Islam Rezwan, Yellu Mahender R, Shaw Gene R
Department of Oncology/Hematology, Marshfield Clinic Weston Center, Weston, Wisconsin, USA.
BMJ Case Rep. 2013 May 22;2013:bcr2013010068. doi: 10.1136/bcr-2013-010068.
Myelofibrosis, either primary or resulting from essential thrombocythemia or polycythemia vera, may present with highly variable white blood cell counts, including progressive leukopaenia with its associated risk of infections. Medications have been developed to reduce splenomegaly and other symptoms, but there are no reports of improved white blood cell counts. We report a case of primary myelofibrosis with marked improvement in leukopaenia and reduced recurrent infections, in addition to reduction in spleen size and improvement in disease-associated symptoms, within 20 weeks after using low-dose ruxolitinib. Although reduction of splenomegaly in myelofibrosis patients is the anticipated benefit of ruxolitinib, the drug may also have the potential to improve leukopaenia if used at a low dose.
骨髓纤维化,无论是原发性的,还是由原发性血小板增多症或真性红细胞增多症引起的,其白细胞计数可能有很大差异,包括进行性白细胞减少及其相关的感染风险。已经开发出药物来减少脾肿大和其他症状,但尚无白细胞计数改善的报道。我们报告了一例原发性骨髓纤维化患者,在使用低剂量鲁索替尼后20周内,白细胞减少明显改善,反复感染减少,此外脾脏大小减小,疾病相关症状改善。虽然骨髓纤维化患者脾肿大的减轻是鲁索替尼预期的益处,但如果低剂量使用,该药物也可能有改善白细胞减少的潜力。