Acute Leukemia Flashcards
What is leukemia?
Clonal neoplastic disease of the hematopoietic system
What is acute lymphoblastic leukemia (ALL)?
Most common malignancy of childhood between 0-15 years old
Rapid onset,rapid progressing
Unknown etiology
Uncommon in adults
What are the clinical features of ALL?
Fatigue/ weakness Fever Lymphadenopathy Splenomegaly Bone pain Bleeding Mediastinal mass in adolescent but more common with T-ALL
What are the lab features of ALL?
Blast forms
Bone marrow involvement
Anemia
Leukopenia
What are the abnormalities associated with poor outcome in ALL?
Translocation t(9:22)- Philadelphia chromosome ( mostly in older children)
What are the abnormalities associated with favorable prognosis in ALL?
Translocation t(12:21) ETV6/RUNX1
What are some adverse prognostic features at diagnosis of ALL?
Age>30 WBC>30,000 T cell variant CNS involvement No complete remission in 30 days
How is ALL treated?
Chemotherapy consisting of 3 phases
Induction:4-6 weeks
Consolidation/ intesification: 6-9 months
Maintenance :2-3 years
CNS therapy ( methotrexate)for all children even if there is no CNS involvement
What is the drugs used for induction and consolidation ?
Vincristine
Dexamethasone or prednisone
Imatinib(Gleevec) included for Philadelphia chromosome
Higher doses are used during consolidation and not induction to avoid tumor lysis syndrome
What are the drugs used for maintenance therapy?
Methotrexate and 6- Mercaptopurine
What are the characteristics of tumor lysis syndrome ?
Hyperphosphatemia Hypocalcemia Hyperuricemia Hyperkalemia Increased creatinine Increased LDH
What are the adverse effects of treatment of ALL?
Thrombosis
Bleeding
Infection
Hypothalamic pituitary adrenal axis suppression
What is the difference in outcomes between childhood and adult ALL?
Both adults and children achieve high initial remission rates
Most children are cured while most adults relapse
What is acute myelogenous leukemia (AML)?
Illness of myeloid or monocytic derivation
Bone marrow replacement by blast cells
Most common leukemia after 15 years old
Increases with age (65 or older)
Associated with radiation exposure, chemotherapy, genetic disease (Downs, Fanconi)
What are the 2 types of AML?
De novo- newly diagnosed patient with no identified exposure to known leukemogenic agents
Secondary- prior myelodysplasia, prior radiation, chemotherapy or certain chemical exposure
How is AML classified?
M0-undifferentiated M1- myeloblastic without maturation M2- myeloblastic with maturation M3- promyelocytic t(15:17) M4- myelomonocytic M5- monocytic M6-erythroid M7-megakaryoblastic
What are the clinical features of AML?
Symptoms related to complications of pancytopenia Weakness and easy fatigue Gingival bleeding, ecchymoses, epistaxis Lymphadenopathy Organomegaly(spleen, liver) Fever Sternal tenderness
What are the labs features of AML?
Pancytopenia
Blast circulation
Coagulopathy
Tumor lysis
How is AML diagnosed?
Bone marrow biopsy
Cytogenetic abnormalities
Flow cytometry for immunophneotyping
What are some adverse prognostic features of AML?
Age<2 or >60
Poor performance status
How is AML treated?
Chemotherapy consisting of:
Induction- cytarabine eor 7 days followed by idarubicin for 3 days
Consolidation- high dose cytarabine for 2-4 cycles
Allogenic stem cell transplant - most common but expensive
What is acute promyelocytic leukemia?
Subset of AML Also known as progranulocytic leukemia or M3 leukemia Has a specific genetic marker t(15:17) High incidence of DIC Medical emergency
How is M3 leukemia treated?
All trans retinoic acid (ATRA) and idarubicin
Can lead to Capillary leak toxicity that is treated with dexamethasone
Complete remission >80%
Cure:40-60%
