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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Ontology: Acute lymphocytic leukemia (C0023449)

Definition (MEDLINEPLUS)

Leukemia is cancer of the white blood cells. White blood cells help your body fight infection. Your blood cells form in your bone marrow. In leukemia, however, the bone marrow produces abnormal white blood cells. These cells crowd out the healthy blood cells, making it hard for blood to do its work. In acute lymphocytic leukemia (ALL), also called acute lymphoblastic leukemia, there are too many of specific types of white blood cells called lymphocytes or lymphoblasts. ALL is the most common type of cancer in children.

Possible risk factors for ALL include being male, being white, previous chemotherapy treatment, exposure to radiation, and for adults, being older than 70.

Symptoms of ALL include:

  • Weakness or feeling tired
  • Fever
  • Easy bruising or bleeding
  • Bleeding under the skin
  • Shortness of breath
  • Weight loss or loss of appetite
  • Pain in the bones or stomach
  • Pain or a feeling of fullness below the ribs
  • Painless lumps in the neck, underarm, stomach, or groin

Tests that examine the blood and bone marrow diagnose ALL. Treatments include chemotherapy, radiation therapy, stem cell transplants, and targeted therapy. Targeted therapy uses substances that attack cancer cells without harming normal cells. Once the leukemia is in remission, you need additional treatment to make sure that it does not come back.

NIH: National Cancer Institute
Definition (NCI) Leukemia with an acute onset, characterized by the presence of lymphoblasts in the bone marrow and the peripheral blood. It includes the acute B lymphoblastic leukemia and acute T lymphoblastic leukemia.
Definition (NCI_CDISC) A progressive, proliferative disease of blood cells, originating from immature lymphoid cells.
Definition (NCI_NCI-GLOSS) An aggressive (fast-growing) type of leukemia (blood cancer) in which too many lymphoblasts (immature white blood cells) are found in the blood and bone marrow.
Definition (CSP) acute leukemia in which lymphoblasts and their progenitor cells predominate; the most common childhood cancer and accounts for 20 percent of adult acute leukemia; common ALL antigen (CALLA) expressed in most cases.
Concepts Neoplastic Process (T191)
ICD9 204.0
ICD10 C91.0 , C91.00, M9821/3
SnomedCT 91857003, 90151006, 154588002, 190034000, 128822004
English Lymphoid leukemia, acute, ALL - Acute lymphobl leukaemia, ALL - Acute lymphobl leukemia, LEUKEMIA ACUTE LYMPHOBLASTIC, [M]Acute lymphoid leukaemia, [M]Acute lymphoid leukemia, Acute lymphocytic leukemia, acute lymphatic leukemia, acute lymphogenous leukemia, ALL (acute lymphocytic leukemia), acute lymphocytic leukemia (diagnosis), acute lymphocytic leukemia, Acute lymphoid leukaemia, disease, Acute lymphoid leukemia, disease, Acute lymphoblastic leukaemia -RETIRED-, Lymphoid leukaemia, acute, Leukemia lymphoblastic acute, Acute Lymphoblastic Leukemia, Acute Lymphogenous Leukemia, Precursor Lymphoblastic Leukemia, Acute Lymphocytic Leukemias, acute lymphocytic leukemia (ALL), Acute lymphoblastic leukemia [ALL], Acute lymphoblastic leukaemia NOS, Acute lymphoblastic leukemia NOS, Leukaemia;acute lymphocytic, acute lymphatic leukaemia, leukemia lymphoblastic, leukemia acute lymphoblastic, acute lymphoblastic leukaemia, acute lymphoblastic leukemias, leukemia - acute lymphoblastic, lymphocytic leukemia acute, acute leukaemia lymphoblastic, acute leukemia lymphocytic, acute leukemia lymphoid, acute leukemia lymphoblastic, acute lymphoid leukemia, lymphoblastic leukaemia, lymphoblastic leukemia, Leukaemias acute lymphocytic, Leukemia, Acute Lymphocytic, Acute Lymphocytic Leukemia, [M]Acute lymphoid leukaemia (disorder), Acute lymphoblastic leukemia (morphologic abnormality), [M]Acute lymphoid leukemia (morphologic abnormality), ACUTE LYMPHOCYTIC LEUKAEMIA, LEUKEMIA, LYMPHOBLASTIC, MALIGNANT, PRECURSOR LYMPHOBLASIC LEUKEMIA, ACUTE LYMPHOGENOUS LEUKEMIA, PRECURSOR CELL LYMPHOBLASTIC LEUKEMIA, Acute lymphoblastic leukemia, NOS, LYMPHOBLASTIC LEUKEMIA, ALL, ACUTE LYMPHOCYTIC LEUKEMIAS, acute lymphoblastic leukemia, ACUTE LYMPHOID LEUKEMIA, ALL - ACUTE LYMPHOCYTIC LEUKEMIA, Leukaemia lymphoblastic acute, Acute lymphoid leukemia, Acute lymphatic leukemia, Lymphoblastic leukemia, ALL - Acute lymphoblastic leukaemia, ALL - Acute lymphoblastic leukemia, Acute lymphoblastic leukaemia, Acute lymphoblastic leukemia, Acute lymphoid leukaemia, Acute lymphatic leukaemia, Acute lymphocytic leukaemia, Acute lymphoblastic leukaemia-lymphoma, Acute lymphoblastic leukemia-lymphoma, Acute lymphoid leukemia, disease (disorder), Lymphoblastic leukaemia, leukemia, acute lymphocytic, leukemia; lymphatic, acute, lymphatic; leukemia, acute, Lymphoblastic leukemia, NOS, Acute lymphoblastic leukemia -RETIRED-, Lymphoblastic Leukemia, Acute Lymphocytic Leukaemia, Acute Lymphoid Leukemia, Precursor Cell Lymphoblastic Leukemia, ALL - Acute Lymphocytic Leukemia, Leukemias acute lymphocytic, Leukemia;acute lymphocytic, acute lymphocytic leukaemia
Spanish leucemia linfoide aguda, enfermedad, Leucemia linfática aguda, Leucemia linfoide aguda, Leucemia linfoblástica aguda, [M] leucemia linfoide aguda (anomalía morfológica), [M] leucemia linfoide aguda, leucemia linfoide aguda, enfermedad (trastorno), leucemia linfoide aguda, Leucemia aguda linfocítica, Leucemia linfocítica aguda
Italian Leucemia linfocitica acuta, Leucemie linfocitiche acute, Leucemia linfoblastica acuta, Leucemia linfatica acuta, ALL, Leucemia linfoide, acuta, Leucemia linfoide acuta
Dutch lymfoïde leukemie, acuut, acute lymfatische leukemie, acute lymfoblastische leukemie, acute lymfoïde leukemie, lymfoblastische leukemie acuut, ALL, leukemie; lymfatisch, acuut, lymfatisch; leukemie, acuut, Acute lymfoblastenleukemie, acute lymfocytaire leukemie, lymfocytische leukemieën acuut
French Leucémie lymphatique aiguë, Leucémie lymphoblastique aiguë, Leucémie lymphoïde aiguë, LLA, Leucémie lymphoïde, aiguë, Leucémie lymphocytaire aiguë, Leucémie aiguë lymphoblastique, Leucémies lymphocytaires aiguës
German akute lymphoblastische Leukaemie, akute Lymphoblastenleukaemie, ALL, akute lymphoide Leukaemie, lymphoide Leukaemie, akut, akute lymphablastische Leukaemie, Leukaemie lymphoblastisch akut, Akute lymphoblastische Leukaemie, Leukaemien akut lymphozytaer, akute lymphatische Leukaemie
Portuguese Leucemia linfóide aguda, Leucemia linfática aguda, Leucemia linfoblástica aguda, Leucemia linfocítica aguda, Leucemias linfocítica aguda
Japanese 急性リンパ球性白血病, 急性リンパ性白血病, 急性リンパ芽球性白血病, ＡＬＬ, ALL, ｷｭｳｾｲﾘﾝﾊﾟｷｭｳｾｲﾊｯｹﾂﾋﾞｮｳ, ｷｭｳｾｲﾘﾝﾊﾟｾｲﾊｯｹﾂﾋﾞｮｳ, ｷｭｳｾｲﾘﾝﾊﾟｶﾞｷｭｳｾｲﾊｯｹﾂﾋﾞｮｳ
Czech Akutní lymfatická leukemie, Akutní lymfoblastické leukemie, Akutní lymfoidní leukemie, Akutní lymfocytická leukemie, Akutní lymfoblastická leukemie, ALL
Korean 급성 림프모구성 백혈병
Hungarian Heveny lymphoblastos leukemia, Heveny lymphoid fehérvérűség, Acut lymphatikus leukaemia, Acut lymphatikus leukemia, Acut lymphoblastos leukemia, ALL, Acut lymphoblastos leukaemia, Lymphoid fehérvérűség, heveny, Acut lymphocytás leukemia, Leukemiák, acut lymphocytás, Fehérvérűség limfoblasztos heveny, Lymphoid fehérvérűség, acut, Acut lymphocytás leukaemia, Acut lymphoid leukaemia
Derived from the NIH UMLS (Unified Medical Language System)

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