Abstract
Juvenile myelomonocytic leukemia is a rare malignancy that occurs in pediatric patients. Previous reports, have described leukemic cells may infiltrate many organs, such as the lungs, skin, liver, spleen, and intestines, but not the central nervous system, although central nervous system infiltration remains a point of concern in every patient with acute leukemia. Here, we present one case of a boy with juvenile myelomonocytic leukemia who developed multiple lesions in the brain while undergoing chemotherapy with 6-mercaptopurine and cytarabine. We diagnosed the central nervous system involvement by magnetic resonance imaging, cerebrospinal fluid cytology, and the patient’s clinical course. He was treated with a high dose of cytarabine and intrathecal chemotherapy, then with unrelated cord blood stem cell transplantation. He has been in a first complete remission for more than 18 months after cord blood stem cell transplantation without any neurological sequelae. In conclusion, we encountered a boy with juvenile myelomonocytic leukemia who developed central nervous system lesions under standard chemotherapy. We subsequently switched treatment to central nervous system-oriented chemotherapy, which resulted in a good clinical condition and successful cord blood stem cell transplantation.
References
Clerk JJ, Berman JN, Look AT. Myeloid leukemia, myelodysplasia and myeloproliferative disease in children. In: Orkin SH, Fisher DE, Look AT, Lux SE, Ginsburg D, Nathan DG, editors. Oncology of infancy and childhood. Philadelphia/Amsterdam: Saunders/Elsevier Inc.; 2009. p. 331–402.
Locatelli F, Nollke P, Zecca M, et al. Hematopoietic stem cell transplantation (HSCT) in children with juvenile myelomonocytic leukemia (JMML): results of the EWOG-MDS/EBMT trial. Blood. 2005;105:410–9.
Yabe M, Sako M, Yabe H, et al. A conditioning regimen of busulfan fludarabine, and melphalan for allogeneic stem cell transplantation in children with juvenile myelomonocytic leukemia. Pediatr Transplant. 2008;12:862–7.
Wilson DB, Isolated CNS. Relapse Following Stem Cell Transplantation for Juvenile Myelomonocytic Leukemia. J Pediatr Hematol Oncol. 2003;25:910–3.
Smith LB. Juvenile Myelomonocytic Leukemia Presenting With Facial Nerve Paresis A Unique Presentation. J Pediatr Hematol Oncol. 2007;29:770–3.
Braumann I. Juvenile myelomonocytic leukemia. In: Swerdlow SH, Campo E, Harris NL, et al., editors. WHO classification of tumors of hematopoietic and lymphoid tissues. USA: International Agency for Research on Cancer; 2008. p. 82–4.
Matsuda K, Shimada A, Yoshida N, et al. Spontaneous improvement of hematologic abnormalities in patient having juvenile myelomonocytic leukemia with specific RAS mutations. Blood. 2007;109:5477–80.
Koike K, Matsuda K. Recent advances in the pathogenesis and management of juvenile myelomonocytic leukemia. Br J Harmatol. 2008;5:567–75.
Acknowledgments
We would like to thank Mr. Charles N. Jones who provided scientific writing assistance.
Conflict of interest
We herein declare that there is no financial support or relationships that may pose conflict of interest.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Fukushima, H., Fukushima, T., Hiraki, A. et al. Central nervous system lesions due to juvenile myelomonocytic leukemia progressed in a boy undergoing first line chemotherapy. Int J Hematol 95, 581–584 (2012). https://doi.org/10.1007/s12185-012-1046-5
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12185-012-1046-5