II. Types

  1. Acute Myelogenous Leukemia
    1. Most cases occur in adults
  2. Acute Lymphocytic Leukemia (or Acute Lymphoblastic Leukemia)
    1. Most cases occur in children

III. Findings: Signs and Symptoms (within 3 months of presentation)

  1. Anemia symptoms and signs
    1. Fatigue
    2. Dyspnea on exertion at even mild intensity
    3. Pallor
  2. Bleeding symptoms and signs
    1. Gingival Bleeding
    2. Gastrointestinal Bleeding
    3. Petechiae
    4. Easy bruisability
  3. Hematologic findings other than bleeding (rare in adults)
    1. Lymphadenopathy
    2. Splenomegaly
    3. Hepatomegaly
  4. Infectious symptoms and signs
    1. Fever
    2. Cellulitis
    3. Gingiva infection
    4. Candida infection
    5. Pneumonia
    6. Perirectal Abscess
    7. Urinary Tract Infection
  5. Chloroma development in any location
    1. Soft Tissue Masses of leukemic cells
  6. Expanding cell mass symptoms (50%)
    1. Bone pain (spine and long bones) is present in 43% of children
      1. Clarke (2016) Arch Dis Child 101(10):894-901 [PubMed]
    2. Sternal tenderness
  7. Renal related symptoms and signs
    1. Nephrolithiasis (Uric Acid stones)
    2. Urate nephropathy

IV. Labs

  1. Complete Blood Count
    1. White Blood Cell Count
      1. Normal Leukocyte count or marked Leukocytosis
    2. Pancytopenia
  2. Peripheral Smear
    1. Circulating blast cells may not be seen
  3. Chemistry panel (Chem 7)
    1. Hyponatremia
    2. Hypokalemia
    3. Hyperuricemia
  4. Liver Function Tests
    1. Lactate Dehydrogenase (LDH) increased

V. Imaging

  1. Bone XRay
    1. Osteopenia
    2. Metaphyseal bands
    3. Lytic lesions
    4. Sclerotic changes
    5. Bone destruction

VI. Complications

  1. Leukostasis
    1. Occurs when circulating blasts > 100 x10^9/L
    2. Examples
      1. Lung hypoperfusion
      2. Brain hypoperfusion
  2. Thrombocytopenia related bleeding complications
    1. Occurs when Platelet Count < 20 x10^9/L
    2. Examples
      1. Gingival Bleeding
      2. Gastrointestinal Bleeding
  3. Neutropenia related infections
    1. Occurs when Neutrophil Count < 0.5 x10^9/L
    2. Examples
      1. Gram NegativeSepsis
      2. Candida infection

VII. Management: Remission

  1. Goal
    1. Successive chemotherapeutic courses
      1. Each course results in fractional cell kill
    2. Results in up to 99.999 elimination of Leukemia cells
      1. Leukemia not detectable below 10^9 cells
    3. Some agents also Induce maturation of Leukemia cells
      1. Results in non-proliferating mature cells
  2. Criteria for Remission
    1. Less then 5 percent blast cells in Bone Marrow
    2. Leukemic cells absent in Bone Marrow
    3. Normal peripheral blood cell counts Restored
    4. Signs of extramedullary Leukemia involvement absent
  3. Overall Management Strategy
    1. Step 1: Remission Induction Chemotherapy
      1. Intensive systemic Chemotherapy
      2. Drives leukemic cell mass below detectable level
    2. Step 2: Early Intensification (Consolidation)
      1. Intensive Chemotherapy after Remission Induction
      2. Further reduce and possibly eradicate Leukemia
    3. Step 3: Maintenance
      1. Lower dose Chemotherapy continued over years
    4. Step 4: Late Intensification
      1. Intensive Chemotherapy restarted after 6 months
    5. Step 5: Local elimination of common relapse sites
      1. Local Chemotherapy or radiation to sanctuary sites

VIII. Management: Supportive care of Pancytopenia

  1. Anemia
    1. Treat with packed Red Blood Cell Transfusion
  2. Thrombocytopenia
    1. Keep Platelet Count above 20 x 10^9/L
    2. Platelet Transfusion effective initially
    3. Antiplatelet Antibody develops over time
      1. Shortens Platelet survival
      2. Render patient unresponsive to transfusion
    4. May respond to HLA Identical Platelets
  3. Granulocytopenia (Neutropenia)
    1. Granulocyte transfusions not recommended
    2. Prevent infection
      1. Reverse isolation rooms
      2. Face Masks
      3. Careful Hand Washing
    3. Observation for Bacterial and fungal infection

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Related Studies

Ontology: Acute leukemia (C0085669)

Definition (NCI_NCI-GLOSS) A rapidly progressing cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of white blood cells to be produced and enter the blood stream.
Definition (NCI) A clonal (malignant) hematopoietic disorder affecting the bone marrow and the peripheral blood. The malignant cells show minimal differentiation and are called blasts, either myeloid blasts (myeloblasts) or lymphoid blasts (lymphoblasts). According to the type of blasts present, acute leukemias are classified as acute myeloid leukemia (AML) and precursor lymphoblastic or acute lymphoblastic leukemia (ALL).
Definition (CSP) leukemia in which the involved cell shows little or no differentiation, usually consisting of blast cells; two types are distinguished, acute lymphocytic and acute myelogenous leukemia.
Concepts Neoplastic Process (T191)
ICD9 208.0
ICD10 M9801/3 , C95.0, C95.00
SnomedCT 91855006, 24072005, 154599000, 188763007
English Leukemia of unspecified cell type, acute, LEUKAEMIA ACUTE, LEUKEMIA ACUTE, Acute leukaemia NOS, Acute leukemia NOS, Acute leukaemia of unspecified cell type, Acute leukemia of unspecified cell type, Acute leukemias, [M]Acute leukaemia NOS, [M]Acute leukemia NOS, acute leukemia, leukemia acute, acute leukemia (diagnosis), Acute leukaemia, disease, Acute leukemia, disease, Acute leukemia, morphology, including blast cell OR undifferentiated leukemia, Acute leukaemia, morphology, including blast cell OR undifferentiated leukaemia, Leukaemia of unspecified cell type, acute, Leukaemia acute, Leukemia acute, acute leukaemia, acute leukaemias, acute leukemias, Acute leukaemia NOS (disorder), Acute leukemia NOS (disorder), Acute leukaemia, Acute leukemia, disease (disorder), Acute leukemia, morphology, including blast cell OR undifferentiated leukemia (morphologic abnormality), leukemia, acute, leukemia; acute, acute; leukemia, Acute leukemia, NOS, Acute leukemia, Acute Leukemia
Spanish leucemia aguda, enfermedad, Leucemia de tipo celular no especificado, aguda, Leucemia aguda de tipo de célula no especificado, Leucemia aguda de tipo celular no especificado, Leucemia aguda NEOM, LEUCEMIA AGUDA, leucemia aguda, SAI, leucemia aguda, SAI (trastorno), Acute leukaemia NOS, Acute leukemia NOS, leucemia aguda (anomalía morfológica), leucemia aguda, enfermedad (trastorno), leucemia aguda, Leucemia aguda
Italian Leucemia acuta, Leucemia di tipo cellulare non specificato, acuta, Leucemia acuta di tipo cellulare non specificato, Leucemia acuta, NAS
Dutch leukemie acuut, acute leukemie van niet-gespecificeerd celtype, acute leukemie NAO, acute leukemie niet-gespecificeerd celtype, leukemie ongekend celtype, acuut, acuut; leukemie, leukemie; acuut, Acute leukemie van niet gespecificeerd celtype, acute leukemie
French Leucémie à cellules de type non précisé, aiguë, Leucémie aiguë de type cellulaire non précisé, Leucémie aiguë à cellules de type non précisé, Leucémie aiguë SAI, LEUCEMIE AIGUE, Leucémie aiguë
German Leukaemie akut, akute Leukaemie NNB, Leukaemie eines unspezifischen Zelltyps, akut, akute Leukaemie eines unspezifischen Zelltyps, Akute Leukaemie nicht naeher bezeichneten Zelltyps, LEUKAEMIE AKUT, akute Leukaemie
Portuguese Leucemia aguda NE, Leucemia aguda de tipo de célula não especificado, Leucemia de tipo celular NE aguda, LEUCEMIA AGUDA, Leucemia aguda
Japanese 急性白血病, 急性白血病NOS, 急性白血病、細胞タイプ不明, キュウセイハッケツビョウ, キュウセイハッケツビョウNOS, キュウセイハッケツビョウサイボウタイプフメイ
Czech Akutní leukemie vycházející z blíže neurčeného buněčného typu, Akutní leukemie blíže neurčeného buněčného typu, Akutní leukemie, Akutní leukemie NOS
Korean 상세불명 세포형의 급성 백혈병
Hungarian Nem meghatározott sejttípusú acut leukemia, Nem meghatározott sejttípusú fehérvérűség, heveny, Meghatározatlan sejttípusú acut leukaemia, Fehérvérűség heveny, Acut leukemia k.m.n., Acut leukaemia, Heveny fehérvérűség, Nem meghatározott sejttípusú acut leukaemia, Acut leukaemia k.m.n.