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
