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Hematology and Oncology > EXAM #3: MDS/MPD > Flashcards

EXAM #3: MDS/MPD Flashcards

1
Q

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

A

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*

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2
Q

What is the major etiology of MDS?

A

Most myelodysplasias result from de novo mutations

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3
Q

What causes secondary MDS?

A
  • Genetic factors
  • Environmental factors
  • Antineoplastic drugs
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4
Q

What are the signs and symptoms of MDS?

A
  • Anemia
  • Infection
  • Bleeding
  • Organomegaly/ Lymphadenopathy
  • Sweet’s Syndrome
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5
Q

What is Sweet’s Syndrome?

A

Acute febrile neutrophilic dermatosis

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6
Q

How is MDS diagnosed?

A

Bone marrow biopsy

PBS

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7
Q

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

A

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

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8
Q

List the differential diagnoses of MDS.

A
  • Vitamin B12 and Folate Deficiency
  • Viral infection e.g. HIV
  • Chemotherapy
  • Alcohol
  • Lead toxicity
  • Benzene
  • Erythropoietic agents e.g. EPO or G-CSF
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9
Q

What types of chemotherapeutic agents cause MDS?

A
  • Alkylating agents

- Topoisomerase inhibitors

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10
Q

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

A

Topoisomerase inhibitors

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11
Q

Which class of chemotheraputic drugs more commonly leads to MDS?

A

Alkylating agents

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12
Q

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

A

Subtype of MDS that causes:

  • Macrocytic anemia
  • Luekopenia
  • Platelet counts elevated

This is seen in FEMALES

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13
Q

What is the prognosis for 5q Syndrome?

A

Good

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14
Q

What drug is used to treat 5q Syndrome?

A

Lenalidomide

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15
Q

What is CMML?

A

Chronic Myelomonocytic Leukemia

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16
Q

What are the characteristics of CMML?

A
  • Increased monocytes

- Variable degrees of dysplasia

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17
Q

What drugs can be used to treat CMML?

A

Imatinib

Azaytidine

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18
Q

What is hypocellular MDS?

A

MDS with clinical features that resemble aplastic anemia

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19
Q

What is low intensity treatment for MDS?

A
  • Supportive care
  • Transfusion
  • Growth factor support
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20
Q

What is high intensity treatment for MDS?

A
  • Stem cell transplant
  • Chemotherapy
  • Clinical trials
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21
Q

What are the “growth factors” that are used to treat MDS?

A

G-CSF
GM-CSF
EPO

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22
Q

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

A

When EPO level is already less than 500

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23
Q

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

A

Hypomethylating agents i.e. Azacytidine

24
Q

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

A

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

25
Q

What is a myeloproliferative disorder?

A

Overproduction of one or more blood cell line

26
Q

What are the classic myeloproliferative disorders?

A

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

27
Q

What mutation is seen with PV and ET?

A

JAK2

28
Q

What is the mutation seen in CML?

A

t(9:22) BCR-ABL

29
Q

What are the symptoms of CML?

A 
Fatigue 
Night sweats 
Weight loss 
Splenomegaly 
Anemia 
Platelet dysfunction
30
Q

What are the labs that are characteristic of CML?

A

1) BASOPHILIA
2) Leukocytosis
3) Neutrophilia

31
Q

What are the three phases of CML?

A

Chronic
Accelerated
Blast

32
Q

What are the characteristics of the chronic phase of CML?

A

Basophilia
Blasts less than 10
BCR-ABL

33
Q

What is the hallmark of the acclerated phase of CML?

A

Worsened splenomegaly

34
Q

What is the blast phase of CML?

A

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

35
Q

How is CML diangosed?

A

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

36
Q

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

A

Imatinib, a tyrosine kinase inhibitor

37
Q

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

A

Dasatinib

38
Q

What causes resistance to Imatinib?

A

Mutations in the ABL kinase domain

39
Q

What is Polycythemia Vera?

A

Elevated RBC mass with concomitant increase in WBC and platelets

40
Q

What are the symptoms associated with PV?

A

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

41
Q

What are the major diagnostic criteria for PV?

A
  • High Hb with JAK2 mutation

- 18.5 in men, and 16.5 women

42
Q

What are the minor criteria for PV?

A
  • Bone marrow hypercellulatiry

- Low serum EPO

43
Q

What does diagnosis of PV require?

A
  • BOTH major and 1 minor

- 1 major and BOTH minor

44
Q

What is the first line treatment for PV?

A

Phlebotomy and low dose ASA

45
Q

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

A

Cytoreductive therapy i.e. hydroxyurea

46
Q

When is hydroxurea contraindicated?

A

Pregnancy

47
Q

What is Essential Thrombocytosis?

A

Isolated increased in platelet count

48
Q

What are the clinical features of ET?

A
  • Visual disturbance
  • Dizziness
  • HA
  • Erythromelalgia
  • Cutaneous ulcers
  • Thrombosis (venous or arterial)
  • Hemorrhage
49
Q

What are the diagnostic criteria for ET?

A

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

50
Q

What are the cause of secondary thrombocytosis?

A 
Iron deficiency 
Infection
Inflammation
Surgery 
Trauma 
Tissue injury/infarction
Malignancy
S/p splenectomy
51
Q

How is ET treated?

A

Hydroxyurea–first line

Anagrelide

52
Q

What is Primary Myelofibrosis?

A

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

53
Q

What is the worst of the myeloproliferative disorders?

A

Primary myelofibrosis

54
Q

What are the hallmarks of Primary Myelofibrosis?

A
  • Bone marrow fibrosis

- Extramedullary hematopoesis

55
Q

What are the clinical features of Primary Myelofibrosis?

A

Splenogmegaly

56
Q

How is Primary Myelofibrosis treated?

A

Palliation and support

57
Q

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

A

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

Hematology and Oncology (38 decks)

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