Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 38
Filtrar
Filtros adicionais











País/Região como assunto
Intervalo de ano
1.
Blood ; 134(3): 277-290, 2019 Jul 18.
Artigo em Inglês | MEDLINE | ID: mdl-31151987

RESUMO

Shwachman-Diamond syndrome (SDS) is a recessive disorder typified by bone marrow failure and predisposition to hematological malignancies. SDS is predominantly caused by deficiency of the allosteric regulator Shwachman-Bodian-Diamond syndrome that cooperates with elongation factor-like GTPase 1 (EFL1) to catalyze release of the ribosome antiassociation factor eIF6 and activate translation. Here, we report biallelic mutations in EFL1 in 3 unrelated individuals with clinical features of SDS. Cellular defects in these individuals include impaired ribosomal subunit joining and attenuated global protein translation as a consequence of defective eIF6 eviction. In mice, Efl1 deficiency recapitulates key aspects of the SDS phenotype. By identifying biallelic EFL1 mutations in SDS, we define this leukemia predisposition disorder as a ribosomopathy that is caused by corruption of a fundamental, conserved mechanism, which licenses entry of the large ribosomal subunit into translation.

3.
Ann Hematol ; 98(6): 1341-1350, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30915499

RESUMO

Eltrombopag (ELT), an oral thrombopoietin receptor agonist, has recently emerged as a promising new drug for the treatment of aplastic anemia (AA). How ELT is used outside of clinical trials in the real-world setting and results of this treatment are not known. We conducted therefore a retrospective survey on the use of ELT in AA among EBMT member centers. We analyzed the 134 patients reported in our survey together with 46 patients recently published by Lengline et al. The median follow-up from start of ELT treatment was 15.3 months, with 85.6% patients alive at last follow-up. Importantly, only 28.9% of our patients received ELT according to the FDA/EMA label as monotherapy in the relapsed/refractory setting, whereas 16.7% received ELT upfront. The overall response rate in our cohort was 62%, very similar to the results of the pivotal ELT trial. In multivariate analysis, combination therapy with ELT/cyclosporine/ATG and response to previous therapy were associated with response. Overall survival was favorable with a 1-year survival from ELT start of 87.4%. We identified age, AA severity before ELT start and response to ELT as variables significantly associated with OS. Two patients transformed to MDS; other adverse events were mostly benign. In sum, ELT is used widely in Europe to treat AA patients, mostly in the relapsed/refractory setting. Response to ELT is similar to the clinical trial data across different age groups, treatment lines, and treatment combinations and results in favorable survival.


Assuntos
Anemia Aplástica/tratamento farmacológico , Benzoatos/uso terapêutico , Hidrazinas/uso terapêutico , Pirazóis/uso terapêutico , Adulto , Idoso , Anemia Aplástica/mortalidade , Avaliação de Medicamentos , Uso de Medicamentos , Europa (Continente) , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/epidemiologia , Síndromes Mielodisplásicas/etiologia , Modelos de Riscos Proporcionais , Receptores de Trombopoetina/agonistas , Estudos Retrospectivos , Adulto Jovem
4.
Crit Care Med ; 47(5): 668-676, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30741755

RESUMO

OBJECTIVES: Neutropenic enterocolitis occurs in about 5.3% of patients hospitalized for hematologic malignancies receiving chemotherapy. Data from critically ill patients with neutropenic enterocolitis are scarce. Our objectives were to describe the population of patients with neutropenic enterocolitis admitted to an ICU and to investigate the risk factors of invasive fungal disease. DESIGN: A multicentric retrospective cohort study between January 2010 and August 2017. SETTING: Six French ICUs members of the Groupe de Recherche Respiratoire en Onco-Hématologie research network. PATIENTS: Adult neutropenic patients hospitalized in the ICU with a diagnosis of enteritis and/or colitis. Patients with differential diagnosis (Clostridium difficile colitis, viral colitis, inflammatory enterocolitis, mesenteric ischemia, radiation-induced gastrointestinal toxicity, and Graft vs Host Disease) were excluded. INTERVENTIONS: None. MEASUREMENT AND MAIN RESULTS: We included 134 patients (median Sequential Organ Failure Assessment 10 [8-12]), with 38.8% hospital mortality and 32.1% ICU mortality rates. The main underlying malignancies were acute leukemia (n = 65, 48.5%), lymphoma (n = 49, 36.6%), solid tumor (n = 14, 10.4%), and myeloma (n = 4, 3.0%). Patients were neutropenic during a median of 14 days (9-22 d). Infection was documented in 81 patients (60.4%), including an isolated bacterial infection in 64 patients (47.8%), an isolated fungal infection in nine patients (6.7%), and a coinfection with both pathogens in eight patients (5.0%). Radiologically assessed enteritis (odds ratio, 2.60; 95% CI, 1.32-7.56; p = 0.015) and HIV infection (odds ratio, 2.03; 95% CI, 1.21-3.31; p = 0.016) were independently associated with invasive fungal disease. CONCLUSIONS: The rate of invasive fungal disease reaches 20% in patients with neutropenic enterocolitis when enteritis is considered. To avoid treatment delay, antifungal therapy might be systematically discussed in ICU patients admitted for neutropenic enterocolitis with radiologically assessed enteritis.

6.
Clin Cancer Res ; 25(8): 2483-2493, 2019 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-30659025

RESUMO

PURPOSE: Biological explanation for discrepancies in patient-related response to chemotherapy depending on the underlying oncogenic events is a promising research area. TLX1- or TLX3-deregulated T-cell acute lymphoblastic leukemias (T-ALL; TLX1/3+) share an immature cortical phenotype and similar transcriptional signatures. However, their prognostic impacts differ, and inconsistent clinical outcome has been reported for TLX3. We therefore hypothesized that the overlapping transcriptional profiles of TLX1+ and TLX3+ T-ALLs would allow identification of candidate genes, which might determine their distinct clinical outcomes. EXPERIMENTAL DESIGN: We compared TLX1+ and TLX3+ adult T-ALL outcome in the successive French national LALA-94 and GRAALL-2003/2005 multicentric trials and analyzed transcriptomic data to identify differentially expressed genes. Epigenetic regulation of asparagine synthetase (ASNS) and in vitro l-asparaginase sensitivity were evaluated for T-ALL cell lines and primary samples. RESULTS: We show that TLX1+ patients expressed low levels of ASNS when compared with TLX3+ and TLX-negative patients, due to epigenetic silencing of ASNS by both DNA methylation and a decrease of active histone marks. Promoter methylation of the ASNS gene correlated with l-asparaginase sensitivity in both T-ALL cell lines and patient-derived xenografts. Finally, ASNS promoter methylation was an independent prognostic factor for both event-free survival [HR, 0.42; 95% confidence interval (CI), 0.24-0.71; P = 0.001] and overall survival (HR, 0.40; 95% CI, 0.23-0.70; P = 0.02) in 160 GRAALL-2003/2005 T-ALL patients and also in an independent series of 47 LL03-treated T lymphoblastic lymphomas (P = 0.012). CONCLUSIONS: We conclude that ASNS methylation status at diagnosis may allow individual adaptation of l-asparaginase dose.

8.
Br J Haematol ; 183(5): 766-774, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30407615

RESUMO

The incidence of acquired aplastic anaemia (AA) peaks in adolescents and young adults (AYA). Although age has been associated with response after immunosuppressive therapy (IST), few data exist about the specific outcome of AYA. We retrospectively compared the outcome of 29 children (aged <15 years), 32 AYA (15-25 years) and 23 adults (>25 years) with AA treated front-line with IST in Saint-Louis Hospital. The cumulative incidence of response was lower in adults compared with AYA (subdistribution hazard ratio [SHR] = 0·38, 95% confidence interval [CI] [0·96-1·00], P = 0·008), but no difference was observed between children and AYA (SHR = 0·84, 95% CI [0·96-1·00], P = 0·56), with a 6 months cumulative incidence of partial response of 44·8% in children, 62·5% in AYA and 21·7% in adults. The 5-year failure-free survival was 48·4%, without impact of age, with a 5-year relapse rate of 20·7%. With a median follow-up of 5·4 years, the 5-year overall survival was 86·5%, without significant difference between children and AYA overall survival (hazard ratio [HR] 1·51, 95% CI [0·25-9·02], P = 0·66), while adults displayed poorer survival than AYA (HR 4·98, 95% CI [1·00-24·73], P = 0·049). This study confirms that age is a prognostic factor in AA patients treated with IST. However, AYA patients have a similar outcome to children in terms of response rate and survival.

9.
Haematologica ; 2018 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-30262561

RESUMO

Aplastic anemia is a rare but potentially life-threatening disease that may affect older patients. Data regarding the treatment of aplastic anemia in this ageing population remains scarce. We conducted a retrospective nationwide multicenter study in France to examine current treatments for aplastic anemia patients over 60 years old. Our aims were to evaluate efficacy and tolerance, and to analyze predictive factors for response and survival. Over the course of a decade, 88 patients (median age 68.5) were identified in 19 centers, with a median follow-up of 2.7 years; 21% had very severe and 36% severe aplastic anemia. We analyzed 184 treatment lines, mostly involving the standard combination of anti-thymocyte globulin and cyclosporine-A (33%), which was also the most frequent first-line treatment (50%). After first-line therapy, 32% of patients achieved a complete response, and 15% a partial response. Responses were significantly better in first line and in patients with good performance status, as well as in those that had followed an anti-thymocyte globulin and cyclosporine-A regimen (overall response rate of 70% after first-line treatment). All treatments were well tolerated by patients, including over the age of 70. Three-year survival was 74.7% (median 7.36 years). Age, Charlson comorbidity index and very severe aplastic anemia were independently associated with mortality. Age per se is not a limiting factor to aplastic anemia treatment with anti-thymocyte globulin and cyclosporine-A; this regimen should be used as a first-line treatment in elderly patients if they have a good performance status and low comorbidity index score.

10.
Bone Marrow Transplant ; 53(10): 1233-1241, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29703972

RESUMO

Outcome of patients undergoing allogenic hematopoietic stem cell transplantation (allo-HSCT) has improved. To investigate if this improvement can be transposed to the ICU setting, we conducted a systematic review and meta-analysis to assess short-term mortality of critically ill allo-HSCT patients admitted to the ICU and to identify prognostic factors of mortality. Public-domain electronic databases, including Medline via PubMed and the Cochrane Library were searched. All full-text articles written-English studies published from 2006 to 2016, including allo-HSCT adults transferred to the ICU were included. Eighteen studies were selected, including 2342 patients. Overall estimated ICU mortality was 51.7%. Prognostic factors associated with an increased ICU mortality were mechanical ventilation (OR = 12.2, 95% CI = 6.2-23.7), vasopressors (OR = 6.3, 95% CI = 3.6-11.1), renal replacement therapy (OR = 4.2, 95% CI = 2.8-6.2), ICU admission for acute respiratory failure (OR = 2.2, 95% CI = 1.1-4.4), acute kidney injury (OR = 2.2, 95% CI = 1.3-4), and acute graft-versus-host disease (OR = 1.6, 95% CI = 1.1-2.3). Factors associated with an increased ICU survival were a single-organ failure (OR = 0.2, 95% CI = 0.1-0.4), neurological failure (OR = 0.4, 95% CI = 0.2-0.8), and reduced-intensity conditioning regimens (OR = 0.7, 95% CI = 0.5-0.9). Septic shock, underlying malignancy, disease status, donor, and graft source did not impact prognosis. Outcome has improved, supporting the usefulness of ICU management. Organ failures at ICU admission, organ support requirement, and GVHD are the main prognostic factors.

11.
Haematologica ; 103(2): 212-220, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29170252

RESUMO

Few therapeutic options are available for patients with aplastic anemia who are ineligible for transplantation or refractory to immunosuppressive therapy. Eltrombopag was recently shown to produce trilineage responses in refractory patients. However, the effects of real-life use of this drug remain unknown. This retrospective study (2012-2016) was conducted by the French Reference Center for Aplastic Anemia on patients with relapsed/refractory aplastic anemia, and patients ineligible for antithymocyte globulin or transplantation, who received eltrombopag for at least 2 months. Forty-six patients with aplastic anemia were given eltrombopag without prior antithymocyte globulin treatment (n=11) or after antithymocyte globulin administration (n=35) in a relapsed/refractory setting. Eltrombopag (median daily dose 150 mg) was introduced 17 months (range, 8-50) after the diagnosis of aplastic anemia. At last followup, 49% were still receiving treatment, 9% had stopped due to a robust response, 2% due to toxicity and 40% due to eltrombopag failure. Before eltrombopag treatment, all patients received regular transfusions. The overall rates of red blood cell and platelet transfusion independence were 7%, 33%, 46% and 46% at 1, 3, 6 months and last follow-up. Responses were slower to develop in antithymocyte treatment-naïve patients. In patients achieving transfusion independence, hemoglobin concentration and platelet counts improved by 3 g/dL (interquartile range, 1.4-4.5) and 42×109/L (interquartile range, 11-100), respectively. Response in at least one lineage (according to National Institutes of Health criteria) was observed in 64% of antithymocyte treatment-naïve and 74% of relapsed/refractory patients, while trilineage improvement was observed in 27% and 34%, respectively. We found high rates of hematologic improvement and transfusion independence in refractory aplastic anemia patients but also in patients ineligible for antithymocyte globulin receiving first-line treatment. In conclusion, elderly patients unfit for antithymocyte globulin therapy may benefit from eltrombopag.

12.
Blood ; 131(7): 717-732, 2018 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-29146883

RESUMO

Bone marrow (BM) failure (BMF) in children and young adults is often suspected to be inherited, but in many cases diagnosis remains uncertain. We studied a cohort of 179 patients (from 173 families) with BMF of suspected inherited origin but unresolved diagnosis after medical evaluation and Fanconi anemia exclusion. All patients had cytopenias, and 12.0% presented ≥5% BM blast cells. Median age at genetic evaluation was 11 years; 20.7% of patients were aged ≤2 years and 36.9% were ≥18 years. We analyzed genomic DNA from skin fibroblasts using whole-exome sequencing, and were able to assign a causal or likely causal germ line mutation in 86 patients (48.0%), involving a total of 28 genes. These included genes in familial hematopoietic disorders (GATA2, RUNX1), telomeropathies (TERC, TERT, RTEL1), ribosome disorders (SBDS, DNAJC21, RPL5), and DNA repair deficiency (LIG4). Many patients had an atypical presentation, and the mutated gene was often not clinically suspected. We also found mutations in genes seldom reported in inherited BMF (IBMF), such as SAMD9 and SAMD9L (N = 16 of the 86 patients, 18.6%), MECOM/EVI1 (N = 6, 7.0%), and ERCC6L2 (N = 7, 8.1%), each of which was associated with a distinct natural history; SAMD9 and SAMD9L patients often experienced transient aplasia and monosomy 7, whereas MECOM patients presented early-onset severe aplastic anemia, and ERCC6L2 patients, mild pancytopenia with myelodysplasia. This study broadens the molecular and clinical portrait of IBMF syndromes and sheds light on newly recognized disease entities. Using a high-throughput sequencing screen to implement precision medicine at diagnosis can improve patient management and family counseling.

13.
Leuk Lymphoma ; 59(6): 1323-1331, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-28901791

RESUMO

Acute leukemia (AL) is the most common hematological malignancy requiring intensive care unit (ICU) management. Data on long-term survival are limited. This is a post hoc analysis of the prospective multicenter data from France and Belgium: A Groupe de Recherche Respiratoire en Réanimation Onco-Hématologique [A Research Group on Acute Respiratory Failure in Onco-Hematological Patients (French)] Study, to identify determinants of 1-year survival in critically ill AL patients. A total of 278 patients were admitted in the 17 participating ICUs. Median age was 58 years and 70% had newly diagnosed leukemia. ICU mortality rate was 28.6 and 39.6% of the patients alive at 1 year. Admission for intensive monitoring was independently associated with better 1-year survival by multivariate analysis. Conversely, relapsed/refractory disease, secondary leukemia, mechanical ventilation and renal replacement therapy were independently associated with 1-year mortality. This study confirms the impact of organ dysfunction on long-term survival in ICU patients with AL. Follow-up studies to assess respiratory and renal recovery are warranted.

14.
J Crit Care ; 40: 69-75, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-28363097

RESUMO

PURPOSE: To describe gastrointestinal emergencies in cancer patients. METHODS: All cancer patients admitted to the medical ICU of Saint-Louis Hospital for an acute abdominal syndrome during the study period (1997-2011) were included. RESULTS: A total of 164 patients were included. The most common diagnoses were: neutropenic enterocolitis (NE) (n=54, 33%), infectious colitis and peritonitis (n=51, 31%), bowel infiltration by malignancy (n=14, 9%), and mucosal toxicity of chemotherapy (n=12, 7%). Microbiologically documented infections were reported in 82 patients (50%), including 12 fungal infections. Twenty-seven patients (16%) underwent urgent surgery. The hospital mortality rate was 35%. Five factors were independently associated with hospital mortality: the Simplified Acute Physiology Score II (SAPS II) score on day 1 (OR 1.03/SAPS II point, 95% CI 1.01 to 1.05), microbiological documentation (OR 0.27, 95% CI 0.11 to 0.64), neutropenia (OR 0.42, 95% CI 0.19 to 0.95), allogenic hematopoietic stem-cell transplantation (HSCT) (OR 5.13, 95% CI 1.71 to 15.4), and mechanical ventilation (OR 3.42, 95% CI 1.37 to 8.51). CONCLUSIONS: Gastrointestinal emergencies in cancer patients are associated with significant mortality. Mortality correlated both with the severity of organ failure upon ICU admission and the underlying diagnosis. Interestingly, patients admitted to the ICU with neutropenia had better survival.


Assuntos
Estado Terminal , Enterocolite Neutropênica/epidemiologia , Neoplasias , Adulto , Emergências , Enterocolite Neutropênica/etiologia , Enterocolite Neutropênica/mortalidade , Feminino , Mortalidade Hospitalar , Hospitalização , Humanos , Unidades de Terapia Intensiva/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Missouri/epidemiologia , Insuficiência de Múltiplos Órgãos/epidemiologia , Insuficiência de Múltiplos Órgãos/etiologia , Insuficiência de Múltiplos Órgãos/mortalidade , Escala Psicológica Aguda Simplificada
15.
J Adolesc Young Adult Oncol ; 6(2): 299-306, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27983886

RESUMO

PURPOSE: Adolescents and young adults (AYAs) with cancer are a unique group of patients in terms of disease incidence and biology, outcome, and psychosocial needs. This study aims to correlate the risk of graft-versus-host disease (GvHD) and age in a population of children and young adults with acute leukemia undergoing hematopoietic stem cell transplantation (HSCT) in first complete remission (CR). METHODS: We analyzed the outcome of 153 consecutive children (<15 years), AYAs (15-24 years), and adults (25-35 years) with lymphoblastic or myeloid acute leukemia in first CR who underwent HSCT with matched donors after myeloablative conditioning. GvHD prophylaxis was methotrexate and cyclosporine A (CsA) in all patients. RESULTS: The cumulative incidence of grade II-IV acute GvHD (aGvHD) was significantly higher in AYA patients than in children (subdistribution hazard ratio (SHR), 2.04, p = 0.005) or adults (SHR 1.59, p = 0.048). Both gut and skin aGvHD occurred more frequently in AYA patients. Increasing CsA blood levels with age could not fully account for this difference. No difference in terms of grade III-IV aGvHD was observed. Chronic GvHD was more frequent in AYAs (SHR 2.81, p = 0.007) and adults (SHR 2.31, p = 0.033) than in children. No difference in terms of nonrelated mortality and overall survival was observed among the age subgroups. CONCLUSION: Since GvHD occurrence is strongly correlated to quality of life, specific attention should be paid to AYAs undergoing HSCT. Further studies should investigate the reasons for the excess of GvHD observed in this population.


Assuntos
Doença Enxerto-Hospedeiro/epidemiologia , Transplante de Células-Tronco Hematopoéticas , Enteropatias/epidemiologia , Leucemia Mieloide Aguda/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Dermatopatias/epidemiologia , Doença Aguda , Adolescente , Adulto , Criança , Doença Crônica , Ciclosporina/uso terapêutico , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Imunossupressores/uso terapêutico , Incidência , Enteropatias/prevenção & controle , Metotrexato/uso terapêutico , Mortalidade , Modelos de Riscos Proporcionais , Qualidade de Vida , Indução de Remissão , Índice de Gravidade de Doença , Dermatopatias/prevenção & controle , Taxa de Sobrevida , Transplante Homólogo , Adulto Jovem
16.
Leuk Lymphoma ; 57(10): 2281-8, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-26849624

RESUMO

Acute myeloid leukemia with high white blood cell count (WBC) is a medical emergency. A reduction of tumor burden with hydroxyurea may prevent life-threatening complications induced by straight chemotherapy. To evaluate this strategy, we reviewed medical charts of adult patients admitted to our institution from 1997 to 2011 with non-promyelocytic AML and WBC over 50 G/L. One hundred and sixty patients were included with a median WBC of 120 G/L (range 50-450), 107 patients received hydroxyurea prior to chemotherapy, and 53 received emergency induction chemotherapy (CT). Hospital mortality was lower for patients treated with hydroxyurea (34% versus 19%, p = 0.047) even after adjusting for age (p < 0.01) and initial WBC count (p = 0.02). No evidence of any difference between treatment groups in terms of WBC decline kinetics and disease free survival (p = 0.87) was found. Oral hydroxyurea prior to chemotherapy seems a safe and efficient strategy to reduce early death of hyperleukocytic AML patients.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Hidroxiureia/uso terapêutico , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/tratamento farmacológico , Leucocitose/sangue , Leucocitose/tratamento farmacológico , Administração Oral , Adulto , Comorbidade , Feminino , Mortalidade Hospitalar , Humanos , Hidroxiureia/administração & dosagem , Quimioterapia de Indução , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/mortalidade , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Resultado do Tratamento
18.
Curr Opin Crit Care ; 21(6): 559-68, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26539930

RESUMO

PURPOSE OF REVIEW: Over the past decades, survival of critically ill hematological patients has dramatically improved, and these patients are more frequently referred to the ICU for intensive treatment, including a rising need for administering anticancer-therapy in this setting. RECENT FINDINGS: The scarce literature on this subject provides evidence for feasibility of administering chemotherapy in the ICU, with expected ICU survival of 60-70%, and one in three patients surviving at least 1 year after discharge. We summarize the recent evidence concerning outcome, dosing and indications of chemotherapy in the ICU, and provide practical guidelines for some special oncological situations. SUMMARY: Anticancer-therapy in the ICU is feasible and no longer futile as long as it is initiated in a selected, well-informed patient population with reasonable prognostic expectations. Accurate recognition of organ failure and early referral to the ICU for both supportive care and timely administration of chemotherapy is recommended before the development of multisystem organ failure.


Assuntos
Antineoplásicos/uso terapêutico , Cuidados Críticos/organização & administração , Estado Terminal , Neoplasias Hematológicas/tratamento farmacológico , Unidades de Terapia Intensiva/organização & administração , Antineoplásicos/administração & dosagem , Antineoplásicos/efeitos adversos , Neoplasias Hematológicas/mortalidade , Humanos , Guias de Prática Clínica como Assunto , Prognóstico , Qualidade de Vida , Índice de Gravidade de Doença , Assistência Terminal
19.
Respiration ; 90(3): 229-34, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26278709

RESUMO

BACKGROUND: Neutropenia recovery (NR) has been associated with worsening preexisting lung injury in up to 50% of critically ill cancer patients. However, only limited relevant data exist in the general population of hematological patients. OBJECTIVES: To assess the incidence of acute respiratory deterioration during NR in patients with hematological malignancies. METHODS: Adult patients with neutropenia expected to last more than 7 days were included. Worsening of respiratory status (WRS) was defined as a decrease in oxygen saturation of ≥5%, the need for oxygen therapy for ≥24 h, an increase in oxygen flow of ≥50% in patients previously treated with oxygen, or the need for mechanical ventilation. NR was defined as the 3 days preceding or following a neutrophil count of >0.5 × 109/l. RESULTS: A total of 16 of 50 patients included in this pilot study experienced WRS during NR (32%), and 13 patients had WRS during neutropenia (26%). The incidence density of WRS was 0.53 (±0.79) episodes per 10 days during NR and 0.20 (±0.39) episodes per 10 days during neutropenia (p = 0.004). Sepsis, stem cell transplantation, preexisting pneumonia, or the use of granulocyte colony-stimulating factor were not associated with WRS during NR. CONCLUSION: Up to one third of noncritically ill hematological patients with expected neutropenia of more than 7 days experience WRS during NR. Clinical consequences and risk factors for WRS during NR remain to be evaluated.


Assuntos
Lesão Pulmonar Aguda/etiologia , Fator Estimulador de Colônias de Granulócitos/administração & dosagem , Neoplasias Hematológicas/tratamento farmacológico , Neoplasias Hematológicas/cirurgia , Transplante de Células-Tronco Hematopoéticas/métodos , Neutropenia/fisiopatologia , Lesão Pulmonar Aguda/fisiopatologia , Adulto , Idoso , Estudos de Coortes , Intervalos de Confiança , Progressão da Doença , Feminino , Seguimentos , França , Fator Estimulador de Colônias de Granulócitos/efeitos adversos , Neoplasias Hematológicas/patologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Projetos Piloto , Estudos Prospectivos , Recuperação de Função Fisiológica , Medição de Risco , Índice de Gravidade de Doença , Fatores de Tempo
20.
Ann Hematol ; 94(11): 1859-63, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26280395

RESUMO

In recent years, the outcome of Burkitt leukemia/lymphoma (BL) has improved significantly. Central nervous system (CNS) involvement continues to be a poor prognostic indicator. High doses of intravenous polychemotherapy, intrathecal chemotherapy, and cranio-spinal radiation therapy are used by numerous groups. Majority of patients are cured after this strategy. The next challenge is to decrease toxicities of treatment, including long-term toxicities secondary to cranio-spinal radiation therapy observed in these cured patients. Liposomal cytarabine could be a good alternative to cranio-spinal radiation therapy as already reported in acute lymphoblastic leukemia. We report here eleven patients treated in our center for BL, with liposomal cytarabine instead of cranio-spinal radiation therapy as prophylactic or curative treatment for CNS involvement. Treatment was safe with no short-term grade >3 adverse events. Moreover, no long-term side effects and no impact on outcome were observed. We conclude that LC could be a good option to decrease short/long-term side effects of cranio-spinal radiation therapy in BL and could be evaluated in a future clinical trial.


Assuntos
Antimetabólitos Antineoplásicos/administração & dosagem , Linfoma de Burkitt/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/prevenção & controle , Citarabina/administração & dosagem , Adolescente , Adulto , Antimetabólitos Antineoplásicos/efeitos adversos , Linfoma de Burkitt/patologia , Linfoma de Burkitt/radioterapia , Neoplasias do Sistema Nervoso Central/radioterapia , Neoplasias do Sistema Nervoso Central/secundário , Quimioprevenção/efeitos adversos , Quimioprevenção/métodos , Irradiação Craniana , Citarabina/efeitos adversos , Feminino , Humanos , Lipossomos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA