ARTICLES IN THREE SENTENCES
Erdheim-Chester disease (ECD), a rare L-group histiocytosis, shows orbital involvement in a third of cases, the so-called ECD-related orbital disease (ECD-ROD); however, few data are available concerning the radiological features of ECD-ROD. Haroche and colleagues performed a retrospective study of 137 patients with ECD, aimed at characterizing the initial radiological phenotype and outcome of patients with ECD-ROD. They concluded that the initial assessment and subsequent monitoring of ECD-ROD by orbital MRI and ophthalmological follow-up is essential to prevent possible functional handicaps, such as visual field limitation.
Total late effect burden in long-term lymphoma survivors after high-dose therapy with autologous stem-cell transplant and its effect on health-related quality of life
There is a growing population of long-term lymphoma survivors after autologous stem-cell transplant who are at increased risk of late effects of the cumulative treatment received. Smeland and colleagues performed the first study assessing late effect burden in adult lymphoma survivors in a real-world national setting, including severity grading based on a comprehensive clinical assessment combined with patient-reported outcomes. Most survivors have a significant late effect burden and experience several different moderate or more severe late effects, affecting their health-related quality of life.
The homeobox transcription factors HoxA9 and Meis1 are causally involved in the etiology of acute myeloid leukemia. Garcia-Cuellar and colleagues applied degron techniques to elucidate the leukemogenic contribution of Meis1. They provided the molecular correlation of the experimental observation that Meis1 enhances leukemogenesis in combination with HoxA9 while on its own it has no discernible effect on hematopoietic development.
Histological and genetic characterization and follow-up of 130 patients with chronic triple-negative thrombocytosis
A number of patients with a clinical presentation of “triple-negative” acquired thrombocytosis do not display the characteristic histological features of essential thrombocytosis or myeloproliferative neoplasm, raising the question of appropriate therapeutic management. In a cohort of 130 patients with chronic, non-reactive triple-negative thrombocytosis, Lemoine and colleagues had bone marrow biopsies reviewed centrally, and then asked whether targeted NGS could help reach a diagnosis. They showed that characterization of triple-negative thrombocytosis relies on thorough clinical, biological, histological and genetic studies.
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