Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ Key Features +++ Essentials of Diagnosis ++ Bruising, pallor, petechiae, and purpura Hepatomegaly Splenomegaly Lymphadenopathy +++ General Considerations ++ 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 +++ Clinical Findings +++ Symptoms and Signs ++ 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 +++ Differential Diagnosis ++ Epstein-Barr virus Cytomegalovirus Immune thrombocytopenic purpura Transient erythroblastopenia of childhood Autoimmune hemolytic anemias Aplastic anemia Juvenile rheumatoid arthritis +++ Diagnosis +++ Laboratory Findings ++ 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 +++ Imaging ++ 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 +++ Treatment +++ Specific Therapy ++ 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 ... Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth