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B&L > Exam 3 - Acute Leukemias, Myeloproliferative Disorders, and MDS > Flashcards

Exam 3 - Acute Leukemias, Myeloproliferative Disorders, and MDS Flashcards

1
Q

TdT

A

marker of lymphoblasts

not expressed by mature lymphocytes or myeloblasts

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2
Q

CD34

A

immature marker of blasts

could be lymphoblasts or myeloblasts

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3
Q

Acute lymphoblastic leukemia (ALL)

A

replacement of marow by lymphoblasts

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4
Q

Types of ALL

A

B-lymphoblastic ALL (80%) - children

T-lymphoblastic ALL (20%) - older

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5
Q

B-lymphoblastic ALL

A

CD34, TdT

CD19, CD22 (No CD20, mature)

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6
Q

T-lymphoblastic ALL

A

Large mediastinal mass
More likely high WBC
CD34, TdT
CD3, CD7, CD4, CD8, CD99

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7
Q

Cytogenetics B-ALL

A

t(9;22) Philadelphia - worst (adult)
11q23 (MLL) - poor (infant, congenital)
t(12;21) ETV6-RUNX1 - favorable (child)

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8
Q

5 worse prognostic factors for ALL

A 
Increased age
Increased WBC
Slow response to therapy
less hyperdiploidy
T-ALL (compared to B-ALL)
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9
Q

Acute myeloid leukemia (AML)
Smear for myelo
Markers for myelo
Markers for other two cell lines

A 
>20% myeloblasts in marrow or blood (unless cytogenetic findings)
Myeloblasts (auer rods)
CD34
CD117, MPO - myeloid markers
CD64, CD14
CD41, CD61
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10
Q

CD64, CD14

A

Monocytic lines

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11
Q

CD41, CD61

A

Megakaryocytic lines

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12
Q

Cytogenetics AML

A 
t(8;21) RUNX1-RUNX1T1: alpha-CBF - good
Inv(16) or t(16;16) CBFB-MYH11: ß-CBF - good
t(15;17) PML-RARA
t(1;22): megakaryoblastic - good
11q23 (MLL): monocyctic - poor
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13
Q

t(15;17) PML-RARA in AML

A

Acute promyelocytic leukemia
Tx with retinoic acid
Check for DIC

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14
Q

Therapy related AML

A

Loss of 5 or 7 (alkylating agents or radiation)
Rearrange MLL 11q23 (topoisomerase II inhibitors)
Very poor prognosis

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15
Q

AML Not Otherwise Specified

A

FLT3 ITD (negative prognostic factor)
NPM1 (positive prognostic factor)
CEBPA (positive prognostic factor)

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16
Q

Myelodysplastic Syndrome

A

Ineffective hematopoiesis (increased ineffective cells)
Increased risk transformation to AML
Replacement of marrow with myeloid lines (to extent)
Primary or secondary

17
Q

MDS: Changes in ___% but less than ___% of which myeloid cell lines and what are the characteristic findings?

A

10%, 20% (=AML)
Dyserythropoiesis (ring sideroblasts)
Dysgranulopoiesis (bilobed nuc, pseudo-Pelger-Heut cells)
Dysmegakaryopoiesis (hypo/non-lobed nuc)

18
Q

Cytogenetic Findings of MDS

A

Deletions 5 or 7 or (5q)

Trisomy 8

19
Q

Secondary MDS

A 
morphologic evidence without cytogenetics or increased blasts
Vitamin def
Toxin exposure
Drug exposure
Viral infection
20
Q

Low grade MDS

A
21
Q

High grade MDS

A 
>5% marrow, >2% blood myeloblasts (>20%=AML)
Refractory anemia with excess blasts 1
5-9%, 2-4%
Refractory anemia with excess blasts 2
10-19%, 5-19%
Dismal prognosis
22
Q

Myeloproliferative Neoplasms

4 cell lines

A

Non-dysplastic proliferation of 1 or more myeloid lines

Granulo, erythoid, megakary, mast

23
Q

Characteristics of MPN

A

Hypercellular marrow
Spleno/hepatomegaly
Insidious onset
Progress to AML, ALL, MDS, marrow fibrosis

24
Q

Classifications of MPN

A 
Chronic myelogenous leukemia (CML)
Polycythemia vera (PV)
Primary myelofibrosis (PMF)
Essential thrombocythemia (ET)
25
Q

CML clinical findings and bone marrow biopsy findings

A

Leukocytosis (neutrophilia)
Increased basophils
Increased platelets
Bone marrow biopsy: hypercellular marrow, small megakaryocytes with round, non-lobated nuclei, no dysplasia

26
Q

CML genetics and treatment

A

BCR-ABL1

Tx: protein tyrosine kinase inhibitors

27
Q

PV clinical findings and unique symptoms

A

Erythrocytosis
Plethora, pruritus, paresthesias
Rare to progress to AML
End with fibrosis

28
Q

PV genetics and secondary PV

A 
JAK2 (V617F) activation
W/out mutation = secondary
    Smokers (carboxyhemoglobin)
    Chronic hypoxia
    Hb disorders
29
Q

Phases of PV

A 
Polycythemic phase (high counts)
Spent phase (post PV, fibrosis of bone marrow)
30
Q

Major complication of PV

A

Venous or arterial thrombosis

Especially: mesenteric, portal or splenic vein

31
Q

PMF characteristics

A

PV w/out erythrocytosis (50% JAK2 mutation)
Instead: granulo/megakaryo line
No JAK2 = MPL or CALR mutation
Progress to fibrotic stage (organ enlargement)
Rare to progress to AML

32
Q

ET characteristics

A 
50% JAK2
No JAK2 = MPL or CALR mutation
No granulo, megkaryocytes are larger
Risk for transient ischemic attacks
NO SPLENOMEGALY

B&L (8 decks)

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