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A 3-year-old girl has had fever, anorexia, and fatigue for the past month. She has lost 5 kg. She has pallor, cervical adenopathy, splenomegaly, skin ecchymoses, and petechiae. Think: Acute leukemia.
The typical presentation is pancytopenia—anemia (pallor and fatigue) and thrombocytopenia (epistaxis, ecchymoses, and petechiae); white cells may be low or high. The initial presentation may be nonspecific and subtle and develop over weeks to months. The final diagnosis depends on the results of bone marrow aspirate and biopsy.
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Leukemia is the most common childhood malignancy (brain tumors are number two).
Leukemia represents 30% of all childhood malignancies.
Acute lymphocytic leukemia (ALL) is five times more common than acute myelogenous leukemia (AML).
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Trisomy 21.
Fanconi anemia and other inherited marrow failure syndromes.
Bloom syndrome.
Immune deficiency syndromes, including ataxia-telangiectasia
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Fever.
Pallor.
Bleeding.
Bone pain.
Lymphadenopathy.
Hepatosplenomegaly.
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Chemotherapy specific to type of leukemia.
Infection prevention: empiric antibiotics for febrile neutropenia and isolation, if necessary.
Monitoring for occurrence of tumor lysis, hydration, allopurinol, or rasburicase.
Transfusions, as needed.
Hyperleukocytosis: leukapheresis and hydroxyurea.
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WARD TIP
Between 70 and 80% of childhood cases of ALL are of the precursor B cell lineage.
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Acute Lymphoblastic Leukemia (ALL)
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Fatigue, anorexia, lethargy, pallor.
Bone pain.
Fever.
Bleeding, bruising, petechiae.
Lymphadenopathy.
Hepatosplenomegaly.
Testicular swelling.
Septicemia.
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WARD TIP
A marrow exam is essential to confirm the diagnosis of ALL.
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CBC: anemia, abnormal white count, low platelet count.
Abnormal electrolytes: low calcium, high phosphorus, high uric acid, high LDH.
Chest x-ray (mediastinal mass).
Bone marrow: hypercellular, increased lymphoblasts.
Cerebrospinal fluid (CSF): blasts.
Cytogenetics and flow cytometry for risk stratification and classification.
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Remission induction: vincristine, prednisone, asparaginase, doxorubicin.
Consolidation and delayed intensification: 6-mercaptopurine (6MP), etoposide, cytarabine, cyclophosphamide, high dose methotrexate.
Maintenance therapy: 2 years—methotrexate, 6MP, vincristine, and prednisone.
CNS prophylaxis/treatment: intrathecal methotrexate, possibly cytarabine and/or irradiation.
Targeted therapy in individual cases.
Allogeneic stem cell transplant in high-risk/refractory cases.
Chimeric antigen receptor or CAR T-cell therapy in high-risk/refractory cases.
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WARD TIP
Tumor lysis syndrome—rapid leukemic cell lysis at presentation or after initiating chemotherapy
Acute renal failure
Hyperphosphatemia
Hyperuricemia
Hyperkalemia
Hypocalcemia
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Acute Myelogenous Leukemia (AML)
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Fifteen to twenty percent of leukemia cases.
Predisposing factors, including toxins (benzene) and medications (etoposide, cyclophosphamide).
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Manifestations of anemia, thrombocytopenia, or neutropenia, including fatigue, bleeding, ...