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Key Features

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Essentials of Diagnosis
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  • Bruising, pallor, petechiae, and purpura

  • Hepatomegaly

  • Splenomegaly

  • Lymphadenopathy

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General Considerations
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  • Defined by the presence of > 25% malignant hematopoietic cells (blasts) in the bone marrow aspirate

  • Accounts for about 25% of all cancer diagnoses in patients younger than 15 years

  • Worldwide incidence is about 1:25,000 children per year, including 3000 children per year in the United States

  • Peak age at onset is 4 years; 85% of patients are diagnosed between ages 2 and 10 years

  • Children with Down syndrome have a 14-fold increase in the overall rate of leukemia

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Clinical Findings

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Symptoms and Signs
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  • Pallor, petechiae, and purpura

  • Intermittent fevers are common

  • Bone pain, especially in the pelvis, vertebral bodies, and legs

  • Hepatomegaly and splenomegaly

  • Lymphadenopathy is common, either localized or generalized to cervical, axillary, and inguinal regions

  • Testes may occasionally be unilaterally or bilaterally enlarged secondary to leukemic infiltration

  • Superior vena cava syndrome is caused by mediastinal adenopathy compressing the superior vena cava

  • Neck may feel full from venous engorgement

  • Face may appear plethoric, and the periorbital area may be edematous

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Differential Diagnosis
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  • Epstein-Barr virus

  • Cytomegalovirus

  • Immune thrombocytopenic purpura

  • Transient erythroblastopenia of childhood

  • Autoimmune hemolytic anemias

  • Aplastic anemia

  • Juvenile rheumatoid arthritis

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Diagnosis

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Laboratory Findings
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  • Complete blood count (CBC) with differential is the most useful initial test

  • WBC count

    • Low or normal (= 10,000/μL) in 50% of patients, but the differential shows neutropenia (absolute neutrophil count < 1000/μL) along with a small percentage of blasts amid normal lymphocytes

    • Between 10,000/μL and 50,000/μL in 30%

    • Over 50,000/μL, occasionally higher than 300,000/μL in 20%

  • Peripheral blood smears also show abnormalities in RBCs

  • Most patients have decreased platelet counts (< 150,000/μL) and decreased hemoglobin (< 11 g/dL) at diagnosis

  • Serum chemistries, particularly uric acid and lactate dehydrogenase, are often elevated as a result of cell breakdown

  • Bone marrow examination confirm diagnosis

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Imaging
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  • Chest radiograph may show mediastinal widening or an anterior mediastinal mass and tracheal compression secondary to lymphadenopathy or thymic infiltration, especially in T-cell ALL

  • Plain radiographs of the long bones and spine may show demineralization, periosteal elevation, growth arrest lines, or compression of vertebral bodies

  • Abdominal ultrasound may show kidney enlargement from leukemic infiltration or uric acid nephropathy as well as intra-abdominal adenopathy

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Treatment

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Specific Therapy
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  • Medications used most commonly in induction

    • Oral prednisone or dexamethasone

    • Intravenous vincristine

    • Daunorubicin

    • Intramuscular or intravenous asparaginase

    • Intrathecal methotrexate

  • Consolidation is the second phase of treatment, during which intrathecal chemotherapy along with continued systemic therapy and sometimes cranial radiation therapy are given to kill lymphoblasts "hiding" in the meninges

  • Several months of intensive chemotherapy follows consolidation, often referred to as intensification

  • Maintenance therapy can include

    • Daily oral mercaptopurine

    • Weekly oral methotrexate

    • Monthly pulses of intravenous vincristine and oral prednisone or dexamethasone

  • Intrathecal chemotherapy, either with methotrexate alone ...

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