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Rapid Fire CP Review > Rapid Review 5 > Flashcards

Rapid Review 5 Flashcards

1
Q

virus a/w ATLL

A

HTLV-1

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2
Q

virus a/w primary effusion lymphoma

A

HHV8

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3
Q

what are the 2 markers that are different btwn Sezary syndrome and ATLL?

A

CD25 and CD26: negative in Sezary

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4
Q

what 4 markers are the same between Sezary and ATLL?

A

CD3+
CD7-
CD4+
CD8-

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5
Q

what’s the chemical name for A antigen

A

N-acetyl-D-galactosamine

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6
Q

what places A antigen on the RBC?

A

alpha-3-N-acetyl-galactosaminyltransferase

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7
Q

what’s the chemical name for B antigen?

A

D-galactose

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8
Q

what places B antigen on the RBC?

A

alpha-3-D-galactosyltransferase

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9
Q

what’s the chemical name for O antigen?

A

aka H substance

L-fucose

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10
Q

what’s H substance?

A

O antigen

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11
Q

what places O antigen on the RBC?

A

alpha-2-fucosyltransferase

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12
Q

what are the 2 things that all of the ABO blood group antigens have in common, structurally?

A

fuc-alpha-1,2
gal beta 1,3 GlcNAc

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13
Q

if you see “popcorn” cells, think of THIS

A

nodular lymphocyte-predominant Hodgkin’s lymphoma

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14
Q

what type of cells “ring” the popcorn cells?

A

PD1+
CD279+
T-cells!!!!!!!!!

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15
Q

what is the biggest difference between RS and popcorn cells?

A

RS cells: 15+/30+
popcorn cells: 15-/30-

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16
Q

all Burkitt lymphomas have translocations involving chromosome 8. what gene is involved on chrom 8?

A

c-MYC

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17
Q

what are the 2 new markers that CLL is positive for? on flow

A

CD200
LEF1

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18
Q

what is the confusing mimic for hairy cell in a peripheral smear?

A

circulating villous lymphocytes in splenic MZL (usu not entirely circumferential)

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19
Q

how does hairy cell look in the bone marrow?

A

interstitial lymphoid aggs or diffuse involvement

LOTS OF FIBROSIS

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20
Q

what 2 IHC/flow markers are positive in Hairy cell as a result of BRAF V600E mut?

A

TRAP and CD25

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21
Q

caribbean lymphoma

A

ATLL

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22
Q

ATLL is a/w what virus?

A

HTLV1

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23
Q

virus a/w extranodal NK/T cell lymphoma

A

EBV

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24
Q

what’s this

A

hallmark cell

ALCL

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25
Q

what is the neoplastic cell in AITL?

A

T follicular helper cells

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26
Q

what happens to B cells in AITL?

A

TFH cells cause differentiation/activation of B cells

B cells are usu EBV+

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27
Q

what are the SENSITIVE IHCs for AITL?

A

PD-1 & ICOS

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28
Q

what are the SPECIFIC IHCs for AITL?

A

CXCL13 > CD10

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29
Q

what are CD8+ T cells doing in AITL?

A

they have an “exhausted phenotype”

POS for:

  • CD8
  • CD45RO
  • CD27
  • PD-1
  • TIGIT
  • ICOS
  • TIM3
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30
Q

90% of AITL cases have this broad abnormality in molecular testing

A

karyotypic abnormalities

tri 3/5/19

gain of X

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31
Q

driver mutation identified in ~70% of AITL cases

A

RHOA p.Gly17Val

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32
Q

AITL has 4 genes that are most commonly mutated. what are they?

A
  1. RHOA (driver in ~70%)
  2. TET2
  3. DNMT3
  4. IDH2-R172
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33
Q

MC mutation in ALCL & what chromosome is the gene on?

A

ALK

chromosome 2

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34
Q

2 characteristic findings for follicular dendritic cell sarcoma

1 morpho & 1 IHC

A

morpho: whorled spindle cells

IHC: CD35+ (and CD21+)

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35
Q

3 mutations that you can see in hemophagocytic syndrome

A

PRF1

UNC13D

STX11

(PRooF of your UNCle on the STyX)

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36
Q

what is the super specific lab finding in HLH?

A

soluble CD25 > 2400 U/mL

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37
Q

what are endothelial cells positive for in bacillary angiomatosis?

A

ulex europaeus

which is also the lectin for H antigen

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38
Q

what’s this

A

ring granuloma

Q fever - Bartonella quintana/henselae

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39
Q

what’s the disease & the 2 specific findings in this pic?

A

lupus/SLE

hematoxylin bodies + karyorrhectic debris/coagulative necrosis

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40
Q

which one has eos?

kikuchi or kimura

A

kimura

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41
Q

which one has plasmacytoid dendritic cells?

kikuchi or kimura

A

kikuchi

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42
Q

which 4 genetic alterations can you use to diagnose AML even if the blast count is less than 20%?

A
  1. t(8;21) RUNX1/RUNX1T1
  2. t(16;16) CBFB/MYH11
  3. inv(16) CBFB/MYH11
  4. t(15;17) PML/RARA
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43
Q

what IHC can you use to clue you into a KIT mutation?

A

CD56

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44
Q

25% of patients with t(8;21) RUNX1-RUNX1T1 have a mutation in THIS gene

A

KIT (it makes it a worse prognosis)

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45
Q

which heme malignancy is a/w abnormal eos with basophilic granules?

A

AML with inv(16) or t(16;16) CBFB-MYH11

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46
Q

what 2 extra mutations confer a worse prognosis in AML with t(16;16) or inv(16)?

A

KIT mutations

FLT3-ITD

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47
Q

which heme malignancy do you treat with ATRA?

A

AML with t(15;17) PML-RARA

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48
Q

there are 2 things that will confer resistance to ATRA. what are they?

A

variant fusions involving RARA and either:

ZBTB1 (11q23)

or

STAT5 (17q11)

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49
Q

AML with mutated NPM1 usually has a mutation where?

A

exon 12

:(

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50
Q

concurrent mutation in THIS negates any good prognosis that an NPM1 mutated AML might have

A

FLT3-ITD

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51
Q

what is the latent period between therapy & the development of therapy-related AML/MDS/MDS-MPN if you are treated with an alkylating agent?

A

5-7 years

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52
Q

what are the therapies a/w therapy-related myeloid neoplasms?

A

radiation/alkylating agents

topoisomerase II inhibitors

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53
Q

mutations in THIS GENE are pathognonomic for down-syndrome related myeloid proliferations

A

GATA1

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54
Q

what IHC stain is a surrogate marker for FLT3 mutation?

A

CD117

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55
Q

chronic neutrophilic leukemia gene

A

CSF3R

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56
Q

what umbrella does chronic neutrophilic leukemia go under?

A

(chronic) myeloproliferative neoplasms

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57
Q

what are the 4 phases of polycythemia vera?

A
  1. prodrome (pre-polycythemic)
  2. proliferative/polycythemic phase
  3. spent phase (post-PV myelofibrosis)
  4. secondary AML (blasting off)
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58
Q

you can get a JAK2 V617F mutation in prefibrotic PMF (primary myelofibrosis), but what other 3 genetic categories are there?

A

CALR exon 9 mut

MPL W515K/L

triple negative

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59
Q

what are the lab (WBC) criteria for CNL diagnosis?

A

leukocytosis > 25 x 109/L

>80% of those are neutrophils

<10% are precursors

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60
Q

genetic mutation a/w systemic mastocytosis

A

KIT D816V

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61
Q

what are the 4 MDS/MPN diseases?

A

aCML

CMML

JMML

MDS/MPN unclassifiable

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62
Q

which AMLs may have eosinophilia?

A

inv(16) / t(16;16)

and

t(8;21)

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63
Q

this is a disease entity: “Myeloid and lymphoid neoplasms with eosinophilia and rearrangements of…”

what are the rearrangement genes?

A

PDGFRA

PDGFRB

FGFR1

PCM1-JAK2

that is as far as I will be remembering that

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64
Q

CMML

2 most common cytogenetic abnormalities

A

+8

-7

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65
Q

CMML

4 most common gene mutations

A

MC: ASXL1

TET2

SRSF2

SETBP1

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66
Q

percentage cutoffs for CMML 0, 1, and 2

A

0: <2% blasts in blood, <5% blasts in BM
1: 2-4% in blood, 5-9% in BM
2: 5-19% in blood, 10-19% in BM

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67
Q

atypical CML does NOT have this genetic alteration, but instead has mutations in these genes

A

NO BCR-ABL1!!!

mut in SETBP1 & ETNK1

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68
Q

MDS/MPN with ring sideroblasts and thrombocytosis

MUTATION

A

SF3B1

but you still need 15% ring sideroblasts

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69
Q

what are the mutations you could potentially have in JMML?

A

mut PTPN11

mut KRAS

mut NRAS

clinical Dx of NF1 or NF1 mut

CBL (germline or LOH)

monosomy 7

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70
Q

T/F: dysplasia of one or more hematopoietic lineages is common in aplastic anemia

A

FALSE

if you’re gonna get a dys-, it’s gonna be dyserythropoiesis

but you do NOT get dysgranulopoiesis or dysmegakaryopoiesis

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71
Q

“standard risk” genetics in plasma cell myeloma

A

t(11;14)

t(6;14)
[14 & std risk: 11 & 6]

hyperdiploidy

B2 microglobulin < 3.5 mg/L

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72
Q

HIGH RISK genetics in plasma cell myeloma

A

del(17p)

t(4;14)

t(14;16)
[14 & high risk: 4 & 16]

+1q21

hypodiploidy

del13q

B2 microglobulin >5.5 mg/L

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73
Q

congenital TTP

eponym

inheritance

gene & chromosome

A

Upshaw-Shulman

autosomal recessive

ADAMTS13 gene on 9q34

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74
Q

what role does calcium play in clotting?

A

provides a positive charge for negative Gla domains on vitamin K-dependent factors

75
Q

what are the ingredients for PT testing?

A

thromboplastin (tissue factor + phospholipid), usu with a heparin neutralizer

calcium

plt-poor plasma

76
Q

what are the ingredients for aPTT testing?

A

partial thromboplastin (phospholipid)

negatively charged surface

plt-poor plasma

~incubate~

add Ca

NO HEPARIN NEUTRALIZER/SENSITIVE TO LUPUS ANTICOAG

77
Q

how do you calculate residual factor activity?

A

factor activity at that dilution

divided by

factor activity in a negative control

78
Q

MoA: heparin

A

antithrombin enhancer

unfractionated: inhibits factor Xa & thrombin equally

LMWH: inhibits predominantly factor Xa

79
Q

what assay is (hypothetically) used to measure the effecct of unfractionated heparin, but it’s too expensive to be practical?

A

anti-Xa assay

80
Q

how does the anti-Xa assay work?

A

chromogenic

factor Xa is a protease > it cleaves a reagent and forms a color > if you have an inhibitor present (ie unfractionated heparin) then you get less color

81
Q

what gets used in actual practice to measure heparin activity?

A

thrombin time

PTT or aPTT

82
Q

which bug has colonies resembling “grains of sand”?

(small pinpoint convex colonies)

A

brucella (gram negative coccobacillus)

83
Q

three biochemical properties of brucella

A
  1. rapid hydrolysis of urea (contains urease)
  2. reaction in 1-4 hours on Christians agar (wtf is this)
  3. positive cytochrome oxidase reaction (spot test)
84
Q

which bacteria does the hockey puck thing?

A

moraxella catarrhalis

85
Q

which bacteria does this, and what is the phenomenon called?

A

clostridium perfringens

double hemolysis: narrow zone of complete/B hemolysis d/t theta toxin + surrounding zone of incomplete/alpha hemolysis d/t alpha toxin

86
Q

who has more vWF: babies or adults?

A

babies

87
Q

which HLA is LEAST important to match in a transplant?

A

HLA DP and HLA DQ

88
Q

which HLA is the MOST important to match in a transplant?

A

HLA A, B, C, and DRB1 (specifically)

89
Q

universal PLASMA donor

A

AB

90
Q

universal PLASMA recipient

A

O

91
Q

universal RBC donor

A

O

92
Q

universal RBC recipient

A

AB

93
Q

preferred specimen for highest yield culture positivity in MUCOR

A

sliced (not ground) tissue

94
Q

plasma for PT

how long is it stable at room temp?

A

24h

95
Q

plasma for aPTT

how long is it stable at room temp?

A

4h

96
Q

plasma for PT

and

plasma for aPTT

what temp do you freeze them at?

how long are they stable at that temp?

A

freeze @ -70C

stable for up to 1 year for coag testing

97
Q

if you’re going to test plasma for FVIII or vWF, what temp can you store it at and for how long?

A

room temp: up to 4 h

frozen at -20 or -70C if testing will be delayed (idk for how long)

do NOT PUT THIS ON ICE

98
Q

reversal agent for factor Xa inhibitors

A

andexanet alpha

99
Q

reversal agent for dabigatran

A

idarucizumab

100
Q

warfarin cytochrome genotype/phenotype with the lowest enzyme activity and therefore the one that requires the lowest doses?

A

CYP2C9 *2*2

VKORC1 AA (warfarin sensitive)

101
Q

what 2 testing derangements will you see with defects in cytoskeletal proteins in RBCs?

A

decreased levels of AE1 (band 3)

decreased staining/decreased fluorescence intensity with eosin-maleimide (EMA) by flow

102
Q

mutation in LPL that confers more symptomatic/active disease and drug resistance?

A

CXCR4

(not MYD88)

103
Q

what is the atypical cell you see in peripheral blood smears of active EBV infxn?

A

it’s a reactive T-cell

(not a fucking monocyte)

104
Q

which mycobacteria (TB complex) is pyrazinamide resistant?

A

bovis (BCG)

105
Q

platelet plug: what are the 2 things that interact?

A

vWF on endothelium

+

GPIb on plts

(as opposed to aggregation, which is GPIIb/IIIa + fibrinogen)

106
Q

mutation in hepatosplenic T-cell lymphoma

A

isochromosome 7q

107
Q

mutation in T-cell large granular lymphocytic leukemia

A

TCR rearrangement

STAT3 mut in 1/3

108
Q

chromogenic anti-Xa test

what does “positive” look like?

what does “negative” look like?

A

“positive” i.e. pt is on a Xa inhibitor: there is less Xa around in pt sample, so less rxn occurs, so less color change

“negative” i.e. pt is NOT on a Xa inhibitor, so MORE Xa is around, so MORE rxn occurs, so stronger color

109
Q

what can you look for in meconium if you are worried about cocaine exposure in the baby?

A

M-hydroxybenzoylecgonine

110
Q

what’s the platelet count for plt-poor plasma?

A

less than 10 x 109/L

111
Q

one gram pos and one gram neg that are intrinsically resistant to colistin

A

gram pos: Enterococcus faecium

gram neg: Burkholderia cepacia

112
Q

which benzo doesn’t show up on a UDS?

A

lorazepam

113
Q

mutation in chronic neutrophilic leukemia

A

CSF3R

114
Q

warfarin mechanism

A

reduce the enzymatic activity of vitamin K epoxide reductase

115
Q

heparin mechanism of action

A

indirect inhibitors of FXa and FII (thrombin)

“indirect thrombin inhibitor”

it binds to antithrombin and makes it do what antithrombin does

116
Q

BNP vs NT-proBNP

name 3 differences

A
  1. NT-proBNP is more stable
  2. NT-proBNP has a longer half-life
  3. BNP is susceptible to degradation by neprilysin
117
Q

what do you use liquid Amies media for?

A

wound/throat specimen for bacterial culture

118
Q

what do you use Cary-Blair media for?

A

stool specimens for bacterial culture

119
Q

what is universal transport media good for transporting?

A

a sample for viral culture

it contains vanc and colistin to inhibit bacteria

120
Q

which bacteria has no peptidoglycan and therefore doesn’t stain with Gram stain?

A

Mycoplasma

121
Q

broadly, defect in

dyskeratosis congenita

A

telomere maintenance defect

122
Q

broadly, defect in

Chediak-Higashi

A

lysosomal trafficking defect

123
Q

broadly, defect in

severe congenital neutropenia

A

primary granule formation defect

124
Q

broadly, defect in

Schwachman Diamond

A

ribosome biogenesis defect

125
Q

broadly, defect in

Fanconi anemia

A

DNA repair pathway defect

126
Q

which bacteria has “bull’s eye” colonies?

A

Yersinia (enterocolitica)

127
Q

what does CIN stand for (agar)

A

cefsulodin-irgasan-novobiocin

128
Q

what are the 3 bacteria that can have elevated minimum inhibitory concentrations (MIC) to imipenem, but are NOT considered carbapenem-resistant enterobacteriaceae?

A

Morganella

Proteus

Providencia

129
Q

classic pentad of TTP

A
  1. thrombocytopenia
  2. microangiopathic hemolytic anemia (MAHA)
  3. fever
  4. renal failure
  5. altered mental status
130
Q

what are the minimum diagnostic criteria for TTP?

A

microangiopathic hemolytic anemia + thrombocytopenia without a better explanation

131
Q

how do you measure bilirubin in serum?

A

van den Bergh or “diazo” rxn

bilirubin + diazotized sulfanilic acid = azobilirubin

132
Q

mutation in factor V leiden

A

Arg 506 Glu

you normally have an Arg, it substitutes a Glutamine

133
Q

AML with monocytic differentiation + eos

A

inv(16)

134
Q

AML with bilobed “apple core” nuclei

A

t(15;17) PML-RARA

“apple core” = APL (acute promyelocytic leukemia)

135
Q

high urine pyridinium collagen cross-links (deoxypyridinoline, Dyd)

A

Paget disease

136
Q

hepatosplenic T-cell lymphoma genetic mutation

A

isochromosome 7q

137
Q

gamma/delta T-cells on flow should make you think of WHAT?

A

hepatosplenic T-cell lymphoma

138
Q

T-prolymphocytic leukemia genetics

A

inv(14)(q11q32)

139
Q

extranodal NK/T cell lymphoma genetics

A

del(6)(q21q25)

140
Q

NK cells are NEGATIVE for this on flow but POSITIVE for this on IHC

A

CD3

141
Q

the thing that is shaped like a banana in plasmodium falciparum is what part of the life cycle?

A

gametocyte

142
Q

how does plasmodium falciparum cause end organ damage?

A

schizonts get sequestered in peripheral tissue capillary beds > sludging

143
Q

secretin stimulation testing

what are you actually measuring?

A

bicarb secretion (from panc into duodenum)

144
Q

AABB Standard 5.12 (31st edition) states that ABO group confirmation is required for units of red cells, whole blood and granulocytes, but you only have to do Rh type confirmation for WHAT units?

A

Rh NEGATIVE

(not positive!)

145
Q

beta globin gene is on what chromosome?

A

11

146
Q

alpha globin gene is on what chromosome?

A

16

147
Q

what Rh type requires confirmation to r/o weak D?

A

Rh negative

148
Q

what test do you use to monitor unfractionated & low molecular weight heparin therapeutic levels?

A

anti-Xa assay

unfractionated: after 6h

LMWH: after 4h

149
Q

testing algorithm for polycythemia vera

A

JAK2 V617F testing

then reflex to JAK2 exon 12 if negative

150
Q

testing algorithm for MPN (PV vs ET vs PMF)

A

JAK2 V617F

reflex to CALR & MPL if negative

151
Q

name 4 species intrinsically resistant to vancomycin

A

GPCs: Leuconostoc, Weisella, Pediococcus

GPRs: Clostridium innocuum

152
Q

4 tests that require platelet poor plasma

A
  1. anti-Xa
  2. coagulation factor activities
  3. vWF
  4. lupus anticoagulant
153
Q

light chain type a/w AL amyloidosis?

A

Lambda

154
Q

light chain type a/w light chain deposition disease?

A

kappa

155
Q

minimum platelets in an apheresis unit?

A

3 x 1011 plts/bag

156
Q

minimum platelets in a whole blood-derived unit of plts?

A

5.5 x 1010 plts/bag

157
Q

amyloidosis is a/w an acquired factor deficiency of what factor?

A

factor X

158
Q

what cell does Ehrlichia infect?

A

monocytes

159
Q

what cell does Anaplasma infect?

A

granulocytes

160
Q

what cell does Babesia infect?

A

MATURE red cells (not reticulocytes)

161
Q

what is special about a stomatocyte

A

it’s Rh NULL

162
Q

lab values diagnostic of acute pancreatitis

A

serum amylase and/or lipase

> 3x upper limit of normal

163
Q

if you can only pick one lab value to evaluate for acute pancreatitis, which should you pick?

A

lipase

it’s more specific and will stay elevated longer than amylase

164
Q

autoimmune lymphoproliferative syndrome

genes affected

germline or somatic?

A

GERMLINE

heterozygous mutations in FAS, FASLG, CASP10

165
Q

most common cause of NAITP (neonatal alloimmune thrombocytopenia) in white people

A

HPA-1a

166
Q

prothrombin gene mutation G20210A specifics

A

guanine to adenine substitution at position 20210 of the 3’ untranslated region of the Factor II gene

167
Q

only human-infecting intracellular helminth

A

Trichinella spiralis

168
Q

dRVVT

the venom directly activates which coagulation factor?

A

factor X

169
Q

which red cell antigen contains disulfide bonds and is therefore destroyed with DTT?

A

Kell!

170
Q

you know how you can get factor XI deficiency in Ashkenazi Jewish patients? what’s another name for that

A

hemophilia C

171
Q

in a karyotype, how many cells have to have the same abnormality for you to call it a clone?

A

3

172
Q

what’s delta bilirubin?

A

bilirubin bound to albumin

173
Q

how do you estimate the concentration of delta bilirubin?

A

it’s like measuring gap

total bili - [conj + unconj bili] = delta bili

174
Q

candida species with intrinsic resistance to fluconazole

A

krusei

175
Q

what can you measure instead of GFR if you’re looking for a sex-independent method?

A

iothalamate clearance

176
Q

mast cell leukemia

mutation

A

C-KIT

177
Q

mast cell leukemia

aberrant flow markers

A

CD2

CD25

178
Q

normal mast cell markers

A

mast cell tryptase

CD117 BRIGHT

179
Q

what antibiotics is pseudomonas NOT intrinsically resistant to?

A

amikacin

cipro

colistin

pip-tazo

180
Q

what lab value should you monitor in a plasma exchange?

A

fibrinogen

181
Q

which mycobacteria is fastidious so it only grows on chocolate agar?

A

mycobacterium haemophilum

182
Q

which developmental stage of plasmodium should you NOT include on your parasitemia percentage?

A

gametocytes! ack!

183
Q
A

Rapid Fire CP Review (7 decks)

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