Raghvan Hari Priya, Ling Caroline Ho Siew, Yui Wee Shiang, Liang Boo Yang, Jamian Ehram, Subbiah Indhira
Haematology Unit, Department of Pathology, Hospital Sultanah Aminah, Ministry of Health, Johor Bahru, Malaysia.
Clinical Haematology, Department of Medicine, Hospital Sultanah Aminah, Ministry of Health, Johor Bahru, Malaysia.
J Yeungnam Med Sci. 2025;42:17. doi: 10.12701/jyms.2025.42.17. Epub 2024 Dec 19.
Chronic myeloid leukemia (CML) typically progresses from a chronic phase to an accelerated phase, and eventually to a blast crisis, often involving the bone marrow and peripheral blood, if left untreated. Central nervous system (CNS) involvement is an uncommon manifestation of CML, particularly as an isolated CNS relapse. Here, we present a rare case of CML in lymphoid blast crisis with an isolated CNS relapse. A 46-year-old female with underlying CML in lymphoid blast crisis, previously treated with chemotherapy and tyrosine kinase inhibitors, presented with visual disturbances. Imaging and cerebrospinal fluid analysis confirmed leukemic infiltration of the CNS without evidence of a systemic disease. Isolated CNS involvement is an atypical complication of CML and presents significant therapeutic challenges owing to the blood-brain barrier, which limits the efficacy of systemic therapies. Subsequently, the patient was treated with intrathecal chemotherapy targeting the CNS. Despite aggressive treatment, CNS relapse remains a major concern due to the limited penetration of standard therapies into the CNS. This case underscores the importance of early recognition of CNS symptoms in CML patients, particularly in those with blast crisis, and highlights the need for tailored therapeutic strategies to manage this rare and challenging manifestation.
慢性髓性白血病(CML)通常从慢性期进展为加速期,并最终发展为急变期,如果不进行治疗,常累及骨髓和外周血。中枢神经系统(CNS)受累是CML的一种不常见表现,尤其是作为孤立的中枢神经系统复发。在此,我们报告一例罕见的CML淋巴母细胞急变期伴孤立性中枢神经系统复发的病例。一名46岁患有CML淋巴母细胞急变期的女性,此前接受过化疗和酪氨酸激酶抑制剂治疗,出现视觉障碍。影像学和脑脊液分析证实中枢神经系统存在白血病浸润,而无全身疾病证据。孤立性中枢神经系统受累是CML的一种非典型并发症,由于血脑屏障限制了全身治疗的疗效,因此带来了重大的治疗挑战。随后,该患者接受了针对中枢神经系统的鞘内化疗。尽管进行了积极治疗,但由于标准疗法进入中枢神经系统的渗透性有限,中枢神经系统复发仍然是一个主要问题。该病例强调了早期识别CML患者,特别是急变期患者中枢神经系统症状的重要性,并突出了需要制定针对性治疗策略来应对这种罕见且具有挑战性的表现。