Alexander 'Sandy' Thomas, is 5 years old, he and his family are well know to you and the practice. Sandy presents today with his father John, who just wants you to have a look at the bruises on his legs. "I'm sure he's fine, you know what he's like always running around tripping over his own feet..but we are just a bit concerned because he seems to be very tired, probably because he has just started school...it takes it out of him a bit"
You note that Sandy is a lot quieter than usual and appears pale.
Sandy is very tried, quieter than usual, and has bruises on his legs and appears pale. A common presentation of leukaemia is a 3-4 week prodrome which may included pallor, increased bruising or bleeding, lethargy anorexia recurrent infections or fevers, anorexia, bone pain or reluctance to walk.
Physical examination in children presenting with ALL (Acute lymphoblastic leukaemia)
80% have pallor,
50% petechiae,
35% lymphadenopathy and
50-60% hepatomegaly or splenomegaly.
The differential diagnosis would include infections (Lymphadenopathy and hepatosplenomegaly would be consistent with infection such as infectious mononucleosis /cytomegalovirus ), juvenile rheumatoid arthritis (if the presentation includes involvement of bones and joints) idiopathic thrombocytic purpura/aplastic anaemia.
Peripheral blood film – usually demonstrate the presence of leukaemic blasts with or without anaemia and thrombocytopenia (and then a bone marrow aspirate would be needed to confirm the diagnosis).
Peripheral blood film ALL usually demonstrates the presence of leukaemic blasts with or without anaemia and thrombocytopenia AML (20% of acute leukaemia/can have similar signs – pallor/bleeding/fever/anorexia/malaise/bone pain).
Abnormal proliferation of lymphoblasts (ALL) or myeloblasts (AML) in the bone marrow- these occupy marrow space, leading to reduced numbers of haematopoietic cells, resulting ultimately in pancytopenia.
Parents often ask: How did this happen to my child?
Did this happen because of something I have done or passed on to my child?
→only a small percentage of paediatric cancers have a clearly hereditary component and despite extensive epidemiological studies few environmental agents have been consistently linked to childhood malignancy. It is thought that cancer initiation results from a series of genetic mutations that result in the inability of a cell to respond normal to signals (intracellular and or extracellular) that control cell proliferation, differentiation or death. In acute lymphoblastic leukaemia a mutant stem cell, capable of indefinite renewal, gives rise to abnormal proliferation of lymphoblasts in the bone marrow.
The Sydney Children's Hospital has 'Ronald McDonald House' which provides accommodation for country families whose children are being treated at Sydney Children's Hospital as inpatients, or outpatients. Cost of accommodation is approximately $30 per night and is claimable through the Isolated Patients Travel Accommodation Assistance Scheme. Many rural areas have local cancer support groups such as Can Assist (the Cancer Council has information on local cancer support groups). Paediatric chemotherapy is usually given in a unit where staff are trained in its use and see the numbers of cases necessary to maintain those skills, although some can be given on an outpatient or day-stay basis. Rural areas need to be able to manage the complications of treatment, such as infections due to a drop in white cell count following chemotherapy.
The first month of therapy is induction (induction aimed at killing leukaemic cells and inducing remission) with 3 – 4 drug combination chemotherapy (vincristine, asparaginase, prednisone, daunorubicin). Remission will be achieved in more than 95% of patients. Further combination therapy is required to prevent relapse (consolidation). Optimal duration of therapy is not known most centres elect to treat for 2 – 3 years. CNS targeted therapy using high-dose intravenous and intrathecal methotrexate has allowed for cranial irradiation to be generally avoided except in patients with overt CNS disease at diagnosis (sparing potential deleterious effects on cognition and growth. Continuation or maintenance therapy, often at lower doses, to kill cancer cells that may re-grow and cause a relapse.
Combination chemotherapy protocols for ALL result in cure of 80%: