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Hematology > Ch18 Chronic Myeloproliferative Disorders Part II > Flashcards

Ch18 Chronic Myeloproliferative Disorders Part II Flashcards

0
Q

Characteristics of polycythemia Vera:

A

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

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1
Q

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

A

Polycythemia Vera

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2
Q

What is the median age of diagnosis for polycythemia Vera?

A

60, slightly predominant in men

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3
Q

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

A

JAK2 Exon 12 mutation

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4
Q

What is the JAK2?

A

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

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5
Q

What are some symptoms of PV?

A 
Headach
Ischemic
Hemorrhage stroke 
Angina
Myocardial infraction
Cramping pain in leg muscle
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6
Q

What is the most common diagnostic finding in PV?

A

Elevated hemoglobin and hematocrit, plasma volume

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7
Q

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

A

Elevated

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8
Q

What is the most common treatment of PV?

A

Phlebotomy - take 1L of blood out to control symptoms

treat patients with iron to replace lost

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9
Q

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

A

Essential thrombocythemia (ET)

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10
Q

What is the most frequent myeloproliferative disorder?

A

ET

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11
Q

Diagnostic criteria of ET?

A

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

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12
Q

The JAK2 gene mutation is found in ______ patients with ET

A

30-50%

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13
Q

What is the most common clinical complication in ET?

A

Thrombosis and hemorrhage

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14
Q

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

A

> 600,000/ul

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15
Q

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

A

Exclusion

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16
Q

What is the recommended treatment for ET patients that are asymptotic

A

Carefully monitoring platelet count

17
Q

What is the ET treatment recommended for high risk patients?

A

Drugs can be used - chemotherapy

Need to control hemorrhaging and manifestations of thrombosis

18
Q

Name a clonal myeoloproliferative disease

A

IMF

19
Q

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

A

IMF

20
Q

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

A

IMF

21
Q

IMF occurs mainly in patients of _____ years old

A

67

22
Q

IMF originates at the level of:

A

CD34 hematopoietic stem cell

23
Q

Splenomegaly is seen in ______ at time of IMF diagnosis

A

85-99%

24
Q

Symptoms of IMF include:

A

Fever
Anorexia
Bone pain

25
Q

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

A

Normalcy tic normochromic anemia

26
Q

What causes significant teardrop poikilocytosis in IMF?

A

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

27
Q

What is the only curative treatment for IMF?

A

Allogenic stem cell transplantation

28
Q

What is the origin of MPDs?

A

Neoplastic transformation of multipotential stem cells

29
Q

The Philadelphia chromosome involves:

A

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

30
Q

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

A

Teardrop cells

31
Q

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

A

Low LAP score and presence of Ph chromosome

32
Q

What factors cause fibroblast proliferation?

A

TGF-B
bFGF
PDGF

33
Q

What are the lab findings in PV?

A

Increased hematocrit; increased RBCs; increased granulocytes and platelets

34
Q

What is the expected erythropoietin value in PV?

A

Decreased

35
Q

When us myelosuppression advocated in patients with PV?

A

When thrombosis-associated risk factors are present

36
Q

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

A

Absence of splenomegaly; normal leukocyte, platelet and LAP levels

37
Q

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

A

Therapeutic phlebotomy

38
Q

What condition is characteristically associated with reactive thrombocytesis?

A

Acute hemorrhage
Chronic inflammatory disorders
Iron deficiency anemia

39
Q

Which MDP is associated with the best prognosis?

A

CML

Hematology (10 decks)

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