Myelodysplasias Flashcards Preview

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Flashcards in Myelodysplasias Deck (30)
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1

Things that should make you suspicious of Myelodysplasia...Epidemiology

Age...usually presents in elderly patients

2

Things that should make you suspicious of Myelodysplasia...CBC

usually unexplained cytopenia, bicytopenia, or pancytopenia

3

Things that should make you suspicious of Myelodysplasia...Diagnostic features

Abnormal "dyspoietic" bone marrow morphologies

Abnormal "dyspoietic" immunophenotypes of maturing precursors

Abnormal cytogenetics

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

4

Things that should make you suspicious of Myelodysplasia...Clinical progression

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

5

5 major adult forms (best to worst prognosis)

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

6

Refractory Cytopenia w/ unilineage dysplasia...Morphological findings

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)

7

Random Note card
Megaloblastoid features (differential diagnosis)

nutritional deficiencies
drugs
HIV
Refractory cytopenia w/ unilineage dysplasia

8

Refractory Cytopenia w/ unilineage dysplasia...Clinical Presentation

Unexplained cytopenias
usually elderly pts (>65-yrs)

9

Refractory Cytopenia w/ unilineage dysplasia...Immunophenotype

Can show abnormal acquisition of surface markers

10

Refractory Cytopenia w/ unilineage dysplasia...Prognosis

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

11

Refractory anemia w/ ring sideroblasts...Diagnosis

Morphological findings + iron stain
Nonspecific cytogenetic abnormalities may be present

12

Refractory anemia w/ ring sideroblasts...Clinical Presentation

Unexplained cytopenias
usually elderly pts

13

Refractory anemia w/ ring sideroblasts...Morphology

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

14

Refractory anemia w/ ring sideroblasts...Immunophenotpe

Can show abnormal acquisition of surface markers

15

Refractory anemia w/ ring sideroblasts...Prognosis

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

16

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

ALL the megakaryocytes are mononuclear

Cytogenetics shows ONLY loss of large arm of chromosome 5!

17

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

Anemia (often severe)
usually elderly pts
more often women

18

MDS w/ isolated del(5q)...Morphology

all the megs are mononuclear

19

MDS w/ isolated del(5q)...Immunophenotype

No known findings

20

MDS w/ isolated del(5q)...Prognosis

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

21

Refractory cytopenia w/ multilineage dysplasia...Key Features

Two or more lineages show dysplastic changes

About half show nonspecific cytogenetic abnormalities

22

Refractory cytopenia w/ multilineage dysplasia...Clinical Presentation

Anemia (often severe)
Usually elderly pts
more often women

23

Refractory cytopenia w/ multilineage dysplasia...Morphology

Granulocytes (if affected) don't granulate normally
nuclei don't lobulate normally

24

Refractory cytopenia w/ multilineage dysplasia...Immunophenotype

Can show abnormal acquisition of surface markers

25

Refractory cytopenia w/ multilineage dysplasia...Prognosis

Median survival 30 months
10% progression to AML in 2 yrs

26

Refractory anemia w/ excess blasts...Key features

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

about half show nonspecific cytogenetic abnormalities

"Pre-leukemia"

27

Refractory anemia w/ excess blasts...Clinical Presentation

Cytopenias
usually elderly pts

28

Refractory anemia w/ excess blasts...Morphology

Blasts and dyspoietic maturation

29

Refractory anemia w/ excess blasts...Immunophenotype

Blast Population (CD34+ and/or CD117) usually

30

Refractory anemia w/ excess blasts...Prognosis

RAEB-1....25% progress to AML
RAEB-2...33% progress to AML