Myeloproliferative neoplasms Flashcards

1
Q

Types of myeloproliferative neoplasms

A

Chronic myeloid leukemia (CML)
Polycythemia vera
Essential thrombocythemia
Primary myelofibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Myeloproliferative neoplasm associated with Philadelphia chromosome

A

CML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Translocation associated with CML

A

t(9;22)(q34;q11)
BCR-ABL
truncated chromosome 22
Ph chromosome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Routinely used for monitoring response to therapy in CML

A

RT-PCR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Generally age of CML

A

40-60 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Common symptoms of CML

A

Mild to moderate anemia
Weight loss and anorexia
Splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Less common symptoms of CML

A

Hyperuricemia –> gout
Platelet dysfunction –> bruising and bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Three phases of CML

A

Chronic phase
Accelerated phase
Blast crisis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Increasing spleen size and/or resistance to therapy in CML

A

Blast crisis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Progression of blast crisis in CML

A

70% develop AML
30% develop B cell ALL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Characteristics of accelerated phase of CML

A

Increasing basophils
Thrombocytopenia
Myeloblasts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Characteristics of peripheral smear in CML

A

High WBC with immature neutrophils beyond blast cell stage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Characteristics of bone marrow histology in CML

A

Hypercellular
Increased granulocytic precursors, eosinophils, and megakaryocytes with micromegakaryocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Mutation in polycythemia vera

A

Point mutation in JAK2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Blood characteristics in polycythemia vera

A

Increased RBC mass
HCT >60%
Increased Hgb
Hyperviscosity and hypervolemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Pt presentation in polycythemia vera

A

HA
Dizziness
HTN
Blurred vision
Plethoric and cyanotic
Splenomegaly

17
Q

Marrow biopsy in polycythemia vera

A

Hypercellular
Panmyelosis
Increased megakaryocytes with frequent hyperlobated forms

18
Q

Possible progression of polycythemia vera

A

Myelofibrosis (30%)
AML (5%)

19
Q

Activating point mutations in essential thrombocythemia

A

JAK2 (50-60%)
CALR (30%)
MPL (5-10%)

20
Q

Causes of reactive thrombocytosis

A

Bleeding
Inflammatory disorders
Iron deficiency

21
Q

Clinical features of essential thrombocythemia

A

Older age
Thrombotic complications
Gangrene
Erythromelalgia

22
Q

Blood smear in essential thombocythemia

A

Increased platelets
Abnormally large platelets

23
Q

Peripheral smear in primary myelofibrosis

A

Tear drop cells/dacrocytes
Variable immature myeloid cells
Nucleated RBCs

24
Q

Marrow biopsy in primary myelofibrosis and stain

A

H&E stain
Extensive fibrosis with collagen fibers

25
Q

Mutations associated with primary myelofibrosis

A

Activating JAK2, CALR, or MPL

26
Q

Pathogenesis of primary myelofibrosis

A

Increased secretion of TGF-beta from marrow cells leads to fibrosis

27
Q

Possible progression of primary myelofibrosis

A

AML (10-20%)

28
Q

Clonal disorders of hematopoietic stem cells

A

Myelodysplastic syndromes

29
Q

Clinical features of myelodysplastic syndromes

A

Refractory anemia
Infections
Abnormal bleeding manifestations

30
Q

Neutrophil changes in myelodysplastic syndromes

A

Abnormal nuclear chromatin condensation

Pseudo-Pelger-Huet cells - bilobed nuclei

Hypo-granularity

31
Q

Erythroid cell changes in myelodysplastic syndromes

A

Megaloblastic change

Dyserythropoiesis

Ringed sideroblasts

32
Q

Megakaryocyte changes in myelodysplastic syndromes

A

Micromegakaryocytes with single hypolobated nuclei

Megakaryocytes with multiple separated nuclei

33
Q

Cytogenic abnormalities in myelodysplastic syndrome

A

Monosomies 5 and 7

-5q
-7q
-20q
+8

34
Q

Outcomes of myelodysplastic syndromes

A

Transform to AML

Medial survival of 9-29 months