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39 Cards in this Set

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What is the definition of acute lymphoblastic leukemia?
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
What is the difference in a peripheral smear between the acute and the chronic leukemias?
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
Where is the differentiation block in Burkitt's lymhoma?
Mature B cells
What population gets acute lymphoid leukemia?
Kids

Peak at 3-4 years; secondary peak in elderly
What population gets acute myeloid leukemia?
Adults

Incidence increases with age.
What is the prognosis for ALL?
Curable in 70% of kids
Curable in a minority of acults
What's more common: ALL or AML?
AML: 5:1

Both diseases are still relatively rare
What mutation is present in the childhood ALL? Adult?
Childhood:  hyperdiploidy, TEL-AML translocation

These give a favorable prognosis

MLL rearrangements (common <1YO) give worse prognosis
Childhood: hyperdiploidy, TEL-AML translocation

These give a favorable prognosis

MLL rearrangements (common <1YO) give worse prognosis
Why is prognosis so much better for kids with ALL than for adults?
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
What mutations give a favorable prognosis in ALL? A poor prognosis?
Favorable:
Hyperploidy
TEL-AML: t(12,21)

Unfavorable:
BCR-AB
MLL rearrangements
What cell is irregular in most of the ALLs?
Precursor B-lymphocytes

Occurs most frequently in kids, also in adutls
What can cause ALL?
Most people, we don't know

Different teratogens:
-Radiation
-Chemicals/drugs
-Oncogenic viruses
-Family history
-Hematologic disorders
What type of cancer is associated with EBV?
Burkitt's lymphoma
What is the clinical presentation of ALL?
Rapid onset! This is staggering!
Bone marrow infiltration
Infiltrating of other organs
Leukostasis
Constitutional symptoms
Bleeding from coagulopathy (DIC)
What type of pain is common in kids with ALL?
Bone pain!
What finding is common with a T-cell ALL?
Mediastinal mass
What organs are more commonly infiltrated in ALL than in AML?
Bones
CNS
Because ALL more typically invades the CNS, what do you have to do?
CNS prophylaxis
What are the hematologic findings in ALL?
WBC elevated, but can be normal or low
Normocytic anemia
Thrombocytopenia
BLASTS IN PERIPHERAL BLOOD
Coagulopathy/DIC sometimes seen
How do you make a diagnosis of ALL?
Bone marrow bipsy:
-Hypercellular marrow
-Excess lymphoblasts
What are signs of ALL on blood chemistry?
Tumor lysis syndrome:
-Increased uric acid, K, phosphate, creatine
-Increased LDH
-Decreased Ca (sequestered by PO4)
What is the diagnosis of acute lymphocytic leukemi?
>20% lymphoblasts on bone marrow biopsy
What do ALL cells look like?
Variable size; indistinct nucleoli
High N/C ratio
Fine chromatin

NO GRANULES, AUER RODS.
What are some cytochemical findings for ALL/
Myeloperoxidase: negative
Nonspecific eterase: negative
Deoxygransferase: positive (some of the time)

The opposite findings are true in AML
What are some common cell surface markers in ALL?
CD19, 20
CD10, CALLA
Surface immunoglobulin

25% of ALL expresses myeloid surface antigens: poor prognostic factor.
What factors indicate a poor prognosis with ALL?
Age: older, <1 year
High WBC count at time of diagnosis
Present myeloid antigens
Late achievment of complete remission
What factors indicate a favorable prognosis for ALL?
Younger, but >1 year
Low WBC count at time of diagnosis
Absent myeloid antigens
Early achievement of complete remission
What's the treatment for leukostasis?
Leukopheresis: take off some of the leukocytes
What's the treatment for tumor lysis syndrome?
Hydration! Hydration! Hydration!
Allopurinol
Rasburicase (antibody that breaks down uric acid)
What does allopurinol do to the bone marrow?
Suppress the bone marrow
What is the generalized treatment for ALL?
1. Induction (killing the marrow) in hospital
2. People have remission
3. Consolidation (keeping the bone marrow down.)
4. Maintenance
5. CNS prophylaxis!!!
1. Induction (killing the marrow) in hospital
2. People have remission
3. Consolidation (keeping the bone marrow down.)
4. Maintenance
5. CNS prophylaxis!!!
What are some different agents for consolidation?
Cytarabine
Cyclophosphamide
Anthracycline
Who with ALL should you transplant?
Philadephia chromosome positive ALL
Why should you give CNS prophylaxis in ALL?
Because without it, 35% will have CNS disease
Who more commonly gets Ph+ ALL: kids or adults?
Atults
What gene product is present in ALL with the philly chromosome?
BCR-ABL

Constituitively active tyrosine kinase
What is the translocation that happens in the philly chromosome?
t(9:22)
What type of ALL has the worst prognosis?
Ph+ ALL
What is the therapy for Ph+ ALL?
Targeted therapy with TKIs in induction and maintenance