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Hematology > Myelodysplasias > Flashcards

Myelodysplasias Flashcards

1
Q

Things that should make you suspicious of Myelodysplasia…Epidemiology

A

Age…usually presents in elderly patients

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2
Q

Things that should make you suspicious of Myelodysplasia…CBC

A

usually unexplained cytopenia, bicytopenia, or pancytopenia

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3
Q

Things that should make you suspicious of Myelodysplasia…Diagnostic features

A

Abnormal “dyspoietic” bone marrow morphologies

Abnormal “dyspoietic” immunophenotypes of maturing precursors

Abnormal cytogenetics

Increased morphologic blasts (>5% but <20%)

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4
Q

Things that should make you suspicious of Myelodysplasia…Clinical progression

A

Some are high risk for progressing to acute leukemia…others low risk

Some the condition doesn’t affect survival and therapies would do more harm than good…others there are effective drugs

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5
Q

5 major adult forms (best to worst prognosis)

A
  1. Refractory cytopenia w/ unilineage dysplasia
  2. Refractory anemia w/ ring sideroblasts
  3. Myelodysplastic syndrome w/ isolated del(5q)
  4. Refractory cytopenia w/ multilineage dysplasia
  5. Refractory anemia w/ excess blasts
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6
Q

Refractory Cytopenia w/ unilineage dysplasia…Morphological findings

A

Usually cytogenetic abnormalities (i.e. trisomies, monosomies), BUT sometimes cytogenetics are normal

Weird looking precursors
binucleation or irregular nuclei
can show fibrosis
high or low cellularity
megaloblastoid features (nonspecific)
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7
Q 
Random Note card
Megaloblastoid features (differential diagnosis)
A

nutritional deficiencies
drugs
HIV
Refractory cytopenia w/ unilineage dysplasia

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8
Q

Refractory Cytopenia w/ unilineage dysplasia…Clinical Presentation

A

Unexplained cytopenias

usually elderly pts (>65-yrs)

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9
Q

Refractory Cytopenia w/ unilineage dysplasia…Immunophenotype

A

Can show abnormal acquisition of surface markers

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10
Q

Refractory Cytopenia w/ unilineage dysplasia…Prognosis

A

Survival not clearly less than normal for age…

Rarely progresses to AML

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11
Q

Refractory anemia w/ ring sideroblasts…Diagnosis

A

Morphological findings + iron stain

Nonspecific cytogenetic abnormalities may be present

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12
Q

Refractory anemia w/ ring sideroblasts…Clinical Presentation

A

Unexplained cytopenias

usually elderly pts

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13
Q

Refractory anemia w/ ring sideroblasts…Morphology

A

Ring sideroblasts usually w/ dyspoietic features (in red cell series ONLY)

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14
Q

Refractory anemia w/ ring sideroblasts…Immunophenotpe

A

Can show abnormal acquisition of surface markers

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15
Q

Refractory anemia w/ ring sideroblasts…Prognosis

A

Survival not clearly less than normal for age….Rarely progresses to AML

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16
Q

MDS w/ isolated del(5q)…Key characteristics

A

ALL the megakaryocytes are mononuclear

Cytogenetics shows ONLY loss of large arm of chromosome 5!

17
Q

MDS w/ isolated del(5q)…Clinical Presentation

A

Anemia (often severe)
usually elderly pts
more often women

18
Q

MDS w/ isolated del(5q)…Morphology

A

all the megs are mononuclear

19
Q

MDS w/ isolated del(5q)…Immunophenotype

A

No known findings

20
Q

MDS w/ isolated del(5q)…Prognosis

A

Good median survival
treatable w/ lenalidomide
10% progress to AML

21
Q

Refractory cytopenia w/ multilineage dysplasia…Key Features

A

Two or more lineages show dysplastic changes

About half show nonspecific cytogenetic abnormalities

22
Q

Refractory cytopenia w/ multilineage dysplasia…Clinical Presentation

A

Anemia (often severe)
Usually elderly pts
more often women

23
Q

Refractory cytopenia w/ multilineage dysplasia…Morphology

A

Granulocytes (if affected) don’t granulate normally

nuclei don’t lobulate normally

24
Q

Refractory cytopenia w/ multilineage dysplasia…Immunophenotype

A

Can show abnormal acquisition of surface markers

25
Q

Refractory cytopenia w/ multilineage dysplasia…Prognosis

A

Median survival 30 months

10% progression to AML in 2 yrs

26
Q

Refractory anemia w/ excess blasts…Key features

A

5-9% morphologic blasts (RAEB-1)
10-19% morphologic blasts (RAEB-2)

about half show nonspecific cytogenetic abnormalities

“Pre-leukemia”

27
Q

Refractory anemia w/ excess blasts…Clinical Presentation

A

Cytopenias

usually elderly pts

28
Q

Refractory anemia w/ excess blasts…Morphology

A

Blasts and dyspoietic maturation

29
Q

Refractory anemia w/ excess blasts…Immunophenotype

A

Blast Population (CD34+ and/or CD117) usually

30
Q

Refractory anemia w/ excess blasts…Prognosis

A

RAEB-1….25% progress to AML

RAEB-2…33% progress to AML

Hematology (9 decks)

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