RESUMO
PURPOSE: To assess the occurrence and possible causes of pulmonary thromboembolism (PTE) in children with hematologic malignancies evaluated in a single pediatric hematology center. PATIENTS AND METHODS: Four hundred fifty-two patients admitted for leukemia in different stages of disease were evaluated whenever they presented with PTE-related acute respiratory failure (ARF). Diagnosis was based on a perfusional lung scan and a digital pulmonary angiography in most cases. When necessary, patients with ARF were transferred to the pediatric intensive care unit (ICU) for cardiorespiratory monitoring and support. Thrombolytic treatment was usually performed with urokinase at a loading dose of 2,000 to 4,560 IU/kg as single bolus followed by 2,000 to 4,530 IU/kg/h for 12 to 42 hours. Before thrombolytic therapy was discontinued, heparin was started at a daily dose of 100 to 500 IU/kg as a continuous infusion and continued for 6 to 26 days. RESULTS: Twelve of 452 children developed 17 PTE episodes, which were resolved completely after appropriate therapy in 15 cases. Univariate analysis showed a statistical correlation between PTE and the diagnosis of acute myeloid leukemia (AML) (P < .001). No major bleeding was observed after thrombolytic treatment. CONCLUSION: Our findings indicate that PTE is not an extremely rare event in children with leukemia and should be ruled out when sudden tachypnea develops in patients with risk factors such as previous tumor lysis, central venous catheter (CVC) malfunction, coagulation abnormalities, and drug-induced pulmonary toxicity. Complete resolution of PTE may be obtained in a high proportion of cases with early diagnosis and proper treatment.
Assuntos
Leucemia/tratamento farmacológico , Embolia Pulmonar/etiologia , Cateterismo Venoso Central/efeitos adversos , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Leucemia/complicações , Leucemia Mieloide Aguda/complicações , Leucemia Mieloide Aguda/tratamento farmacológico , Masculino , Prognóstico , Embolia Pulmonar/tratamento farmacológico , Insuficiência Respiratória/etiologia , Fatores de Risco , Terapia TrombolíticaRESUMO
We retrospectively analyzed red blood cell (RBC) support and alloimmunization rate in 218 consecutive patients - 128 from the Pediatric Department and 90 from the adult Hematology Department - undergoing hematopoietic stem cell transplantation (HSCT) between 1994 and 2000. In the pre-HSCT period, the pediatric patients undergoing auto-HSCT required more RBC support. In the post-HSCT period, pediatric patients transplanted with an unrelated donor required more RBC support (median 13.5 U/10 kg bw) than patients receiving HSCT from a related donor (median 6 U/10 kg bw) or from an autologous source (median 4 U/10 kg bw, P=0.0004). In the pre-HSCT period, 159 out of 218 patients (73%) received a total of 1843 RBC units, with an overall median of 9 U/patient over a median of 24 months (range 4-62); 10 patients (6%) developed a total of 12 alloantibodies, with an alloimmunization rate of 5.4/1000 RBC units. In the post-HSCT period, all but three patients were given a total of 2420 RBC units, with an overall median of 6 U/patient over a median of 4 months (range 1-18); all but one of the pre-existing alloantibodies disappeared and three patients (1%) developed new alloantibodies with an alloimmunization rate of 1.2/1000 RBC units. These newly produced alloantibodies (one anti-M and two anti-E) were detected at +58, +90 and +210 days after HSCT. These findings might suggest a different approach to alloantibody screening tests in patients receiving HSCT, with a subsequent reduction of costs and laboratory workload.
Assuntos
Formação de Anticorpos , Transfusão de Eritrócitos/estatística & dados numéricos , Eritrócitos/imunologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Isoanticorpos , Adolescente , Adulto , Idoso , Antígenos de Grupos Sanguíneos , Criança , Pré-Escolar , Feminino , Neoplasias Hematológicas/terapia , Humanos , Lactente , Isoantígenos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Hypoxaemia that is induced by physical exercise (EIH) in some athletes, who are however capable of enduring intense muscolar work, is a phenomenon that has been known for some time. However, assumptions such as alveolar hypoventilation, veno-arterial shunt, limitation of diffusion, or mismarch of the VA/Q ratio, have not to date been able to exhaustively explain this phenomenon. In this study five athletes displaying exercise-induced hypoxaemia were evaluated by increasing-load exercise tests, as proposed by other authors, and by means of intermittent tests with supermaximal exercise steps (130% VO2 max) with breaks for incomplete recovery (3 min). The fundamental fact arising from our study is that the intermittent tests did not bring about hypoxaemia in the tests subjects. Analysis of the ventilator and metabolic parameters, of the alveolar pressure of the O2, and of the partial pressures of the CO2 in the arterial blood, all measured during the two different types of muscular exercise, lead to the belief that the different distribution of the pulmonary blood flow, which has been documented in highly trained athletes, plays a very important role in inducing EIH.
Assuntos
Ciclismo/fisiologia , Hipóxia/fisiopatologia , Troca Gasosa Pulmonar/fisiologia , Adaptação Fisiológica , Adolescente , Dióxido de Carbono/sangue , Teste de Esforço , Frequência Cardíaca , Humanos , Hipoventilação/fisiopatologia , Contração Muscular , Oxigênio/sangue , Consumo de Oxigênio , Pressão Parcial , Resistência Física , Alvéolos Pulmonares/fisiopatologia , Circulação Pulmonar , Respiração , Volume de Ventilação Pulmonar , Relação Ventilação-PerfusãoAssuntos
Esportes/fisiologia , Adulto , Frequência Cardíaca , Humanos , Masculino , Consumo de OxigênioRESUMO
Hydroxyurea is a usually well-tolerated cytostatic agent, but its side effects include cutaneous lesions that appear after several years of maintenance therapy with hydroxyurea. The reported incidence of such adverse reactions varies from 10 to 35%; in our Center it is 2%. We describe a patient with essential thrombocythemia who presented with ulcers of the hands only 15 days after hydroxyurea treatment.
Assuntos
Hidroxiureia/efeitos adversos , Úlcera Cutânea/induzido quimicamente , Idoso , Humanos , Hidroxiureia/uso terapêutico , Masculino , Úlcera Cutânea/patologia , Trombocitose/tratamento farmacológico , Fatores de TempoRESUMO
Mesenteric cysts are rare intra-abdominal tumors with an incidence around one case per 100,000 hospital admissions. The clinical presentation is variable; patients may be asymptomatic or present with either acute or chronic abdominal pain. Physical examination commonly demonstrates a smooth, round and mobile abdominal mass. Differential diagnosis includes any abdominal cyst or tumor. Laboratory tests are usually helpless. Ultrasonography and CT scans are the best diagnostic tools. The treatment of choice is the total resection of the cyst, which is regularly performed by open surgery. This paper reports a case of a mesenteric cyst successfully resected by laparoscopy, and addresses the possible uses of this approach.
Assuntos
Laparoscopia , Cisto Mesentérico/cirurgia , Adulto , Humanos , Masculino , Cisto Mesentérico/diagnósticoRESUMO
We describe a case of congenital dyserythropoietic anaemia (CDA) presenting at week 20 of pregnancy with hydrops fetalis and very severe anaemia. Fetal blood examination showed marked erythroblastosis with morphologic alterations while the basic haematological and biochemical tests were in the normal range. The fetus received intravascular red blood cells transfusion at 20 and 25 weeks of gestation. After a caesarean section at week 33 the child became transfusion dependent. Haematological investigations demonstrated that the child is affected by a CDA-like picture which, although morphologically similar to CDA II, is Ham test negative and does not show the typical membrane proteins alterations.
Assuntos
Anemia Diseritropoética Congênita/complicações , Hidropisia Fetal/etiologia , Adulto , Anemia Diseritropoética Congênita/sangue , Transfusão de Sangue , Eritroblastose Fetal/sangue , Eritroblastose Fetal/etiologia , Feminino , Humanos , Hidropisia Fetal/sangue , Recém-Nascido , Microscopia Eletrônica , GravidezRESUMO
BACKGROUND AND OBJECTIVES: Splenectomy is the treatment of choice in the majority of patients affected by idiopathic thrombocytopenic purpura refractory to corticosteroid therapy, but it is not free from early and late complications. As the available literature does not seem to contain any precise indications concerning possible factors predicting the response to splenectomy, the aim of this retrospective study of 65 splenectomized patients was to attempt to identify potentially predictive clinical or laboratory parameters. DESIGN AND METHODS: For the purposes of statistical analysis, the patients were divided into two groups: the first included those with a complete (platelets > 100x10(9)/L) or partial response (platelets 50-100 x10(9)/L) to splenectomy; the second, the non-responders (platelets < 50x10(9)/L). The non-parametric tests were based on the Kruskal-Wallis method for independent samples, and the independent samples were compared using the Chi-square test according to Pearson. RESULTS: Univariate analysis did not reveal any significant correlation between successful splenectomy and age, sex, platelet count at diagnosis, anti-platelets antibody positivity, the site of platelet sequestration, the time between diagnosis and surgery, or the response to high intravenous immunoglobulin doses. However, the probability of success was greater in the patients with a complete or partial pre-operative response to steroid therapy (p<0.05). INTERPRETATION AND CONCLUSIONS: The factor most frequently associated with the success of splenectomy is the site of autologous platelet sequestration. Our study did not identify any clinical or laboratory parameter clearly predictive of post-splenectomy cure other than a transient response to steroid treatment. This finding needs further confirmation in larger patient populations.
Assuntos
Púrpura Trombocitopênica Idiopática/cirurgia , Esplenectomia , Adolescente , Adulto , Fatores Etários , Idoso , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Contagem de Plaquetas , Valor Preditivo dos Testes , Prognóstico , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/fisiopatologia , Fatores Sexuais , Resultado do TratamentoRESUMO
BACKGROUND AND OBJECTIVE: The diagnostic and prognostic value of bone marrow biopsy (BMB) has been widely investigated in patients with chronic myeloproliferative disorders (CMPD). The present study is based on a review of the results of routine BMBs taken from 93 essential thrombocythemia (ET) patients at the time of diagnosis. DESIGN AND METHODS: The common BMB histologic parameters and clinico-hematologic variables were considered for diagnostic and prognostic purposes. Clinico-pathologic correlations were looked for univariately. Moreover, the diagnostic significance of the histologic findings was tested by means of cluster analysis. Overall survival and event-free survival were considered as prognostic endpoints. RESULTS: There were no correlations between the clinic and pathologic findings, and none of the histologic and clinical parameters was predictive of survival or the occurrence of major clinical events. Cluster analysis of the BMB findings revealed two distinct morphologic patterns: one was clearly myeloproliferative; the other had somewhat dysplastic features. The event-free and overall survival rates in the latter group were significantly worse (p = 0.0377 and p = 0.0162 respectively), with major ischemic events accounting for most of the difference in event-free survival. INTERPRETATION AND CONCLUSIONS: These results have no clearcut counterpart in the literature, but we feel that dysplastic BMB findings could be included in the definition of ET prognostic scores in order to allow therapeutic strategies to be adapted to the level of risk.
Assuntos
Exame de Medula Óssea , Medula Óssea/patologia , Trombocitemia Essencial/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Crise Blástica/epidemiologia , Crise Blástica/etiologia , Diferenciação Celular , Criança , Aberrações Cromossômicas , Intervalo Livre de Doença , Feminino , Fibrose , Seguimentos , Humanos , Hiperplasia , Isquemia/etiologia , Isquemia/mortalidade , Masculino , Megacariócitos/patologia , Pessoa de Meia-Idade , Neoplasias/complicações , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Trombocitemia Essencial/complicações , Trombocitemia Essencial/mortalidade , Trombose/etiologia , Trombose/mortalidadeRESUMO
A recent study has shown the reproducibility of time to exhaustion (time limit: tlim) at the lowest velocity that elicits the maximal oxygen consumption (vVO2 max). The same study found an inverse relationship between this time to exhaustion at vVO2 max and vVO2 max among 38 élite long-distance runners (Billat et al. 1994b). The purpose of the present study was to compare the time to exhaustion at the power output (or velocity) at VO2 max for different values of VO2 max, depending on the type of exercise and not only on the aerobic capacity. The time of exhaustion at vVO2 max (tlim) has been measured among 41 élite (national level) sportsmen: 9 cyclists, 9 kayak paddlers, 9 swimmers and 14 runners using specific ergometers. Velocity or power at VO2 max (vVO2 max) was determined by continuous incremental testing. This protocol had steps of 2 min and increments of 50 W, 30 W, 0.05 m s-1 and 2 km-1 for cyclists, kayak paddlers, swimmers and runners, respectively. One week later, tlim was determined under the same conditions. After a warm-up of 10 min at 60% of their vVO2 max, subjects were concluded (in less than 45 s) to their vVO2 max and then had to sustain it as long as possible until exhaustion. Mean values of vVO2 max and tlim were respectively equal to 419 +/- 49 W (tlim = 222 +/- 91 s), 239 +/- 56 W (tlim = 376 +/- 134 s), 1.46 +/- 0.09 m s-1 (tlim = 287 +/- 160 s) and 22.4 +/- 0.8 km h-1 (tlim = 321 +/- 84 s), for cyclists, kayak paddlers, swimmers and runners. Time to exhaustion at vVO2 max was only significantly different between cycling and kayaking (ANOVA test, p < 0.05). Otherwise, VO2 max (expressed in ml min-1 kg-1) was significantly different between all sports except between cycling and running (p < 0.05). In this study, time to exhaustion at vVO2 max was also inversely related to VO2 max for the entire group of élite sportsmen (r = -0.320, p < 0.05, n = 41). The inverse relationship between VO2 max and tlim at vVO2 max has to be explained, it seems that tlim depends on VO2 max regardless of the type of exercise undertaken.