Acute Lymphocytic Leukemia

Aka: Acute Lymphocytic Leukemia, Acute Lymphoblastic Leukemia, ALL

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II. History

  1. Success story of Twentieth Century Oncology
  2. Previously ALL was 100% fatal in 3 months
  3. Current Chemotherapy regimens hold >50% cure

III. Epidemiology

  1. Disease of children and young adults
    1. Onset is at under age 20 years old in more than half of patients
    2. Highest Incidence is between ages 2-3 years
  2. Most common childhood (25% of cancers diagnosed under age 15 years)
    1. Incidence: 35-40 cases per 1 Million
    2. Annual Incidence: 2900 children and adolescents per year in U.S
  3. Ethnicity
    1. Highest Incidence in hispanic and white children
    2. Lowest rate among black children

IV. Risk Factors

  1. Prenatal Radiation Exposure
  2. Genetic Syndromes
    1. Down Syndrome
    2. Neurofibromatosis
    3. Ataxia Telangiectasia
    4. Shwachman syndrome
    5. Bloom syndrome

V. Symptoms

  1. See Acute Leukemia
  2. Fever
  3. Lethargy
  4. Bleeding or Bruising
  5. Musculoskeletal pain
  6. Gastrointestinal symptoms from Hepatosplenomegaly
    1. Nausea
    2. Abdominal fullness
    3. Early Satiety

VI. Signs

  1. See Acute Leukemia
  2. Hepatomegaly or Splenomegaly (50% to 75%)
  3. Lymphadenopathy
  4. Testicular involvement may occur
  5. Anterior Mediastinal Mass (T-Cell Variant)
  6. CNS Involvement in 5-8% of adults with Acute Lymphoblastic Leukemia

VII. Labs: Pathology

  1. Immature Lymphoblasts (blast cells)
    1. Round or convoluted nuclei
    2. Express Common ALL Antigen (CALLA) in 60% of cases
    3. Contrast with Acute Myelogenous Leukemia
      1. Smaller blast cells with ALL
      2. No Auer rods in ALL
      3. Contain deoxynucleotidyl transferase in 90% of cases (rare in AML)
  2. Lymphocyte Cell Types
    1. T-Cell Type (20% of cases)
    2. B-Cell Type (5%)
    3. Null Cell Type (15%)

VIII. Diagnosis

  1. Sources
    1. Peripheral Blood Smear
    2. Bone Marrow Biopsy
  2. Studies to distinguish between AML and ALL
    1. Flow cytometry with immunophenotyping
      1. Sorts and counts cells by cell surface markers
    2. Cytogenetic testing
      1. Chromosome evaluation (karyotype or fluorescence in situ hybridization analysis)

IX. Management: Acute

  1. Remission-Induction Chemotherapy
    1. Vincristine
    2. Prednisone
    3. Daunorubicin or L-asparaginase
  2. CNS Prophylaxis (prevents Leukemic Meningitis)
    1. Whole Brain Radiation (18 to 24-Gy)
    2. Intrathecal Methotrexate
  3. Maintenance Chemotherapy for 2-3 years
    1. 6-Mercaptopurine
    2. Methotrexate

X. Management: Surveillance of survivors treated with Chemotherapy and radiation

  1. Initial surveillance
    1. Year 1
      1. Monthly Physical Exam and Complete Blood Count (CBC)
      2. Every 2 month Liver Function Tests until normal
      3. Bone Marrow Aspirate or Lumbar Puncture as indicated
      4. Echocardiogram as indicated
    2. Year 2
      1. Every 3 month physical exam (with testicular exam) and Complete Blood Count
    3. Year 3 (and after)
      1. Every 6 month physical exam (with testicular exam) and Complete Blood Count
  2. Routine periodic exams
    1. Eye Examination
    2. Dental care
  3. Annual lab testing
    1. Complete Blood Count with differential
      1. Obtain for up to 10 years following last treatment
    2. Comprehensive metabolic panel
      1. Includes serum Electrolytes, Serum Creatinine, Blood Urea Nitrogen, Serum Calcium and Liver Function Tests
    3. Serum Phosphorus
    4. Serum Magnesium
    5. Thyroid Stimulating Hormone
    6. Urinalysis
  4. Hepatitis testing (one time screening)
    1. Hepatitis C Antibody testing (if treated before 1993)
    2. Hepatitis B Surface Antigen (if treated before 1972)
  5. Other tests as indicated
    1. Respiratory symptoms
      1. Chest XRay
      2. Pulmonary Function Testing
    2. Hearing changes
      1. Audiometry
    3. Cardiac symptoms (e.g. CHF)
      1. Repeat testing every 3-5 years if pretreatment cardiac testing was abnormal
      2. Echocardiogram
      3. Electrocardiogram

XI. Management: Surveillance of survivors miscellaneous

  1. Treated with cranial or craniospinal radiation
    1. Neuroimaging is indicated for neurologic symptoms
  2. Treated with Hematopoietic Stem Cell Transplantation
    1. See Hematopoietic Stem Cell Transplant for protocol

XII. Prognosis

  1. Child: Probable cure in >50%
    1. Age under 50 years: Five-year survival 75%
  2. Adult: Long term survival <30%
    1. Age over 50 years: Five-year survival 25%

XIII. Complications

  1. See Acute Leukemia
  2. Leukemic Meningitis in relapse
    1. Headache and Nausea
    2. Cranial Nerve palsy
    3. Seizures
    4. Altered Level of Consciousness
  3. General Chemotherapy complications
    1. Lymphocytopenia
    2. Immunodeficiency (predominantly T-Cells)
      1. Pneumocystis carinii Pneumonia
    3. Growth retardation (most attain normal growth)
    4. Sterility (most resolves)
    5. Joint osteonecrosis (hips, Shoulders and knees)
      1. Cummulative 20 year Incidence 2.8% in adolescents treated for ALL
  4. Cyclophosphamide related
    1. Bladder Cancer
  5. Anthracycline (doxorubicin or daunorubicin) related
    1. Cardiomyopathy
  6. Cranial Radiotherapy related
    1. CNS tumor
    2. Papillary Thyroid Carcinoma
    3. Acute monocytic Leukemia
    4. Cognitive decline
    5. Osteoporosis
    6. Obesity
    7. Periodontal Disease
    8. Cataracts

XIV. Resources

  1. National Cancer Institute
    1. http://www.cancer.gov/cancertopics/pdq/treatment/childALL/HealthProfessional

XV. References

  1. Davis (2014) Am Fam Physician 89(9): 731-8 [PubMed]
  2. Wilbur (2014) Am Fam Physician 91(1):29-36 [PubMed]

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