II. History
- Success story of Twentieth Century Oncology
- Previously ALL was 100% fatal in 3 months
- Current Chemotherapy regimens hold >50% cure
III. Epidemiology
- Disease of children and young adults
- Onset is at under age 20 years old in more than half of patients
- Highest Incidence is between ages 2-3 years
- Most common childhood (25% of cancers diagnosed under age 15 years)
- Ethnicity
- Highest Incidence in hispanic and white children
- Lowest rate among black children
IV. Risk Factors
- Prenatal Radiation Exposure
-
Genetic Syndromes
- Down Syndrome
- Neurofibromatosis
- Ataxia Telangiectasia
- Shwachman syndrome
- Bloom syndrome
V. Symptoms
- See Acute Leukemia
- Fever
- Lethargy
- Bleeding or Bruising
- Musculoskeletal pain
- Gastrointestinal symptoms from Hepatosplenomegaly
- Nausea
- Abdominal fullness
- Early Satiety
VI. Signs
- See Acute Leukemia
- Hepatomegaly or Splenomegaly (50% to 75%)
- Lymphadenopathy
- Testicular involvement may occur
- Anterior Mediastinal Mass (T-Cell Variant)
- CNS Involvement in 5-8% of adults with Acute Lymphoblastic Leukemia
VII. Labs: Pathology
- Immature Lymphoblasts (blast cells)
- Round or convoluted nuclei
- Express Common ALL Antigen (CALLA) in 60% of cases
- Contrast with Acute Myelogenous Leukemia
- Smaller blast cells with ALL
- No Auer rods in ALL
- Contain deoxynucleotidyl transferase in 90% of cases (rare in AML)
-
Lymphocyte Cell Types
- T-Cell Type (20% of cases)
- B-Cell Type (5%)
- Null Cell Type (15%)
VIII. Diagnosis
- Sources
- Studies to distinguish between AML and ALL
- Flow cytometry with immunophenotyping
- Sorts and counts cells by cell surface markers
- Cytogenetic testing
- Chromosome evaluation (karyotype or fluorescence in situ hybridization analysis)
- Flow cytometry with immunophenotyping
IX. Management: Acute
- Remission-Induction Chemotherapy
- Vincristine
- Prednisone
- Daunorubicin or L-asparaginase
- CNS Prophylaxis (prevents Leukemic Meningitis)
- Whole Brain Radiation (18 to 24-Gy)
- Intrathecal Methotrexate
- Maintenance Chemotherapy for 2-3 years
- 6-Mercaptopurine
- Methotrexate
X. Management: Surveillance of survivors treated with Chemotherapy and radiation
- Initial surveillance
- Year 1
- Monthly Physical Exam and Complete Blood Count (CBC)
- Every 2 month Liver Function Tests until normal
- Bone Marrow Aspirate or Lumbar Puncture as indicated
- Echocardiogram as indicated
- Year 2
- Every 3 month physical exam (with testicular exam) and Complete Blood Count
- Year 3 (and after)
- Every 6 month physical exam (with testicular exam) and Complete Blood Count
- Year 1
- Routine periodic exams
- Eye Examination
- Dental care
- Annual lab testing
- Complete Blood Count with differential
- Obtain for up to 10 years following last treatment
- Comprehensive metabolic panel
- Includes serum Electrolytes, Serum Creatinine, Blood Urea Nitrogen, Serum Calcium and Liver Function Tests
- Serum Phosphorus
- Serum Magnesium
- Thyroid Stimulating Hormone
- Urinalysis
- Complete Blood Count with differential
- Hepatitis testing (one time screening)
- Hepatitis C Antibody testing (if treated before 1993)
- Hepatitis B Surface Antigen (if treated before 1972)
- Other tests as indicated
- Respiratory symptoms
- Hearing changes
- Cardiac symptoms (e.g. CHF)
- Repeat testing every 3-5 years if pretreatment cardiac testing was abnormal
- Echocardiogram
- Electrocardiogram
XI. Management: Surveillance of survivors miscellaneous
- Treated with cranial or craniospinal radiation
- Neuroimaging is indicated for neurologic symptoms
- Treated with Hematopoietic Stem Cell Transplantation
- See Hematopoietic Stem Cell Transplant for protocol
XII. Prognosis
- Child: Probable cure in >50%
- Age under 50 years: Five-year survival 75%
- Adult: Long term survival <30%
- Age over 50 years: Five-year survival 25%
XIII. Complications
- See Acute Leukemia
- Leukemic Meningitis in relapse
-
General Chemotherapy complications
- Lymphocytopenia
- Immunodeficiency (predominantly T-Cells)
- Growth retardation (most attain normal growth)
- Sterility (most resolves)
- Joint osteonecrosis (hips, Shoulders and knees)
- Cummulative 20 year Incidence 2.8% in adolescents treated for ALL
- Cyclophosphamide related
- Anthracycline (doxorubicin or daunorubicin) related
- Cranial Radiotherapy related
- CNS tumor
- Papillary Thyroid Carcinoma
- Acute monocytic Leukemia
- Cognitive decline
- Osteoporosis
- Obesity
- Periodontal Disease
- Cataracts
XIV. Resources
- National Cancer Institute