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Strom - Hematology > 3.24.14* Hematologic Malignancies I & II > Flashcards

3.24.14* Hematologic Malignancies I & II Flashcards

PPT* Lecture 1* PPT 2* Lecture 2* Reading (Ch. 11)* Pictures

1
Q

leukemia

A

abnormal proliferation of cells in the bone marrow or bloodstream

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2
Q

acute leukemia

A

rapidly proliferating leukemia, mainly blasts. (slow growing is chronic leukemia)

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3
Q

lymphoma

A

abnormal cell growth only involving lymphoid tissue (lymph nodes, spleen, subepithelium of GI tract)

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4
Q

t(15;17)

A

Acute myeloblastic leukemia

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5
Q

Characteristics of a blast

A

a. Large cells
b. High nuclear/cytoplasmic ratio
c. Prominent, single or multiple nucleoli
d. Immature (faint/smudgy) chromatin
e. Their appearance is shared by many cells on a slide
* *AUER RODS are diagnostic of myeloid blasts

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6
Q

what proportion of bone marrow aspirate cells are blasts

A

5%

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7
Q

myeloid cells (granulocytes plue monocytes) should outnumber erythroid precursors in bone marrow aspirate by what proportion?

A

2:1 to 5:1

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8
Q

what is the cellularity estimate for a normal bone marrow core biopsy?

A

100 - age

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9
Q

How is immunophenotyping performed?

A

flow cytometry:
a. lyse red cells/precursors
b. add fouorescent Ab to desired cell surface proteins
c. run through cytometer
d. forward scatter measures size
e. side scatter intensity measures internal granules or segmented nuclei
f. fluorescent antibodies give wavelengths telling presence of desired antigens
immunohistochemistry
a. enzyme for color is conjugated to antibody for desired cell surface protein
b. add chromogenic substrate

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10
Q

what marrow blast number implies acute leukemia?

A

> 20% (if less then need to do cytogenetic study)

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11
Q

immunophenotype with CD34+

A

blasts

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12
Q

immunophenotype with CD34+, CD33+, CD117+

A

myeloid blasts -> AML

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13
Q

immunophenotype with Tdt+, CD10+

A

lymphoid blasts -> ALL

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14
Q

immunophenotype with CD19+, CD20+

A

mature lymphocytes (lymphoma)

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15
Q

CBC most suggestive of a hematologic malignancy?

a. anemia, left shift of granulocytes
b. increased platelet count
c. increased monocytes, target cells
d. myelocytes, auer rods, giant platelets
e. polymorphic lymphocytes

A

D

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16
Q

what cell type is most abundant in normal bone marrow?

a. erythroid precursors
b. myeloid precursors
c. blasts
d. megakaryocytes
e. lymphocyte precursors

A

B

Remember a neutrophil only lasts 24 hours, they have many precursors

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17
Q

What does the pathologist use to count blasts?

a. aspirate
b. core biopsy
c. flow cytometry

A

A

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18
Q

which of the following methods can directly tel the immunophenotype of the abnormal cells in a bone marrow biopsy?

a. FISH
b. cytogenetics
c. flow cytometry
d. targeted DNA sequencing
e. differential count

A

C

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19
Q

You suspect a patient has a hematologic malignancy involving a particular translocation, but his cytogenetic studies are normal. What is the best way to follow up?

a. FISH
b. flow cytometry
c. immunohistochemistry
d. whole exome sequencing

A

A

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20
Q

A clonal proliferation of megakaryoctes causing the platelet count in PBS to triple would be an example of

a. myelodysplastic syndrome
b. myeloproliferative disease
c. acute myeloid leukemia
d. acute undiferentiated leukemia

A

B

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21
Q

myeloblasts proliferating in a location outside bone marrow and outside the bloodstream are called?

a. myeloysplastic syndrome
b. myeloproliferative disease
c. acute myeloid leukemia
d. acute undifferentiated leukemia
e. myeloid sarcoma

A

E

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22
Q

6y/o in ER with WBC count of 230 K/uL (normal is 10). What is the diagnosis

a. sepsis
b. AML
c. ALL
d. CML
e. polycythemia

A

C. (more common in kids, AML is more common in adults). Sepsis (40-60)

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23
Q

ALL (acute lymphoblastic leukemia)

A

a. BCR-ABL1 t(9;22). Fusion protein of part of a serine-threonine kinase (BCR) to a tyrosine kinase (ABL1).
b. 25% of all adult blood cancers
c. immunophenotype (CD10, CD19, TdT)

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24
Q

which of the following immunophenotypes is most consistent with ALL?

a. CD10, TdT
b. CD34, CD33
c. CD117, CD11b
d. HL-DR, CD33
e. CD34,

A

A

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25
Q

Leukemic blasts in 5 y/o are Tdt, CD3, CD5 and show the following translocation: t(14q11, 10q24) (TCR-alpha; HOX11). Where outside the bone marrow would you expect these cells to form a mass?

a. lung
b. skin
c. brain
d. testes
e. thymus

A

E

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26
Q

73 year old woman in Er with weakness and SOB. Her WBC is 33 (n 4-10) with 85% neutrophils. What is the next study you need from the lab?

a. RT-PCR assay
b. cytogenetics
c. flow cytometry
d. bone marrow aspirate differential count
e. peripheral blood manual differential count

A

E.

This is probably a bacterial infections, may be acute neoplastic so check with PBS

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27
Q

60 y/o male smoker with hgb of 21 (n 14-18). His pulmonary function and oxygenation are normal, low serum erythropoietin. What do you need next from the lab?

a. molecular testing Jak2-V617F mutation
b. RT-PCR for BCR-ABL1 mRNA
c. flow cytometry
d. bone marrow aspirate differential count

A

A

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28
Q

49 year old woman with platelet count 635 K/ul (n 150-400). Hgb 9 (12-16) and microcytic (MCV 72, n 80-100). What is the next study?

A

iron studies

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29
Q

AML genotypes that are diagnostic regardless of blast count

A

t(15;17) PML-RARA(5-8%)
t(8;21) RUNX1-RUNXT1 (5%)
inv(16) CBFB-MYH11 (5-8%)

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30
Q

ALL genotypes

A

t(12;21) TEL-AML1

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31
Q

t(15,17)

A

(AML genotype)
PML-RARA
a. Fusion of PML (tf) with RARA (tf, retinoic acid receptor alpha) leading to dominant negative blockade of normal RARA and inhibits granulocyte differentiation.
b. good prognosis

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32
Q

what is the retinoic acid analogue that can block PML-RARA fusion gene?

A

ATRA. Induced differentiation of the blasts to granulocytes (clinical remission)

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33
Q

What is the clinical presentation of t(15,17) AML?

A

severe thormbocytopenia

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34
Q

PBS of t(15,17) AML?

A

big blasts, cleaved “bat wing” nuclei. Many cytoplasmic granules. Auer rods in stacks.

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35
Q

Immunophenotype of t(15,17) AML

A

Weak/absent CD34, HLA-DR, CD13+, CD33+

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36
Q

t(8,21)

A

(AML genotype)
Runx1-Runx1T1
a. Fusion protein of two tf’s. Dominant negative repressor of myeloid maturation (same as APL)
b. good response to chemo

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37
Q

What is the clinical presentation of t(8,21)?

A

in kids

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38
Q

PBS of t(8,21) AML

A

Some maturation to myelocytes

Occasional crystallization of granule contents (“Auer rods”)

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39
Q

Immunopheonotype of t(8,21) AML

A

CD34+, HLA-DR+, CD13+, CD33 weak

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40
Q

Runx1

A

a. protein part of transcription factor complex, core binding factor (CBF).
b. AML fusion gene t(8,21) Runx1-Runx1T1

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41
Q

inv(16)(p12.1;q22)

A

(AML)
inv(16) CBFB-MYH11
a, fusion protein of a transcription factor with MYH1. Dominant negative repressor of myeloid maturation.
b. better than most prognosis if “risk adapted” therapy is used

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42
Q

CBFB

A

component of CBF heterodimer with Runx1

43
Q

clinical presentation of inv(16) AML

A

younger patients/kids

44
Q

PBS morphology of inv(16) AML

A

Mixed granulocyte-monocyte features (“Myelomonocytic”)

Increased eosinophils in blood and marrow

45
Q

AMLs with normal cytogenetics take up how many causes

A

50%

46
Q

Morphology of AML with normal cytogenetics

A

undifferentiated or variably granulocytic or monocytic/ monoblastic that are blasts

47
Q

AML with complex karyotype

A

5-10% in patients with 3 or more cytogenic findings (translocation, trisomy, monosomy

a. poor prognosis
b. blasts

48
Q

B cell subtype genetic abnormalities for ALL

A

t(9;22)(q34;q11.2); BCR-ABL1 (bad prognosis)
t(v;11q23); MLL rearranged (bad prognosis)
t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1) (good prognosis)
t(5;14)(q31;q32); IL3-IgH
t(1;19)(q23;p13.3); E2A-PBX1
Hyperdiploid (>50 chromosomes) (good prognosis)
Hypodiploid (<45 chromosomes)

49
Q

t(9,22)

A 
(ALL, B cell)
BCR-ABL1
fusion protein of a serine-threonine kinase (BCR) to a tyrosine kinase (ABL1). Proliferation activator
b. mostly adults
c. poor prognosis
50
Q

morphology seen in t(9,22) ALL

A

Big agranular blasts

51
Q

Immunophenotype of t(9,22)

A

CD10+, CD19+, TdT+

52
Q

t(v;11q23)

A 
(B cell ALL)
MLL rearranged
a. Fusion of transcription regulator (histone methyl transfferase) to any of several partners inhibits differentiation
b. kids
c. poor prognosis
53
Q

morphology seen in t(v;11q23)

A

big agranular blasts

54
Q

Immunophenotype for t(v;11q23)

A

CD10-, CD19+, TdT+

55
Q

t(12;21

A 
(B  cells ALL)
TEL-AML1
a. Fusion protein that acts as a dominant negative transcription factor with multiple effects on gene expression. 
b. kids
c. good prognosis (90%)
56
Q

Morphology seen in t(12,21) B-ALL

A

TdT+, CD34+, CD10+, CD20-

57
Q

T-ALL

A

a. most have a translocation of a oncogene to a TCR promoter
b. Kids
c. High risk prognosis

58
Q

morphology seen in T-ALL

A

big agranular blasts

59
Q

immunophenotype seen in T-ALL

A

TdT+, CD3+, CD5+; can express myeloid or B-cell antigens as well

60
Q

myeloproliferative disorder

A

proliferating clones with differentiation into blood cells. Subtypes are myeloid, erythroid and megakaryocyte.

61
Q

Types of myeloproliferative diseases

A 
CML
CMML
CEL
CNL
Mastocytosis
ET
P vera
primary myelofibrosis
62
Q

CML (chronic myelogenous leukemia

A

High WBC due to all stages of granulocyte maturation in blood

63
Q

CMML (chronic myelomonocytic leukemia)

A

High WBC due to monocyte, promonocytes and hybrids of monocyte/granulocytes

64
Q

Chronic eosinophillic leukemia (CEL, aka PDGFR neoplasm)

A

increased eosinophils

65
Q

Chronic neutrophilic leukemia

A

increased mature neutrophils (rare)

66
Q

Mastocytosis

A

increased mast cells (primarily tissue targeted, bone marrow often involved)

67
Q

Primary myelofibrosis

A

elevated platelet count

68
Q

what myeloproliferative disorders often present with thrombosis?

A

a. polycythemia vera

b. essential thrombocythemia or primary myelofibrosis

69
Q

What features in a PBS suggest sepsis instead of a myeloproliferative disorder

A

a. toxic granulation of PMNs

b. left shift (fewer cells representing the PMN precursors)

70
Q

What is the order of precursors by abundance seen in a left shift

A

neutrophils
bands
metamyelocytes
myelocytes

71
Q

myeloid bulge

A

patient has more myelocytes than metamyelocytes. Suggestive of myeloproliferative disorder.

72
Q

What is the next step after seen a CBC/PBS with many myeloid precursors?

A

get RT-PCR assay for BCR-ABL1, because CML is common and treatable.

73
Q

What is the drug used for CML

A

imatinib (inhibits ABL kinase)

74
Q

what can CML progress to if untreated?

A

blast phase: acute leukemia (myeloid and sometimes lymphoid)

75
Q

Polycythemia vera

A

a. elevated red cell count
b. Activating Jak2 mutation (95%) or Epo receptor mutations.
c. presents with thrombosis, hypertension, stroke or MI
d. no immunphenotype for RBCs
e. >10 yr survival

76
Q

What is the differential diagnosis for Polycythemia vera with increased RBC?

A

lung disease

77
Q

What is the common clinical presentation for polycythemia vera

A

thrombosis, hypertension, stroke or MI.

78
Q

What is the common morphology seen in polycythemia vera

A

Hypercellular marrow, erythroid hyperplasia, increased Megs

79
Q

What can polycythemia vera and ET progress to if untreated

A

myelofibrosis
MDS (myelodysplastic syndrome)
acute leukemia

80
Q

What is the function of Jak2 that leads to polycythemia vera

A

constituent activation of Epo/Tpo receptor to cause RBC hyperproliferation (and increased Megs)

81
Q

What myeloproliferative disorders give increased platelets and thus increased risk of thrombosis?

A

essential thrombocythemia

primary myelofibrosis

82
Q

Essential thrombocythemia

A

a. Jak2 mutation leading to elevated platelet count. Also mutation in thrombopoeitin receptor (MPL)
b. clinical presentation thrombosis.
c. no immunophenotype for platelets
d. >10 year survival

83
Q

What is the differential diagnosis for essential thrmbocythemia with increased platelets?

A

iron deficiency
infection
chronic inflammation

84
Q

What is the morphology seen in ET?

A

Increased Megs; they’re large and weird looking even for Megs, and tend to cluster

85
Q

Primary myelofibrosis

A

a. Jack 2 mutation, increased platelets, very similar to ET

b. may may increased reticulin fibers.

86
Q

Mastocytosis clinical presentation in kins

A

a. Neoplasms of mast cells usually present as benign cutaneous lesions in kids (the most common is “urticaria pigmentosa”, aka itchy pigmented skin lesions).
b. can become systemic and released histamine and other mediators to cause flushing, nausea, tachycardia, hypotension)

87
Q

What test can indicate mastocytosis?

A

serum typtase level (indicative or hyperactive mast cells)

88
Q

Bone marrow findings for mastocytosis

A

bland looking, round or spindle shaped cells.

89
Q

Mastocytosis

A

(increased mast cells)

a. cKIT mutations or PDGFRA activation (by FIP1 translocations)
b. immunophenotype: tryptase, CD117 (cKIT, the SCF receptor), CD25
c. variable prognosis

90
Q

Mastocytosis genetics

A

Either cKIT mutants OR PDGFRA activation (FIP1 translocation)

91
Q

Mastocytosis morphology

A

Aggregates of bland looking cells, round or spindle shaped, sometimes with eosinophilia

92
Q

reactive cause for increase eosinophils

A

parasites

drug reactions

93
Q

Jak2-V617F mutation seen in

A

ET

P vera

94
Q

Myelodysplastic syndrome

A

poorly functioning clones

95
Q

what are the 5 major adult subtypes of myelodysplasia (best to worst prognosis

A

a. Refractory cytopenia with unilineage dyplasia
b. Refractory anemia with ring sideroblasts
c. Myelodysplastic syndrome with isolated del(5q)
d. Refractory cytopenia with multilineage dysplasia
e. Refractory anemia with excess blasts

96
Q

Refractory cytopenia with unilineage dysplasia

A

a. presentation - unexplained cytopenia (s), usually over age 65
b. morphology- megablastoid features
c. prognosis- variable, rarely progresses to AML

97
Q

Refractory anemia with ring sideroblasts

A

a. presentation - unexplained cytopenia(s) usually over 65 years
b. morphology- ringed sideroblasts, dyspoietic features (all only in RBCs)

98
Q

Myelodysplastic syndrome with isolated del(5q)

A

a. loss of large arm of chromosome 5)
b. presentation- unknow cytopenia over 65
c. morphology- all megs are mononuclear
d. treatable with lenalidomide; 10% progress to AML

99
Q

morphology of refractory cytopenia with unilineage dysplasia

A

Weird looking precursors; binucleation or irregular nuclei; can show fibrosis, high or low cellularity, megaloblastoid features.

100
Q

treatment for MDS with isolated del (5q)

A

lenalidomide

101
Q

Refractory cytopenia with multilineage dysplasia

A

a. presentation- often severe anemia, usually elderly women
b. morphology- dysplastic changes in two or more lineages. Granulocytes (if affected) don’t granulate normally; nuclei don’t lobulate normally.
prognosis: median survival 30 months. 10% progress to AML.

102
Q

Refractory anemia with excess blasts

A

a. presentation- cytopenias, elderly
b. morphology- blast and syspoietic maturation
c. immunophenotype- Blast population (CD34+, and/or CD117+) usually evident
d. prognosis: RAEB-1: 25% progress to AML. RAEB-2: 33% do so.

103
Q

genetic defect common in refractory anemia with excess blasts

A

5% - 9% morphologic blasts (RAEB-1)

10% - 19% morphologic blasts (RAEB-2)

104
Q

immunophenotype seen in refractory anemia with excess blasts

A

Blast population (CD34+, and/or CD117+) usually evident

Strom - Hematology (6 decks)

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