Certain cytogenetic abnormalities are typically associated with these disorders and may help to predict response to treatment and outcome. The diagnosis may not be clear cut at initial presentation and an observation period may be necessary prior to any form of therapy. The treatment of these disorders has changed over the last decade. This is a rare condition that affects children. It has a higher incidence in association with certain other clinical conditions like neurofibromatosis.
Children often present with low platelet count and recurrent infections.
The spleen is often enlarged and causes marked abdominal distension. This type of leukaemia is usually resistant to standard chemotherapy and the only curative treatment is bone marrow transplantation from a family member or, in its absence, from an unrelated donor.