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CNS Involvement by North American-Adult T-cell Leukemia/Lymphoma Is Associated With Discrete Dissemination Patterns
Nishi Shah1,2, Melissa Suchanek1,3, Astha Thakkar1
1Department of Oncology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY.
Purpose:
CNS involvement in North American adult T-cell leukemia/lymphoma (NA-ATLL) remains poorly understood. This study examined the CNS involvement in patients with ATLL treated at a tertiary hospital in New York City.
Methods:
CNS involvement was defined by positive cerebrospinal fluid (CSF) cytology, flow cytometry, positive CNS imaging, or neurological examination findings.
Results:
Among 94 patients with NA-ATLL, 21 (22.3%) had CNS involvement. CSF was involved in 13 patients at diagnosis and five at relapse. Magnetic resonance imaging detected brain and spinal involvement in 24% and 14% of the patients, respectively. Results of neurological examinations were abnormal in 33% and 14% of the patients at diagnosis and relapse, respectively. The XPO1 E571K mutation was found in two patients with extensive, treatment-refractory CNS disease, with a median overall survival (OS) of 2 months. Other mutations, including KLF2 and PI3KCD, were noted in two patients. The median OS was 8.5 months, and the median relapse-free survival (RFS) was 6.5 months in our series. In most cases (5/21), the lymphomatous phenotype appeared to have a direct mass-like extension, whereas in patients with predominant blood involvement tended to spread to the CSF by traversing the blood-brain barrier.
Conclusion:
In this report, we describe the patterns of CNS involvement in ATLL and their association with mutations. We also describe two rapidly fatal cases with the XPO1 E571K mutation, which may represent a novel therapeutic target for T-cell lymphomas.
Insights
Central nervous system (CNS) involvement affects over 20% of North American adult T-cell leukemia/lymphoma (NA-ATLL) patients. The XPO1 E571K mutation was linked to poor outcomes, suggesting a potential therapeutic target.
Area of Science:
- Hematology
- Neurology
- Oncology
Background:
- Central nervous system (CNS) involvement in North American adult T-cell leukemia/lymphoma (NA-ATLL) is not well understood.
- This study investigates CNS involvement patterns in NA-ATLL patients treated in New York City.
Purpose of the Study:
- To examine the prevalence and characteristics of CNS involvement in NA-ATLL.
- To identify potential prognostic factors and therapeutic targets associated with CNS disease in NA-ATLL.
Main Methods:
- Defined CNS involvement by cerebrospinal fluid (CSF) analysis, CNS imaging, and neurological exams.
- Analyzed patient data for demographic, clinical, and molecular features, including mutations like XPO1 E571K.
Main Results:
- 22.3% of 94 NA-ATLL patients had CNS involvement, with CSF affected in 13 at diagnosis and 5 at relapse.
- Neurological abnormalities were observed in 33% at diagnosis and 14% at relapse; XPO1 E571K mutation correlated with refractory disease and poor survival (median OS 2 months).
- Median overall survival (OS) was 8.5 months and relapse-free survival (RFS) was 6.5 months; disease spread occurred via mass-like extension or blood-brain barrier traversal.
Conclusions:
- CNS involvement is a significant complication in NA-ATLL, with distinct patterns of spread.
- The XPO1 E571K mutation may indicate a rapidly fatal course and represents a potential therapeutic target for T-cell lymphomas.
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