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