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Hematology > Hematology Week 3: Myelodysplastic Syndrome > Flashcards

Hematology Week 3: Myelodysplastic Syndrome Flashcards

(45 cards)

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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

Study These Flashcards
A

Hypogranular platelets

20
Q

Identify Dysplasia

Study These Flashcards
A

Hypolobated Megakaryocytes

21
Q

Identify Dysplasia

Study These Flashcards
A

Multinucleated Megakaryocytes

AKA Pon ball megakaryocyte

22
Q

List of dysplasias in Myelodysplasia Syndrome

8 listed

Study These Flashcards
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

24
Q

Vitamin/Nutrient Deficiency

6 listed

25
denture cream has ______ and can cause \_\_\_\_\_\_\_\_.
* zinc * copper deficiency
26
Infection as a diff dx of myelodysplasia syndrome
27
Tests for Parvo Virus 2 listed
Parvo virus titers/PCR
28
Tests for HIV 2 listed
HIV serology/NAT
29
Tests for Hepatitis 2 listed
* HSV * CMV *
30
Tests for EBV 2 listed
* EBV serology * NAT (Nucleic acid Testing)
31
Toxins as a diff dx of myelodysplasia Syndrome
* liver function tests * coagulation studies * Lead levels (lead exposure)
32
The leading cause of macrocytosis in adults
Alcohol use
33
Neoplastic Differential Dxs of Myelodysplasia Syndrome 7 listed
Myeloproliferative neoplasms Acute Myeloid Leukemia
34
Revised International Prognostic Scoring System (IPSS-R)
* Assigns a risk category that correlates with survival and risk of transformation to AML * most important component by far is the cytogenetic abnormalities
35
cytogenetic abnormalities that are looked for 5 most common
These are the most common * del(20q) * Del(7q) * Trisomy 8 * i(17q) * del(5q) - women
36
Y chromosome as men age
men can lose their Y chromosome as they age doesn't mean they have a neoplasm
37
IPSS-R prognostic score values
38
Treatment of Myelodysplasia Syndrome Low Risk
* Growth Factors * Supportive Care
39
Treatment of Myelodysplasia Syndrome High Risk 2 listed
* Induction chemotherapy * HSCT * if these treatments can be tolerated as most patients are \>70
40
Supportive care/Growth Factors for Treatment of Myelodysplasia Syndrome 7 listed
* 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
Immune Treatments for Treatment of Myelodysplasia Syndrome
Thalidomide/Lenalidomide
42
Thalidomide/Lenalidomide
Decrease transfusion need most effective in patients with del(5q)
43
Pharmacology of thalidomide
Angiogenesis inhibitor and immunomodulator
44
Chemotherapy in MDS
patients with high-risk disease Conventional chemotherapy * Cytarabine (ara-C) hypomethylating agents * Azacitidine * Decitabine * Decrease cell proliferation * improve blood counts
45
Only curative treatment option for MDS
HSCT usually for healthy younger patients and is the only curative option

Hematology (26 decks)

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