Conflict of interest: Nothing to declare.
Clinical features and early treatment response of central nervous system involvement in childhood acute lymphoblastic leukemia
Article first published online: 12 FEB 2014
© 2014 Wiley Periodicals, Inc.
Pediatric Blood & Cancer
Volume 61, Issue 8, pages 1416–1421, August 2014
How to Cite
Levinsen, M., Taskinen, M., Abrahamsson, J., Forestier, E., Frandsen, T. L., Harila-Saari, A., Heyman, M., Jonsson, O. G., Lähteenmäki, P. M., Lausen, B., Vaitkevičienė, G., Åsberg, A., Schmiegelow, K. and on behalf of the Nordic Society of Paediatric Haematology and Oncology (NOPHO) (2014), Clinical features and early treatment response of central nervous system involvement in childhood acute lymphoblastic leukemia. Pediatr. Blood Cancer, 61: 1416–1421. doi: 10.1002/pbc.24981
- Issue published online: 10 JUN 2014
- Article first published online: 12 FEB 2014
- Manuscript Accepted: 16 JAN 2014
- Manuscript Received: 9 NOV 2013
- Danish Childhood Cancer Foundation, Rigshospitalets forskningspuljer, Dansk Kræftforsknings Fond, Elna og Jørgen Fagerholt Pedersens Kræftforskningsfond, Anders Hasselbalchs fond til leukæmiens bekæmpelse
- Swedish Cancer Foundation
- minimal residual disease
Central nervous system (CNS) involvement in childhood acute lymphoblastic leukemia (ALL) remains a therapeutic challenge.
To explore leukemia characteristics of patients with CNS involvement at ALL diagnosis, we analyzed clinical features and early treatment response of 744 patients on Nordic-Baltic trials. CNS status was classified as CNS1 (no CSF blasts), CNS2 (<5 leukocytes/µl CSF with blasts), CNS3 (≥5 leukocytes/µl with blasts or signs of CNS involvement), TLP+ (traumatic lumbar puncture with blasts), and TLP− (TLP with no blasts).
Patients with CNS involvement had higher leukocyte count compared with patients with CNS1 (P < 0.002). Patients with CNS3 more often had T-ALL (P < 0.001) and t(9;22)(q34;q11)[BCR-ABL1] (P < 0.004) compared with patients with CNS1. Among patients with CNS involvement headache (17%) and vomiting (14%) were most common symptoms. Symptoms or clinical findings were present among 27 of 54 patients with CNS3 versus only 7 of 39 patients with CNS2 and 15 of 75 patients with TLP+ (P < 0.001). The majority of patients with CNS involvement received additional induction therapy. The post induction bone marrow residual disease level did not differ between patients with CNS involvement and patients with CNS1 (P > 0.15). The 12-year event-free survival for patients with leukemic mass on neuroimaging did not differ from patients with negative or no scan (0.50 vs. 0.60; P = 0.7) or between patients with symptoms or signs suggestive of CNS leukemia and patients without such characteristics (0.50 vs. 0.61; P = 0.2).
CNS involvement at diagnosis is associated with adverse prognostic features but does not indicate a less chemosensitive leukemia. Pediatr Blood Cancer 2014; 61:1416–1421. © 2014 Wiley Periodicals, Inc.