RESULTS OF INTENSIVE CHEMOTHERAPY IN CHILDREN WITH JUVENILE CHRONIC MYELOMONOCYTIC LEUKEMIA - A PILOT-STUDY

Background. The use of chemotherapy in juvenile chronic myelomonocytic leukemia (J-CMML) has not generally been successful. Our previous exp erience in 11 patients demonstrated that chemotherapy with low doses o f daunorubicin or cytarabine resulted in a 90% fatal outcome and a med ian survival rate of only 7 months. Procedure and Results. Between 198 5 and 1997, six children (five boys and one girl) aged 3-67 months (me dian age: 20.5) were diagnosed with J-CMML and underwent intensive com bination chemotherapy similar to that used for acute myeloblastic leuk emia. Two patients with high-risk factors developed progressive diseas e with fatal outcome at 5 and 18 months postdiagnosis, respectively. H owever, four patients without poor prognosis indices responded and wer e 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). Conc lusions. The use of intensive combination chemotherapy in children wit h J-CMML can result in long-term survival in some patients. (C) 1998 W iley-Liss, Inc.