Acute Lymphoblastic Leukemia
Leukemias The leukemias are the most common malignant neoplasms in childhood, accounting for approximately 31% of all malignancies that occur in children younger than 15 yr of age.
Leukemias • group of malignant diseases in which genetic abnormalities in a hematopoietic cell give rise to an unregulated clonal proliferation of cells.
ALL
AML
Leukemia
JMML
CML
ALL ALL has a striking peak incidence at 2-3 yr of age and occurs more in boys than in girls at all ages.
ALL
85% Blymphoblastic
15% TLymphoblastic
1% Mature B cells
Signs and Symptoms • • • •
Anorexia Fatigue Malaise Irritability
Signs and Symptoms • low-grade fever • Bone or joint pain
Signs and Symptoms • Pallor • Listlessness • Purpuric and petechial skin lesions or mucous membrane
Diagnosis 1. Blood Tests
Usually shows ANEMIA and THROMBOCYTOPENIA
Diagnosis 2. Bone Marrow Aspiration ALL is diagnosed by a bone marrow evaluation that demonstrates >25% of the bone marrow cells as a homogeneous population of lymphoblasts
Diagnosis 3. Flow cytometry 4. Cytogenetics 5. Molecular studies.
Treatment • The single most important prognostic factor in ALL is the treatment: “without effective therapy, the disease is fatal”
Treatment • Risk-directed therapy has become the standard of current ALL treatment and takes into account age at diagnosis, initial white blood cell count, immunophenotypic and cytogenetic characteristics of blast populations and rapidity of early treatment response and
PHASES REMISSION INDUCTION
CONSOLIDATION
MAINTENANCE
RELAPSE The major impediment to a successful outcome is relapse of the disease. • Relapse occurs in the bone marrow in 15-20% of patients with ALL Intensive chemotherapy with agents not previously used in the patient followed by allogeneic stem cell transplantation can result in long-term survival for some patients with bone marrow relapse
PROGNOSIS • 5 yr survival around 90%