acute leukemia

Question 1

what is the etiology of acute leukemias

Answer 1

Down syndrome
Alkylating agents, Topoisomerase II inhibitors
Chloramphenicol 
phenylbutazone
Benzene, pesticides, herbicides

Question 2

what is an important theme with cancer

Answer 2

cancer cells of the cancer seem to have stopped differentiating at some point in their maturation
some cancers are names after the cell at which maturation arrest occurred

Question 3

the WHO classification system for AML includes

Answer 3

pt Hx
Cell morphology
Histochemistry
Flow Cytometry
Chromosomal analysis
Molecular evaluation

Question 4

what are the presenting symptoms of AML

Answer 4

case present < 3months
Fever, bleeding, fatigue and SOB
Stroke Symptoms
Retinal Hemorrhages
Gingival hyperplasia (monocytic leukemia)
solid tumors (myeloid sarcomas)

Question 5

what are the lab like for AML

Answer 5

anemia: normochromic and normocytic in De novo
Macrocytic in MDS related cases
~35% < 5 x 103 WBC/mm3
~ 45%  5 x 103 – 99 x 103 WBC/ mm3
~ 20% > 100 x 103 / mm3
 The Characteristic Peripheral Smear: Blast with a “Leukemic Hiatus”  (Poly and Blasts, but no intermediate forms)
Aeur rod
Large Nucleus

Question 6

what is the treatment for AML

Answer 6

7 days of cytarabine given by continuous IV infuction and 3 days of an Anthracycline usually daunorubicin

Question 7

what is the treatment for pt >70 who can not tolerate standard therapy

Answer 7

low dose decitabine
Allo-HSCT
intensive supportive therapy with Abx, Blood Products, care of mucous membranes and skin

Question 8

How do APL present

Answer 8

most present like acute leukemia except bleeding and thrombosis is common which is due to DIC

Question 9

what do the labs look like for APL

Answer 9

marrow reveals a sea of large Granular Pro-myelocyted with frequent prominent Auer rods
t(15:17), this translocation juxtaposes the retinoic acid receptor gene from chromosome 17 next to the PML gene of chromosome 15

Question 10

what is the Tx for APL

Answer 10

tretinoin or ATRA
this can causes SOB, chest pain, fever hypoxia
another option is Arsenic trioxide

Question 11

who does ALL affect the most

Answer 11

largely disease of children & young children
there is B-cell and T-Cell
Increased risk with: Trisomy 21, Down syndrome
radiation exposure

Question 12

Unclassified ALL genes and gene rearrangements

Answer 12

+TdT
CD34
T(9;22) (BRC/ABL)

Question 13

Pre B Cell ALL gene and gene rearrangement

Answer 13

\+HCR
\+TdT
\+k or lambda light chain
\+HLA-DR
\+CD19 T(9;22) (BRC/ABL)

Question 14

Burkitts Leukemia genes and gene rearrangement

Answer 14

IgM
\+IgM
\+HLA-DR
\+CD 19 & CD 20
T(8,-) (q24,-)
(C-MYC)

Question 15

T cell ALL genes and gene rearrangement

Answer 15

+CD 2, CD7, CD 38

Question 16

what is the standard in all ALL patients

Answer 16

lumbar puncture because of CNS effects

Question 17

What will bone marrow show

Answer 17

mostly nuclei

Question 18

what is the TX of ALL

Answer 18

vincristine and prednisone
the addition of anthracycle
L-asparaginase and cyclophosphamide
7 & 3 
7 days of cytrabine and 3 days of anthracycline