RESUMEN
In 2010, we reported the outcome of hematopoietic stem cell transplantation (HSCT) in 11 children with Griscelli syndrome type 2 (GS2). We report here the update on this cohort to include 35 patients. Twenty-seven (77%) patients received conditioning regimen including busulfan, cyclophosphamide with etoposide. Eight (23%) were given busulfan, fludarabine. Thiotepa was added to busulfan and fludarabine regimen in two patients; one received haploidentical marrow and one unrelated cord blood. Posttransplant clinical events included veno-occlusive disease (n = 7), acute (n = 8), or chronic (n = 1) graft-versus-host disease II-IV. With a mortality rate of 37.1% (n = 13) and a median follow-up of 87.7 months of the survivors, 5-year cumulative probability of overall survival (OS) for our cohort of patients was 62.7% (±8.2%). Cumulative probability of 5-year OS was significantly better in those who did not have hemophagocytic lymphohistiocytosis (HLH) prior to HSCT (100% vs. 53.3 ± 9.5%, P value: 0.042). Of the 16 patients with neurologic involvement before HSCT, 8 survived and 3 presented sequelae. OS at 5-year was 50 ± 12.5% and 73.3 ± 10.2% (P value: 0.320) in patients with and without CNS involvement, respectively. In conclusion, HSCT in patients with GS2 is potentially curative with long-term disease-free survival. Early HSCT before the development of the accelerated phase is associated with a better outcome.
Asunto(s)
Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Linfohistiocitosis Hemofagocítica , Piebaldismo , Enfermedades de Inmunodeficiencia Primaria , Busulfano , Niño , Humanos , Linfohistiocitosis Hemofagocítica/terapia , Recurrencia Local de Neoplasia , Piebaldismo/terapia , Enfermedades de Inmunodeficiencia Primaria/terapia , Estudios Retrospectivos , Acondicionamiento Pretrasplante , VidarabinaRESUMEN
GOALS: The aim of this study is to investigate the prevalence of depression among cancer patients in Jordan and to study the relation between several socio-demographic, disease- and treatment-related factors, and the occurrence of depression among those cancer patients. PATIENTS AND METHODS: A cross-sectional survey study was conducted at a major university hospital in Jordan. Cancer patients were interviewed for socio-demographic information and medical records were checked for information about disease and treatment of patient. Patients' psychological status was assessed using The Hospital Anxiety and Depression Scale (HADS). RESULTS: The prevalence of depression in our sample was 51.9%. Significant correlation was detected between depression and appetite among cancer patients. Knowledge of having cancer and stage of the disease were also significantly associated with occurrence of depression. CONCLUSION: In an effort to reduce the occurrence of depression among cancer patients, special attention is needed for changes in the psychological status in patients with knowledge about their diagnosis and patients in advanced disease stage.