RESUMO
BACKGROUND: The quantitative assessment of neuroblastoma cell content in bone marrow aspirates for response evaluation has been introduced recently. Data on the concordance of interobserver reports are lacking so far. METHODS: Investigators of seven European countries representing national reference or large oncological centers convened in 2016. They agreed to quantitatively assess routine bone marrow smears of the participating institutions and to discuss the discrepant results in joint meetings. RESULTS: From 2017 through 2019, three cytology rounds with 24, 28, and 28 bone marrow samples were run evaluating the representativity of the smears (yes/[restricted]/no) and the presence of tumor cells (yes/no and %). The comparison of the reports using κ (Fleiss) and α (Krippendorff) statistics demonstrated no robust reliabilities. The agreement on the representativity was moderate to poor, on the presence of tumor cells moderate to good, and on the percentage of tumor cells slight to moderate. Though the value of cytology is unquestioned to detect even tiny metastatic cells in bone marrow, the investigators unanimously agreed that a reliable quantification of the tumor cell content in bone marrow smears is unrealistic. For the key issue of representativity, a new practical definition was developed. CONCLUSION: For any work with bone marrow aspirates, the representativity of the material is of paramount importance. A practical definition is proposed. A reliable quantitative cytological assessment of tumor cell content in bone marrow aspirates is not feasible in metastatic neuroblastoma. Therefore, its use as response criterion should be reconsidered.
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Exame de Medula Óssea/métodos , Neoplasias da Medula Óssea/secundário , Citodiagnóstico/métodos , Neuroblastoma/patologia , Seguimentos , Humanos , Prognóstico , Reprodutibilidade dos TestesRESUMO
About a quarter of children with Down syndrome and transient abnormal myelopoiesis (TAM) progress to acute megakaryoblastic leukemia (AMKL). We describe isolated dysmegakaryopoiesis despite complete resolution of TAM in an 18-month-old girl, who developed AMKL 6 months later.
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Síndrome de Down/sangue , Síndrome de Down/complicações , Fator de Transcrição GATA1/genética , Leucemia Megacarioblástica Aguda/etiologia , Reação Leucemoide/etiologia , Pré-Leucemia/etiologia , Trombocitopenia/etiologia , Trombopoese , Linhagem da Célula , Núcleo Celular/ultraestrutura , Células Clonais , Progressão da Doença , Síndrome de Down/etiologia , Síndrome de Down/genética , Feminino , Humanos , Recém-Nascido , Leucemia Megacarioblástica Aguda/genética , Leucemia Megacarioblástica Aguda/patologia , Reação Leucemoide/genética , Megacariócitos/patologia , Pré-Leucemia/patologia , Vacúolos/ultraestruturaRESUMO
OBJECTIVE: The need for pediatric antifungal stewardship programs has been driven by an increasing consumption of antifungals for prophylactic and empirical use. Drivers and rational of antifungal prescribing need to be identified to optimize prescription behaviors. METHODS: A prospective modified weekly Point Prevalence Survey capturing antifungal prescriptions for children (> 90 days to < 18 years of age) in 12 centers in England during 26 consecutive weeks was performed. Demographic, diagnostic and treatment information was collected for each patient. Data were entered into an online REDCap database. RESULTS: One thousand two hundred fifty-eight prescriptions were included for 656 pediatric patients, 44.9% were girls, with a median age of 6.4 years (interquartile range, 2.5-11.3). Most common underlying condition was malignancy (55.5%). Four hundred nineteen (63.9%) received antifungals for prophylaxis, and 237 (36.1%) for treatment. Among patients receiving antifungal prophylaxis, 40.2% did not belong to a high-risk group. In those receiving antifungal treatment, 45.9%, 29.4%, 5.1% and 19.6% had a diagnosis of suspected, possible, probable of proven invasive fungal disease (IFD), respectively. Proven IFD was diagnosed in 78 patients, 84.6% (n = 66) suffered from invasive candidiasis and 15.4% (n = 12) from an invasive mold infection. Liposomal amphotericin B was the most commonly prescribed antifungal for both prophylaxis (36.6%) and empiric and preemptive treatment (47.9%). Throughout the duration of the study, 72 (11.0%) patients received combination antifungal therapy. CONCLUSIONS: Antifungal use in pediatric patients is dominated by liposomal amphotericin B and often without evidence for the presence of IFD. A significant proportion of prophylactic and empiric antifungal use was seen in pediatric patients not at high-risk for IFD.
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Antifúngicos/uso terapêutico , Infecções Fúngicas Invasivas/tratamento farmacológico , Adolescente , Anfotericina B , Gestão de Antimicrobianos/métodos , Antiprotozoários/uso terapêutico , Azóis/uso terapêutico , Candidíase/tratamento farmacológico , Candidíase Invasiva/tratamento farmacológico , Criança , Pré-Escolar , Inglaterra , Feminino , Humanos , Lipossomos/uso terapêutico , Masculino , Prescrições , Estudos ProspectivosRESUMO
OBJECTIVE: The objective of this study was to report our multidisciplinary diagnostic approach for patients with anterior mediastinal masses (AMM). METHODS: A retrospective review of patients with AMM at a tertiary pediatric surgical oncology centre (January 2011-December 2016) was performed. We analyzed data on clinical presentation, mode of tissue diagnosis, anesthetic techniques, and complications. RESULTS: Of the 44 patients admitted with AMM (median age 11â¯years, 27 males and 17 females), 22 had respiratory symptoms. Imaging revealed tracheobronchial compression in 26 children. Twenty patients had a lymph node biopsy. Ten patients had image-guided core biopsy of the mediastinal mass, and 2 had mediastinoscopic biopsy of a paratracheal lymph node. One patient with likely recurrence of a relapsed metastatic ethmoid carcinoma did not have a biopsy. The diagnosis was made from alternative tissues, such as pleural fluid in 4 and peripheral blood in 7 patients. Twenty-five anesthetics were assessed, as 14 patients required no or only local anesthesia, and 5 had unavailable anesthetic notes. Eighteen of 25 patients were anesthetized maintaining spontaneous breathing, mostly by means of ketamine sedation. There were no major anesthetic complications. CONCLUSION: Safe tissue diagnosis of anterior mediastinal masses can be obtained by a personalized multidisciplinary approach. Use of alternative tissues, local anesthesia, and ketamine sedation help minimize the need for general anesthesia, muscle paralysis, and controlled ventilation. LEVEL OF EVIDENCE: IV (Case Series with no Comparison Group).
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Linfonodos/patologia , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/patologia , Adolescente , Anestesia Geral , Anestesia Local , Anestésicos Dissociativos , Criança , Pré-Escolar , Feminino , Humanos , Biópsia Guiada por Imagem , Ketamina , Masculino , Neoplasias do Mediastino/complicações , Mediastinoscopia , Equipe de Assistência ao Paciente , Derrame Pleural/etiologia , Doenças Respiratórias/etiologia , Estudos RetrospectivosRESUMO
INTRODUCTION: For patients with primary refractory and relapsed acute leukaemias allogeneic stem cell transplantation is the only hope for cure, but morphological remission is not always achieved after standard salvage regimens. Here we review the experience with high-dose etoposide and cyclophosphamide (HD-Et/Cy) in relapsed/refractory acute leukaemias at the Royal Marsden Hospital. PATIENTS AND METHODS: Twenty-three patients (15 adults, 8 children) with refractory/relapsed acute myeloblastic (n = 18; 78%), lymphoblastic (n = 4; 17%) or biphenotypic (n = 1; 4%) leukaemia who had failed to respond to at least one previous line of chemotherapy received HD-Et/Cy at our institution between 2006 and 2015. RESULTS: Overall response rate was 21.7% (95%CI 4.0-40.0). Median overall survival was 14.8 months (95%CI 9.1-49.1). Eight (35%) patients (7 AML, 1 biphenotypic leukaemia) proceeded to allogeneic transplant after one cycle of HD-Et/Cy: four of them (50%; 3 adults, 1 child) in complete remission and another four children (50%) with aplastic bone marrow with scattered blasts. Among the transplant recipients, three with AML (38%), ie. one adult (responder) and two children with aplastic bone marrow with scattered blasts, became long-term survivors 9.8, 4.4 and 2.5 years post-HD-Et/Cy, respectively. Toxicity profile was comparable to similar regimens with no treatment-related deaths. The most common grade 3-4 toxicity was febrile neutropenia (96%). CONCLUSIONS: HD-Et/Cy can salvage patients with refractory/relapsed AML who remain candidates for allogeneic stem cell transplantation after failure of standard salvage regimens and do not have access to clinical trials.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/tratamento farmacológico , Adolescente , Adulto , Fatores Etários , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Biomarcadores , Criança , Ciclofosfamida/administração & dosagem , Resistencia a Medicamentos Antineoplásicos , Etoposídeo/administração & dosagem , Feminino , Humanos , Imunofenotipagem , Leucemia Mieloide Aguda/mortalidade , Masculino , Recidiva , Retratamento , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Diagnostic challenges combined with the vulnerability of neonates to develop invasive candidiasis (IC) may lead to antifungal administration in the absence of IC. A modified point-prevalence study was performed to obtain an improved insight and understanding of antifungal prescribing in this specific patient population. METHODS: Neonates and infants ≤90 days of age receiving systemic antifungals from 12 centers in England were included. Data were collected prospectively during 26 consecutive weeks and entered into an online REDCap database. RESULTS: Two hundred eighty neonates and infants were included, the majority ≤1 month of age (68.2%). Prematurity was the commonest underlying condition (68.9%). Antifungals were prescribed for prophylactic reason in 79.6%; of those, 64.6% and 76.3% were extreme low birth weight infants and prematurely born neonates, respectively. Additional risk factors were present in almost all patients, but only 44.7% had ≥3 risk factors rendering them more susceptible to develop IC. Nonpremature and non extremely low birth weight premature infants only scored ≥3 risk factors in 32.6% and 15%, respectively. Fluconazole was the most common antifungal used (76.7% of all prescriptions), and commonly underdosed as treatment. The number of microbiologic proven IC was low, 5.4%. CONCLUSIONS: Neonatal antifungal prophylaxis is commonly prescribed outside the recommendations based on known risk profiles. Fluconazole is the main antifungal prescribed in neonates and infants, with underdosing frequently observed when prescribed for treatment. Number of proven IC was very low. These observations should be taken into consideration to develop a national pediatric Antifungal Stewardship program aiming to guide rational prescribing.
Assuntos
Antifúngicos/administração & dosagem , Gestão de Antimicrobianos , Doenças do Prematuro/prevenção & controle , Prescrições/estatística & dados numéricos , Absorção Fisiológica , Candidíase/prevenção & controle , Candidíase Invasiva/prevenção & controle , Quimioprevenção , Inglaterra , Feminino , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro/microbiologia , Recém-Nascido de muito Baixo Peso , Masculino , Prescrições/normas , Prevalência , Estudos Prospectivos , Fatores de RiscoRESUMO
OBJECTIVE: To assess the outcome of children older than one year with neuroblastoma treated at King Abdul-Aziz Medical City, Jeddah, Kingdom of Saudi Arabia. METHODS: We retrospectively reviewed the files of 52 children older than one year with neuroblastoma (NBL) treated at our center between September 1987 and May 2003. Treatment consisted of OPEC chemotherapy regimen (vincristine, cisplatin, etoposide, and cyclophosphamide) or alternating OPEC/OJEC (carboplatin in place of cisplatin), surgical resection +/- radiotherapy (RT). No patient received high dose therapy (HDT). RESULTS: Thirty-four patients (65%) were stage 4, 12 (23%) stage 3, and 6 (11%) stage 2. Three stage 2 patients were treated with surgery only, all are alive in complete remission (CR). All stage 3 and 4 patients were treated with chemotherapy and surgery +/- RT. After induction chemotherapy, CR was achieved in 17 patients (32%) and partial remission in 10 (19%). Complete surgical resection was possible in 11 patients (22%). Disease recurrence or progression occurred in 27 patients (51%). With a median follow-up of 24 months (range 4-120), the 2-year event free survival was 10%, 82%, and 87% and the overall survival was 12%, 83%, and 100% for stage 4, 3, and 2. CONCLUSION: Children older than one year with localized NBL have good prognosis compared to those with stage 4. The use of HDT may improve the outcome in the latter group. Toxicity was significant, and adoption of risk-stratified treatment may help to reduce treatment complications.
RESUMO
OBJECTIVE: To review the clinical features and outcome of all cases of stage 4S neuroblastoma treated at our center. METHODS: We retrospectively reviewed the files of all patients (n=75) with neuroblastoma treated at King Abdul-Aziz Medical City, Jeddah, Kingdom of Saudi Arabia between 1986 and 2005. We studied the clinical features and outcome of patients with stage 4S neuroblastoma. RESULTS: Six patients (8%) were confirmed to have stage 4S neuroblastoma. Three were boys with a median age at diagnosis of 4.5 months (range 28 days-11 months). Four patients required no intervention. The remaining 2 patients were treated with chemotherapy due to progressive hepatomegaly and respiratory distress. No patient required radiotherapy or surgical intervention. With a median follow up of 4 years (range 9 months-15.5 years), all patients are alive and well. Two patients continue to have a residual abdominal mass, while complete resolution occurred in the others. CONCLUSION: Stage 4S neuroblastoma is a special tumor that carries excellent prognosis. Spontaneous regression may occur and intervention is only required in symptomatic patients.
Assuntos
Neoplasias das Glândulas Suprarrenais , Neoplasias Hepáticas , Neuroblastoma , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/tratamento farmacológico , Neoplasias das Glândulas Suprarrenais/patologia , Glândulas Suprarrenais/patologia , Fatores Etários , Antineoplásicos/administração & dosagem , Antineoplásicos/uso terapêutico , Antineoplásicos Alquilantes/administração & dosagem , Antineoplásicos Alquilantes/uso terapêutico , Antineoplásicos Fitogênicos/administração & dosagem , Antineoplásicos Fitogênicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Carboplatina/administração & dosagem , Carboplatina/uso terapêutico , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Etoposídeo/administração & dosagem , Etoposídeo/uso terapêutico , Feminino , Seguimentos , Hepatomegalia/diagnóstico , Hepatomegalia/etiologia , Humanos , Lactente , Recém-Nascido , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/patologia , Masculino , Regressão Neoplásica Espontânea , Estadiamento de Neoplasias , Neuroblastoma/diagnóstico , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/tratamento farmacológico , Neuroblastoma/mortalidade , Neuroblastoma/patologia , Neuroblastoma/secundário , Prognóstico , Estudos Retrospectivos , Arábia Saudita , Pele/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/secundário , Análise de Sobrevida , Fatores de TempoRESUMO
OBJECTIVE: To compare differences in demographics and outcomes in childhood Hodgkin lymphoma (HL) presenting at the Children's Hospital Lahore (CHL), and Royal Marsden Hospital (RMH), UK. STUDY DESIGN: An observational comparative study. PLACE AND DURATION OF STUDY: From January 2011 to February 2012 at CH, Lahore and from October 2008 to February 2012 at RMH, UK. METHODOLOGY: Consecutive HL patients (50 from each hospital) were inducted. Data regarding age, gender, staging, histopathology and outcome were analysed. Clinical and pathological staging done according to Ann-Arbor and World Health Organization classification. Treatment duration was 6-8 months. They were followed for 6 months post-treatment. Frequencies of variables were noted and compared. Chi-square test was used for determining significance. RESULTS: Patients from Children's Hospital, Lahore were younger (mean 7.9 years) with male predominance (n=42, 84%). Histopathology showed Mixed Cellularity (MC) in 32 (64%), Nodular Sclerosis (NS) in 5 (10%), Lymphocyte Rich in 4 (8%) and lymphocyte depleted in 1 (2%), nodular lymphocyte predominant (NLP) in 1 (2%) each. Majority presented in stage IV (n=25,50%), or stage III (n=20,40%). Constitutional B symptoms were present in 37 (74%). Bone marrow involvement observed in 23 (46%). Remission was achieved in 42 (84%) patients; 2 (4%) relapsed, 4 (8%) expired and 2 (4%) left against medical advice. In contrast, RMH patients were older (mean 11.8 years.) and 30 (60%) were males. NS (n=40,80%) and NLP (n=6,12%) types were predominant. Two (4%) patients were in stage I, 27 (54%) in stage II, 12 (24%) in stage III and 9 (18%) presented in stage IV. Fourteen (28%) had B-symptoms. None had bone marrow disease. Event free survival was 46 (92%). Four (8%) patients relapsed. Three responded to second line therapy and one relapsed postautologous transplant. CONCLUSION: Significant differences were observed in age at presentation, stage, histopathology and extent of bone marrow involvement between the groups. Of interest is the bone marrow involvement in stage IV patients in Pakistan. Delayed diagnosis account for advanced stage but difference in pathological subtype needs further study.
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Doença de Hodgkin/mortalidade , Doença de Hodgkin/patologia , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Doença de Hodgkin/diagnóstico , Hospitais Pediátricos , Humanos , Lactente , Masculino , Estadiamento de Neoplasias , Paquistão/epidemiologia , Características de Residência , Fatores Socioeconômicos , Taxa de Sobrevida , Resultado do TratamentoAssuntos
Células da Medula Óssea/patologia , Síndrome de Chediak-Higashi/diagnóstico , Síndrome de Chediak-Higashi/patologia , Células Precursoras de Granulócitos/patologia , Síndrome de Chediak-Higashi/fisiopatologia , Pré-Escolar , Hematopoese/fisiologia , Humanos , Lisossomos/patologia , MasculinoAssuntos
Neoplasias da Mama/líquido cefalorraquidiano , Carcinoma Ductal de Mama/líquido cefalorraquidiano , Carcinoma Ductal de Mama/secundário , Linfocitose/líquido cefalorraquidiano , Neoplasias da Medula Espinal/líquido cefalorraquidiano , Neoplasias da Medula Espinal/secundário , Neoplasias da Mama/genética , Carcinoma Ductal de Mama/genética , Carcinoma Ductal de Mama/imunologia , Líquido Cefalorraquidiano/citologia , Contagem de Eritrócitos , Feminino , Genes erbB-2 , Granulócitos/patologia , Humanos , Contagem de Leucócitos , Linfocitose/etiologia , Pessoa de Meia-Idade , Neoplasias da Medula Espinal/genética , Neoplasias da Medula Espinal/imunologia , Punção EspinalRESUMO
Soft tissue sarcomas (STS) are highly malignant tumours that constitute 5-6% of all malignant childhood neoplasms. Of these, rhabdomyosarcoma (RMS) is the most common in children, and has a characteristic two-peak age incidence, 2-5 and 15-19 years. Most children with RMS are cured with conventional chemotherapy and local therapy (surgery with or without radiotherapy). Children with metastatic disease at presentation, particularly those older than 10 years or with bone marrow or bone involvement have a much poorer outcome. In this subgroup, high-dose therapy with stem cell rescue has been studied over the last two decades. Various single or multiagent chemotherapy regimens with or without radiotherapy and autologous stem cell rescue have been used as consolidation treatment with little success. Recent trials using sequential high-dose chemotherapy in the early phase of treatment have proved to be feasible, but the beneficial effect has to be confirmed. The role of purging remains unclear. Collaboration between different international groups is urgently required, in an attempt to improve the poor outcome of children with high risk STS.
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Antineoplásicos/administração & dosagem , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Transplante de Medula Óssea , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Terapia Combinada , Humanos , PrognósticoAssuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia Mieloide Aguda/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Citarabina/administração & dosagem , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Feminino , Humanos , Lactente , Leucemia Mieloide Aguda/mortalidade , Masculino , Estudos Retrospectivos , Arábia Saudita/epidemiologia , Taxa de Sobrevida , Resultado do Tratamento , Vidarabina/administração & dosagem , Vidarabina/análogos & derivadosRESUMO
This case of genital ulceration and social concerns in a preschool aged child was highly suspicious for child sexual abuse. However, the lesions presumed to be herpes simplex did not respond to antiviral medication. Specialist input from paediatric oncology and endocrinology resulted in a rare diagnosis of langerhans cell histiocytosis. Furthermore, the complication of diabetes insipidus later developed, making this the youngest child described to our knowledge with vulval lesions of langerhans cell histiocytosis and a central nervous system complication.
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Abuso Sexual na Infância/diagnóstico , Histiocitose de Células de Langerhans/patologia , Úlcera Cutânea/etiologia , Doenças da Vulva/patologia , Pré-Escolar , Diagnóstico Diferencial , Feminino , Herpes Genital/diagnóstico , Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/tratamento farmacológico , Humanos , Doenças da Vulva/virologiaRESUMO
Severe hyperlipidaemia with asparaginase therapy is rare. We report six cases, four of which developed significant problems with severe hyperlipidaemia during induction therapy for ALL and lymphoblastic lymphoma. The median triglyceride level was 22.3 mmol/L and the median cholesterol level was 12.3 mmol/L. None of the patients showed signs or symptoms of pancreatitis. Three children were re-exposed with Peg asparaginase, and one with Erwinia asparaginase, without recurrence of hyperlipidaemia or other symptoms. These cases highlight the dilemma in managing such rare cases of symptomatic hypertriglyceridaemia secondary to asparaginase and steroid therapy.
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Asparaginase/efeitos adversos , Asparaginase/uso terapêutico , Terapia de Reposição de Enzimas/efeitos adversos , Hipertrigliceridemia/induzido quimicamente , Polietilenoglicóis/efeitos adversos , Polietilenoglicóis/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , MasculinoRESUMO
Viral haemorrhagic cystitis (HC) is a significant complication after haematopoietic stem cell transplantation (HSCT), with a potential for major morbidity. The aim of this 7-year analysis of 1160 HSCT patients was to evaluate risk factors for the incidence, severity, toxicity of therapy, clinical course, and outcome of this condition. The overall incidence of HC was 5·8%, with most cases occurring after allogeneic HSCT. Unrelated donors (P = 0·001), non-peripheral blood stem cell source (P = 0·005), myeloablative conditioning (P<0·001), use of alemtuzumab in conditioning (P = 0·001), and severe acute graft versus host disease (P<0·001) were independent risk factors for an increased incidence of HC post-allogeneic transplant on multivariate analysis. Severe forms of HC were associated with grades II-IV acute graft versus host disease and a longer duration of haematuria. Contrary to previous studies which were carried out on smaller patient populations, busulphan, cyclophosphamide, anti-thymocyte globulin, and total body irradiation were not found to independently increase the risk of viral HC, unless used in a myeloablative combination. Neither duration of viriuria nor peak viral load in urine influenced the severity of HC on multivariate analysis. Severe HC contributed to the deaths of two patients. Overall survival was not statistically different between patient subgroups with non-severe and severe HC.
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Cistite/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hemorragia/etiologia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Cistite/terapia , Coleta de Dados , Feminino , Hemorragia/terapia , Humanos , Incidência , Lactente , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Resultado do Tratamento , Adulto JovemRESUMO
We reviewed the results of two consecutive United Kingdom Childrens' Cancer Study Group (UKCCSG) studies of children with stage IV Hodgkin's Disease (HD) treated between January 1982 and December 1999. Among 697 children with HD, 67 were diagnosed to be stage IV. The median age at diagnosis was 12.7 years (range 4.4-16.2). Thirty-five (52%) were boys. Thirty-nine patients (58%) had B symptoms at diagnosis. All were treated with 6-8 cycles of ChlVPP chemotherapy regimen (Chlorambucil, Vinblastine, Procarbazine and prednisolone) and only 12 had radiotherapy. The overall survival (OS) at 5 and 10 years was 80.8% and 77.2%, respectively, whilst the event-free survival (EFS) at the same time intervals was 55.2% and 48.8% respectively. Twenty-eight patients (41.79%) relapsed/progressed, 18 (64%) survived after further chemotherapy with or without high-dose therapy and stem cell rescue. Twelve patients died, seven of HD, three from infections and one from secondary acute myeloblastic leukaemia (AML). Although the EFS in this study was lower than other studies, 64% of relapsed patients were salvaged with second-line therapy. It is also anticipated that survivors treated with this non-anthracycline-containing regimen will have less long-term toxicity.