Chronic myeloid leukemia extramedullary blast crisis presenting as central nervous system leukemia

Abstract

Rationale:Childhood chronic myeloid leukemia (CCML) is a malignant disease of granulocyte abnormal hyperplasia that is caused by clonal proliferation of pluripotent stem cells. The condition is relatively rare, accounting for 2.0% to 3.0% of cases of childhood leukemia. In addition, the incidence of extramedullary blast crisis in CCML presenting as central nervous system (CNS) blast crisis remaining chronic phase of the disease in bone marrow is extremely unusual.Patient concerns:We report a case of childhood chronic myelogenous leukemia that abandoned treatment, resulting in chronic myelogenous leukemia transforming into extramedullary blast crisis resulting in CNS leukemia, accompanied by the chronic phase of the disease in bone marrow.Diagnoses:Chronic myeloid leukemia extramedullary blast crisis presenting as CNS leukemia without blast crisis in bone marrow.Interventions:Following high-dose systemic and intrathecal chemotherapy, the patient continued to do well.Lessons:High-dose systemic and intrathecal chemotherapy is safe and helpful for CCML extramedullary blast crisis. A long-term follow-up is crucial.

Cerebrospinal fluid infiltration by chronic myeloid leukaemia: Evaluation of cytomorphological features

“…2 Approximately 4%-16% of all CML cases develop BC during the course of the disease. 7,8 This phase often presents as acute leukaemia. 5 Lymph nodes, skin, soft tissues, bones, serosal surfaces, and the gastrointestinal and genitourinary tracts are the most common sites of extramedullary BC.…”

“…5 CNS involvement by CML leukaemic cells occurs via hematogenous dissemination, meningeal implantation or infiltration of cranial bone marrow, or due to procedural injury during lumbar puncture. 4,5,7 As these cells gain entry into the CNS milieu, they remain shielded from the effect of TKI, owing to the poor penetration of the blood-brain barrier by these drugs. 5,10 Subsequently, these cells also participate in the formation of a blood-tumour barrier.…”

“…Usually, second-generation TKIs are initiated for more efficient BBB penetration, along with intrathecal chemotherapy, high-dose systemic chemotherapy and radiotherapy. [7][8][9][10]15,17 Also, lymphoid CML-BC responds more readily to chemotherapy than myeloid CML-BC. However, such extensive therapy is accompanied by substantial toxicity and a short-lived response.…”

“…Extramedullary BC of CML is defined as the proliferation of leukaemic blasts at all sites apart from the bone marrow 2 . Approximately 4%‐16% of all CML cases develop BC during the course of the disease 7,8 . This phase often presents as acute leukaemia 5 .…”

Isolated blast crisis relapse in the central nervous system of a patient treating for a chronic myelogenous leukemia

“…Lymph nodes, skin and soft tissues, bone, spleen are the commonest sites of extramedullary blast crises. However, isolated CNS blast crisis is uncommon and is limited to occasional case reports in adults or exceptional in children patients receiving imatinib mesylate treatment [ 9 , 10 ]. CNS blast crisis is usually presented with the clinical and radiological features of encephalitis and/or meningitis, which includes symptoms such as headache, cognitive changes, raised intracranial pressure and visual disturbances.…”