| Autor: |
Maschan AA; Federal Research Center for Pediatric Hematology, Oncology, and Immunology, Moscow, Russian Federation. amaschan@mail.ru, Khachatrian LA, Solopova GG, Ossipova EY, Baidun LV, Dmitrieva SV, Maschan MA, Resnik IB |
| Jazyk: |
angličtina |
| Zdroj: |
Journal of pediatric hematology/oncology [J Pediatr Hematol Oncol] 2011 Jan; Vol. 33 (1), pp. e32-4. |
| DOI: |
10.1097/MPH.0b013e3181f46e3e |
| Abstrakt: |
Juvenile myelomonocytic leukemia (JMML) occurs with an incidence of 1.2 per million children a year, and represents 18% to 30% of all myelodysplastic (MDS) and myeloproliferative (MPS) disorders in the age group below 15, being by far the most common MDS/MPS in children younger than 4 years. The only therapeutic approach which results in a definitive cure of patients with JMML is myeloablative chemo-therapy/radio-therapy, followed by allogeneic hematopoietic cell transplantation. Few cases of transformation of JMML in acute lymphoblastic leukemia have been reported. We describe a child with JMML diagnosed at the age of 4 months in whom complete remission was achieved with 13-cis retinoic acid and cytosine-arabinoside and was sustained for 7 years with no maintenance therapy. Ninety-eight months after the diagnosis of JMML was established, overt T-cell leukemia developed. Treatment with acute lymphoblastic leukemia (ALL)-directed chemotherapy induced complete restoration of normal hemopoiesis, but testicular involvement persisted. The patient died after transplantation with unrelated cord blood. This case suggests that JMML is a true stem cell disorder and that stem cell transplantation should be considered, even in patients with a very favorable clinical course. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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