Malignant granulocytic Disorders

Question 0

CML

Answer 0

Myeloproliferative, chronic
Increase in granulocytes, IGs, with or without blasts
No toxic features (toxic granulation/Dohle bodies)
Can transition into Acute Leukemia with >5% blasts and go into “blasts crisis” with 2/3 granulocytic and 1/3 lymphoid blasts
Key: has Philadelphia c’some with the BCR/ABL gene at (9;21) which controls a tyrosine kinase that keeps cells from maturing
Does NOT have the JAK2 oncogene present

Question 1

Myeloproliferative

Answer 1

An increase in 1 cell line (clonal); all cell lines technically increase, but one “outraces” the others

Question 2

t(9;21)

Answer 2

Philadelphia c’some assoc. with CML

Question 3

Essential Thrombocythemia (Thrombocytosis)

Answer 3

Myeloproliferative, chronic
High numbers of bizarre plt that cause spontaneous thrombosis
Can lead to marrow fibrosis, leading to myelophthistic and leukoerythroblastic leukemias
Has high EPO levels

Question 4

Polycythemia Vera

Answer 4

Myeloproliferative, chronic
Extremely high red count (Hct >65%)
Lose EPO requirement and have low EPO levels
Undergo therapeutic phlebotomy to reduce RBC mass
And increase in fibrous cells in BM can lead to myelophthistic leukemia

Question 5

Myelodysplastic

Answer 5

Decrease in one or more cell lines, cells are still made but die before leaving the marrow

Question 6

Characteristics of Myelodysplastics

Answer 6

PB: neutrophils that have (pseudo) Pelger-Huet or hypersegmentation and hypogranulation
RBC: megaloblastic changes, increase in MCV, nRBCs with “popcorn” nuclei
PLT: increase in size (giant), hypogranulation, with(out) megakaryocytic nuclei

Question 7

“Acute” malignancies

Answer 7

Containing >20% blasts in BM

Question 8

M0

Answer 8

Acute

Surface markers on blasts mark them as myelocytic, but they do not stain with cytochemicals

Question 9

M1

Answer 9

AML
>90% blasts in BM with Auer Rods
MPO/SBB/SE (+)

Question 10

M2

Answer 10

AML with maturation
<90% blasts in marrow with(out) Auer Rods
T(8;21) abnormality
MPO/SBB/SE (+)

Question 11

M3

Answer 11

Acute Promyelocytic Leukemia
>30% pros in BM with a high # of Auer Rods (called “faggot cells”)
C’some abnormality t(15;17) with involvement from PML/RARA
VERY MPO/SBB/SE (+)
Can be treated with ATRA (All Trans Retinoic Acid) that forces cells to mature

Question 11

t(8;21)

Answer 11

Assoc. with M2

Question 13

M4

Answer 13

Acute Myelomonocytic Leukemia
>20% monocytes cells in BM with(out) Auer Rods
MPO/SBB/SE (+) AND NSE (+), can have hybrid cells that take both stains

Question 14

t(15;17)

Answer 14

Retinoic Acid receptor gene assoc. with M3

Question 15

M5A

Answer 15

Acute Monocytic Leukemia
>80% monoblasts in children
NSE (+)

Question 16

M5B

Answer 16

Acute Monocytic Leukemia
<80% monoblasts with some maturation in adults
NSE (+)

Question 17

M6 (unique stain)

Answer 17

Acute Erythroleukemia
Bizarre RBCs with >20% myeloblasts in BM along with 50% dysplastic normoblasts
MPO/SBB/SE (+), normoblasts are PAS (+)

Question 18

M7

Answer 18

Acute Megakaryocytic Leukemia
Bizarre PLT and acute fibrosis
Find PLT surface markers on blasts
Gives BM “dry tap”

Question 19

Refractory anemia

Answer 19

Myelodysplastic syndrome, <5% in marrow

Question 20

RARS

Answer 20

> 15% ringed sideroblasts in bone marrow

Question 21

RAEB

Answer 21

<5% blasts in peripheral blood, 5-20% blasts in bone marrow

Question 22

Chronic myelomonocytic leukemia

Answer 22

Leukocytosis, increased monocytes into peripheral blood