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.
Extramedullary plasmacytoma (EMP) is a rare disease that occurs in 3% to 5% of patients with plasma cell disorder. It occurs most commonly in the upper respiratory tract and the oral cavity. Very few EMP cases have been reported in the central nervous system (CNS). We report herein an unusual case of EMP in the nasal cavity that recurred in the CNS without systemic involvement. A 67-year-old man visited our hospital due to a month-long bout with exophthalmos. He was diagnosed with EMP in the nasal cavity, paranasal sinus, and orbital cavity. He received radiotherapy to which he had complete responses. After 2 years, he visited our hospital because of a month-long headache. He was diagnosed with EMP recurrence in the CNS via brain magnetic resonance imaging and cerebrospinal fluid analysis. He was treated with whole brain radiotherapy and intrathecal chemotherapy with methotrexate, but he expired due to pneumonia.
Allogeneic hematopoietic stem cell transplantation (HSCT) is considered the optimal curative treatment for acute myeloid leukemia (AML), but some patients develop bone marrow relapse due to remnant leukemia, and few patients develop extramedullary relapse without bone marrow relapse. Isolated extramedullary relapse (IMER) is defined as extramedullary relapse without bone marrow relapse. IMER has been reported in various sites, including the skin, soft tissue, and central nervous system(CNS). Isolated CNS relapse is relatively rare and is associated with poor prognosis due to the absence of an optimal treatment for it. Reported herein is a case involving an adult AML woman who suffered from isolated extramedullary relapse in the CNS after allogeneic HSCT. She was treated with intrathecal chemotherapy and whole-brain and spine radiotherapy, followed by systemic chemotherapy. She is currently well, with no evidence of leukemia recurrence for over six years.
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Post-transplant leukemia relapse in organs: biology. and behavior in 585 reports Isabel Cunningham Critical Reviews in Oncology/Hematology.2021; 157: 103170. CrossRef