EXAM #3: MDS/MPD Flashcards Preview

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Flashcards in EXAM #3: MDS/MPD Deck (57):
1

What is MDS? What are the general characteristics of MDS?

MDS= Myelodysplastic Syndrome
- Dysplasia or bad growth of the bone marrow cells
- Increased number of cells i.e. hypercellular bone marrow with poor products
- Results in various cytopenias (low numbers)
- Blasts less than 20% in the bone marrow

****Greater than 20% blasts= leukemia*****

2

What is the major etiology of MDS?

Most myelodysplasias result from de novo mutations

3

What causes secondary MDS?

- Genetic factors
- Environmental factors
- Antineoplastic drugs

4

What are the signs and symptoms of MDS?

- Anemia
- Infection
- Bleeding
- Organomegaly/ Lymphadenopathy
- Sweet's Syndrome

5

What is Sweet's Syndrome?

Acute febrile neutrophilic dermatosis

6

How is MDS diagnosed?

Bone marrow biopsy
PBS

7

What are some of the cell types seen on PBS that are typical of MDS?

1) Giant platelets
2) Pseudo-Pegler Huet anomaly
3) Ringed Sideroblasts

8

List the differential diagnoses of MDS.

- Vitamin B12 and Folate Deficiency
- Viral infection e.g. HIV
- Chemotherapy
- Alcohol
- Lead toxicity
- Benzene
- Erythropoietic agents e.g. EPO or G-CSF

9

What types of chemotherapeutic agents cause MDS?

- Alkylating agents
- Topoisomerase inhibitors

10

Which class of chemotheraputic drugs can more quickly cause lead to MDS?

Topoisomerase inhibitors

11

Which class of chemotheraputic drugs more commonly leads to MDS?

Alkylating agents

12

What is 5q-Syndrome? What patient population is this seen in?

Subtype of MDS that causes:
- Macrocytic anemia
- Luekopenia
- Platelet counts elevated

****This is seen in FEMALES****

13

What is the prognosis for 5q Syndrome?

Good

14

What drug is used to treat 5q Syndrome?

Lenalidomide

15

What is CMML?

Chronic Myelomonocytic Leukemia

16

What are the characteristics of CMML?

- Increased monocytes
- Variable degrees of dysplasia

17

What drugs can be used to treat CMML?

Imatinib
Azaytidine

18

What is hypocellular MDS?

MDS with clinical features that resemble aplastic anemia

19

What is low intensity treatment for MDS?

- Supportive care
- Transfusion
- Growth factor support

20

What is high intensity treatment for MDS?

- Stem cell transplant
- Chemotherapy
- Clinical trials

21

What are the "growth factors" that are used to treat MDS?

G-CSF
GM-CSF
EPO

22

When does EPO tend to work better in patients with MDS?

When EPO level is already less than 500

23

In patients with MDS, what class of drugs have been shown to decrease time to leukemia and transfusion dependence?

Hypomethylating agents i.e. Azacytidine

24

What is the role of transplant in the treatment of MDS? What type of transplant is curative?

Allogeneic transplant is curative; however, patients have to be less than 40

25

What is a myeloproliferative disorder?

Overproduction of one or more blood cell line

26

What are the classic myeloproliferative disorders?

1) Chronic myeloid leukemia
2) Polycythemia vera
3) Essential thrombocytosis
4) Primary myelofibrosis

27

What mutation is seen with PV and ET?

JAK2

28

What is the mutation seen in CML?

t(9:22) BCR-ABL

29

What are the symptoms of CML?

Fatigue
Night sweats
Weight loss
Splenomegaly
Anemia
Platelet dysfunction

30

What are the labs that are characteristic of CML?

1) BASOPHILIA
2) Leukocytosis
3) Neutrophilia

31

What are the three phases of CML?

Chronic
Accelerated
Blast

32

What are the characteristics of the chronic phase of CML?

Basophilia
Blasts less than 10
BCR-ABL

33

What is the hallmark of the acclerated phase of CML?

Worsened splenomegaly

34

What is the blast phase of CML?

Greater than 20% blasts i.e. transformation to Leukemia

35

How is CML diangosed?

1) BCR-ABL
2) Basophilia
3) Confirm with FISH or RT-BCR

36

What is the primary treatment for CML? What class of drug is this?

Imatinib, a tyrosine kinase inhibitor

37

What is the second line treatment for CML when patients are resistant to Imatinib?

Dasatinib

38

What causes resistance to Imatinib?

Mutations in the ABL kinase domain

39

What is Polycythemia Vera?

Elevated RBC mass with concomitant increase in WBC and platelets

40

What are the symptoms associated with PV?

1) Puritis, especially after bathing
2) Headache
3) Blurry vision
4) Venous thrombosis-->Budd Chiari

41

What are the major diagnostic criteria for PV?

- High Hb with JAK2 mutation
- 18.5 in men, and 16.5 women

42

What are the minor criteria for PV?

- Bone marrow hypercellulatiry
- Low serum EPO

43

What does diagnosis of PV require?

- BOTH major and 1 minor
- 1 major and BOTH minor

44

What is the first line treatment for PV?

Phlebotomy and low dose ASA

45

For patients with PV and intermediate risk/ high risk, what is the second line treatment?

Cytoreductive therapy i.e. hydroxyurea

46

When is hydroxurea contraindicated?

Pregnancy

47

What is Essential Thrombocytosis?

Isolated increased in platelet count

48

What are the clinical features of ET?

- Visual disturbance
- Dizziness
- HA
- Erythromelalgia
- Cutaneous ulcers
- Thrombosis (venous or arterial)
- Hemorrhage

49

What are the diagnostic criteria for ET?

1) Platelet count greater than 450,000
2) BM biopsy with proliferation of megakaryocytic lineage
3) JAK2 mutation or ruled out reactive thrombocytosis

50

What are the cause of secondary thrombocytosis?

Iron deficiency
Infection
Inflammation
Surgery
Trauma
Tissue injury/infarction
Malignancy
S/p splenectomy

51

How is ET treated?

Hydroxyurea--first line
Anagrelide

52

What is Primary Myelofibrosis?

Proliferation of mature myeloid cells i.e. megakaryoctes that produce PDGF, causing bone marrow fibrosis

53

What is the worst of the myeloproliferative disorders?

Primary myelofibrosis

54

What are the hallmarks of Primary Myelofibrosis?

- Bone marrow fibrosis
- Extramedullary hematopoesis

55

What are the clinical features of Primary Myelofibrosis?

Splenogmegaly

56

How is Primary Myelofibrosis treated?

Palliation and support

57

What are the supportive measures that are commonly undertaken in Primary Myelofibrosis?

1) Recombinant EPO
2) Iron chelation
3) Splenectomy
4) JAK2
5) Allo SCT