RESUMEN
The Langerhans cell histiocytosis (LCH) in children is relatively rare and the long-term analysis of therapy results has not been done yet in Hungary. The aim of this study was to investigate the incidence, clinical features, prognostic risk factors, and treatment results of children's LCH in Hungary in a 20-year period. Children less than 18 years of age with newly diagnosed LCH in Hungary were entered in this study. Clinical data of all children with LCH were reported to the National Childhood Cancer Registry in Hungary from 1981 to 2000. The clinical files were collected and abstracted for information regarding age at diagnosis, gender, disease characteristics, treatment, and outcome of treatment. Median follow-up duration of surviving patients is 10.98 years. Between January 1981 and December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The annual incidence of LCH in children younger than 18 years of age was 2.24/million children. The male-female ratio was 1.36:1; the mean age was 4 years 11 months. Thirty-eight children had localized disease and in 73 cases systemic dissemination was found already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation, and 5 children got only local irradiation. In 2 cases remission was achieved with local steroid administration. Seventy-five patients received chemotherapy. In the 20 years of the study 14 children died, 9 due to the progression of the disease. Sixteen patients had relapse with a mean of 2.16 +/- 1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n = 111) was 88.3 +/- 3.1% at 5 years and 87.3 +/- 3.2% at 10 and 20 years. Childhood LCH is a well-treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood.
Asunto(s)
Histiocitosis de Células de Langerhans/epidemiología , Histiocitosis de Células de Langerhans/terapia , Adolescente , Niño , Preescolar , Femenino , Histiocitosis de Células de Langerhans/mortalidad , Humanos , Hungría/epidemiología , Incidencia , Lactante , Masculino , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
Wilms tumor was discovered in a 22-month-old otherwise healthy girl. Her pretreatment karyotype proved to be normal 46,XX with total premature division (PCD) in 21% of her lymphocyte mitoses. This phenomenon was not seen in the parents. PCD was found in less than 4% of lymphocyte mitoses of five other children with Wilms tumor. The individual finding provides further evidence for a relation of PCD to tumorigenesis; however, elucidation of the question needs further systematic studies.
Asunto(s)
Centrómero/fisiología , Fragilidad Cromosómica , Tumor de Wilms/genética , Femenino , Humanos , LactanteRESUMEN
Between 1981 and 1997 seven children and adolescents (5 boys and 2 girls) were treated for colorectal carcinomas in two paediatric centres. The case notes of the patients were studied to determine the presentation, clinical findings, prognosis and the differences of colorectal carcinomas in the young patients compared to adults. Carcinoma of the colon and rectum is uncommon in this age group and has a poor prognosis. The age range was 9 - 15 years, mean age 11.8 years. All segments of the large bowel were represented as sites of the primary tumour. Vague abdominal pain, vomiting and weight loss were the commonest presenting symptoms. The duration of symptoms varied from one month to twelve months (median: four months). Contrast enema was the most useful diagnostic investigation. Five patients had Dukes' stage C and two had Dukes' stage D tumour. Mucin-secreting adenocarcinoma was the commonest histological diagnosis. Five patients had complete resection, two had palliative procedures. Post-operative chemotherapy was given to six patients and two had post-operative radiotherapy.
Asunto(s)
Adenocarcinoma/complicaciones , Neoplasias Colorrectales/complicaciones , Adenocarcinoma/mortalidad , Adenocarcinoma/cirugía , Adolescente , Niño , Neoplasias Colorrectales/mortalidad , Neoplasias Colorrectales/cirugía , Femenino , Humanos , Masculino , Pronóstico , Estudios RetrospectivosRESUMEN
In the present days the molecular genetical investigations are the mainstream of establishing the etiology of malignant diseases. Beside these surveys, prenatal, neonatal, environmental and developmental risk factors for malignancy have repeatedly been investigated during the last few years. Mounting evidences show the importance of the intrauterine and perinatal period in tumor genesis and health quality in later life. This review article summarizes the results of traditional epidemiologic studies which identified a number of risk factors for malignancy. These easily detectable anamnestic data, developmental, physical features can further support the prenatal origin of tumors and can give new directions for the modern molecular genetical investigations.
Asunto(s)
Neoplasias/etiología , Peso al Nacer , Neoplasias Óseas/etiología , Neoplasias de la Mama/etiología , Carcinoma Hepatocelular/etiología , Femenino , Humanos , Neoplasias Renales/etiología , Neoplasias Hepáticas/etiología , Masculino , Neoplasias/epidemiología , Neoplasias/genética , Neuroblastoma/etiología , Osteosarcoma/etiología , Neoplasias de la Próstata/etiología , Factores de Riesgo , Neoplasias Testiculares/etiología , Tumor de Wilms/etiologíaRESUMEN
In B-cell non-Hodgkin's lymphomas (NHL), clonal rearrangement of the immunoglobulin heavy chain (IgH) gene provides a useful marker for the detection of minimal residual disease (MRD) after treatment. To explore clinical usefulness of polymerase chain reaction (PCR) analysis of clonal IgH gene rearrangement in the detection of MRD a follow up study of 10 patients with B-cell NHL have been performed. At the time of diagnosis, tumor DNAs were PCR-amplified using sense primer specific for the heavy chain variable region (VH) and antisense primer specific for the heavy chain joining region (JH) of the IgH gene. The clonal rearrangement of IgH gene detected by PCR was used as clonal marker to determine MRD after treatment. In three cases, where clinical remission was not achieved, clonal IgH gene rearrangement was detected after the treatment. In seven cases, clinical remission was achieved after induction therapy but the PCR analysis revealed clonal IgH gene rearrangement in three of the cases. In all of the three cases, where MRD was detected by PCR, clinical relapse developed after 7-28 months of the therapy. In all cases that have relapsed, the IgH gene rearrangement was identical at the time of initial diagnosis and at the relapse. This study demonstrates that PCR analysis of clonal IgH gene rearrangement is a useful method to monitor and detect MRD before clinical relapse.
Asunto(s)
Biomarcadores de Tumor/genética , Reordenamiento Génico de Linfocito B , Genes de Inmunoglobulinas/genética , Cadenas Pesadas de Inmunoglobulina/genética , Linfoma de Células B/diagnóstico , Linfoma de Células B/genética , Reacción en Cadena de la Polimerasa/métodos , ADN de Neoplasias/genética , Humanos , Neoplasia Residual/diagnósticoRESUMEN
Analysis of lineage involvement was performed in 17 Philadelphia chromosome-positive acute lymphoblastic leukemia patients with no history of chronic myeloproliferative disorder. The percentage of blastic cells as defined by flow cytometry matched that of the Ph-positive cells in 14 out of 17 patients. The bcr-abl rearrangement was investigated by fluorescent in situ hybridization in morphologically identified blastic cells, myeloid elements, lymphocytes and erythroblasts using a combined light and fluorescent microscopical imaging. Lymphoid lineage restriction could be determined in all but three of the patients. These 14 patients exhibited heterogeneity in terms of m-bcr and M-bcr types of translocation as revealed by reverse transcription polymerase chain reaction. The three patients with multilineage involvement and M-bcr type of translocation reverted to chronic phase and the percentage of Ph-positive cells remained high. Thus, we could identify an uncommitted stem cell origin among Ph-positive ALLs only in those patients whose disease subsequently proved to be a lymphoid blastic crisis with clinically silent chronic phase.
Asunto(s)
Linaje de la Célula , Proteínas de Fusión bcr-abl/genética , Reordenamiento Génico , Cromosoma Filadelfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/patología , Adulto , Anciano , Secuencia de Bases , Niño , Preescolar , Cartilla de ADN , Femenino , Humanos , Hibridación Fluorescente in Situ , Masculino , Persona de Mediana EdadRESUMEN
The highly variable biological behaviour of neuroblastoma, a neoplasm which belongs to the family of primitive neuroectodermal tumours, is determined by its molecular pathological characteristics. Among them amplification of the N-myc gene is the most important factor in both therapeutic and prognostic points of view. Value of the amplification can be determined by different methods. The latest of them is the competitive polymerase chain reaction (PCR), essence of which is the parallel reaction of the target N-myc gene (exon 3.) and the endogen competitor CF gene (exon 3.) in the same reaction solution. The authors applied the method on 11 neuroblastoma cases diagnosed between 1994 and january of 1999. In three cases the amplification was determined also by fluorescens in situ hybridization (FISH). Six of the 11 cases were detected to have more than 10-fold, two of the six about 100-fold N-myc amplification. Results of the PCR and FISH correlated well. The two methods applied by the authors complete each other and are appropriate for determining the gene amplification which gives valuable prognostic and therapeutic information about the examined tumour.
Asunto(s)
Genes myc/genética , Neuroblastoma/genética , Reacción en Cadena de la Polimerasa , Humanos , Hibridación in SituRESUMEN
We present a case of a germinoma in the sellar region of a 10-year-old female patient who presented with a history of polydipsia, polyuria and visual disturbances. The tumor was resected and histologically analyzed. Interphase cytogenetics was performed using chromosome specific (peri)-centromeric DNA probes for all the somatic and X chromosomes on fresh frozen tissues. Fluorescent in situ cell hybridization demonstrated accumulated cytogenetic abnormalities involving significant alterations of chromosome 1, 4, 5/19 and 15. The child was treated postoperatively by radiation and now appears well with only minor neurological deficits. At 3-year follow-up no recurrent tumor mass could be demonstrated.
Asunto(s)
Aberraciones Cromosómicas , Trastornos de los Cromosomas , Análisis Citogenético/métodos , ADN de Neoplasias/análisis , Germinoma/genética , Neoplasias de Células Germinales y Embrionarias/genética , Neoplasias Hipofisarias/genética , Niño , Aberraciones Cromosómicas/genética , Cromosomas Humanos Par 1/genética , Cromosomas Humanos Par 15/genética , Cromosomas Humanos Par 19/genética , Cromosomas Humanos Par 4/genética , Cromosomas Humanos Par 5/genética , Femenino , Germinoma/diagnóstico , Humanos , Hibridación Fluorescente in Situ , Imagen por Resonancia Magnética , Metafase , Neoplasias Hipofisarias/diagnósticoRESUMEN
Chromosomal instability was examined in 20 apparently healthy children of survivors of childhood malignancy. As compared to controls, no increase of spontaneous or bleomycin-induced aberrations (including gaps, breaks, sister chromatid exchanges, pulverization, and premature centromere divisions) were found in these "index children." The results suggest that the offspring of subjects previously receiving chemotherapy and/or radiotherapy for childhood malignancy are probably at no increased risk of latent chromosomal instability.
Asunto(s)
Aberraciones Cromosómicas , Predisposición Genética a la Enfermedad , Neoplasias/genética , Bleomicina/efectos adversos , Centrómero/genética , Niño , Femenino , Humanos , Cariotipificación , Leucemia/genética , Linfoma/genética , Masculino , Núcleo Familiar , Intercambio de Cromátides Hermanas , SobrevivientesRESUMEN
Eleven pediatric acute lymphoid leukemia patients were investigated for chromosomal aneuploidy by interphase cytogenetics using chromosome specific (peri)centromeric probes for all the somatic and sex chromosomes. Results were compared with metaphase cytogenetic and flow cytometric derived DNA aneuploidy data. Experiments performed on normal human cells using chromosome specific (peri)centromeric probes indicated that disomy could be recognized in a range of 89.1+/-2.7% (12.9)-96.8+/-0.2% (0.9) for the somatic chromosomes and in 98.1+/-0.4% (1.3) for the sex chromosomes. Using the cutoff level of the mean false monosomy and trisomy in the control cells +2 S.D., chromosome loss or gain for the somatic chromosomes could be revealed beyond a clonal ratio of 3.6-13.2% and 1.1-6.8%, respectively. The same value for the sex chromosomes was 3.5% and 0%, respectively. In 5 of 11 patients the leukemic cells proved to be diploid with all three methods at both gross DNA and chromosome levels. Interphase cytogenetics revealed chromosome loss or gain in all of the remaining six patients, however, the metaphase analysis indicated numerical aberration in only two patients. In one of them only the increased chromosome number could have been detected without identifying the chromosomes involved and in the other one the two methods indicated trisomy for a different chromosome. Flow cytometric data showed aneuploidy in three of the six aneuploid leukemia patients. The results suggest that interphase cytogenetics might be more accurate compared with flow cytometry and metaphase analysis to reveal aneuploidy.
Asunto(s)
Aberraciones Cromosómicas , ADN de Neoplasias/análisis , Citometría de Flujo/métodos , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Adolescente , Niño , Preescolar , Femenino , Humanos , Hibridación Fluorescente in Situ , Lactante , Interfase , Masculino , MetafaseRESUMEN
The prevalence of 55 well-defined mild errors of morphogenesis (MEMs) was determined in 100 children with acute lymphoblastic leukemia (ALL), their 80 sibs, 91 mothers, and 76 fathers. Seventy-four patients were treated in Pécs (Hungary) and 26 in Tübingen (Germany). Only white Caucasian index cases were included in the study. Two-hundred children examined for acute infections served as controls. In addition, we analyzed the family history for birth defects and malignancies, associated major malformations, birth weight, birth order, and pretreatment height of the patients. The results of the Pécs and Tübingen patients were at first evaluated separately but since no differences were found only the cumulative data were analyzed further. A significantly increased prevalence of MEMs was found in the ALL patients and their sibs of both sexes: their MEM/subject ratios were 1.59 and 1.51, respectively, whereas the same parameter was 0.74 in the mothers, 0.67 in the fathers and 0.69 in the controls. The same tendency was observed when familial cases and/or age-dependent MEMs were excluded and when malformation-type and variant-type MEMs were evaluated separately. No association of ALL with specific MEMs or combinations was recorded. Family history, associated major malformations, parity and birth weight of the patients did not differ significantly from the local reference values, whereas the pretreatment height of the male probands proved to be greater than expected.
Asunto(s)
Anomalías Congénitas/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Adolescente , Peso al Nacer , Estatura , Niño , Preescolar , Anomalías Congénitas/epidemiología , Anomalías Congénitas/fisiopatología , Discapacidades del Desarrollo/epidemiología , Discapacidades del Desarrollo/genética , Discapacidades del Desarrollo/fisiopatología , Femenino , Alemania/epidemiología , Humanos , Hungría/epidemiología , Lactante , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiología , Leucemia-Linfoma Linfoblástico de Células Precursoras/fisiopatología , PrevalenciaRESUMEN
Anthropometric, dysmorphologic, and cytogenetic investigations of 21 children of 20 survivors of childhood malignancy revealed no signs of congenital anomalies in any of the subjects examined. No increase of mild errors of morphogenesis (minor anomalies) was observed in the well-developing children; no latent chromosome instability was found in their Bleomycin-treated lymphocyte cultures either. The suggestion that previous oncological therapy does not lead to an increased risk of congenital disorders in the offspring was confirmed by the present findings obtained with various, in part new methods.
Asunto(s)
Neoplasias/genética , Antropometría , Bleomicina/farmacología , Niño , Preescolar , Aberraciones Cromosómicas , Citogenética , Femenino , Humanos , Lactante , Prueba de Cultivo Mixto de Linfocitos/métodos , Linfocitos/efectos de los fármacos , Masculino , Morfogénesis , Neoplasias/congénito , SobrevivientesRESUMEN
Current intensive chemotherapies cure about 70% of the children with ALL. On the other hand a significant number of the children are not cured despite intensive treatment. At the same time some highly curable patients are treated too intensively and suffer from unnecessary side effects of the chemo- and radiotherapy applied. In order to further improve the therapeutic results in this disease, we have to distinguish between the cases with a better and a worse prognosis. The initial karyotype (both numerical and structural chromosome abnormalities) proved to be one of the most reliable prognostic parameters, leading to the suggestion of developing genotype-specific therapies. Although the prognosis in patients with pseudodiploid karyotype is usually unfavorable, a significantly better prognosis can be observed in those with more than 50 chromosomes. Because the latter patients can achieve remission on a metabolite-based therapy, the toxic effects of more aggressive chemotherapy with anthracyclines and genotoxic agents can be avoided; thus, the reliable and accurate identification of patients with > 50 chromosomes is of particular importance. For this purpose three methods: chromosome analysis, DNA flow cytometry, and fluorescence in situ hybridization can be used. In 1993 it was decided to develop a comprehensive nationwide project in order to perform the initial genetic analysis of all ALL children diagnosed in the hematological/oncological centers of Hungary. Here the data obtained on 187 ALL patients diagnosed in the period from 1993 to 1995 are presented. In about 75% of patients (in 140 of 187) chromosome analysis was performed, in 78 cases (55.7%) successfully. The proportion of patients with abnormal karyotype was 36 of 78 (46.1%), and hyperdiploidy with more than 50 chromosomes was detected in 13 of 78 (16.6%) children. The lower ratio of hyperdiploid cases in our patients as compared to the data in the literature may be due to technical difficulties and the small number of patients studied, but it may reflect real geographic characteristics. Using flow cytometry, seven of 31 patients investigated (22.5%) proved to be hyperdiploid with a DNA index above 1.16. A higher ratio of hyperdiploid patients in this study calls attention to the significance of simultaneous application of the two methods. Taken together, 16 of 80 (20.0%) successfully studied patients proved to be hyperdiploid (> 50 chromosomes and/or DNA index above 1.16). The pattern of chromosome involvement in our study determined by chromosome analysis and/or FISH technique proved also to be different from the data of large international series. In addition to trisomies of chromosomes 4, 6, 10, 14, 17, 18, 21, and X, which are known to be the most frequently involved chromosomes, trisomies of chromosomes 3, 8, 11, and 13 were also observed with a high frequency. Comparison of survival curves of various cytogenetic subgroups showed a significant difference between diploid-pseudodiploid and diploid-hyperdiploid A (with 47-50 chromosomes) subgroups. No favorable prognosis of hyperdiploid patients (> 50 chromosomes) could be proved. Because of the small number of patients studied, prognostic differences of cytogenetic subgroups need further confirmation. The clinical and genetic differences observed, however, call attention to the necessity for further genetic studies of ALL patients in Hungary, because these differences may reflect real geographic characteristics and may be related to different environmental mutagen/carcinogen effects of the given geographic area. It is essential to determine whether or not these differences really exist and if they do to reveal the causes leading to these differences. In our view this is one of the routes by which the therapeutic results in childhood ALL can be further improved simultaneously with the avoidance of early and late toxicity of chemotherapy.
Asunto(s)
Aberraciones Cromosómicas , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Niño , Humanos , Hibridación Fluorescente in Situ , Cariotipificación , PronósticoRESUMEN
The authors investigated the usefulness of the interphase cytogenetic approach to reveal numerical chromosomal abnormalities. Experiments performed on normal human cells using chromosome specific (peri)centromeric probes indicated that disomy was recognized in a range of 89.1 +/- 5.4%-96.8 +/- 0.4% for the somatic chromosomes and in 98.1 +/- 0.8% for the sex chromosomes. Using positivity threshold of mean percentage of the fals monosomy and trisomy + 2 SD, chromosome loss or gain for the somatic chromosomes could be revealed beyond clonal ratio of 3.6-13.2% and 1.1-6.8%, respectively. The same value for the sex chromosomes was 3.2% and 0%, respectively. Eleven pediatric acute lymphoid leukaemia were investigated for chromosomal aneuploidy by interphase cytogenetics using chromosome specific (peri)centromeric probes for all the somatic and sex chromosomes. Results were compared with metaphase cytogenetic and flow cytometry derived gross DNA aneuploidy data. In 5 cases the leukaemic cells proved to be diploid with all three methods at both gross DNA and chromosome levels. Interphase cytogenetics revealed chromosome loss or gain in all the remaining 6 cases, however, metaphase analysis indicated numerical aberration in only 2 patients. In one of them only the increased chromosome number could have been detected without identifying the chromosomes involved and in the other one the two methods indicated trisomy for not the same chromosome. Flow cytometry data showed aneuploidy in 3 out of the 6 aneuploid leukaemia. The results imply that interphase cytogenetics might be more accurate as compared with flow cytometry and metaphase analysis to reveale aneuploidy.
Asunto(s)
ADN , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Factores de Edad , Aneuploidia , Niño , Citogenética , Citometría de Flujo , Humanos , MetafaseRESUMEN
A total of nine children with previously untreated stage IV Wilms' tumor of favorable histology were treated according to the Austrian/Hungarian Wilms' Tumor Protocol 89 and received a preoperative single dose of carboplatin as an "up front" window therapy. The treatment consisted of carboplatin as a single-dose of 600 mg/m2 over 30 minutes on day 1. Response evaluation by chest X-ray, serial CT scans, and sonography was performed on day 22. Investigation of the abdominal tumors revealed seven partial responses (78%), one nonresponse, and one progressive disease with a median tumor volume reduction of 62%. Response of metastases evaluated by CT scans was as follows: four complete remission, four partial response, and one nonresponse. Thrombocytopenia (WHO grade III 1, grade II 2, grade I 2) and leukocytopenia (WHO grade II 1, grade I 5) were the main side effects. No renal or liver toxicity were observed. The overall response rate after a preoperative single-dose of 600 mg/m2 carboplatin in untreated patients with stage IV Wilms' tumor is encouraging and the toxicity acceptable. This data indicate that carboplatin seems to be an additional effective drug in patients with previously untreated Wilms' tumor of favorable histology.
Asunto(s)
Carboplatino/uso terapéutico , Neoplasias Renales/tratamiento farmacológico , Tumor de Wilms/tratamiento farmacológico , Austria , Carboplatino/administración & dosificación , Niño , Preescolar , Femenino , Humanos , Hungría , Lactante , Neoplasias Hepáticas/tratamiento farmacológico , Neoplasias Hepáticas/secundario , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Pulmonares/secundario , Masculino , Estadificación de Neoplasias , Tumor de Wilms/secundarioRESUMEN
A 2-year-old girl presented with thrombocytopenic purpura. Clinical examination and follow-up documented severe bone marrow hypoplasia associated with bilateral progressive Coats retinopathy, nail dystrophy, fine hair, and apparent chromosome instability. The syndrome is regarded as a variant of the Révész syndrome sharing some findings of dyskeratosis congenita.
Asunto(s)
Anemia Aplásica/genética , Queratosis/genética , Enfermedades de la Retina/genética , Preescolar , Aberraciones Cromosómicas , Trastornos de los Cromosomas , Femenino , Enfermedades del Cabello/genética , Humanos , Cariotipificación , Leucoplasia/genética , SíndromeRESUMEN
Earlier studies suggested an increased prevalence of minor morphological aberrations and variants, also termed informative morphogenetic variants (IMVs) in children with acute lymphoblastic leukaemia (ALL) and possibly in their siblings, however, family investigations have not been performed in this field. In the present survey the occurrence of 54 well-defined IMVs was determined in 50 children with ALL, in their 53 siblings, 49 mothers, and 41 fathers. 170 children examined for acute infections served as controls. A significantly increased prevalence on IMVs was found in the ALL-patients and in their siblings: their IMV/subject ratios were 1.32 and 1.38, respectively, whereas the same parameter was 0.75 in the mothers, 0.66 in the fathers, and 0.76 in the controls. The same tendency was observed when familial cases and/or age-dependent IMVs were excluded. No association of ALL with given specific IMVs or combinations was found. On the basis of an increased prevalence of minor morphological aberrations no conclusion concerning predisposition to ALL or any other malignancy can be drawn at the moment. However, further investigation of the problem is justified, since the research of such association may reveal new details of prenatal origin and predisposition of childhood malignancy.
Asunto(s)
Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Adulto , Análisis de Varianza , Niño , Preescolar , Anomalías Congénitas/epidemiología , Anomalías Congénitas/genética , Familia , Femenino , Humanos , Hungría/epidemiología , Masculino , Morfogénesis , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiología , PrevalenciaRESUMEN
The case histories of two patients with acute lymphocytic leukemia, who developed central nervous system complication during combined chemotherapy are described. The neurological picture could be characterized by symptoms of headache, mental deterioration, hemiparesis and seizures. Following L-asparaginase administration one patient had intracranial thrombosis with focal seizures and hemiparesis associated with clotting abnormalities, including severe hypofibrinogenemia and decreased antithrombin III activity. In the other patient, it was after intrathecal administration of Methotrexate when mental deterioration associated with the symptoms of progressive leukoencephalopathy occurred. It arises the possibility that with increasing complexity of combined chemotherapy the occurrence rate of neurological complications will also increase.
Asunto(s)
Encefalopatías/inducido químicamente , Metotrexato/efectos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Asparaginasa/efectos adversos , Niño , Femenino , Hemiplejía/inducido químicamente , Humanos , Embolia y Trombosis Intracraneal/inducido químicamente , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicacionesRESUMEN
The case history of a 15 year old boy in whom thrombosis of the internal carotid artery was associated with severe disseminated intravascular thrombosis (DIC) is described. Both peripheral blood smear and bone marrow aspirate revealed acute myelogenous leukemia FAB M-2 type as the cause of the disease. Consumption coagulopathy is common sign of hemostasis disturbances in leukemia. It is frequently observed in acute promyelocytic leukemia, but rarely it may be seen in the other forms of hemoblastosis, too.