Ch18 Chronic Myeloproliferative Disorders Part II

Question 0

Characteristics of polycythemia Vera:

Answer 0

Cyanosis, polycythemia, and splenomegaly
Granulocytes and megakaryocytic precursors mildly elevated
Bone marrow fibrosis
RBC levels can be normal or decreased

Question 1

A chronic abnormality of the hematopoietic stem cell characterized by uncontrolled proliferation of erythroid, granulocytic and megakaryocytic cells defines:

Answer 1

Polycythemia Vera

Question 2

What is the median age of diagnosis for polycythemia Vera?

Answer 2

60, slightly predominant in men

Question 3

What is the genetic abnormality found in nearly all PV patients?

Answer 3

JAK2 Exon 12 mutation

Question 4

What is the JAK2?

Answer 4

A cytoplasmic tyrosine kinase encoded by a gene on the short arm of chromosome 9

Question 5

What are some symptoms of PV?

Answer 5

Headach
Ischemic
Hemorrhage stroke 
Angina
Myocardial infraction
Cramping pain in leg muscle

Question 6

What is the most common diagnostic finding in PV?

Answer 6

Elevated hemoglobin and hematocrit, plasma volume

Question 7

The LAP score is ________ in PV in 97% of cases

Answer 7

Elevated

Question 8

What is the most common treatment of PV?

Answer 8

Phlebotomy - take 1L of blood out to control symptoms

treat patients with iron to replace lost

Question 9

A chronic myeloproliferative disorder characterized by marked thrombocytosis associated with abnormal platelet function and increased risk of thrombosis and hemorrhage:

Answer 9

Essential thrombocythemia (ET)

Question 10

What is the most frequent myeloproliferative disorder?

Answer 10

ET

Question 11

Diagnostic criteria of ET?

Answer 11

Elevated platelet count
Megakaryocytic hyperplasia in the BM
Absence of significant marrow fibrosis
Normal hematocrit and MCV

Question 12

The JAK2 gene mutation is found in ______ patients with ET

Answer 12

30-50%

Question 13

What is the most common clinical complication in ET?

Answer 13

Thrombosis and hemorrhage

Question 14

The peripheral blood smear of ET patients will show ________ marked thrombocytes

Answer 14

> 600,000/ul

Question 15

ET has no unique marker therefore it is by nature a diagnosis of:

Answer 15

Exclusion

Question 16

What is the recommended treatment for ET patients that are asymptotic

Answer 16

Carefully monitoring platelet count

Question 17

What is the ET treatment recommended for high risk patients?

Answer 17

Drugs can be used - chemotherapy

Need to control hemorrhaging and manifestations of thrombosis

Question 18

Name a clonal myeoloproliferative disease

Answer 18

IMF

Question 19

Fibrosis of the BM, extra medullary hematopoiesis involving the liver and spleen, and leukoerthroblastosis in the peripheral blood are characteristic what what myeoloproliferative disorder

Answer 19

IMF

Question 20

Characterized by the proliferation of mainly granulocytes and megakaryocyte leading to the characteristic finding of BM fibrosis

Answer 20

IMF

Question 21

IMF occurs mainly in patients of _____ years old

Answer 21

67

Question 22

IMF originates at the level of:

Answer 22

CD34 hematopoietic stem cell

Question 23

Splenomegaly is seen in ______ at time of IMF diagnosis

Answer 23

85-99%

Question 24

Symptoms of IMF include:

Answer 24

Fever Anorexia Bone pain

Question 25

What type of anemia is seen in 50-90% of IMF patients?

Answer 25

Normalcy tic normochromic anemia

Question 26

What causes significant teardrop poikilocytosis in IMF?

Answer 26

When RBC pass through the marrow, the fibrotic sinusoids of the BM and the spleen

Question 27

What is the only curative treatment for IMF?

Answer 27

Allogenic stem cell transplantation

Question 28

What is the origin of MPDs?

Answer 28

Neoplastic transformation of multipotential stem cells

Question 29

The Philadelphia chromosome involves:

Answer 29

t(9:22) BCR and c-ABL genes 210-kD protein possessing increased tyrosine kinase activity

Question 30

What is the predominant abnormal erythrocytes morphology associated with idiopathic myelofibrosis?

Answer 30

Teardrop cells

Question 31

Which featured of chronic myelogenous leukemia are the most important characteristics that distinguish it from myelofibrosis?

Answer 31

Low LAP score and presence of Ph chromosome

Question 32

What factors cause fibroblast proliferation?

Answer 32

TGF-B bFGF PDGF

Question 33

What are the lab findings in PV?

Answer 33

Increased hematocrit; increased RBCs; increased granulocytes and platelets

Question 34

What is the expected erythropoietin value in PV?

Answer 34

Decreased

Question 35

When us myelosuppression advocated in patients with PV?

Answer 35

When thrombosis-associated risk factors are present

Question 36

What features help distinguish secondary erythrocytes is and relative erythrocytosis from PV?

Answer 36

Absence of splenomegaly; normal leukocyte, platelet and LAP levels

Question 37

What is the safest and least expensive treatment for patients with PV?

Answer 37

Therapeutic phlebotomy

Question 38

What condition is characteristically associated with reactive thrombocytesis?

Answer 38

Acute hemorrhage Chronic inflammatory disorders Iron deficiency anemia

Question 39

Which MDP is associated with the best prognosis?

Answer 39

CML