EXAM #3: MDS/MPD Flashcards Preview

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