Myeloproliferative and myelodyplasia Flashcards Preview

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Flashcards in Myeloproliferative and myelodyplasia Deck (76):
1

Who is usually diagnosed with MDS?

Older
Women

2

What is the phenotype of MDS?

Peripheral cytopenias with normocellular or hypercellular bone marrow

3

Those with MDS have an increase risk for what?

Acute leukemia

4

What is the cause of primary MDS?

Unclear

5

What are the causes of secondary MDS?

Genetic factors
Environmental factors
Antineoplastic agents

6

HOw do you characterize MDS?

What cell line is decreased

7

Leukemia is caused by bad type of what cells?

Blasts

8

SSx of MDS?

anemia (if low Hb/RBCs)
infections (if low WBCs)
Bleeding manifestations (if low platelets)
Organomegaly

9

What is sweet syndrome?

Acute neutrophilic dermatosis and pyoderma gangrenosum

10

What is the cause of sweet syndrome?

Unknown

11

How do you confirm MDS?

Bone marrow biopsy with normo or hypercellular bone marrow

12

What is trilineage dyspoiesis?

Abnormal WBC, RBC, platelet precursors

13

What is the value of Fe studies in MDS?

r/o Fe deficiency anemia

14

What is pseudo-Pelger huet anomaly?

Bilobed nucleus with a string between, characteristic of MDS

15

Ringed sideroblasts = ?

MDS or sideroblastic anemia

16

Can B12/folate deficiencies result in decrease WBCs or platelets?

yes

17

True or false: ethanol exposure cannot cause bone marrow suppression

False

18

What is the function of G-CSF?

Macrophage released cytokine that increases PMNs

19

What are the two types of antineoplastics that can cause MDS?

Alkylating agents
Topoisomerase inhibitors

20

Treatment with what chemotherapeutic agents can lead to MDS, prior to AML?

Alkylating agents

21

Treatment with what chemotherapeutic agents can lead to AML, without MDS as an intermediary?

Topoisomerases

22

What are the factors that go into the prognostic scoring for MDS, and probability for developing to AML? (3)

Marrow blasts
Karyotypes
cytopenia

23

What is 5q syndrome? What are the three blood anomalies? Who gets this?

MDS subtype more common in older women

macrocytic anemia
Leukopenia
Platelet count normal to increase

24

What is the usual cause of death with MDS?

Infection

25

What is the prognosis for 5q syndrome?

(favorable)--Transformation to AML is less common that other subtypes of MDS

but require transfusions

26

What do you treat MDS with?

Lenalidomide

27

What is CMML?

Form of MDS/MPD, characterized by increased monocytes in BM and pS

28

What is the treatment for CMML?

Imatinib

29

What is hypocellular MDS?

MDS that has the same clinical features as hypercellular MDS

Resembles aplastic anemia

30

What is the low intensity treatment for MDS?

Supportive care
Transfusions PRN
Growth factor support

31

What is the high intensity treatment for MDS?

Stem cell transplant
Chemo
Clinical trials of novel therapies

32

What are the growth factors used for MDS treatment?

G-CSF or GM-CSF


EPO if low Hb

33

What is the drug that is used to treat 5q syndrome?

Lenalidomide

34

What are the two drugs that are used to treat MDS outside of 5q syndrome?

Hypomethylating agents like azacytidine and decitabine

35

What is the only cure to MDS?

Allogenic stem cell transplant

36

What can MDS turn into if untreated?

Leukemia

37

What are MPD?

Overproduction of one or more blood cell lines, but normal maturation

38

Is hematopoiesis effective in MPD?

yes

39

What can MPD turn into if left untreated?

Leukemia

40

What are the four myeloproliferative disorders?

CML
Polycythemia vera
Essential thrombocytosis
Primary myelofibrosis

41

What is the gene mutation that causes MPD?

JAK2 mutations (tyrosine kinase inhibitor)

42

What is the cause of CML?

t(9;22) BCR-ABL

43

What are the ssx of CML?

Fatigue, night sweats

Anemia
Platelet dysfunction
Neutrophilia

44

What are the labs outside of the CBC that are elevated with CML?

LDH and uric acid

45

What are the CBC results in CML?

Neutrophilia
Absolute basophilia (invariable)
Eosinophilia

46

What is the chronic phase of CML?

Peripherl bone marrow blasts

BCR-ABL rearrangement

47

What is the accelerated phase of CML?

Essentially leukemia
Bloood or bone marrow blasts >10%

48

What is the blast phase f CML?

Blood or marrow blasts >20%

49

How do you diagnose CML?

Cytogenetics/FISH
bone marrow biopsy

50

What is the treatment for CML?

Imatinib

51

What are the second generation Y-kinase inhibitors?

Dasatinib
Nilotinib

52

What is the cause of imatinib failure? How do you overcome this?

Development of mutations in the ABL kinase domain

Increase dose or change meds

53

What is polycythemia vera?

Elevated RBC mass in the absence of secondary conditions

54

What happens to WBCs and platelets in polycythemia vera?

Also increases

55

What are the symptoms of polycythemia vera?

Pruritus

56

Who usually has polycythemia vera?

Older males

57

What is the cause of polycythemia vera?

JAK2 mutation

58

What are the signs of polycythemia vera?

HTN
HSM
gout
PHTN

59

What are the major criteria for polycythemia vera?

Hg >18.5 in men (>16.5 women)

Presence of JAK2 mutation

60

What are the minor criteria for polycythemia vera?

Bone marrow with hypercellularity
Serum EPO below reference range for normal

61

What is needed to diagnose polycythemia vera?

Both major criteria and 1 minor,

62

What is the treatment for polycythemia vera?

ASA
Phlebotomy

63

What is the HCT goal with polycythemia vera?

64

What is the drug that can be used for polycythemia vera if not prego?

Hydroxyurea

65

What is anagrelide?

Drug that selectively inhibits platelet production

66

What is essential thrombocytosis? Who get is?

Isolated increase in platelet count

Older women

67

What is the genetic cause of essential thrombocytosis?

JAK2 mutation

68

What are the SSx of essential thrombocytosis?

High Hb
Neutrophilia

Thrombosis or hemorrhage, usually GI tract

69

What is the problem with essential thrombocytosis?

First trimester spontaneous abortions (but rare)

70

What are the major criteria of essential thrombocytosis?

1. Sustained high platelet count
2. bone marrow biopsy with proliferation of megakaryocytes
3. Not polycythemia vera or other causes
4. JAK2 mutation

71

What is the treatment of essential thrombocytosis?

Hydrea
Anagrelide or IFN-alpha

72

What is the goal platelet count for essential thrombocytosis if symptomatic?

73

Which MPD has the worst prognosis?

Primary myelofibrosis

74

What is primary myelofibrosis?

FIbrotic changes of one marrow

75

What are the ssx of primary myelofibrosis? (4)

Marrow fibrosis
HSM
Portal HTN
Hemochromatosis

76

What is the treatment for primary myelofibrosis?

Palliative and supportive

JAK2 inhibitors
Deferoxamine