Childhood monosomy 7 revisited

Abstract

Monosomy 7 is found in acute myeloid leukaemia (AML) and myelodysplasia and is characteristic of a rare chronic myeloproliferative disease (MPD) of young children. We have seen 16 children with monosomy 7. Their clinical features and response to treatment are discussed. Monosomy 7 diseases appear to have a particularly poor prognosis. The AML is often resistant to treatment and relapse is common. Children with chronic MPD die of bone marrow failure or evolve to AML or myelofibrosis. We have treated these children intensively with combination chemotherapy and allogeneic bone marrow transplantation. Four children with MPD received supportive care and low dose chemotherapy alone. They all died, surviving between 4 months and 4 years. Six children with MPD received intensive chemotherapy:three remitted, one relapsing after 9 months, the others remaining in remission at 18 months and 3 years. One child with MPD has undergone successful BMT and survives 71/2 years after presentation. Remission was achieved in three of four cases of AML. They all relapsed within 9 months. Bone marrow transplantation was successful in one child with myelofibrosis. Intensive chemotherapy and early bone marrow transplantation is likely to offer these children their best chance of survival.