RESUMO
An adequate bone marrow aspirate is essential for a rapid diagnosis of acute leukaemia by multicolour flow cytometry enabling the simultaneous assessment of multiple antigens on the cell surface as well as intracellular or nuclear ones. In the context of acute leukaemia, it is important to have a diagnosis of the blasts lineage as soon as possible to decide the appropriate treatment. This is sometimes delayed due to difficulties in obtaining a bone marrow aspirate due to a "dry tap". In this study we evaluated retrospectively cell markers results by flow cytometry of unfixed bone marrow trephines of 65 patients with leukaemia at diagnosis and including a few after treatment. Our aims were: 1) To compare cell markers results between bone marrow trephine (BMT) and bone marrow aspirate (BMA) 24 cases and BMT with peripheral blood (PB) 14 cases in paired samples to establish if they were reproducible with results of the unfixed bone marrow trephine biopsies. 2) To ascertain a precise diagnosis in 27 (42%) of the cases in which only a bone marrow trephine was available. We demonstrated that unfixed bone marrow trephine provides an adequate and representative cell suspension for flow cytometry and it is a powerful tool when no other material (bone marrow aspirate or peripheral blood) is available to make a rapid diagnosis. Furthermore when marrow aspirate or peripheral blood paired samples were available, flow cytometry results obtained were identical across all the sample types. Applicability to the clinical laboratory: We described a method to obtain a cell suspension from core biopsies that can easily be implemented routinely in a laboratory that performs diagnostic flow cytometry immunophenotyping. This method is simple, inexpensive and it doesn't require extra equipment.
Assuntos
Biomarcadores Tumorais/sangue , Citometria de Fluxo/métodos , Neoplasias Hematológicas/sangue , Imunofenotipagem/métodos , Biópsia , Células da Medula Óssea/patologia , Neoplasias Hematológicas/imunologia , Neoplasias Hematológicas/patologia , Humanos , Baço/patologiaRESUMO
BACKGROUND: Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy (SHML), usually affects young adults and commonly presents with massive painless cervical lymphadenopathy. Extranodal disease is present in a third of patients, and it is recognised that this can involve the central nervous system. Intracranial RDD is rare in adults and fewer than 10 paediatric cases have been reported. CASE: A 10-year-old boy with isolated intracranial RDD presents with a painless forehead mass. The management is discussed and the literature reviewed. CONCLUSION: This case of isolated intracranial RDD highlights the importance of considering RDD in the differential of paediatric intracranial mass lesions and outlines the diagnostic and treatment challenges faced when managing this rare condition.
Assuntos
Histiocitose Sinusal , Criança , Histiocitose Sinusal/diagnóstico , Histiocitose Sinusal/fisiopatologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Tomógrafos ComputadorizadosRESUMO
We reviewed the pattern of acute neurotoxicity in children with B-non-Hodgkin's lymphoma (B-NHL) and B-acute lymphoblastic leukaemia (ALL) treated with the UKCCSG 9002/9003 protocols. Among 175 patients, 21 (12%) developed acute neurotoxicity: 9002 protocol (n=11/112) and 9003 (n=10/63). There were 20 boys and the median age was 10 years. Patients with neurological symptoms due to other causes were excluded. Acute neurological symptoms developed following induction chemotherapy in 7 patients, or after a more intensive course of chemotherapy containing high-dose methotrexate (n=14). Nine patients required their chemotherapy to be altered because of the acute neurotoxicity. One patient died of cerebral haemorrhage but none of the remaining six deaths was attributed to acute neurotoxicity. We conclude that acute neurotoxicity is common in children treated with the 9002/9003 protocols and tends to be transient. Intrathecal and systemic chemotherapy including high-dose methotrexate is probably the most common predisposing factor. Modification of subsequent chemotherapy is not invariably necessary.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Linfoma de Burkitt/tratamento farmacológico , Linfoma de Células B/tratamento farmacológico , Doenças do Sistema Nervoso/induzido quimicamente , Doença Aguda , Adolescente , Anticonvulsivantes/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma de Burkitt/complicações , Criança , Pré-Escolar , Protocolos Clínicos , Feminino , Humanos , Linfoma de Células B/complicações , Imageamento por Ressonância Magnética , Masculino , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/tratamento farmacológico , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Reino UnidoRESUMO
The VEEP regimen (vincristine, etoposide, epirubicin, prednisolone), with or without involved field radiotherapy, has been shown to be an effective treatment in adult Hodgkin's disease. In an attempt to avoid the late sequelae of both alkylating agents and radiotherapy this regimen has been studied in a series of 54 children and young adults. Early analysis suggested that the relapse rate was higher with VEEP than with standard alkylating agent-based regimens. Sufficient follow-up has now been achieved to evaluate the likelihood of sustained remission following second-line treatment and therefore the overall long term survival with this treatment approach. The 5-year Overall Survival (OS) and 5-year Progression Free Survival (PFS) for patients with stage I-III disease was 93% and 82% respectively. However, the 5-year OS and PFS for stage IV patients was only 44% and 50%, respectively. Of 13 patients who were initial treatment failures on VEEP, 7 of whom had advanced disease, only 6 were salvaged with second-line therapy. 8 of 33 who attained a complete response (CR) relapsed and there were 2 relapses in those achieving a partial response (PR) (n = 8). All those relapsing from CR/PR were salvaged by second-line alkylating agent chemotherapy +/- radiotherapy, +/- high dose chemotherapy. In conclusion, patients with stage I-IIIA, non-bulky disease, the moderately high relapse rate did not adversely affect the overall high cure rate, although VEEP failures were subjected to a high total treatment burden. VEEP alone is inadequate in patients with stage IV disease, bulky mediastinal disease in/or those with B symptoms in whom there is a high primary failure rate and relatively poor results with second line therapy.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença de Hodgkin/tratamento farmacológico , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Progressão da Doença , Intervalo Livre de Doença , Epirubicina/administração & dosagem , Etoposídeo/administração & dosagem , Feminino , Seguimentos , Humanos , Masculino , Prednisolona/administração & dosagem , Recidiva , Terapia de Salvação , Vincristina/administração & dosagemRESUMO
Relapse patterns have been documented in 191 children with localised Ewing's sarcoma treated with the United Kingdom Children's Cancer Group (UKCCSG) Ewing's Tumour regimen ET2. All received chemotherapy comprising ifosfamide, vincristine, doxorubicin and actinomycin D. Local treatment modality was excision and or radiotherapy depending on tumour site and response to primary chemotherapy. Although not strictly comparable, due to the clinical indications used for each modality, local relapse rates were very low and were similar, irrespective of the type of local treatment modality: radiotherapy (3/56), surgery (7/114) or a combination (0/20). Combined relapse (local + distant) rates were similarly low irrespective of the type of local therapy: radiotherapy (4/56), surgery (4/114) or a combination (0/20). Overall survival was lower in females (P = < 0.04), older children (P = < 0.002) and those with primaries at sites other than long bones (P = < 0.02). It is concluded that with effective intensive chemotherapy combined with either radiotherapy or surgery, local control in this study was excellent at sites other than the pelvis. Preventing distant relapse, predominantly to lung and bone, remains the major challenge.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/terapia , Sarcoma de Ewing/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Terapia Combinada , Dactinomicina/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Feminino , Seguimentos , Humanos , Ifosfamida/administração & dosagem , Lactente , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Recidiva Local de Neoplasia , Resultado do Tratamento , Vincristina/administração & dosagemRESUMO
Eighteen patients with poor risk Ewing's sarcoma (including 11 patients with metastatic disease at presentation) received consolidation therapy of busulphan and melphalan with autologous stem cell rescue. There were nine females. The median age at diagnosis was 14.2 years (range 2.75-30 years). There was one early death due to cytomegalovirus pneumonitis. One patient developed a single generalised convulsion during busulphan therapy. Severe renal toxicity was not encountered. One patient developed veno-occlusive disease of the liver (VOD) which eventually resolved. With a median follow up of 2 years, 13 patients survive including six with initial metastatic disease. We conclude that high-dose busulphan/melphalan is well-tolerated and should be evaluated for efficacy in a larger series of patients with high risk Ewing's sarcoma.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/terapia , Transplante de Células-Tronco Hematopoéticas , Sarcoma de Ewing/terapia , Adolescente , Adulto , Anticonvulsivantes/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/patologia , Bussulfano/administração & dosagem , Bussulfano/efeitos adversos , Criança , Pré-Escolar , Clonazepam/uso terapêutico , Terapia Combinada , Contraindicações , Intervalo Livre de Doença , Feminino , Hepatopatia Veno-Oclusiva/etiologia , Humanos , Masculino , Melfalan/administração & dosagem , Metástase Neoplásica , Fenitoína , Indução de Remissão , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/patologia , Convulsões/induzido quimicamente , Convulsões/prevenção & controle , Análise de Sobrevida , Condicionamento Pré-Transplante/efeitos adversos , Resultado do TratamentoRESUMO
Thalidomide was used to treat acute (n=21) or chronic (n=59) graft-vs-host disease (GVHD) in 80 haematopoietic stem cell allograft recipients after failure to respond to the combination of cyclosporine and corticosteroids with or without other agents. The median time to onset of acute GVHD was 11 days, and thalidomide was started at a median of 48 days post transplant. In addition to corticosteroids and cyclosporine, 13 patients had also received other agents before thalidomide. None of the patients responded and all died of acute GVHD. For chronic GVHD (limited in 13, extensive in 46), thalidomide was started at a median of 385 days post transplant. In addition to corticosteroids and cyclosporine, 34 patients received azathioprine concomitantly. In all patients, thalidomide was added to the ongoing immunosuppressive regimen. The median duration of therapy with thalidomide was 60 days (range, 11-1210; <2 weeks in 11). In total, 13 patients (22%) had complete response, eight (14%) partial response and 38 (64%) no response. Response rates were comparable for limited (39%) and extensive (33%) chronic GVHD. At a median of 53 months, 19 patients are alive, 13 without evidence of chronic GVHD. Survival was significantly better in patients who responded to thalidomide. The principal causes of death were progressive chronic GVHD (n=29) and relapsed leukaemia (n=7). In conclusion, thalidomide has no activity in acute GVHD, but has some activity in chronic GVHD in combination with other agents.
Assuntos
Doença Enxerto-Hospedeiro/tratamento farmacológico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Talidomida/uso terapêutico , Doença Aguda , Adolescente , Corticosteroides/uso terapêutico , Adulto , Criança , Pré-Escolar , Doença Crônica , Ciclosporina/uso terapêutico , Feminino , Doença Enxerto-Hospedeiro/mortalidade , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/mortalidade , Neoplasias Hematológicas/terapia , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante de Células-Tronco Hematopoéticas/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Análise de Sobrevida , Transplante HomólogoRESUMO
From 1984 to 1996, 31 consecutive children without sibling donors, aged 5-19 years (median 8) with acute lymphoblastic leukaemia (ALL) in second complete remission (CR), received unpurged autologous bone marrow transplantation (ABMT) after melphalan and single fraction total body irradiation (TBI). ABMT was performed using fresh unmanipulated marrow harvested after standard reinduction and consolidation therapy 2-11 months (median 5) after relapse. With a median survival of 2.9 years the probability of survival for all patients in continuing second CR was 45.1% (95% CI, 24%-62%) after 5 years. Regimen-related and non-leukaemia mortality was 7% (95% CI, 2%-26%). The longest time to second relapse from ABMT was 3.1 years. Pituitary and gonadal dysfunction requiring hormonal replacement therapy occurred in the majority of long-term survivors. Twelve patients developed cataracts. ABMT with melphalan/single fraction TBI has proved an effective anti-leukaemia treatment with low regimen-related mortality but significant long-term morbidity. The current approach of allogeneic BMT from an unrelated donor when no sibling donor is available, following conditioning with cyclophosphamide/ fractionated TBI has resulted in a reduced relapse rate and improved short-term overall survival in the treatment of relapsed childhood ALL. However, long-term results are awaited.
Assuntos
Transplante de Medula Óssea , Leucemia-Linfoma Linfoblástico de Células Precursoras/cirurgia , Adolescente , Catarata/etiologia , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Febre/microbiologia , Seguimentos , Transtornos Gonadais/tratamento farmacológico , Transtornos Gonadais/etiologia , Sobrevivência de Enxerto , Hormônio do Crescimento Humano/deficiência , Hormônio do Crescimento Humano/uso terapêutico , Humanos , Lactente , Masculino , Melfalan/administração & dosagem , Melfalan/toxicidade , Mucosa Bucal , Neutrófilos , Contagem de Plaquetas , Infecções por Pneumocystis , Pneumonia por Pneumocystis/microbiologia , Estudos Prospectivos , Sepse , Estomatite/microbiologia , Taxa de Sobrevida , Tiroxina/uso terapêutico , Fatores de Tempo , Condicionamento Pré-Transplante , Transplante Autólogo , Irradiação Corporal TotalRESUMO
As there is concern regarding the high carcinogenic potential of melphalan (Mel), 725 patients with haematological malignancies who received allogeneic (n = 714) or syngeneic (n = 11) transplants over the last 26 years were followed-up to evaluate if melphalan was more likely to result in secondary malignant neoplasms (SMNs) than cyclophosphamide (Cy). Three hundred and ninety-five were treated with Cy/TBI and 330 with Mel/TBI. Twelve patients developed non-haematological SMN. Median time to develop a SMN was 7 years (range 2-17 years). Age-adjusted rate was significantly higher than in the general population (observed 12 expected 1.2, risk 10; P < 0.0001). The cumulative overall risk of developing a SMN at 2, 5, 10 and 15 years post transplant was 0.4% (95% CI 0.1-2.6%), 1.7% (95% CI 0.6-4.4%), 6.4% (95% CI 2.8-10.8%) and 6.6% (95% CI 3.4-12.4%), respectively. Even though age-adjusted rates were higher than the general population melphalan/TBI was not associated with higher age-adjusted risk than Cy/TBI (increased risk 7.9 vs 11.4; P = NS). The cumulative overall risk of SMNs was not different with CY/TBI or Mel/TBI (8/393 vs 4/363; 10 year risk 4.4%, 95% CI 1.8-10.6 vs 8.4%, 95% CI 2.9-22.9; P = NS). The risk was significantly higher with use of additional cranial or cranio-spinal irradiation (17.5% vs 2.7% at 10 years; P = 0.0241). Transplants for acute lymphatic leukaemia resulted in a higher incidence of SMNs than did transplants for other diseases (ALL: 17.4%, 95% CI 6.3-42.6%; other diseases: 3.4% (95% 1.3-8.5%, P = 0.0469). The risk of SMN for patients with chronic GVHD was 8.4% (95% CI 3.7-18.7%) as compared to 3.5% (95% CI 1-11.1%) for patients without chronic GVHD (P = NS). No factor was associated with independently increased risk in multivariate analysis. Use of melphalan and TBI for transplant conditioning does not appear to be associated with higher risk of second malignant neoplasms than cyclophosphamide and TBI.
Assuntos
Antineoplásicos Alquilantes/administração & dosagem , Transplante de Medula Óssea , Ciclofosfamida/administração & dosagem , Neoplasias Hematológicas/terapia , Melfalan/administração & dosagem , Adolescente , Adulto , Antineoplásicos Alquilantes/efeitos adversos , Criança , Pré-Escolar , Terapia Combinada , Ciclofosfamida/efeitos adversos , Feminino , Seguimentos , Humanos , Masculino , Melfalan/efeitos adversos , Pessoa de Meia-Idade , Segunda Neoplasia Primária/induzido quimicamente , Condicionamento Pré-Transplante , Transplante Homólogo , Transplante IsogênicoRESUMO
Acute lymphoblastic leukaemia (ALL) presenting as a transient pancytopenia is known to occur in children and less commonly in adults. The period of pancytopenia usually resolves after about 5-38 weeks, to be followed by overt ALL. The pathogenesis is not known and there are no specific cytogenetic abnormalities. Diagnosis is often difficult during the period of bone marrow hypoplasia. Quantitative flow cytometry can help to establish early diagnosis, and can be used on more patients presenting in a similar way.
Assuntos
Pancitopenia/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Adolescente , Citometria de Fluxo , Humanos , MasculinoRESUMO
Erythroid leukemia is an uncommon form of acute myeloid leukemia (AML) which has previously been associated with a poor prognosis. We present the outcome of 27 patients with AML-M6 (19 de novo and 8 secondary) treated with intensive regimens including bone marrow transplantation (BMT). In the de novo group, median age was 30 years (2-72); 5 cases were under 15 years. Remission rate after induction chemotherapy was 95%. Consolidation in those achieving remission with BMT was 82%. Transplant related mortality was 36%. Median survival for de novo M6 was 2.9 years which was not significantly different to matched controls with AML (non M6). Overall relapse rate was 35%. In contrast, patients with secondary disease had a poor prognosis with lower remission rates (57%) and higher relapse rates (75% of those achieving remission after induction chemotherapy). In our series, the prognosis of patients with AML-M6 was most closely related to age and disease status at presentation (de novo or secondary). The disease is sensitive to AML induction regimens and long-term survival can be achieved with BMT in first complete remission.
Assuntos
Transplante de Medula Óssea , Leucemia Eritroblástica Aguda/terapia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Demografia , Humanos , Imunofenotipagem , Cariotipagem , Laboratórios , Leucemia Eritroblástica Aguda/mortalidade , Pessoa de Meia-Idade , Indução de Remissão/métodos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
Over a period of 14 years, 50 patients (12 children and 38 adults) of whom 46 had acute myeloid leukemia (AML) and 4 had myelodysplastic syndrome characterized by the t(8;21)(q22;q22) translocation were referred to the Royal Marsden Hospital. The clinicopathological features of these cases were analyzed to determine the influence of age, secondary karyotype abnormalities, and expression of the lymphoid marker CD19 on event free survival, and presence of extramedullary leukemia on overall survival. They were treated with a variety of chemotherapy protocols and some had bone marrow transplantation. There appeared to be no difference in survival between children (age <17 years) and adults (age >16 years). Out of the 50 cases, 16 (32%) had the (8;21) translocation alone, 17 (34%) had additional loss of a sex chromosome and the remaining 17 (34%) had other karyotype abnormalities of which deletion or translocation of the long arms of a #9 was most common (observed in 8 of the 17 patients). The karyotype groups had a significant impact on survival, the group with loss of a sex chromosome having a poorer outcome and the group with abnormalities of chromosome 9 having a better outcome. CD19 positivity was seen in 21 of the 33 cases (63%) in whom it was measured compared to 11% observed in controls with AML without a t(8;21). CD19 status did not exert any influence on event free survival. Extramedullary leukemia (EML) occurred in 5 of the 50 cases (10%). In one patient it was observed at diagnosis but in the others it presented concurrent with bone marrow relapse. The overall survival of patients with EML was worse than that of the other patients but did not achieve statistical significance and was probably adversely affected by other factors.
Assuntos
Cromossomos Humanos Par 21 , Cromossomos Humanos Par 8 , Leucemia Mieloide/diagnóstico , Leucemia Mieloide/genética , Translocação Genética , Doença Aguda , Adolescente , Adulto , Fatores Etários , Antígenos CD19/análise , Sistema Nervoso Central/patologia , Criança , Pré-Escolar , Aberrações Cromossômicas , Transtornos Cromossômicos , Intervalo Livre de Doença , Feminino , Humanos , Infiltração Leucêmica , Masculino , Pessoa de Meia-Idade , Prognóstico , Taxa de SobrevidaRESUMO
Studies that have assessed the use of granulocyte colony-stimulating factor (G-CSF) following bone marrow transplantation have shown a significantly reduced time to neutrophil recovery with the use of this agent, which may translate into a reduced duration of antimicrobial therapy and hospitalisation. We performed a pharmacoeconomic study evaluating the elective use of G-CSF after bone marrow transplantation in children. 22 consecutive children who underwent bone marrow transplantation and received G-CSF 5 micrograms/kg/day were compared with 18 such children (control group) who did not receive G-CSF. Despite a significant reduction in time to recovery of the absolute neutrophil count (ANC) to > 0.5 x 10(9)/L in G-CSF recipients compared with the control group (14 days vs 20.9 days; p < 0.0001), there was only a trend towards a reduction in the duration of intravenous antimicrobial therapy (14.5 days vs 18.6 days; p = 0.15), and there was no significant difference in the duration of hospitalisation (25.3 days vs 29.8 days). Reasons for prolonged hospitalisation beyond ANC recovery included continued use of total parenteral nutrition, treatment of graft-versus-host disease and treatment of ongoing infection. Overall, the mean total cost for patients receiving G-CSF was Pounds 15001, compared with Pounds 15482 for the control group (1995 values). In conclusion, while there appears to be no benefit in financial terms, the release of a child from strict isolation as a result of early ANC recovery must be taken into consideration.
Assuntos
Transplante de Medula Óssea/economia , Transplante de Medula Óssea/fisiologia , Fator Estimulador de Colônias de Granulócitos/economia , Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Criança , Pré-Escolar , Feminino , Humanos , MasculinoRESUMO
Aspergillus species can cause life-threatening infection in immunocompromised children. Pulmonary infections are the most common, and are usually acquired through inhalations of aspergillus spores in unfiltered air. Some patients acquire invasive aspergillus infection from endogenous spread of colonized para-nasal sinuses.
Assuntos
Aspergilose/etiologia , Fungemia/etiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Antifúngicos/uso terapêutico , Aspergilose/tratamento farmacológico , Aspergillus fumigatus , Criança , Feminino , Fungemia/tratamento farmacológico , Humanos , Itraconazol/uso terapêutico , MasculinoRESUMO
There are several reports of osteosarcoma metastases to the heart. A rare case of a metastatic osteosarcoma to the endocardium of the left atrium in a 17 year old patient is described. The radiological features are discussed in detail.
Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Femorais/patologia , Neoplasias Cardíacas/secundário , Neoplasias Pulmonares/secundário , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/secundário , Adolescente , Evolução Fatal , Feminino , Neoplasias Femorais/diagnóstico por imagem , Átrios do Coração , Humanos , Radiografia , CintilografiaRESUMO
Hepatocellular carcinoma (HCC) is the second most common primary malignant hepatic tumor in children. It often develops in patients with underlying liver disease. We report the clinicopathologic features of an unusual HCC occurring in an infant who presented with features of Cushing's syndrome due to bilateral adrenal hyperplasia. The tumor is characterized by epithelial syncytial giant cells. Giant cell carcinoma of the liver has been previously reported, but the cells were osteoclast-like (ie, mesenchymal type) and not epithelial type as it is in this patient. We propose to use the term HCC, syncytial giant cell type, to denote this apparently novel lesion.
Assuntos
Carcinoma de Células Gigantes/patologia , Carcinoma Hepatocelular/patologia , Neoplasias Hepáticas/patologia , Hiperplasia Suprarrenal Congênita , Carcinoma de Células Gigantes/diagnóstico , Carcinoma de Células Gigantes/metabolismo , Carcinoma Hepatocelular/diagnóstico , Carcinoma Hepatocelular/metabolismo , Síndrome de Cushing , Feminino , Humanos , Lactente , Queratina-8/metabolismo , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/metabolismo , beta Catenina/metabolismoRESUMO
High-dose chemoradiotherapy (HDCRT) followed by autologous stem cell (ASC) rescue is now widely used in a number of childhood malignancies. The most common to date is neuroblastoma, where it now has an established role as consolidation of initial complete remission in children over the age of 1 year with stage 4 disease. High-dose melphalan alone prolongs progression-free survival, with a small increase in long-term survival. The value of a total body irradiation (TBI)-based regimen is currently under randomized evaluation. In soft-tissue sarcoma, such as rhabdomyosarcoma or Ewing's sarcoma of bone or soft tissue, high-dose therapy has been used to consolidate initial complete or partial remission. The benefit has not been demonstrated in randomized studies but chemotherapy-alone regimens based on combined alkylating agents appear to be of potential value. Other tumours have been treated in this way but the place of high-dose therapy remains entirely unclear. It is possible that inherently chemosensitive tumours, such as Wilm's tumour, may be candidates where standard treatment has failed. The replacement of bone marrow reinfusion with cytokine-primed peripheral blood stem cell (PBSC) rescue has reduced the morbidity of these procedures. The issue of the potential risk of reinfusing tumour cells has been addressed in neuroblastoma where purging using immunodepletion or in vitro chemotherapy has been carried out.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transplante de Células-Tronco Hematopoéticas , Neoplasias/terapia , Neoplasias Encefálicas/terapia , Criança , Pré-Escolar , Terapia Combinada , Humanos , Neoplasias/patologia , Neoplasias Embrionárias de Células Germinativas/terapia , Neuroblastoma/terapia , Prognóstico , Radioterapia Adjuvante , Rabdomiossarcoma/terapia , Sarcoma de Ewing/terapia , Taxa de Sobrevida , Transplante AutólogoRESUMO
The pattern of remission deaths was examined in 842 children with acute lymphoblastic leukaemia (ALL) treated at a single centre over 18 years. The mortality rate from leukaemia fell significantly during three consecutive time periods during which treatment became progressively more intensive and that during remission induction fell from 3.5% to under 1%, but the rate of death in remission stayed constant at 5-6%. The factors associated with an increased risk of remission death were: young age, a higher leucocyte count, bone marrow transplantation, and Down's syndrome. The pattern of remission deaths changed over the years; measles and herpes viruses decreased while deaths associated with periods of intensification and gut toxicity increased. Four children developed second neoplasms. Treatment of ALL is still associated with a significant risk of death in remission but the pattern of infective deaths has changed. Many should be avoidable by provision of adequate supportive care, close supervision after periods of intensive treatment, and appropriate antibiotic, antifungal, and cytokine therapy.
Assuntos
Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Fatores Etários , Transplante de Medula Óssea/mortalidade , Criança , Pré-Escolar , Síndrome de Down/complicações , Síndrome de Down/mortalidade , Feminino , Humanos , Lactente , Contagem de Leucócitos , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Indução de Remissão , Fatores de RiscoRESUMO
Three children, aged 7-10 years, with acute lymphoblastic leukaemia presented with back pain, along with a mild kyphosis. Collapse of the vertebral bodies at multiple levels was shown on imaging. Chemotherapy resulted in pain resolution and spontaneous remodelling of the vertebrae.