Pre-TBL independent study

Question 1

What is leukemia (generally speaking)

Answer 1

A neoplasm involving hematopoietic stem cells– can derive into leukemia involving any of the cells that arise from the main precursor, incl myeloid leukemia and lymphoid leukemia (from myeloid cells and lymphoid/lymphocytes!)

Question 2

AML (Acute Myeloid Leukemia) vs CML

Answer 2

caused by BLOCK in differentiation process (called LEFT SHIFT) bc lots of immature blasts
-Chronic has overprolif of ALL lvls of cell maturation

Question 3

AML
pop
cells
sx

Answer 3

mostly adults
periph cytopenias (cell deficiencies), eg decr plates/rbc/grans, lots of blasts
-malig clones of immature cells replace healthy marrow
-sx: anemia, fatigue, infections, bleeding (thrombocytopenia), fever etc, if invasive can be in skin/gums (thin tissue)

Question 4

Four types of AML

Answer 4

1. AML with recurrent genetic abnormalities (usu balanced translocations)
2. AML with myelodysplasia-related changes
3. Therapy-related myeloid neoplasms
4. Idiopathic AML

Question 5

Dx of AML

Answer 5

high # of blasts in periph blood smear (if no auer rods, >20%), hypercellularity
might have auer rods (red lines)

Question 6

What do red lines inside cell confirm Dx of?

Answer 6

auer rods in AML!

Question 7

AML prognosis? and why

Answer 7

only a quarter survive, bc AML reduces ability to fight off infxns
(some translocations indicate better px)

Question 8

AML tx?

Answer 8

blood transfusions, antibios, bone marrow transplant, chemo

Question 9

ALL (acute lymphoblastic leukemia/lymphoma)
pop
sx and diff from AML

Answer 9

mostly children

similar sx to AML but with HEPATOSPLENOMEGALY

Question 10

2 types of AML based on lymphocytes?

Answer 10

-precursor B-cell ALL (B-ALL)
can have recurrent genetic abnormalities in chrom, or translocs
-T-ALL: also affects medistinal (THYMIC) involvement (obv)
BOTH affect bone marrow and periph blood

Question 11

ALL dx?

Answer 11

many lymphocytes in periph blood smear
-IHC cytochem to dist bn lymphos and myeloblasts
(blasts should be >~20-25% of cells)

Question 12

Tx of ALL

Answer 12

treatable in kids
adults with ALL worse than with AML
-early induction, consol, prolonged maint of tx use
-tx with intrathecal chemo/ brain/spine radiation bc ALL likes to hide in the spinal cord

Question 13

ALL with favorable prognosis?

Answer 13

12;21 and hyperdiploid

Question 14

ALL with poor px?

Answer 14

9;22, v;11q23, and hyPO diploid

Question 15

between hyper and hypodiploid ALL, which has better px?

Answer 15

HYPERdiploid ALL!

Question 16

MDS (MyeloDysplastic Syndrome)

Answer 16

Disorder where stem cells cannot properlY become mature cells, causing problems down the line incl cytopenias, ineff hematopoeisis, dysplasia in cell lines ,and incr risk of AML

Question 17

What else besides AML does MDS increase risk for and why?

Answer 17

MDS increases risk for LEUKEMIA bc cells are unstable, usu bc of genetic abnormalities (soemtimes chemo)

Question 18

MDS sx and pop

Incr risk for?

Answer 18

much older pts, >70
anemia, freq blood transfusions, otherwise asymptomatic (dont know they have it)
-Can dev AML, and infections/bleeding
-Dysplasia in marrow and periph smear

Question 19

What does MDS periph blood smear show?

Answer 19

Decr plates/RBCs

Histo shows unusual WBCs with PSEUDO-PELGER HEUT CELLS (bilobed dumbell shaped nuclei)

Question 20

What does MDS bone marrow show? (stain?)

Answer 20

HYPERcellularity
decr cell counts (blasts LESS than 20%)
Erytrhopoetic issues such as RC precursers, nucl irreg, IRON STAIN
issues with grans/megakars

Question 21

Subtypes of MDS

Answer 21

• low risk (usu dont dev into AML), refractory anemia with unilineage dysplasia (RA) or with Ring sideroblasts (RARS), RAs are refactory to b12 or folate tx
• intermed risk: refractory anemia with multilin dysplasia or w.o ring sidereoblasts, or with excess blasts (more acronyms)
• high risk: RAEB-1 (RA with excess blasts 2)

Question 22

clonal cytogenic abnormalities are observed in approx how much of MDS cases?

Answer 22

50%

(look for del(5q)/7q issues

Question 23

Tx for MDS?

Answer 23

NONE (bone marrow ineffective bc older pts), colony stim factors $$$

Question 24

Which MDS genome may have + clin course?

Answer 24

isolated del(5q)

Question 25

What does imunophenotyping in hematopoeitic issues determine? What are 2 methods?

Answer 25

whether cell is B/T/myeloid and what lvl of maturation
(tells us cell type via staining)
Examples are: IHC and Flow cytometry

Question 26

How IHC works

Answer 26

uses ab’s to detect certain prot in sample

Non-fluor, uses LM, catalyzes rxn to give colored product etc

Question 27

How Flow cytometry works

Answer 27

Laser based
fluor ab’s attach to ag’s to count/sort/detect cells, ag/ab stained complex detected electronically in fluid, cells scanned for diff properties

Question 28

Which is better: IHC or flow cytometry and why (and what is its disadv?)

Answer 28

FLOW CYTOMETRY!
improved sensitivity, more detail, buuut loss of cell structure
map cell pop on XY plane, in C shape, so top RIGHT quadrant is positive for both X and Y, whereas top LEFT is positive only for Y

Question 29

What might pseudo-pelger-huet cells (bilobed nuclei) indicate?

Answer 29

MYELODYSPLASTIC SYNDROME (MDS)!