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Hematology > Myeloproliferative and myelodyplasia > Flashcards

Myeloproliferative and myelodyplasia Flashcards

1
Q

Who is usually diagnosed with MDS?

A

Older

Women

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2
Q

What is the phenotype of MDS?

A

Peripheral cytopenias with normocellular or hypercellular bone marrow

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3
Q

Those with MDS have an increase risk for what?

A

Acute leukemia

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4
Q

What is the cause of primary MDS?

A

Unclear

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5
Q

What are the causes of secondary MDS?

A

Genetic factors
Environmental factors
Antineoplastic agents

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6
Q

HOw do you characterize MDS?

A

What cell line is decreased

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7
Q

Leukemia is caused by bad type of what cells?

A

Blasts

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8
Q

SSx of MDS?

A

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

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9
Q

What is sweet syndrome?

A

Acute neutrophilic dermatosis and pyoderma gangrenosum

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10
Q

What is the cause of sweet syndrome?

A

Unknown

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11
Q

How do you confirm MDS?

A

Bone marrow biopsy with normo or hypercellular bone marrow

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12
Q

What is trilineage dyspoiesis?

A

Abnormal WBC, RBC, platelet precursors

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13
Q

What is the value of Fe studies in MDS?

A

r/o Fe deficiency anemia

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14
Q

What is pseudo-Pelger huet anomaly?

A

Bilobed nucleus with a string between, characteristic of MDS

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15
Q

Ringed sideroblasts = ?

A

MDS or sideroblastic anemia

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16
Q

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

A

yes

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17
Q

True or false: ethanol exposure cannot cause bone marrow suppression

A

False

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18
Q

What is the function of G-CSF?

A

Macrophage released cytokine that increases PMNs

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19
Q

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

A

Alkylating agents

Topoisomerase inhibitors

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20
Q

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

A

Alkylating agents

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21
Q

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

A

Topoisomerases

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22
Q

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

A

Marrow blasts
Karyotypes
cytopenia

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23
Q

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

A

MDS subtype more common in older women

macrocytic anemia
Leukopenia
Platelet count normal to increase

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24
Q

What is the usual cause of death with MDS?

A

Infection

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25
Q

What is the prognosis for 5q syndrome?

A

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

but require transfusions

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26
Q

What do you treat MDS with?

A

Lenalidomide

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27
Q

What is CMML?

A

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

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28
Q

What is the treatment for CMML?

A

Imatinib

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29
Q

What is hypocellular MDS?

A

MDS that has the same clinical features as hypercellular MDS

Resembles aplastic anemia

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30
Q

What is the low intensity treatment for MDS?

A

Supportive care
Transfusions PRN
Growth factor support

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31
Q

What is the high intensity treatment for MDS?

A

Stem cell transplant
Chemo
Clinical trials of novel therapies

32
Q

What are the growth factors used for MDS treatment?

A

G-CSF or GM-CSF

EPO if low Hb

33
Q

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

A

Lenalidomide

34
Q

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

A

Hypomethylating agents like azacytidine and decitabine

35
Q

What is the only cure to MDS?

A

Allogenic stem cell transplant

36
Q

What can MDS turn into if untreated?

A

Leukemia

37
Q

What are MPD?

A

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

38
Q

Is hematopoiesis effective in MPD?

A

yes

39
Q

What can MPD turn into if left untreated?

A

Leukemia

40
Q

What are the four myeloproliferative disorders?

A

CML
Polycythemia vera
Essential thrombocytosis
Primary myelofibrosis

41
Q

What is the gene mutation that causes MPD?

A

JAK2 mutations (tyrosine kinase inhibitor)

42
Q

What is the cause of CML?

A

t(9;22) BCR-ABL

43
Q

What are the ssx of CML?

A

Fatigue, night sweats

Anemia
Platelet dysfunction
Neutrophilia

44
Q

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

A

LDH and uric acid

45
Q

What are the CBC results in CML?

A

Neutrophilia
Absolute basophilia (invariable)
Eosinophilia

46
Q

What is the chronic phase of CML?

A

Peripherl bone marrow blasts

BCR-ABL rearrangement

47
Q

What is the accelerated phase of CML?

A

Essentially leukemia

Bloood or bone marrow blasts >10%

48
Q

What is the blast phase f CML?

A

Blood or marrow blasts >20%

49
Q

How do you diagnose CML?

A

Cytogenetics/FISH

bone marrow biopsy

50
Q

What is the treatment for CML?

A

Imatinib

51
Q

What are the second generation Y-kinase inhibitors?

A

Dasatinib

Nilotinib

52
Q

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

A

Development of mutations in the ABL kinase domain

Increase dose or change meds

53
Q

What is polycythemia vera?

A

Elevated RBC mass in the absence of secondary conditions

54
Q

What happens to WBCs and platelets in polycythemia vera?

A

Also increases

55
Q

What are the symptoms of polycythemia vera?

A

Pruritus

56
Q

Who usually has polycythemia vera?

A

Older males

57
Q

What is the cause of polycythemia vera?

A

JAK2 mutation

58
Q

What are the signs of polycythemia vera?

A

HTN
HSM
gout
PHTN

59
Q

What are the major criteria for polycythemia vera?

A

Hg >18.5 in men (>16.5 women)

Presence of JAK2 mutation

60
Q

What are the minor criteria for polycythemia vera?

A

Bone marrow with hypercellularity

Serum EPO below reference range for normal

61
Q

What is needed to diagnose polycythemia vera?

A

Both major criteria and 1 minor,

62
Q

What is the treatment for polycythemia vera?

A

ASA

Phlebotomy

63
Q

What is the HCT goal with polycythemia vera?

A
64
Q

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

A

Hydroxyurea

65
Q

What is anagrelide?

A

Drug that selectively inhibits platelet production

66
Q

What is essential thrombocytosis? Who get is?

A

Isolated increase in platelet count

Older women

67
Q

What is the genetic cause of essential thrombocytosis?

A

JAK2 mutation

68
Q

What are the SSx of essential thrombocytosis?

A

High Hb
Neutrophilia

Thrombosis or hemorrhage, usually GI tract

69
Q

What is the problem with essential thrombocytosis?

A

First trimester spontaneous abortions (but rare)

70
Q

What are the major criteria of essential thrombocytosis?

A
  1. Sustained high platelet count
  2. bone marrow biopsy with proliferation of megakaryocytes
  3. Not polycythemia vera or other causes
  4. JAK2 mutation
71
Q

What is the treatment of essential thrombocytosis?

A

Hydrea

Anagrelide or IFN-alpha

72
Q

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

A
73
Q

Which MPD has the worst prognosis?

A

Primary myelofibrosis

74
Q

What is primary myelofibrosis?

A

FIbrotic changes of one marrow

75
Q

What are the ssx of primary myelofibrosis? (4)

A

Marrow fibrosis
HSM
Portal HTN
Hemochromatosis

76
Q

What is the treatment for primary myelofibrosis?

A

Palliative and supportive

JAK2 inhibitors
Deferoxamine

Hematology (32 decks)

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