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Meningeal leukemia in the blastic phase of chronic granulocytic leukemia
Insights
Meningeal leukemia developed in nearly 7% of patients with Ph-positive chronic granulocytic leukemia (CGL) in blastic phase. Intrathecal methotrexate effectively treated this complication, eradicating symptoms and cerebrospinal fluid abnormalities.
Area of Science:
- Hematology
- Oncology
- Neurology
Background:
- Ph-positive chronic granulocytic leukemia (CGL) in blastic phase presents significant treatment challenges.
- Improved survival in CGL blastic phase may increase the risk of central nervous system involvement.
Observation:
- Meningeal leukemia developed in 6.9% of 101 patients treated for CGL blastic phase.
- Neurologic signs included cranial nerve palsies and papilledema; cerebrospinal fluid showed pleocytosis with myeloblasts.
- Meningeal leukemia was more frequent in complete responders (42%) compared to incomplete responders (2.3%).
Findings:
- Complete remission in CGL blastic phase patients was associated with a median survival of 12 months.
- Intrathecal methotrexate demonstrated high efficacy in treating meningeal leukemia.
- Treatment led to eradication of cerebrospinal fluid pleocytosis and resolution of neurologic symptoms in most patients.
Implications:
- Increased survival in CGL necessitates vigilance for meningeal leukemia development.
- Intrathecal methotrexate is a successful therapeutic strategy for meningeal leukemia in CGL.
- Further research into CNS prophylaxis and treatment in CGL is warranted.
Abstract:
One hundred one patients were treated for Ph' positive chronic granulocytic leukemia (CGL) in the blastic phase. In seven of these (6.9 per cent), meningeal leukemia developed. Of the 99 patients who died of their disease, a complete remission was achieved in 12 with a median survival of 12 months (three to 28 months). Incomplete responders had a median survival of only 2.5 months (one to 14 months). In five of the 12 complete responders (42 per cent), but in only two of the incomplete responders (2.3 per cent), meningeal leukemia developed. The principal neurologic signs were cranial nerve palsies and papilledema. All patients had pleocytosis with myeloblasts in the cerebrospinal fluid. As in patients with acute leukemia and diffuse histiocytic lymphoma, increased survival of patients in whom hematologic remission from the blastic phase of CGL is achieved may allow sufficient time for the development of meningeal leukemia. Intrathecal methotrexate is extremely successful in treating this complication. Cerebrospinal fluid pleocytosis was eradicated in all seven of our patients, and neurologic symptoms and signs were completely eliminated in five patients. No evidence of meningeal leukemia was found in three of the five patients in whom an autopsy was performed.