Exam 2 Material

Question 1

General: What are the four (4) characteristics of Acute Myeloid Leukemia?

Answer 1

1. Clonal Disorder
2. Autonomous Proliferation
3. Single or multilineage involvement
4. Minimal cell maturation

Question 2

General: Clinical presentation of Acute Myeloid Leukemia (AML)?

Answer 2

*Pallor, fatigue
*Fever/Infection
*Bruising/bleeding
*Malignant cell infiltration into other tissues

Question 3

General: Peripheral blood presentation of Acute Myeloid Leukemia (AML)?

Answer 3

*Variable leukocytosis
*Variable single/multilineage dysplasia
*Myeloblasts present (leukemic hiatus)

Question 4

General: Bone marrow presentation of Acute Myeloid Leukemia (AML)?

Answer 4

*Hypercellular increased blasts
* >20% blasts with limited maturation
*Variable single/multilineage dysplasia

Question 5

Objective 3 (AML): Describe AML Myelodysplasia Related with respect to:
a. Defining criteria of AMLs in this category (etiology & genetics)
b. Typical age of patients

Answer 5

a. >20% blasts
AML evolving from a previous MDS or MDS/MPN
AML with genetic abnormalities that mirror MDS
>/= 3 genetic abnormalities (i.e. -7, -5, del(5q), del(7q), etc.)

b. Elderly

Question 6

Objective 1 (MPN): Describe myeloproliferative neoplasms (MPNs)

Answer 6

Clonal disorder of HSC diseases characterized by excessive production/over-accumulation of RBCs, granulocytes, and platelets (in some combination)

Question 7

Objective 2 (MPN): List the diseases included in the classification of MPNs and recognize their
abbreviations.

Answer 7

Chronic Myeloid Leukemia (CML)
Polycythemia Vera (PV)
Essential Thrombocythemia (ET)
Primary Myelofibrosis (PMF)
Chronic Neutrophilic Leukemia (CNL)
Chronic Eosinophilic Leukemia (CEL)
Juvenile Myelomoncytic Leukemia (JMML)
MPN, not otherwise specified

Question 8

Objective 3 (MPN): Define chronic myeloid leukemia (CML) with emphasis on the affected cell line(s).

Answer 8

Overproduction of defective granulocytes (cells do differentiate and mature)

Question 9

Objective 4 (MPN): Discuss the theory of pathogenesis of CML

Answer 9

t(9;22) – fusion of BCR:ABL1 leading to production of a protein with enhanced TK activity

Question 10

Objective 5 (MPN): Describe the peripheral blood and bone marrow in CML.

Answer 10

PB:
*Marked leukocytosis
*Bimodal granulocyte pattern
*Basophilia
*Thrombocytosis
*Decreased LAP

BM:
*Marked hypercellular
*Pseudo-Gaucher cells
*Increased fibrosis

Question 11

Objective 7 (MPN): Describe the clinical phases of CML with an emphasis placed on blast count and response to tyrosine kinase inhibitors.

Answer 11

Chronic/Stable Phase: <10% blasts, TKI has reduced the progression

Blast phase: >20% blasts, TKI resistance

Question 12

Objective 11 (MPN): Define polycythemia vera (PV) with emphasis on the affected cell line(s).

Answer 12

Clonal disorder of HSCs characterized by an increase in RBCs, granulocytes, and platelets

Question 13

Objective 12 (MPN): Discuss clinical symptoms commonly observed in patients with PV

Answer 13

*CNS – headache, etc.
*Hypertension and vascular abnormalities
*Pruritus (excessive itching)

Question 14

Objective 13 (MPN): Identify major morphologic changes in the bone marrow and peripheral
blood in patients with PV. Included in this objective: recognize that PV is normally normocytic- normochromic but repeated phlebotomies will lead to
microcytic-hypochromic anemia.

Answer 14

PB:
*Erythrocytes - increased RBC mass
*Granulocytes - neutrophilia, but mature cells
*Platelets - thrombocytosis, normal morphology

BM
*Hypercellular, but M:E normal
*Iron stores are decreased
*Megakaryocytes are increased

Question 15

Objective 14 (MPN): List diagnostic criteria for PV

Answer 15

Hgb >16.5 or HCT >49 [Men]
Hgb >16.0 or HCT >48 [Women]
BM hypercellularity w/ panmyelosis
Presence of JAK2 V617F mutation

Question 16

Objective 15 (MPN): Discuss the progression of PV

Answer 16

Prodromal/Stable Phase: borderline to mild erythrocytosis
Overt Phase: significantly increased red cell mass
Spent Phase: large and overactive spleen, pancytopenia due to BM fibrosis

Question 17

Objective 16 (MPN): Define essential thrombocythemia (ET) with emphasis on the affected cell line(s)

Answer 17

Clonal disorder of HSCs characterized by increased megakarypoiesis

Question 18

Objective 17 (MPN): List the diagnostic criteria for ET

Answer 18

*Sustained platelet count of >450
*Proliferation of megakaryocytic line with increased dysplasia
*Does not meet the criteria for any other MPN, MDS, or myeloid neoplasm
*Positive for JAK2 or other clonal marker
*Absence of reactive thrombocytosis, if clonal marker not present

Question 19

Objective 18 (MPN): List the morphologic changes in the peripheral blood in patients with ET

Answer 19

*Thrombocytosis with dysplasia
*Platelet clustering and accumulation near feather of
blood film
*Neutrophils may be increased; basophils are not

Question 20

Objective 19 (MPN): List two complications that may occur in patients with ET.

Answer 20

1. Progression to PMF
2. Transformation to acute leukemia

Question 21

Objective 20 (MPN): Define primary myelofibrosis (PMF) with emphasis on the affected cell line(s) and pathogenesis

Answer 21

Clonal disorder of HSCs characterized by proliferation of megakaryocytes and granulocytes

Question 22

Objective 21 (MPN):