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Liz's Hematology Module 2 > Myelodysplastic Syndromes (MDSs) > Flashcards

Myelodysplastic Syndromes (MDSs) Flashcards

1
Q

Group of acquired clonal hematologic disorder that affect maturation of erythroid, myeloid, and/or megakaryocytic lines
- PROGRESSIVE PB CYTOPENIAS (MAY EVENTUALLY PROGRESS TO AML)

A

Myelodysplastic Syndromes (MDSs)

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2
Q

Mechanism of MDSs

A

Hypomethylation of DNA followed by hypermethylation of DNA

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3
Q

Which MDS mechanism leads to reduced transcription leading to cytopenias?

A

Hypomethylation of DNA

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4
Q

Which MDS mechanism leads to enhanced transcription leading to an acute leukemia (usually AML)

A

Hypermethylation of DNA

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5
Q

MDS PB findings

A
  • Cytopenias
  • Anemia (N/N or macrocytic anemia)
  • Dysplasia
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6
Q

MDS BM findings

A
  • Hypercellular
  • Dysplasia
  • Ineffective hematopoiesis
  • M/E ratio decreased
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7
Q

Is flow cytometery and cytogenetics diagnostic for MDS?

A

NO! Important for providing prognostic risks and therapy but not diagnostic

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8
Q

Criteria for calling ringed sideroblasts in the erythrocyte line

A

At least 5 iron granules that circle at least 1/3 of the nucleus

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9
Q

What are the most common lab findings in MDS?

A

Macrocytic or normocytic anemia (w/ low retics)

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10
Q

Differentiating MDS from other conditions:

- MDS may present with ____ ____ RBCs but still have normal iron stores

A

Microcytic/hypochromic

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11
Q

Differentiating MDS from other conditions:

- MDS may present w/ megaloblastoid cellular development but still have ____ vitamin B12 and folate values

A

Normal

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12
Q

Most common MDSs

A
  • Refractory Anemia w/ Excess Blasts (RAEB-1 and 2)
  • Refractory Cytopenia w/ Multilineage Dysplasia
  • Refractory Cytopenia w/ Unilineage Dysplasia
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13
Q

RAEB-1

  • PB blasts
  • BM blasts
  • Auer rods present?
  • % transform into AML
  • Survival
A
  • PB blasts: < 5%
  • BM blasts: 5-9%
  • Auer rods: NO
  • 25% will transform to AML
  • Survival: 16 months
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14
Q

RAEB-2

  • PB blasts
  • BM blasts
  • Auer rods present?
  • % transform into AML
A
  • PB blasts: 5-19%
  • BM blasts: 10-19%
  • Auer rods: Possibly
  • 33% will transform into AML
  • Survival: 9 months
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15
Q

MDS w/ Isolated del(5q)

  • What deletion?
  • Predominately in what sex?
  • What anemia is characteristic?
A
  • Deletion of a 5q presumed tumor suppressor gene
  • Predominately in women
  • Macrocytic anemia is characteristic
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16
Q

What % of BM erythroid precursors are ringed sideroblasts in RARS (refractory anemia w/ ringed sideroblasts)?

A

More than 15%

17
Q

How do you disitnguish myelodysplastic/myeloproliferative neoplasms from CML?

A
  • BCR-ABL1 NEGATIVE
  • Persistent monocytosis > 1.0 x 10^9/L
  • Granulocytes < 10% of differential
18
Q

One specific type of a myelodysplastic/myeloproliferative neoplasm

A

Juvenile Myelomonocytic Leukemia

19
Q

How do you score the prognosis for a MDS?

A

International Prognostic Scoring System (IPSS)

20
Q

International Prognostic Scoring System

  • % BM blasts
  • Karyotype
  • # of cytopenias
A

< 2 is good; higher than 2 is bad

  • More blasts is bad
  • More aberrations is worse
  • More cell lines shwoing cytopenai is worse
21
Q

MDS

- Treatment

A

Lenalidomide and farnesyltransferase inhibitors

22
Q

What prognostic score and what age must the patient be to receive the “cure” for an MDS?

A
  • “cure” = stem cell transplant
  • Score: > 2 IPSS
  • Age: < 70
23
Q

MDS etiology

A
  • Somatic mutation
  • Production of a pathologic clone
  • Clonal expansion
  • Possible further mutations
  • Disruped apoptosis early in the disease could lead to increased apoptosis as disease progresses
24
Q

Morphological features of dyserthropoiesis

A
  • Oval macrocytes
  • Hypochromatic microcytes
  • Dimorphic RBC population
  • RBC precursors w/ more than one nucleus
  • Abnormal nuclear shapes (budding, bridging, hyperlobation, megaloblastic changes)
  • Uneven cytoplasmic staining
  • Ringed sideroblasts
  • Vacuolization
  • PAS positivity
25
Q

Morphological features of dysmyelopoiesis

A
  • Persistant basophilic cytoplasm
  • Hypogranulation, agranulation, pseudo Chediak-Higashi granules, abnormally sized primary and secondary granules
  • Pseudo Pelger-Huet, irregular hypersegmentation
  • Uneven cytoplasmic staining
26
Q

Morphological features of dysmegakaryopoiesis

A
  • Giant platelets
  • Abnormal granulation, hypogranulation or agranulation
  • Micromegakaryocytes
  • Large mononuclear megakaryocytes
  • Bilobed or multiple small separated nuclei
27
Q

Abnormal cellular function associated w/ dyserythropoiesis

A
  • RBCs have shortened lifespan

- Erythroid precursors may have decreased sensitivity to EPO leading to anemia

28
Q

Abnormal cellular function associated w/ dysmyelopoiesis

A
  • Granulocytes have ↓ adhesion
  • Granulocytes have deficient phagocytosis
  • Granulocytes have ↓ chemotaxis
  • Granulocytes have ↓ levels of MPO and alkaline phosphatase
29
Q

Differentiating MDS from other conditions:

- MDS may present w/ myelodysplasia but have normal ____ levels

A

Copper

30
Q

Differentiating MDS from other conditions:

- MDS may present w/ hypersegmented polys but still have normal ____ and ____ values

A

Vitamin B12; Folate

31
Q

Abnormal cellular function associated w/ dysmegakaryopoiesis

A

Platelets may have increased bleeding depsite adequate platelet counts

32
Q

RARS PB findings

A
  • Anemia

- No blasts

33
Q

RARS BM findings

A
  • ≥15% of erythroid precurosrs are ringed sideroblasts
  • Erythroid dysplasia only
  • < 5% blasts
34
Q

MDS associated w/ isolated del(5q) PB findings

A
  • Anemia
  • Usually normal or increased platelet count
  • Nor or rare blasts (< 1%)
35
Q

MDS associated w/ isolated del(5q) BM findings

A
  • Normal to ↑ megakaryocytes w/ hypolobated nuclei
  • < 5% blasts
  • Isolated del(5q) cytogenic abnormality
  • No Auer rods
36
Q

MDSs are most common in which age group?

A

> 50 years old

37
Q

What is a major indication of MDS in the PB and BM?

A

Dyspoiesis

38
Q

According to the WHO classification of MDS, what percentage of blasts would constitute transformation into an acute leukemia?

A

20%

39
Q

MDS most likely will transform into acute ____ leukemia

A

Myeloid

Liz's Hematology Module 2 (13 decks)

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