BACKGROUND: The use of chemotherapy in juvenile chronic myelomonocytic leukemia (J-CMML) has not generally been successful. Our previous experience in 11 patients demonstrated that chemotherapy with low doses of daunorubicin or cytarabine resulted in a 90% fatal outcome and a median survival rate of only 7 months. PROCEDURE AND RESULTS: Between 1985 and 1997, six children (five boys and one girl) aged 3-67 months (median age: 20.5) were diagnosed with J-CMML and underwent intensive combination chemotherapy similar to that used for acute myeloblastic leukemia. Two patients with high-risk factors developed progressive disease with fatal outcome at 5 and 18 months postdiagnosis, respectively. However, four patients without poor prognosis indices responded and were alive 145, 82, 51, and 6 months after diagnosis. Overall survival in this small series at 7 years from diagnosis is 60% (CI: 17-100). CONCLUSIONS: The use of intensive combination chemotherapy in children with J-CMML can result in long-term survival in some patients.
BACKGROUND: The use of chemotherapy in juvenile chronic myelomonocytic leukemia (J-CMML) has not generally been successful. Our previous experience in 11 patients demonstrated that chemotherapy with low doses of daunorubicin or cytarabine resulted in a 90% fatal outcome and a median survival rate of only 7 months. PROCEDURE AND RESULTS: Between 1985 and 1997, six children (five boys and one girl) aged 3-67 months (median age: 20.5) were diagnosed with J-CMML and underwent intensive combination chemotherapy similar to that used for acute myeloblastic leukemia. Two patients with high-risk factors developed progressive disease with fatal outcome at 5 and 18 months postdiagnosis, respectively. However, four patients without poor prognosis indices responded and were alive 145, 82, 51, and 6 months after diagnosis. Overall survival in this small series at 7 years from diagnosis is 60% (CI: 17-100). CONCLUSIONS: The use of intensive combination chemotherapy in children with J-CMML can result in long-term survival in some patients.
Authors: Anna Hecht; Julia Meyer; Farid F Chehab; Kristie L White; Kevin Magruder; Christopher C Dvorak; Mignon L Loh; Elliot Stieglitz Journal: Pediatr Blood Cancer Date: 2019-07-26 Impact factor: 3.167