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Hematology > Hematology Week 3: Myelodysplastic Syndrome > Flashcards

Hematology Week 3: Myelodysplastic Syndrome Flashcards

1
Q

Myelodysplasia Definition

A

abnormal appearance of myeloid lineage

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2
Q

Myelodysplasia Syndrome is a

A
  • hematological malignancy
  • clonal stem cell disorder
  • characterized by dysplasia ineffective hematopoiesis
  • increased risk of AML (“preleukemia”)
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3
Q

Myelodysplasia Syndrome is characterized by?

A
  • Cytopenias
  • ineffective hematopoiesis
  • dysplasia
    *
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4
Q

Myelodysplasia Syndrome is a clonal stem cell disorder meaning that?

A

BM is replaced by clonal cells

Progeny of an abnormal clonal multipotent stem cell

  • cytogenetic abnormalities
  • gene mutations
  • epigenetic changes

retains ability to differentiate

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5
Q

Myelodysplasia Syndrome Pathogenesis

A
  • a normal stem cell gains some type of insult which can cause increased apoptosis cells don’t survive as well as usual
  • cells accrue more genetic alterations that can lead to increased cell survival and reduced apoptosis
  • leads to AML
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6
Q

Myelodysplasia Syndrome Clinical Presentations

3 listed

A
  • Cytopenias
  • Dysplasia
  • Ineffective Hematopoiesis
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7
Q

Dx of Myelodysplasia Syndrome

4 listed

A
  • Cytopenias
  • Evidence of dysplasia
  • Careful assessment of blasts <20%
  • Exclude causes of secondary dysplasia (vitamin deficiency, HIV+, etc..)
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8
Q

Patients with Myelodysplasia Syndrome have increased risk for?

3 listed

A

10-40% will progress to AML

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9
Q

Epidemiology of MDS

A

more common in med except for del(5q) subtype in females

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10
Q

Erythroid Dysplasia in Myelodysplasia Syndrome

2 listed

A
  • Erythroid multinucleation
  • Ring sideroblasts
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11
Q

Neutrophil Dysplasia in Myelodysplasia Syndrome

2 listed

A
  • Hypolobation (2 nuclei pseudo-paltrow hewwit cells)
  • Hypogranulation
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12
Q

Platelet Dysplasia in Myelodysplasia Syndrome

4 listed

A
  • Giant platelets
  • hypogranular platelets
  • Hypolobated megakaryocytes
  • Multinucleated megakaryocyte
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13
Q

Identify Dysplasia

A

Erythroid Multinucleation

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14
Q

Identify Dysplasia

A

Ring sideroblasts

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15
Q

Identify Dysplasia

A

Neutrophil hypolobation

Pseudo-Pelger-Huet cells

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16
Q

Identify Dysplasia

A

Neutrophil hypogranulation

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17
Q

Identify Dysplasia

A

abnormal granules and blasts

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18
Q

Identify Dysplasia

A

Giant Platelets

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19
Q

Identify Dysplasia

A

Hypogranular platelets

20
Q

Identify Dysplasia

A

Hypolobated Megakaryocytes

21
Q

Identify Dysplasia

A

Multinucleated Megakaryocytes

AKA Pon ball megakaryocyte

22
Q

List of dysplasias in Myelodysplasia Syndrome

8 listed

A
  • Erythroid multinucleation
  • Erythroid Ring sideroblasts
  • Neutrophil Hypolobation
  • Neutrophil Hypogranulation
  • Giant Platelets
  • Hypogranular platelets
  • Hyperlobated Megakaryocytes
  • Multinucleate Megakaryocytes
23
Q

Non-neoplastic Differential Dx of Myelodysplasia Syndrome

7 listed

A
24
Q

Vitamin/Nutrient Deficiency

6 listed

A
25
Q

denture cream has ______ and can cause ________.

A
  • zinc
  • copper deficiency
26
Q

Infection as a diff dx of myelodysplasia syndrome

A
27
Q

Tests for Parvo Virus

2 listed

A

Parvo virus titers/PCR

28
Q

Tests for HIV

2 listed

A

HIV serology/NAT

29
Q

Tests for Hepatitis

2 listed

A
  • HSV
  • CMV
    *
30
Q

Tests for EBV

2 listed

A
  • EBV serology
  • NAT (Nucleic acid Testing)
31
Q

Toxins as a diff dx of myelodysplasia Syndrome

A
  • liver function tests
  • coagulation studies
  • Lead levels (lead exposure)
32
Q

The leading cause of macrocytosis in adults

A

Alcohol use

33
Q

Neoplastic Differential Dxs of Myelodysplasia Syndrome

7 listed

A

Myeloproliferative neoplasms

Acute Myeloid Leukemia

34
Q

Revised International Prognostic Scoring System (IPSS-R)

A
  • Assigns a risk category that correlates with survival and risk of transformation to AML
  • most important component by far is the cytogenetic abnormalities
35
Q

cytogenetic abnormalities that are looked for

5 most common

A

These are the most common

  • del(20q)
  • Del(7q)
  • Trisomy 8
  • i(17q)
  • del(5q) - women
36
Q

Y chromosome as men age

A

men can lose their Y chromosome as they age doesn’t mean they have a neoplasm

37
Q

IPSS-R prognostic score values

A
38
Q

Treatment of Myelodysplasia Syndrome Low Risk

A
  • Growth Factors
  • Supportive Care
39
Q

Treatment of Myelodysplasia Syndrome High Risk

2 listed

A
  • Induction chemotherapy
  • HSCT
  • if these treatments can be tolerated as most patients are >70
40
Q

Supportive care/Growth Factors for Treatment of Myelodysplasia Syndrome

7 listed

A
  • if someone is not very symptomatic and their risk score is low can try to decrease the symptoms
  • If having anemia with fatigue may consider transfusing them
  • if transfusion dependent but don’t meet criteria for aggressive therapy or cannot tolerate it then consider EPO
  • if they are thrombocytopenic and are bleeding or if platelets are very low will give platelet transfusions
  • If they have neutropenia then infection control (antibiotics) and GCSF for neutrophil count
41
Q

Immune Treatments for Treatment of Myelodysplasia Syndrome

A

Thalidomide/Lenalidomide

42
Q

Thalidomide/Lenalidomide

A

Decrease transfusion need

most effective in patients with del(5q)

43
Q

Pharmacology of thalidomide

A

Angiogenesis inhibitor and immunomodulator

44
Q

Chemotherapy in MDS

A

patients with high-risk disease

Conventional chemotherapy

  • Cytarabine (ara-C)

hypomethylating agents

  • Azacitidine
  • Decitabine
  • Decrease cell proliferation
  • improve blood counts
45
Q

Only curative treatment option for MDS

A

HSCT usually for healthy younger patients and is the only curative option

Hematology (26 decks)

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