MDS Flashcards

1
Q

Meaning of Auer rods

A

MDS excess blasts

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2
Q

common RBC morphology on blood smear

A

Ovalomacrocytosis

also:
Basophilic stippling
Howell-Jolly bodies
Megaloblastoid nucleated RBC
Elliptocytes
teardrop cells
Atomatocytes
Acanthocytes
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3
Q

Cytogenetic abnormailties-unbalanced

A
Loss of chromosome 7 or del(7q)
del(5q) or t(5q)
Isochromosome 17q or t(17p)
Loss of chromosome 13 or del(13q)
del(11q)
del(12p) or t(12p)
del(9q)
idic(X)(q13)
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4
Q

BM myeloid morphology

A

relative maturation arrest

abnormal localization of immature precursors (ALIP)- immature cells cntered centrally and not peripherally

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5
Q

BM erytroid morphology

A

ineffective erythropoiesis
large RBC
nuclear multilobation and budding
cytoplasm- vacuolization, PAS positive granules, ring sideroblasts

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6
Q

BM megakaryocyte morphology

A

usually normal

Large, small (“dwarf”/micromegakaryocyte), multiple dispersed nuclei ( “pawn ball megakaryocytes”), hypogranular

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7
Q

BM fibrosis

A

up to 50%

usually mild-moderate

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8
Q

Cytogentic abnormaility- balanced

A
t(11;16)(q23.3;p13.3)
t(3;21)(q26.2;q22.1)
t(1;3)(p36.3;q21.2)
t(2;11)(p21;q23.3)
inv(3)(q21q26.2) or t(3;3)(q21.2;q26.2)
t(6;9)(p23;q34.1)
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9
Q

cytogenetic abnormailtes diagonstic of AML

A

t(8;21)(q22;q22); RUNX1-RUNX1T1 (previously AML1-ETO)
inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11
t(15;17)(q22;q21.1); PML-RARA

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10
Q

WHO Classification criteria

A

MDS with single lineage displasia
MDS with multi-lineage displasia
MDS with ring sidroblasts (>15%)
MDS with isolated del(5q)
MDS with excess blasts 1 (BM 5-9%, PB 2-4%)
MDS with excess blasts 2 (BM 10-19%, PB 5-19% or Auer rod)
MDS uncalssifiable

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11
Q

SF3B1

A

Associated with ring sidroblasts

favorable prognosis

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12
Q

DD of ring sidroblasts

A
Alcohol
Benzene
Lead
Isoniazid
Copper deficiency
Congenital
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13
Q

MDS with del(5q)

A

Macrocytic anemia w/wo trombocytosis
Usually no neutropenia
Pretty benign

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14
Q

DD of MDS

A

Deficiency of vitamin B12, folate, or copper
Zinc, lead or arsenic poisoning
HIV
VEXAS

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15
Q

IPSS-R

A
Cytogenetics
Blasts
HB
ANC
PLT
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16
Q

Molecular prognostic associations

A

Adverse – ASXL1, SRSF2, DNMT3A, RUNX1, U2AF1, TP53, EZH2, STAG2, CBL, NRAS, BCOR
Favorable – SF3B1
Neutral or uncertain – TET2, ZRSR2, IDH1, IDH2

17
Q

Clinical prognostic factors

A

age
Performance status
number and severity of cytopenia

18
Q

Timing of IPSS-R assessment

A

At diagnosis

19
Q

Outcomes by risk

A
Very low risk (≤1.5 points) – 8.8 year OS; >14.5 years to 25 percent AML
Low risk (>1.5 to 3 points) – 5.3 year OS; >10.8 years to 25 percent AML
Intermediate risk (>3 to 4.5 points) – 3.0 year OS; 3.2 years to 25 percent AML
High risk (>4.5 to 6 points) – 1.6 year OS; 1.4 years to 25 percent AML
Very high risk (>6 points) – 0.8 year OS; 0.7 year to 25 percent AML
20
Q

Lower-intensity therapies

A
EPO, TPO
Luspatercept,
Hypomethylating agents
IDH inhibitors
Immunosuppressive therapy
lenalidomide
20
Q

Lower-intensity therapies

A

EPO, TPO
Lusbatercept
Hypomethylating agents
IDH inhh

21
Q

Treatment of low risk

A

Asymptomatic- supportive care

Symptomatic- lower-intensity therapies

22
Q

Treatment of high risk

A

Medically fit- allogenic-HSCT
Unfit, not frail- lower-intensity therapies
frail- supportive care

23
Q

Anemia treatment in low risk

A

if EPO < 500 - ESA

if EPO> 500 - HMA

24
Q

Thrombocytopenia Tx in low risk

A

TPO-RA

unless excess blasts

25
Q

Tx of MDS-RS

A

Luspatercept

26
Q

Tx of del(5q)

A

Lenalidomide

27
Q

Luspatercept mechanism

A

Inhibition of TGF-β

28
Q

Features associated with immunosuppressive therapy responsiveness

A
Age <60 years
≤5 percent blasts
Hypocellular bone marrow
PNH-positive clones,
STAT3-mutant T cell clones
29
Q

Immune suppressive therapy

A

ATG + cyclosporine

30
Q

Luspatercept indication

A

MDS-RS

31
Q

IDH inhibitors

A

Ivosidenib

Enasidenib

32
Q

Tx og high risk

A
Allo- HSCT
Induction chemo (3+7) w/wo allo HSCT
low intensity therapies