UNIT 3 MPN

Question 1

If matured platelets are produced

Answer 1

THROMBOSIS

Question 2

if immatured,
non-functional—

Answer 2

bleeding

Question 3

occurs because the body senses the
immature/nonfunctional production of cells (e.g., in erythrocytes, the body
senses lack of oxygen when RBC’s are nonfunctional). The body “recruits” other
organs

Answer 3

extramedullary hematopoiesis

Question 4

MPN arising from a single genetic translocation in a pluripotential HSC producing a clonal
overproduction of the myeloid cell line, resulting in a preponderance of immature cells in the
neutrophilic line.
First detected cancer with known genetic abnormality.

Answer 4

CML

Question 5

CML Occurs at all ages but is seen predominantly in those aged

Answer 5

46-53 yrs old

Question 6

Diagnosis is often accidental and is diagnosed late because there are no manifestations
in its early stages.
Seen more often in populations exposed to ionizing radiation.

Answer 6

CML

Question 7

Chromosome 9 contains

Answer 7

ABL

Question 8

Chromosome 22 contains

Answer 8

BCR

Question 9

Chronic has gradual progression, unlike acute
where it is sudden, hence, more commonly
seen in older populations.
In general, leukemias have unknown causes.
However, _____ is seen in populations exposed
to ionizing radiation like X-ray.

Answer 9

CML

Question 10

Producing a clonal overproduction of the myeloid cell line resulting in a preponderance of immature cells in the
neutrophilic line

Answer 10

CML

Question 11

Consistently associated with the BCR-ABL 1 fusion gene located in the Philadelphia chromosome

Answer 11

CML

Question 12

It is the _____ that is
involved in the Philadelphia translocation.

Answer 12

BCR GENE 1

Question 13

Frequent infection
Anemia
Bleeding
Splenomegaly
All secondary to massive pathologic accumulation of myeloid progenitor cells in bone marrow,
peripheral blood, and extramedullary tissue

Answer 13

CML

Question 14

Leukocyte count can be as high as 50,000/ cu.mm
High WBC

Answer 14

CML

Question 15

increase in uric acid in the blood.

Answer 15

hyperuricemia - CML

Question 16

increase in uric acid in the urine.

Answer 16

URICOSURIA - CML

Question 17

LAP OF CML

Answer 17

DECREASED

Question 18

Serum cobalamin and transcobalamin of CML

Answer 18

INCREASED

Question 19

Increased granulopoiesis, megakaryocytes, and reticulin presence.
● Gaucher-like cells (sea blue cells)

Answer 19

CML

Question 20

First synthetic tyrosine kinase inhibitor
Designed to selectively bind the ATP binding site and inhibit the tyrosine kinase activity
of the BCR-ABL1 fusion protein

Answer 20

imatinib mesylate

Question 21

2 to 5 yrs

Highly treatable

Most CML cases (85%) are diagnosed in the _____

Answer 21

INITIAL (CHRONIC)

Question 22

6-18 months

Resistance develops

Splenomegaly starts
Increase in basophils, leukocytosis.
Symptoms start to appear

Answer 22

ACCELERATED

Question 23

3-4 months

Generally unresponsive

“Acute on top of chronic”
High in immature/abnormal leukocytes
30% or more blasts present in BM and peripheral blood
There is extramedullary infiltrate of blood cells
Excessive bleeding, bruising

Answer 23

blast crisis (acute)

Question 24

Panmyelosis in the Bone Marrow
Increase in erythrocytes, granulocytes, and platelets in the peripheral blood

Answer 24

PV

Question 25

PV Rarely occurs in children; ages: ____ Men > women

Answer 25

40-60

Question 26

Genetically characterized by mutation of Janus kinase 2 (JAK2V617F) ○ found on chromosome band ____.

Answer 26

PV ; 9p24

Question 27

major criteria

Answer 27

HGB - M=18.5 g/dL F= 16.5 g/dL HCT - M= 49% F= 48% RCM = 0.25 OF THE MEAN NORMAL

Question 28

minor criteria of PV

Answer 28

low serum erythropoeitin levels

Question 29

Symptoms: Cyanosis Splenomegaly Thrombotic or hemorrhagic phenomena Headaches, dizziness Paresthesia (because blood is too viscous due to elevated blood cells)

Answer 29

PV

Question 30

BM showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic proliferation with pleomorphic, mature megakaryocytes. Identification of the JAK2 V617F mutation or the JAK2 exon 12 mutation.

Answer 30

PV

Question 31

Independent of normal regulatory mechanisms that are associated with increased red blood cell (RBC) mass. Hyperviscosity (resulting in cardiovascular disease, hypertension). The heart is affected.

Answer 31

proliferative phase of PV

Question 32

Continuation of proliferative phase.

Answer 32

stable phase

Question 34

Progressive splenomegaly (palpable spleen) or hypersplenism (large spleen with BM hyperplasia and peripheral blood cytopenias) and pancytopenia. ○ There is already enlargement of the spleen (splenomegaly) Triad of BM fibrosis, splenomegaly, and anemia with teardrop-shaped poikilocytes (post polycythemic myeloid metaplasia).

Answer 34

spent phase of PV

Question 35

● A clonal MPN with increased megakaryopoiesis and thrombocytosis ○ Count >600 x 109/L and sometimes with a count >1000 x 109 /L.

Answer 35

EV

Question 36

Primary thrombocytosis Idiopathic thrombocytosis Hemorrhagic thrombocythemia

Answer 36

EV

Question 37

Accumulation of platelets = thrombosis Vascular occlusions due to platelet accumulation ○ Leads to heart attack and ischemic stroke.

Answer 37

EV

Question 38

EV Majority occurs in individuals between ____ Second peak of cases occur in women in childbearing age (30yrs of age)/ Women > men

Answer 38

50-60 yrs old

Question 39

Demonstration of JAK2V617F or related mutation, or in the absence of JAK2V617F, no evidence for reactive thrombocytosis (e.g., inflammation).

Answer 39

EV

Question 40

50% has asymptomatic thrombocytosis. 50% hemorrhage or thrombosis. Vascular occlusions Pulmonary emboli

Answer 40

EV

Question 41

Persistent elevation of platelets (>450 x 1012 L) in peripheral blood. Significant increase (hyperplasia) of megakaryocytes in the bone marrow. Not meeting criteria of other MPNs. *Diagnosis requires all four criteria

Answer 41

EV

Question 42

Reactive thrombocytosis ○ Increased platelets due to inflammation Increased platelets but decreased function of platelets

Answer 42

EV

Question 43

Used to quickly reduce the platelet count.

Answer 43

plateletpheresis of PV

Question 44

Preferred in ET patients with concomitant thrombocytosis and leukocytosis.

Answer 44

hydroxyurea of PV

Question 45

Preferred if only the platelet count is elevated (because of fewer side effects compared with hydroxyurea).

Answer 45

anagrelide

Question 46

Least common but most aggressive clonal HSC MPN.

Answer 46

PMF

Question 47

Chronic idiopathic myelofibrosis ● Agnogenic myelofibrosis ● Myelofibrosis with myeloid metaplasia

Answer 47

PMF

Question 48

● A reactive process causes the overproduction of collagen that eventually disrupts the normal architecture of the BM and replaces hematopoietic tissue resulting in pancytopenia. ○ Fibroblasts are normally found in the bone marrow and produce collagen to provide structural support for HSCs

Answer 48

myelofibrosis

Question 49

PMF common in patients with ages 50 yrs old and above ○ Occurs in patients older than age 60. Male = female

Answer 49

PMF

Question 50

Driver Gene Mutations in PMF:

Answer 50

JAK2 (V617F) CALR MPL

Question 51

Patients lacking JAK2, CALR, and MPL mutations ○ Associated with worse prognosis

Answer 51

triple-negative PMF

Question 52

TET2 ASXL1 DNMT3A AND EZH2

Answer 52

epigenetic regulator mutations

Question 53

Dacryocytes (teardrop cells), nucleated RBCs, polychromatophilia. ○ Normocytic, normochromic anemia with reticulocytosis (2%–15%).

Answer 53

PMF

Question 54

Megakaryocytic proliferation and atypia, without reticulin fibrosis ≥grade 1, accompanied by increased age-adjusted BM cellularity, granulocytic proliferation, and often decreased erythropoiesis

Answer 54

PRE PRIMARY MYELOFIBROSIS

Question 55

Megakaryocytic proliferation and atypia, accompanied by either reticulin and/or college fibrosis grades 2 or 3.

Answer 55

primary myelofibrosis (overt)

Question 56

Hepatosplenomegaly: most common Bleeding: 20-30% ○ Bleeding diathesis is an important clinical feature because it can contribute to the cause of death. Gout Pruritus

Answer 56

chronic neutrophilia leukemia

Question 58

staining with trichome

Answer 58

TYPE 1

Question 59

silver impregnation techniques

Answer 59

TYPE 3

Question 60

increased radiographic bone density (presence of osteosclerosis)

Answer 60

TYPE 4

Question 61

Bleeding diathesis: thrombocytopenia, abnormal platelet function, hemostatic abnormalities, DIC

Answer 61

PMF

Question 62

PMF is further subdivided into

Answer 62

prefibrotic or early stage PMF overt fibrotic stage PMF

Question 63

minor criteria of PMF

Answer 63

leukoeryhthroblastosis anemia increased serum lactic splenomegaly

Question 64

PMF: Diagnosis requires meeting all three major and two minor criteria

Answer 64

TRUE

Question 65

by far the worst MPN, and patients are at risk for fatal outcomes associated with disease progression, leukemic transformation, thrombohemorrhagic complications, and infections.

Answer 65

PMF

Question 66

EXTREMELY INCREASED IN PV

Answer 66

ERYTHROCYTES

Question 67

EXTREMELY INCREASED IN CML

Answer 67

LEUKOCYTES

Question 68

TEARDROP-SHAPED ERYTHROCYTES

Answer 68

PM

Question 69

LAP SCORE

Answer 69

EXTREMELY INCREASED IN PV

Question 70

LAP SCORE IN CML

Answer 70

DECREASED

Question 71

PH1 CHROMOSOME

Answer 71

POSITIVE FOR CML