay of treatment in BP RMS, but the method to be used for local control is controversial, and may vary from case to case in this heterogeneous disease.