Successful Chemotherapy with A-Triple-V Regimen in Juvenile Myelomonocytic Leukemia

Bae, Won Jin; Keon, Eun Jee; Park, Ji Kyoung; Lee, Soon Yong

Abstract

PURPOSE: Juvenile myelomonocytic leukemia (JMML) is a rare, fatal hematopoietic malignancy in childhood, characterized by hepatosplenomegaly, monocytosis, leukocytosis and the presence of immature myeloid precursors in the peripheral blood with absence of the Philadelphia chromosome. We evaluated the treatment outcome of the intensive chemotherapy (A-Tiple-V regimen) for the JMML patients.
METHODS
Five patients with JMML (3 males and 2 females with the age range of 15 months~6 years) received 4~36 cylces of the A-Triple-V regimen (Ara-C, VP-16, Vincristine, and Vinblastine). Response to therapy was defined as the disappearance of the immature myeloid cells in the peripheral blood with normalization of WBC and platelet counts, and reduction of hepatosplenomegaly.
RESULTS
Of the patients, 3 cases received 24~36 cycles of the chemotherapy until normalizaton of the clinical pictures, including especially reduction of splenomegaly, and they have been alive in the disease-free state with the duration of 26, 78 and 156 months, respectively. Another one case received bone marrow transplantation after 18 cycles of the chemotherapy because of persistent splenomegaly, and since then, she has been alive in the disease-free state with the duration of 77 months. The remaining 1 case developed blastic crisis after 4 cycles of the chemotherapy and thereafter we lost the follow-up.
CONCLUSION
We conclude that A-triple-V regimen is a novel chemotherapy for the treatment of JMML patients.