SummaryHypereosinophilic syndrome (HES) is defined as chronic, unexplained hypereosinophilia with organ involvement. A subset of HES patients presents an interstitial deletion in chromosome 4q12, which leads to the expression of an imatinib‐responsive fusion gene, FIP1L1‐PDGFRA. These patients are diagnosed as chronic eosinophilic leukaemia (CEL). We treated seven CEL and HES patients, six of which expressed FIP1L1‐PDGFRA, with imatinib using initial daily doses ranging from 100 to 400 mg. In a remission maintenance phase, the patients were treated with imatinib once weekly. All imatinib‐treated patients achieved a complete haematological remission (CHR), and five of the six patients with FIP1L1‐PDGFRA expression exhibited molecular remission. The decreased imatinib doses were as follows: 200 mg/week in three patients, 100 mg/week in two patients and 100 mg/d in the remaining two patients. For remission maintenance, imatinib doses were set at 100 mg/week in five patients and 200 mg/week in two patients. At a median follow‐up of 30 months all patients remained in CHR and FIP1L1‐PDGFRA expression was undetectable in five of the six FIP1L1‐PDGFRA‐expressing patients. These data suggest that a single weekly dose of imatinib is sufficient to maintain remission in FIP1L1‐PDGFRA‐ positive CEL patients.
The aim of the study was to evaluate mental distress and health-related quality of life in patients with bilateral partial deafness (high-frequency sensorineural hearing loss) before cochlear implantation, with respect to their audiological performance and time of onset of the hearing impairment. Thirty-one patients and 31 normal-hearing individuals were administered the Beck Depression Inventory (BDI), the State-Trait-Anxiety-Inventory (STAI) and the World Health Organization Quality of Life-BREF questionnaire (WHOQOL-BREF). Patients also completed the Nijmegen-Cochlear-Implant-Questionnaire (NCIQ), a tool for evaluation of quality of life related to hearing loss. Patients revealed increased depressive and anxiety symptoms, as well as decreased health-related quality of life (psychological health, physical health), in comparison with their healthy counterparts (t tests, p < 0.05). Furthermore, a General Linear Model demonstrated in patients with a prelingual onset of hearing loss enhanced self-evaluated social interactions and activity (NCIQ), when their outcomes were contrasted with those obtained in individuals with postlingual partial deafness (p < 0.05). The study failed to show any effect of collateral tinnitus. Patients not using hearing aids had better audiological performance and, therefore, better sound perception and speech production, as measured with NCIQ. There was no effect of hearing aid use with respect to mental distress. Additional statistically significant correlations seen in patients included those between a steeper slope hearing loss configuration (averaged pure-tone thresholds at 1 and 2 kHz with subtracted threshold at 0.5 kHz) and better audiometric speech detection, between audiometric thresholds and the subjectively rated sound perception (NCIQ), as well as left-ear audiometric word recognition scores and the subjectively perceived ability to recognize advanced sounds (NCIQ). In addition, a longer duration of postlingual deafness, as well as a younger age at the onset were both related to worse speech detection thresholds. The results of the study provide evidence that successful rehabilitation in patients with partial deafness might have to go beyond the standard speech therapy. Enhancement of the regular diagnostic assessment with additional psychological tools is highly recommended. Further investigation is required as to the role of functional residual hearing, hearing aid use and tinnitus, in relation to future outcomes of cochlear implantation.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.