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1.
Blood ; 139(14): 2173-2185, 2022 04 07.
Artigo em Inglês | MEDLINE | ID: mdl-34871373

RESUMO

Chimeric antigen receptor (CAR) T-cell therapy can induce durable remissions of relapsed/refractory B-acute lymphoblastic leukemia (ALL). However, case reports suggested differential outcomes mediated by leukemia cytogenetics. We identified children and young adults with relapsed/refractory CD19+ ALL/lymphoblastic lymphoma treated on 5 CD19-directed CAR T-cell (CTL019 or humanized CART19) clinical trials or with commercial tisagenlecleucel from April 2012 to April 2019. Patients were hierarchically categorized according to leukemia cytogenetics: High-risk lesions were defined as KMT2A (MLL) rearrangements, Philadelphia chromosome (Ph+), Ph-like, hypodiploidy, or TCF3/HLF; favorable as hyperdiploidy or ETV6/RUNX1; and intermediate as iAMP21, IKZF1 deletion, or TCF3/PBX1. Of 231 patients aged 1 to 29, 74 (32%) were categorized as high risk, 28 (12%) as intermediate, 43 (19%) as favorable, and 86 (37%) as uninformative. Overall complete remission rate was 94%, with no difference between strata. There was no difference in relapse-free survival (RFS; P = .8112), with 2-year RFS for the high-risk group of 63% (95% confidence interval [CI], 52-77). There was similarly no difference seen in overall survival (OS) (P = .5488), with 2-year OS for the high-risk group of 70% (95% CI, 60-82). For patients with KMT2A-rearranged infant ALL (n = 13), 2-year RFS was 67% (95% CI, 45-99), and OS was 62% (95% CI, 40-95), with multivariable analysis demonstrating no increased risk of relapse (hazard ratio, 0.70; 95% CI, 0.21-2.90; P = .7040) but a higher proportion of relapses associated with myeloid lineage switch and a 3.6-fold increased risk of all-cause death (95% CI, 1.04-12.75; P = .0434). CTL019/huCART19/tisagenlecleucel are effective at achieving durable remissions across cytogenetic categories. Relapsed/refractory patients with high-risk cytogenetics, including KMT2A-rearranged infant ALL, demonstrated high RFS and OS probabilities at 2 years.


Assuntos
Imunoterapia Adotiva , Leucemia-Linfoma Linfoblástico de Células Precursoras , Antígenos CD19 , Criança , Análise Citogenética , Humanos , Lactente , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Receptores de Antígenos de Linfócitos T/uso terapêutico , Recidiva , Adulto Jovem
2.
J Pediatr Hematol Oncol ; 46(3): e254-e258, 2024 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-38408123

RESUMO

BACKGROUND: Pediatric B-lymphoblastic lymphoma is an uncommon subtype of non-Hodgkin lymphoma. Studies regarding the biology, clinical course, and approach to relapse are limited. OBSERVATIONS: We present a series of children with B-lymphoblastic lymphoma to describe the clinical course at diagnosis and relapse as well as the role of tumor cytogenetics, immunotherapy, and hematopoietic stem cell transplant. CONCLUSIONS: The prognostic significance of cytogenetic changes in B-lymphoblastic lymphoma is not well described but may offer improved risk stratification. Immunotherapy may offer salvage options for relapsed disease and can serve as a bridge to transplant.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Linfoma não Hodgkin , Leucemia-Linfoma Linfoblástico de Células Precursoras , Criança , Humanos , Recidiva Local de Neoplasia , Linfoma não Hodgkin/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Recidiva , Progressão da Doença
3.
Pediatr Blood Cancer ; 70(8): e30401, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37158503

RESUMO

There are limited data pertaining to the prognostic features and optimal therapeutic approach for the 20%-25% of children with lymphoblastic lymphoma (LLy) who have the B-lymphoblastic subtype. Outcomes are favorable following treatment modeled after acute lymphoblastic leukemia (ALL) regimens, but prognosis is dismal after relapse, and there are no established features for predicting therapy response. Ongoing US and international trials will include the largest cohort of uniformly treated patients with B-LLy to date, providing an opportunity to define clinical and molecular predictors of relapse and to establish a standard of care for treatment to improve outcomes for this rare pediatric cancer.


Assuntos
Linfoma de Células B , Linfoma não Hodgkin , Leucemia-Linfoma Linfoblástico de Células Precursoras , Humanos , Criança , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Prognóstico , Recidiva
4.
Pediatr Transplant ; 27(7): e14583, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37485777

RESUMO

BACKGROUND: After solid organ transplantation, children are at risk for Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and smooth muscle tumors. Little is known about the clinical course, Epstein-Barr viral load variations, and optimal treatment for such patients. We set forth to understand the course of repeated episodes of post-transplant lymphoproliferative disorder and smooth muscle tumors. METHODS: We performed a retrospective chart review of patients up to 21 years old with solid organ transplantation and post-transplant lymphoproliferative disorder at the Children's Hospital of Philadelphia from January 2003 through June 30, 2020. RESULTS: Six patients had multiple episodes of Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and smooth muscle tumors. When the second episode was discovered, only one patient was symptomatic. Histology differed from diagnosis in four patients. Treatment included viral-specific T-lymphocytes (2), rituximab (3), reduction in immunosuppression alone (1). Five patients had complete response, and one had stable disease, but three patients developed a subsequent tumor. Two patients developed Epstein-Barr virus-associated smooth muscle tumors. Of these six patients, four are alive. The deaths were not related to their tumors. CONCLUSIONS: Despite a complete response to initial therapy, children are at risk for repeated episodes of Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and smooth muscle tumors. Histology and location were not typically consistent with initial diagnosis, suggesting these are second primaries rather than recurrences. Disease may be managed with individualized treatment plans but EBV-specific T cells need further study in such tumors.

5.
J Pediatr Hematol Oncol ; 45(8): e966-e971, 2023 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-37661306

RESUMO

As hospitalized pediatric patients have grown in number and complexity, and residency structural changes have reduced resident coverage, inpatient care models have changed to include additional providers at the "front line." Hospitalists are increasingly employed in general pediatric units, but in specialized inpatient areas, hospitalist care models are less common. Hospitalist programs in pediatric oncology are few and unique, and thus there are limited data assessing this role. Here we describe the oncology/stem cell transplant hospitalist program at the Children's Hospital of Philadelphia with a survey project to assess the perceptions of physicians in the role. Hospitalists from 2017 to 2019 (n=26) were surveyed to assess nonclinical roles and job satisfaction. With a response rate of 84.6%, all respondents concurred work-life balance, hours, and flexibility are attractive and found the field intellectually stimulating. Most (86.4%) agreed there were significant academic opportunities. The vast majority felt this job was valuable in attaining career and personal goals; 95.5% were happy they accepted this position. As the pediatric oncology/stem cell transplant hospitalist position is a viable, versatile career path providing ample academic opportunities and job satisfaction, the expansion of such a model within our institution and others should be well received.


Assuntos
Médicos Hospitalares , Humanos , Criança , Inquéritos e Questionários , Satisfação no Emprego , Hospitais Pediátricos , Hospitalização
6.
J Pediatr Hematol Oncol ; 44(4): e859-e865, 2022 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-35235547

RESUMO

Children with cancer and those undergoing hematopoietic stem cell transplantation frequently require anesthesia for imaging as well as diagnostic and therapeutic procedures from diagnosis through follow-up. Due to their underlying disease and side effects of chemotherapy and radiation, they are at risk for complications during this time, yet no published guideline exists for preanesthesia preparation. A comprehensive literature review served as the basis for discussions among our multidisciplinary panel of oncologists, anesthesiologists, nurse practitioners, clinical pharmacists, pediatric psychologists, surgeons and child life specialists at the Children's Hospital of Philadelphia. Due to limited literature available, this panel created an expert consensus guideline addressing anesthesia preparation for this population.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Neoplasias , Anestesia Geral/efeitos adversos , Criança , Consenso , Diagnóstico por Imagem , Humanos , Neoplasias/terapia
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