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1.
Am J Hematol ; 99(5): 844-853, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38357714

RESUMO

Splenomegaly is the clinical hallmark of myelofibrosis. Splenomegaly at the time of allogeneic hematopoietic cell transplantation (HCT) is associated with graft failure and poor graft function. Strategies to reduce spleen size before HCT especially after failure to Janus kinase (JAK) inhibition represent unmet clinical needs in the field. Here, we leveraged a global collaboration to investigate the safety and efficacy of splenic irradiation as part of the HCT platform for patients with myelofibrosis. We included 59 patients, receiving irradiation within a median of 2 weeks (range, 0.9-12 weeks) before HCT. Overall, the median spleen size prior to irradiation was 23 cm (range, 14-35). Splenic irradiation resulted in a significant and rapid spleen size reduction in 97% of patients (57/59), with a median decrease of 5.0 cm (95% confidence interval, 4.1-6.3 cm). The most frequent adverse event was thrombocytopenia, with no correlation between irradiation dose and hematological toxicities. The 3-year overall survival was 62% (95% CI, 48%-76%) and 1-year non-relapse mortality was 26% (95% CI, 14%-38%). Independent predictors for survival were severe thrombocytopenia and anemia before irradiation, transplant-specific risk score, higher-intensity conditioning, and present portal vein thrombosis. When using a propensity score matching adjusted for common confounders, splenic irradiation was associated with significantly reduced relapse (p = .01), showing a 3-year incidence of 12% for splenic irradiation versus 29% for patients with immediate HCT and 38% for patients receiving splenectomy. In conclusion, splenic irradiation immediately before HCT is a reasonable approach in patients experiencing JAK inhibition failure and is associated with a low incidence of relapse.


Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Mielofibrose Primária , Trombocitopenia , Humanos , Baço , Esplenomegalia/etiologia , Esplenomegalia/radioterapia , Mielofibrose Primária/radioterapia , Mielofibrose Primária/complicações , Transplante de Células-Tronco Hematopoéticas/métodos , Trombocitopenia/complicações , Recidiva , Condicionamento Pré-Transplante/métodos , Doença Enxerto-Hospedeiro/etiologia
2.
Endocrinol Metab Clin North Am ; 52(4): 629-641, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37865478

RESUMO

Racial and ethnic disparities exist in the prevalence and management of osteoporosis, metastatic cancer, and sickle cell disease. Despite being the most common metabolic bone disease, osteoporosis remains underscreened and undertreated among Black women. Skeletal-related events in metastatic cancer include bone pain, pathologic fractures, and spinal cord compression. Disparities in screening for and treating skeletal-related events disproportionately affect Black patients. Metabolic bone disease contributes significantly to morbidity in sickle cell disease; however, clinical guidelines for screening and treatment do not currently exist. Clinical care recommendations are provided to raise awareness, close health care gaps, and guide future research efforts.


Assuntos
Anemia Falciforme , Neoplasias , Osteoporose , Humanos , Feminino , Osteoporose/etiologia , Osteoporose/terapia , Osteoporose/diagnóstico , Atenção à Saúde , Anemia Falciforme/complicações , Anemia Falciforme/terapia
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