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39 Cards in this Set
- Front
- Back
What is the definition of acute lymphoblastic leukemia?
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A clonal disorder with all leukemic cells arising from a hematopoietic progenitor in the lymphoid lineage, characterized by the accumulation of excess lymphoid blasts in the bone marrow
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What is the difference in a peripheral smear between the acute and the chronic leukemias?
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Chronic: retain the ability to differentiate to the endpoint of their lineage
Acute: suffer a differentiation block: can't get to the most mature form |
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Where is the differentiation block in Burkitt's lymhoma?
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Mature B cells
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What population gets acute lymphoid leukemia?
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Kids
Peak at 3-4 years; secondary peak in elderly |
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What population gets acute myeloid leukemia?
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Adults
Incidence increases with age. |
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What is the prognosis for ALL?
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Curable in 70% of kids
Curable in a minority of acults |
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What's more common: ALL or AML?
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AML: 5:1
Both diseases are still relatively rare |
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What mutation is present in the childhood ALL? Adult?
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Childhood: hyperdiploidy, TEL-AML translocation
These give a favorable prognosis MLL rearrangements (common <1YO) give worse prognosis |
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Why is prognosis so much better for kids with ALL than for adults?
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In kids, the mutations that give a favorable prognosis (hyperdiploidy, TEL-AML) are more common and the mutations that give a less favorable prognosis (BCR-ABL and MLL rearrangements) are more rare
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What mutations give a favorable prognosis in ALL? A poor prognosis?
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Favorable:
Hyperploidy TEL-AML: t(12,21) Unfavorable: BCR-AB MLL rearrangements |
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What cell is irregular in most of the ALLs?
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Precursor B-lymphocytes
Occurs most frequently in kids, also in adutls |
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What can cause ALL?
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Most people, we don't know
Different teratogens: -Radiation -Chemicals/drugs -Oncogenic viruses -Family history -Hematologic disorders |
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What type of cancer is associated with EBV?
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Burkitt's lymphoma
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What is the clinical presentation of ALL?
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Rapid onset! This is staggering!
Bone marrow infiltration Infiltrating of other organs Leukostasis Constitutional symptoms Bleeding from coagulopathy (DIC) |
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What type of pain is common in kids with ALL?
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Bone pain!
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What finding is common with a T-cell ALL?
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Mediastinal mass
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What organs are more commonly infiltrated in ALL than in AML?
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Bones
CNS |
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Because ALL more typically invades the CNS, what do you have to do?
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CNS prophylaxis
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What are the hematologic findings in ALL?
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WBC elevated, but can be normal or low
Normocytic anemia Thrombocytopenia BLASTS IN PERIPHERAL BLOOD Coagulopathy/DIC sometimes seen |
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How do you make a diagnosis of ALL?
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Bone marrow bipsy:
-Hypercellular marrow -Excess lymphoblasts |
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What are signs of ALL on blood chemistry?
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Tumor lysis syndrome:
-Increased uric acid, K, phosphate, creatine -Increased LDH -Decreased Ca (sequestered by PO4) |
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What is the diagnosis of acute lymphocytic leukemi?
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>20% lymphoblasts on bone marrow biopsy
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What do ALL cells look like?
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Variable size; indistinct nucleoli
High N/C ratio Fine chromatin NO GRANULES, AUER RODS. |
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What are some cytochemical findings for ALL/
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Myeloperoxidase: negative
Nonspecific eterase: negative Deoxygransferase: positive (some of the time) The opposite findings are true in AML |
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What are some common cell surface markers in ALL?
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CD19, 20
CD10, CALLA Surface immunoglobulin 25% of ALL expresses myeloid surface antigens: poor prognostic factor. |
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What factors indicate a poor prognosis with ALL?
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Age: older, <1 year
High WBC count at time of diagnosis Present myeloid antigens Late achievment of complete remission |
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What factors indicate a favorable prognosis for ALL?
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Younger, but >1 year
Low WBC count at time of diagnosis Absent myeloid antigens Early achievement of complete remission |
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What's the treatment for leukostasis?
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Leukopheresis: take off some of the leukocytes
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What's the treatment for tumor lysis syndrome?
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Hydration! Hydration! Hydration!
Allopurinol Rasburicase (antibody that breaks down uric acid) |
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What does allopurinol do to the bone marrow?
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Suppress the bone marrow
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What is the generalized treatment for ALL?
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1. Induction (killing the marrow) in hospital
2. People have remission 3. Consolidation (keeping the bone marrow down.) 4. Maintenance 5. CNS prophylaxis!!! |
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What are some different agents for consolidation?
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Cytarabine
Cyclophosphamide Anthracycline |
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Who with ALL should you transplant?
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Philadephia chromosome positive ALL
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Why should you give CNS prophylaxis in ALL?
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Because without it, 35% will have CNS disease
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Who more commonly gets Ph+ ALL: kids or adults?
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Atults
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What gene product is present in ALL with the philly chromosome?
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BCR-ABL
Constituitively active tyrosine kinase |
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What is the translocation that happens in the philly chromosome?
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t(9:22)
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What type of ALL has the worst prognosis?
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Ph+ ALL
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What is the therapy for Ph+ ALL?
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Targeted therapy with TKIs in induction and maintenance
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