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Hematology > EXAM 3 review > Flashcards

EXAM 3 review Flashcards

1
Q

CGD

A

Cytochrome B dificiency + Granulomas accumulation

  • Cytochrome B needed for respiratory burst
  • H202 / hydrogen peroxide need to kill catalse positive organisms
  • X linked recessive
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2
Q

Chediak higashi

A

deficiency: defective fusion protein
accumulation: giant azurophilic granules

Autsomal recessive

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3
Q

Gauchers disease

A

deficiency: Betaglucocerebrosidase
Accumulation: glucocerebroside

most common lipid storage disorder
Autosomal recessive
Gaucher cell
Type II: infantile, most severe

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4
Q

Niemann Pick

A

deficiency: Sphingomyelinase
accumulation: sphingomyelim and cholesterol

foamy histocytes
2nd most common lipid storage disease
Autosomal recessive

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5
Q

Tay Sachs

A

deficiency: Hexosaminidse A
Accumulation: lipids and gangliosides

Autosomal recessive

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6
Q

Alder Reilly anomaly

A

Deficiency: Alpha L iduronidase
accumulation: mucopolysaccharides

  • halo around the granule
  • can be interpreted as toxic granulatio
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7
Q

AML with t(8;21)(q22;q22)

A

RUNX1-RUNX1T1

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8
Q

AML with inv(16)(p13q22) or t(16;16)(p13;q22)

A

CBFB-MYH11

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9
Q

APL (acute prolymyelocytic leukemia) with t(15;17)(q22;q12)

A

PML-RARA

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10
Q

AML with t(9;11)(p21;q23)

A

MLLT3-KNT2A

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11
Q

AML with t(6;9)(p23;q43)

A

DEK-NUP214

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12
Q

AML with inv(3)(q21q26) or t(3;3)(q21;q26)

A

GATA-2, MECOM

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13
Q

AML with t(1;22)(p13;q13)

A

RBM15-MKL1

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14
Q

B cell ALL with t(9;22)(q34;q11)

A

BCR-ABL1

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15
Q

B cell ALL with t(v;11q23)

A

KMT2A rearranged

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16
Q

B cell ALL with t(12;21)(p13;q22)

A

ETV6-RUNX1

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17
Q

B cell ALL with hyperploidy

A

50-66 chromosomes

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18
Q

B cell ALL with hypoploidy

A

<45 chromosomes

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19
Q

B cell ALL with t(5;14)(q31;q32)

A

IL3-IGH

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20
Q

B cell ALL with t(1;19)(q23;p13)

A

TCF3-PBX1

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21
Q

M0 (AML NOS)

A
  • myeloblast
  • No maturation
  • minimal differentation
  • Cyto: negative

CD34,CD38, HLA-DR

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22
Q

M1

A
  • Myeloblast
  • MPO(+), SBB(+), SE(+)

CD13, CD33, CD34, CD117

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23
Q

MPO stain
Myeloperoxidase

A
  • Stains myeloperoxidase in promary granules
  • positive: Bluish-black granules

Can differentiate AML (+) from ALL (-)

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24
Q

SBB - Sudan Black B Stain

A
  • stains phospholipids
  • positive: brownish-black granules
  • present in granulocyte and monocyte lysosomal granules

AML(+); ALL (-)

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25
Q

SE - Specific esterase

A
  • Stains specific esterase in primary granules
  • positive: bright red granules

AML (+); ALL(-)

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26
Q

M2

A
  • maturation
  • myeloblast
  • Auer rods
  • MPO+, SBB+, SE+

CD11b,CD13,CD15,CD33,CD34

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27
Q

M4

A
  • Myeloblasts + Monocytic
  • MPO+, SBB+, SE+, NSE+
  • Muramidase - antimicrobial enzyme in monocytic line. increased serum and urine muramidase

CD13, CD15, CD33, CD65

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28
Q

NSE Stain - Non specifc esterase in monocytic cell

A
  • positive = Brick Red Stain
  • Both = stains strongly positive in monocytic cell line
  • acetate = positive in megakaryocyte and light in T lymphocytes

AML - Monocytic (+)
AML - Granulocytic (AML with minimal differentation) (-)

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29
Q

combined esterase

A

Stains both specific and nonspecific.
Specific: blue
Nonspecific: red/brown

  • positive, or both -> myelomonocytic leukemia
  • positive, for non specific only -> acute monocytic leukemia
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30
Q

M5(A andB )

A

A - monoblast
B - Promonocytes

MPO+/-, SBB+/-, NSE+, SE-

Extramedullary disease
- Cutaneous and gum infiltration -> gingival hypertrophy

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31
Q

monoblast vs promonocyte

A

monoblast
- round nuclei, promonint nucleoli, delicate chromatin
- Basophilic cytoplasm

Promonocyte
- Peaked crease

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32
Q

M6

A

Erythroid Leukemia
BM
- >80 immature erythroid precursors
- >30% pronoromoblast

PAS+

CD235a ( glycophorin A)

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33
Q

PAS stain

A

Stains glycogen
- Positive = bright magenta

Used to differentiate
- strongly + pure eryhtroid leukemia

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34
Q

M7

A

Megakaryoblastic leukemia
- >20% blasts
- >50% megakaryoblasts

Electron microscopy platelet peroxidase positive

One or more platelet glycoproteins
- CD41
- CD42
- CD61

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35
Q

Acute Basophilic Leukemia

A
  • basophilic lineage, but blast like morphology

MPO-,SBB-, NSE-, PASblock positivity

CD11b, CD33, CD34, CD124, CD203c Basophil marker

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36
Q

APMF

A

CD34
- BM - panmyeloif hyperplasia
- Reticulin fibrosis - collagen
- PB: pancytopenia

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37
Q

Myelodysplasia related changes

A

> 2 lines with dysplasia

MDS, that goes beyond 20% blasts

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38
Q

Neutrophilc myelodsyplasia related changes

A
  • hypogranulation
  • hyposegmentatio
  • pseudo pelger-huet
  • bizarre segmentation
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39
Q

Erythrocytic - myelodsyplasia related changes

A

Howel jolly bodies
Multi nucleated,
megaloblastic,
vacuoles

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40
Q

Thrombocytic myelodsyplasia related changes

A

micormegakaryocytes with decreased lobulation

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41
Q

Therapy related AML

A
  • Cytotoxic
  • radiation treeatment

Alkylating agents/radiation (5-6 years post exposure)
Topoisomerase II inhibitors (2-3 years post exposure)

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42
Q

AML - downs syndrome

A

Transient abnormal myelopoiesis
- 10% of children with downs
- temporary AML presentation

Myeloid Leukemia assiciated with Downs Syndrome
- M7 - acute megakaryoblastic leukemia

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43
Q

Myeloid sarcoma - AML

A

outside red bone marrow

MPO+, SE+, NSE+

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44
Q

Germline predisposition AML

A

Fanconi’s anemia
- development of myelodysplastic syndrome with excess blasts

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45
Q

Ambigious lineage AML

A
  • doesnt match anything
  • or mixture
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46
Q

MDS definition

A

heterogenous group of clonal hematological malignancies characterized by
- PB cytopenias
- Transform into acute leukemia
- Dysplastic features

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47
Q

In MDS there is an

A

abnormal increase in intramedullarry apoptosis in BM
- imbalance of cytokines

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48
Q

PB dyserythropoiesis

A
  • internuclear bridging
  • anistocytosis with macrocytosis
  • howell jolly body
  • basophilic stippling
  • teardrop cell
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49
Q

BM dyserythropoiesis

A
  • Asynchronous development
  • Nuclear budding
  • internuclear bridging
50
Q

Dysgranulocytopoiesis

A

PB
- Hyposegmentation
- Bilobulation (Pseudo-Pelger huet)

BM
- Hyposegmentation (pseudo - pelger - huet )
- hypogranulation/agranulation

51
Q

Dysmegakaryocytopoiesis

A

PB
- giant platelet

BM
- megakaryocytes with detached nuclei
- hypogranulation

52
Q

MDS –> AML

A

Blasts increase
Leukopenia - changes to leukocytosis

53
Q

MDS affects + genetics

A

elderly, 70

LOSS of genetic material
- chromosome deletions

54
Q

Myeloproliferative neoplasm definition

Mature Myeloid proliferation

A

Group of disorders that are considered clonal malignancies of the hematopoietic stem cell

adults >40 years old

  • BM hyperplasia and spelonmegaly or hepatosplenomegaly
55
Q

Draw table for MDS

56
Q

CML affected cell

A

granulocyte

57
Q

CNL affected cell

A

neutrophils

58
Q

CEL affected cell

A

eosinophil

59
Q

PV cell

A

pancytosis

60
Q

ET affected cell

A

megakaryocytes/PLT

61
Q

PMF affected cell

62
Q

CML gene

63
Q

CML definition

A

increase in granulocytes in PB and marked granulocytic hyperplasia in BM

LOW LAP score

64
Q

1st human disease traced to a chrom. abnormality:

A

Philadelphia chromosome

CML

  • 90-95% of patients with CML
65
Q

Philadelphia chromosome

66
Q

BCR-ABL gene produces

A

p210 tyrosine kinase

67
Q

CML clinical features

symptoms

A

malaise/fatugie
- anemia
Abdomen fullness
loss of apetite
- splemomegaly
Night sweats
Weight loss
Bone tenderness/aching

68
Q

3 CML phases

A

Chronic
Accelerated
Blast phase

69
Q

CML chronic phase

A
  • usualy diagnosed in this phase
  • disease remains stable for several years
  • responsive to chemo
70
Q

CML accelerated phase

A
  • 3-5 years after onset (untreated)
  • ## worsening symptoms
71
Q

blast phase CML

A

conversion of CML to aggressive form of acute leukemia that is difficult to treat

72
Q

CML lab findings

A

WBC count >100,000 microL - leukocytosis

Decreased Hgb and Hct
- anemia (normochromic, normocytic)
- thrombocytosis

73
Q

First indicator of CML

A

basophilia
>2% in chronic phase

74
Q

Myelocyte bulge
chronic phase

A

CML

more myelocytes than metamyelocytes

75
Q

PB smear CML

chronic phase

A
  • giant platelets
  • nRBCs
  • Anisocytosis
  • Basophilic stippling
  • polychromasia
76
Q

CML- chronic phase BM

A

Marked granulocytic hyperplasia

Increased
- megakaryocytes
- basophilic
- eosinophilic
- neutrophils

77
Q

CML accelerated phase BM and PB

A

BM
- increasing blasts 5-19%
- megakaryocyte clusters
- fibrosis

PB
- increasing blasts and promyelocytes
- basophils >20%
- thrombocytopenia

worsening splenomegaly, bone pain and anemia

78
Q

CML blast phase findings

A

BM
- Blasts more than or equal to 20% and in clusters

PB
blasts>20%

79
Q

CML cytogenetics

A

t(9;22)(q34;q11)(BCR-ALB1+)

80
Q

FISH
PT-PCR
CML

A

fluorescence in situ hybridization

PT-PCR, reverse transcriptase polymerase chain reaction
- extremely sensitive
- used to detect residual disease or recurrence after treatment

81
Q

LAP stains

A

leukocyte alkaline phosphatase activity in granules
- present in normal neutrophilic granules
- decreased/absent in malignantion neutrophilic granules

returns to normal or increased
- remission of disease with therapy
- infect

82
Q

toxic vacuoles

A

Leukemoid RXN: increased
CML: absent

83
Q

Toxic granulation

A

Leukemoid RXN: increased
CML: absent

84
Q

Dohle bodies

A

Leukemoid RXN: increased
CML: absent

85
Q

Eosinophilia

A

Leukemoid RXN: normal
CML: increased

86
Q

Basophilia

A

Leukemoid RXN: normal
CML: increased

87
Q

Myelocyte bulge

A

Leukemoid RXN: absent
CML: present

88
Q

LAP score

Leukemoid vs CML

A

Leukemoid RXN: high
CML: low

89
Q

Ph chromosome

A

Leukemoid RXN: negative
CML: positive

90
Q

CML treatment

A

only proven cure: allogenic bone marrow transplant

91
Q

CNL major criteria

A

WBC count >25,000 microL
and
>80% band or segmented neutrophils

92
Q

BM CNL

A

granulocytic proliferation

93
Q

CNL mutation

94
Q

CNL important

how does CNL differ from CML?

A

Basophilia and Eosinophilia NOT present

No philadelphia chromosome

LAP increased

95
Q

CEL basics

A

clonal expansion of eosinophil precursor

96
Q

CEL major criteria

A

PB eosinophils >1,500ul

normal (0-500)

NO philadelphia chromosome

97
Q

PV definition

A

chronic abnormality of the hematopoietic stem cell characterized by uncontrolled proliferation of
- erythroid cells
- granulocytic cells (neutrophils)
- megakaryocytic cells (PLT)

thoery: JAK2 V617F mutation

98
Q

PV clincal feature

A

chronic disease with insidious onset
(asymptomatic/routine doctor visit)
- thrmobosis/bleeding
- splenomegaly/hepatomegaly
- hypervscosity
- hypertension
- gout

99
Q

PV lab findings

A

pancytosis
- increased RBC count, Hgb, Hct
- Increased PLT
- Increased granulocytosis

100
Q

PV BM

A

hyperceullarity with pancytosis

101
Q

PV lab findings coag

A

Normal PT/APTT, but
- adjust anticoagulant for high Hct
- High Hct = less plasma
- failure to adjust anticoagulant (sodium citrate) = falsely increased PT/APTT

102
Q

Secondary Polycythemia

A

increased RBC due to response to other factors
- Hypoxia
- EPO producing tumor

Increased cells
- secondary RBC only; PV - multiple cell lines
- EPO
- secondary increased; PV - decreased

103
Q

relative polycythemia

A

increased RBC due to decreased plasma volume
- dehydration/burns

aka tertiary polycythemia

increased cells:
relative - RBC only
PV - multiple cell lines

104
Q

PV treatment

A

Therapeutic phlebotomy
1 unit weekly, decrease Hct

men <45%
women <40%

105
Q

ET definition

A

chronic myeloproliferative disorder characterized by
- marked thrombocytosis
- abnormal platelet function
- increased risk of thrmobsis and hemorrhage

106
Q

ET clinical features

A

chronic disrase with insidious onset (asymptomatic/routine doctor visit)

possible symptoms
- thrombosis /hemorrhage - most common

107
Q

ET lab findings

A

increased PLT count >450,000 uL
can be as high as 1 million/uL

Normocytic + normochromic RBCs
- can develop IDA from GI/mucosal bleeds

splenic atrophy
- howell jolly
- target cells
- acanthocytes

108
Q

ET BM

A

increase in megakaryocytic cells
- larger than usual
- clusters
- hyperlobulated

109
Q

ET lab findings

A

abnormal platelet aggregation
can also have
- decreased platelet adhesion
- storage pool defects
- prolonged bleeding times

Cytogenetics:
- JAK2 (50-60% cases)

110
Q

Reactive thrombocytosis

A

increased platelet count due to something else
- acute hemorrhage
- postsplenectomy
- chronic infection
- drug induced
- trauma

111
Q

Reactive thrombocytosis vs. ET

A

PLT count >1,000,000/uL
- RT - infrequent
- ET - frequent

BM megakaryocyte clusters
- RT - not present
- ET - commong

112
Q

disease often based on exclusion

113
Q

PMF basics

Primary myelofibrosis

A

fibrosis - excessive formation of fibrous (connective) tissue

114
Q

PrePMF

A

BM is hypercellular
increased granulocytic and megakaryocytic proliferation mimics ET and PV

115
Q

Overt PMF
FIbrotic stage

A

-“classic triad”

  • BM fibrosis
  • PB leukoerythrooblastosis and Teardrop cell
    1. immature myeloid cells and nRBCs
  • Myeloid metaplasia (abnormal change in myeloid tissue)
    1. extramedullary hematopoiesis due to BM fibrosis
116
Q

PMF clinical features

A
  • insidious onset - symptom free for years
  • diagnosis made at a routine doctor visiti

symp:
- fever
- anorexia/weight loss
- night sweats
- bone pain
- anemia symptoms

117
Q

PMF lab findings

A

Decreased RBC, Hgb, Hct

WBC and PLT variable

smear
- Teardrop cell
- megakaryocyte fragments and large PLT

118
Q

fibrotic stage of PMF - BM

A
  • fibrosis
  • often results in dry tap
  • due to increased fibrosis. andlow cellularity
119
Q

PMF coagulation

A

increased fibrin degradation products (D-dimer)

extramedullary hematopoiesis in liver can cause dysfunction
- liver = coagulation factor producer

120
Q

CML vs PMF

A

CML
- marked leukocytosis
- RBC morphology - normal
- Ph chrom
- LAP decreased

PMF
- WBC count: <30x10^3 /uL
- RBC morphology - teardrop cell
- Ph chrom. absent
- LAP normal/increased

121
Q

PMF treatment

A

asymptomatic
- no treatment/monitor

Symptomatic: cytotoxic therapies/and or radiation

Splenectomy
- risk of operative thrombovytosis

allogenic stem cell transplant
- only curative treatment

Hematology (36 decks)

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