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Rapid Fire CP Review > Rapid Review 4 > Flashcards

Rapid Review 4 Flashcards

1
Q

type 1 Crigler-Najjar has *** UGT activity

A

has NO UGT activity

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2
Q

type 2 Crigler-Najjar has *** UGT activity

A

has SOME/REDUCED UGT activity

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3
Q

how do Gilbert and Crigler-Najjar bilirubin levels compare?

A

Gilbert: only slightly increased (3-6)
CN type 1: highest bili (>20)
CN type 2: intermediate (5-20)

(unconjungated bili)

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4
Q

pure red cell aplasia is a paraneoplastic syndrome related to what tumor?

A

thymoma

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5
Q

what mutation is associated with AML with maturation?

A

t(6;9) DEK-NUP214
[you have to be ~mature to do 69]

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6
Q

what labs favor cholestatic overr hepatocellular jaundice?

A

alk phos >>> transaminases

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7
Q

cirrhosis SPEP characteristics

A

beta-gamma bridging & low albumin

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8
Q

broadly, which method do you use for hematocrit?

A

conductometry

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9
Q

TTP is an auto-Ab to what

A

ADAMTS13

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10
Q

what blood product should you use in TTP?

A

cryo-poor plasma

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11
Q

why should you use ~this blood product~ in TTP?

A

cryo-poor plasma
bc it has ADAMTS13 (vWF-cleaving metalloprotease)

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12
Q

which worm is a/w megaloblastic anemia?

A

diphyllobothrium latum

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13
Q

CD markers in
T-ALL vs ATLL

A

T-ALL: CD4+/8+ sCD3-/cCD3+ [4 & 8 double positive makes it TALLer]
ATLL: CD4+/8- CD3+ popcorn cells [eating popcorn 43 times in atlanta]

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14
Q

mutation in Langer-Giedion

A

del8q24

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15
Q

what level of sweat chloride is suggestive of cystic fibrosis?

A

>60 mmol/L

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16
Q

mutation in Wilson disease

A

ATP7B

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17
Q

what is the gold standard for diagnosing Wilson disease?

A

elevated hepatic copper

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18
Q

are these low or high in Wilson disease?
ceruloplasmin
urinary copper excretion

A 
LOW ceruloplasmin (can appear normal d/t inflammatory states [APR]) 
HIGH urinary copper excretion
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19
Q

what pathway does Cowden syndrome impact?

A

mTOR pathway (PTEN in 85%, PIK3CA, AKT1)

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20
Q

inheritance of Cowden syndrome

A

auto DOMINANT

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21
Q

inheritance of Peutz-Jeghers syndrome

A

auto DOMINANT

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22
Q

mutation in Hartnup disease

A

SLC6A19
auto recessive

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23
Q

what’s the specific weird lab value in Hartnup?

A

increased urinary tryptophan

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24
Q

what is the problem in PKU/phenylkaptonuria?

A

phenylalanine hydroxylase deficiency
auto recessive mut in PAH gene on chrom 12

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25
Q

what is the problem in benign hyperphenylalaninemia?

A

deficiency of pterin-4a-carbinolamine dehydratase (altered BH4 cofactor)

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26
Q

what is the inheritance of ornithine transcarbamylase deficiency?

A

x-linked recessive
(complete or partial lack of ornithine transcarbamylase)
[urea cycle]

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27
Q

what is the problem in maple syrup urine disease?

A

BCKD deficiency: branched chain keto acid dehydrogenase
genes: BCKDHA, BCKDHB, DBT

auto recessive

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28
Q

what amino acids does BCKD break down?

A

leucine
isoleucine
valine
(LIV)

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29
Q

what is the deficiency in tyrosinemia type I?

A

fumarylacetoacetate hydrolase (FAH) deficiency

last step in tyrosine breakdown

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30
Q

what is the inheritance of tyrosinemia type I?

A

auto recessive

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31
Q

how do you diagnose tyrosinemia type I?

A

elevated succinylacetone in urine

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32
Q

what malignancy is tyrosinemia type I a/w?

A

HCC

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33
Q

what is the disease with “sweaty feet” odor?

A

isovaleric acidemia :(

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34
Q

what is the problem in isovaleric acidemia?

A

isovaleryl-CoA dehydrogenase deficiency

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35
Q

what is the disease with “boiled cabbage” odor + fetid breath?

A

hypermethioninemia

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36
Q

what is the issue in hypermethioninemia?

A

methionine adenosyltransferase deficiency (buildup of methionine > demyelination)

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37
Q

what is the disease that is aka fish-odor disease?

A

trimethylaminuria

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38
Q

what is the issue in trimethylaminuria/fish odor disease?

A

mutations in flavin monooxygenase 3 (FMO3) gene
buildup of trimethylamine

aka fish odor disease

Fish odor - Flavin monooxygenase

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39
Q

what is the mutation in hyperhomocysteinemia?

A

C677T mut in MTHFR [methylene tetrahydrofolate reductase]

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40
Q

which inborn error of metabolism is a/w an increased risk of thrombotic disorders?

A

hyperhomocysteinemia > elevated homocysteine causes endothelial cell toxicity and disrupted vascular hemostatic mechanisms

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41
Q

what are the 3 peroxisome biogenesis disorders, in order of severity?

A

least bad: Refsum disease (PHYH mut)
middle bad: neonatal adrenoleukodystrophy (ABCD1 mut)
worst: Zellweger syndrome (PEX mut)

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42
Q

which peroxisome biogenesis disorder can be treated with diet?

A

Refsum disesase

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43
Q

which peroxisome biogenesis disorder is a/w Leydig cell damage?

A

neonatal adrenoleukodystrophy

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44
Q

what is like, the core issue in peroxisome biogenesis disorders?

A

buildup of very long chain fatty acids (Refsum is phytanic acid specifically)

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45
Q

what is the mode of inheritance of the glycogen storage diseases?

A

all are auto recessive EXCEPT
TYPE IXa

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46
Q

what’s the eponym
type I GSD

A

von Gierke’s

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47
Q

what’s the deficiency
type I GSD

A

G6PC mut > glucose-6-phosphatase deficiency

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48
Q

what will TTP look like on a vWF gel?

A

increased high-molecular-weight multimers

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49
Q

vWD 2M

A

decreased ability of vWF to bind to GPIb receptor on plt (this is the opposite of 2B)

normal multimers on gel

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50
Q

vWD 2N

A

mutated vWF-factor VIII binding site > vWF can’t protect factor VIII from degradation (increased factor VIII clearance) > factor VIII levels fall

normal multimers on gel

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51
Q

vWD 2A

A

small, less active vWF bc large multimers can’t assemble

No HMWM or IMWM
only has LMWM on gel

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52
Q

vWD 2B

A

increased affinity of vWF for plt GPIb/V/IX (on plt) > rapid vWF clearance & low plts (this is the opposite of 2M)

No HMWM
Has IMWM and LMWM on gel

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53
Q

vWD type 3

A

NO vWF!

gel has an empty lane

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54
Q

which von willebrand disease types are quantitative?

A

type 1 and type 3

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55
Q

which von willebrand disease types are qualitative?

A

type 2A, 2B, 2M, 2N

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56
Q

which vWD can you NOT give DDAVP to?

A

type 2B

2B has increased affinity btwn vWF and GPIb > so you’ll just keep using up all your plts and worsen thrombocytopenia

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57
Q

what makes vWF?

A

endothelial cells & megakaryocytes

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58
Q

where is vWF stored?

A

endothelial cell: Weibel-Palade bodies

plts: alpha granules

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59
Q

3 normal functions of vWF

A
  1. bind to collagen + plt GPIb/V/IX complex > plt adhesion
  2. bind to plt GPIIb/IIIa > increased plt aggregation
  3. bind to FVIII > prolong half-life of FVIII
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60
Q

what is the gold standard for measuring vWF activity?

A

vWF:RCo

asks “how well does vWF agglutinate plts in response to ristocetin?”

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61
Q

what does vWF:Ag test?

A

measures amount of vWF present, via LIA or ELISA

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62
Q

what should you suspect if vWF activity (vWF:RCo) is decreased out of proportion to the decrease in vWF:Ag?

so RCo/Ag <0.5-0.7

A

type 2 vWD (esp 2A, 2B, 2M)

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63
Q

which vWD are auto recessive?

A

2N and 3

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64
Q

which vWD are auto DOMINANT?

A

1, 2A, 2B, 2M

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65
Q

what chromosome is the vWF gene on?

A

chromosome 12

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66
Q

what should you be thinking of if they give you a child with an abdominal mass and like, flow?

A

Burkitt

***multiple nucleoli, vacuolated cytoplasm***

CD19/20+, but also CD10+!!!

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67
Q

which glycogen storage disease is the most common one?

A

type I: von Gierke’s

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68
Q

which glycogen storage disease is the most severe?

A

type II: Pompe’s

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69
Q

what’s the eponym for type II glycogen storage disease?

A

Pompe’s

(pompeii has 2 ii’s)

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70
Q

what is the defect in Pompe’s?

A

alpha-1,4 glucosidase deficiency

aka

acid maltase deficiency

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71
Q

what’s the eponym for type III glycogen storage disease?

A

Cori’s/Forbes

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72
Q

what’s the defect in Cori’s/Forbes?

A

debranching enzyme deficiency

AGL mutation

(ABCD: andersen’s branching cori debranching)

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73
Q

what’s the eponym for type IV glycogen storage disease?

A

Andersen’s

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74
Q

what’s the defect in Andersen’s?

A

branching enzyme deficiency

(ABCD: andersen branching, cori debranching)

GBEI mutation

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75
Q

what is the eponym for type V glycogen storage disease?

A

McArdle’s

(ardle has 5 letters)

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76
Q

what’s the defect in McArdle’s?

A

muscle phosphorylase deficiency

(think of the IHC duh)

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77
Q

what’s the eponym for type VI glycogen storage disease?

A

Hers’

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78
Q

what’s the defect in Hers’ dz?

A

liver phosphorylase deficiency

PHKA2 mutation

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79
Q

what’s the eponym for type VII glycogen storage disease?

A

Tarui/Tauri

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80
Q

what’s the defect in Tarui’s?

A

muscle phosphofructokinase (pfk)

(causes deficiency of M subunit > myocytes & rbcs can’t use carbs for energy)

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81
Q

what’s the defect in type VIII glycogen storage disease?

A

hepatic phosphorylase kinase deficiency

82
Q

what’s the eponym for type VIII glycogen storage disease?

A

there isn’t one, lololol

83
Q

what’s the eponym for type IX glycogen storage disease?

A

there isn’t one AGAIN

84
Q

what’s the defect in type IX glycogen storage disease?

A

deficiency of phosphorylase b kinase

alpha subunit: PHKA1 (muscle) and 2 (liver)

beta subunit: PHKB (muscle & liver)

gamma subunit: PHKG2 (liver)

i have no idea if i need to know this

85
Q

what’s the defect in type XI glycogen storage disease?

A

absent GLUT2 transporter

86
Q

what’s the eponym for type XI glycogen storage disease?

A

fanconi-bickel

87
Q

which glycogen storage disease is inherited in an autosomal recessive fashion?

A

PHKA1 & 2: x-linked recessive

PHKB & G2: auto recessive (like the rest of the GSDs)

88
Q

which GSD has heart failure and death within 2 years?

A

type II: Pompe’s

89
Q

which GSD has liver cirrhosis & death within 5 years?

A

type IV: andersen’s

90
Q

which GSD has exercise-induced rhabdomyolysis & renal failure 2/2 myoglobinuria?

A

type V: McArdle’s

91
Q

which steps of heme synthesis happen in the mitochondria?

A

First step (ALAS)

Last 3 steps (CPO, PPO, FECH)

92
Q

which steps of heme synthesis happen in the cytosol?

A

ALAD

PBGD

URO3S

UROD

(steps 2-5)

93
Q

order of heme synthesis enzymes

A
  1. ALAS
  2. ALAD
  3. PBGD
  4. URO3S
  5. UROD
  6. CPO
  7. PPO
  8. FECH
94
Q

disease name & inheritance

aminolevulinic acid dehydratase (ALAD) deficiency

A

ALAD porphyria

95
Q

disease name & inheritance

porphobilinogen deaminase deficiency

A

acute intermittent porphyria (AIP)

auto DOMINANT

96
Q

what cofactor does ALAS need?

A

vitamin B6 (pyridoxine)

97
Q

which forms of ALAS control hgb synthesis and where?

A

ALAS2: bone marrow

ALAS1: hepatocytes

feedback inhibition: heme blocks ALAS from entering mt

98
Q

what is ALAD extremely sensitive to?

A

LEAD

(this also inhibits FECH)

99
Q

how do you die of lead poisoning?

A

CNS damage

(it is NOT buildup of ALA d/t ALAD inactivation)

100
Q

what is the most common porphyria?

A

acute intermittent porphyria

(PBGD def)

101
Q

what’s another name for PBGD (porphobilinogen deaminase)?

A

hydroxymethylbilane synthase

(this step forms hydroxymethylbilane)

102
Q

if you suspect AIP, what should your first test be?

A

urinary porphobilinogen

and then? total porphyrin testing

103
Q

what is a normal level of porphobilinogen excretion?

104
Q

disease name & inheritance

uroporphyrin III synthase (URO3S) deficiency

A

congenital erythropoietic porphyria (no proto)

auto recessive

105
Q

disease name & inheritance

uroporphyrinogen decarboxylase (UROD) deficiency

A

porphyria cutanea tarda

80% acquired, 20% inherited auto DOM

106
Q

disease name & inheritance

coproporphyrinogen oxidase deficiency

A

hereditary coproporphyria

auto DOMINANT

107
Q

disease name & inheritance

protoporphyrinogen oxidase deficiency

A

variegate porphyria

autosomal DOMINANT

108
Q

disease name & inheritance

ferrochelatase (FECH) deficiency

A

erythropoietic protoporphyria

auto DOMINANT with poor penetrance

109
Q

where does most transferrin come from?

A

99% comes from the digestion of old RBCs!

110
Q

which porphyrias are the erythropoietic ones?

A

they have erythro in the names

erythropoietic protoporphyria (FECH)

congenital erythropoietic porphyria (URO3S)

(the rest are hepatic)

111
Q

which porphyrias are autosomal recessive?

A

ALAD porphyria (ALAD)

congenital erythropoietic porphyria (URO3S)

112
Q

which porphyrias result in photosensitivity & why?

A

def in UROD, CPO, PPO, or FECH

[anything after URO3S]

d/t buildup of tetrapyrrole rings

113
Q

which photosensitive porphyrias are delayed & which are immediate & why?

A

delayed: uro & copro buildup: UROD & CPO def
immediate: proto buildup: PPO & FECH def

114
Q

which porphyrias are acute?

A

AIP (PBGD)

variegate (PPO)

coproporphyria (CPO)

ALAD def

115
Q

which porphyrias are non-acute?

A

congenital erythropoietic porphyria (URO3S)

cutanea tarda (UROD)

erythropoietic protoporphyria (FECH)

116
Q

which aspergillus spp are uniseriate?

117
Q

which aspergillus spp are biseriate?

A

niger

terreus

flavus

118
Q

which aspergillus species have circumferential phialides?

A

flavus & niger

119
Q

which aspergillus species have NON circumferential phialides?

A

fumigatus (single layer)

terreus (double layer)

120
Q

what fluid should you use for EBV dx?

121
Q

what thyroid neoplasm is a/w amyloid?

A

medullary thyroid cancer

122
Q

what tumor marker should you use to monitor medullary thyroid cancer?

A

calcitonin

123
Q

WBC count for adequate leukoreduction in a whole blood unit

A

fewer than 8.3 x 105

124
Q

explain the dilute Russell viper venom test

A
  1. RVV activates factor X > Xa
  2. RLS: Xa + prothrombin/factor II + factor V + phospholipid > thrombin

if there’s not enough phospholipids (such as in the presence of antiphospholipid antibodies), then it won’t be as available for thrombin creation, and clotting time is prolonged

[less phospholipids > less thrombin > less clotting > prolonged clotting time]

125
Q

what syndrome presents with livedo reticularis + multiple embryo losses?

(+/- clots, preeclampsia, placental abruption)

A

antiphospholipid antibody syndrome (APLAS)

126
Q

how do you test for a lupus anticoagulant?

what is the principle screening test?

A

Staclot-LA test

or

dRVVT (this is the principle one)

127
Q

what is Staclot-LA test?

A

hexagonal phase phospholipid neutralization test

if you have lupus anticoagulant/antiphospholipid antibody in a tube, and you add phosphatidylethanolamine > prolonged PTT

128
Q

if you have antiphospholipid antibody syndrome (APLAS), then what specific antibodies might you have?

A
  1. lupus anticoagulant
  2. anticardiolipin antibody (MOST COMMONLY MEASURED)
  3. anti-beta-2-glycoprotein-I
129
Q

what causes epidemic typhus?

A

Rickettsia prowazekii

130
Q

what causes murine typhus?

A

Rickettsia typhi

131
Q

what causes sylvatic typhus

A

Rickettsia prowazekii

132
Q

what transmits Rickettsia prowazekii?

A

human body louse

flying squirrel ectoparasites

Amblyoma ticks

(so this is like the other Rickettsia)

133
Q

what causes scrub typhus?

A

orienta tsutsugamushi

134
Q

how is orienta tsutsugamushi transmitted?

135
Q

T/F: ALL viruses are obligate intracellular organisms.

A

TRUE

ALL OF THEM!

136
Q

what test helps you differentiate between immune & non-immune hemolysis?

A

DAT

(although sometimes it’s abn in non-immune too so idk)

137
Q

is staph saprophyticus resistant or sensitive to novobiacin?

138
Q

what is the most common chromosomal translocation in childhood ALL?

A

t(12;21) ETV6-RUNX1

WHICH IS THE SAME FUCKING THING AS

t(12;21) TEL-AML1

139
Q

name the 4 sources of the metabolite morphine in the body

A
  1. codeine
  2. heroin
  3. morphine
  4. poppy seeds
140
Q

what is the name of the unique metabolite to heroin?

141
Q

how long does 6-MAM stay in the urine after herin use?

142
Q

what is the metabolite of methadone?

A

EDDP

2-ethylidene-1,5-dimethyl-3,3-diphenylpyrrolidine

143
Q

what drugs can cause a false positive on methadone specific immunoassays?

A

verapamil

quetiapine

diphenhydramine

doxylamine

144
Q

what is the deficiency in hyperhomocysteinemia?

A

cystathionine-β-synthase

145
Q

long-term immunity in Hep B

146
Q

acute stage Hep B

A

HBsAg & HBeAg

147
Q

“core window” period of Hep B

A

anti HBc total and anti HBc IgM

148
Q

what is the core window period of Hep B exactly?

A

the 6 months between acute onset and permanent immunity

149
Q

you need 2 things for computer crossmatch, what are they

A
  1. no record of previous detection of an antibody
  2. T W O determinants of the patient’s ABO group
150
Q

which viruses take ~2 weeks to show viral cytopathic effect?

151
Q

which virus has retractile fiber formation

152
Q

most common genetic abnormality identified in FISH in CLL

153
Q

there are 3 FISH cytogenetic abnormalities and three additional things that portend poor survival in CLL. what are they?

A

FISH

  1. del17p
  2. del11q
  3. “complex abnormalities”

Unmutated immunoglobulin heavy chain variable gene (IgHCV gene)

ZAP-70 expression (>20%)

CD38 expression (>30%)

***please note that the most common cytogenetic abnormality in CLL, del13q, is NOT included in this list.

154
Q

rearrangements involving what chromosome/gene are usually found in ALCL?

A

chromsome 2: ALK gene

classic translocation is t(2;5) ALK-NPM

155
Q

what test do you use to monitor therapy in a patient being treated for TTP?

A

platelet count, keep it simple

156
Q

screening test for lupus anticoagulant

A

LA sensitive aPTT

colloidal silica clotting time

kaolin clotting time

dRVVT

157
Q

which heme malignancy is a/w basophilia?

158
Q

whose egg is this

A

diphylidium caninum

159
Q

what’s this

A

diphylidium caninum proglottid

160
Q

3 ways you can tell mycobacterium bovis apart from tuberculosis

A
  1. tuberculosis reduces nitrate very strongly, bovis does not
  2. tuberculosis is positive for niacin accumulation, bovis does not
  3. tuberculosis will grow on thiophene-2-carboxylic hydrazine (TCH) medium, bovis does not
161
Q

what peripheral blood finding should clue you in to hairy cell?

A

monocytopenia

162
Q

specific marker for hairy cell

A

annexin A1

(TRAP, CD103, CD11c, CD25 also all positive)

163
Q

how does hairy cell look/infiltrate in bone marrow?

164
Q

how does ascorbic acid interfere with UA results?

A

most of the UA strip tests are oxidating rxns, and vitamin C is an antioxidant, so it quenches the rxns

165
Q

what vitamin is ascorbic acid?

166
Q

what rxns does ascorbic acid interfere with on a UA?

A

blood

urobilinogen

bilirubin

glucose

nitrite

leukocyte esterase

167
Q

what’s this?

A

morulae of anaplasma/ehrlichia

168
Q

what’s this?

A

Rickettsia rickettsii

169
Q

what cells does Rickettsia infect?

A

vascular endothelial cells

170
Q

reference standard for RMSF diagnosis

171
Q

what bug is implicated in trench fever?

A

bartonella/rochalimaea Quintana

172
Q

what transmits bartonella/rochalimaea quintana?

A

human body louse

173
Q

what stain can you use to dx bartonella?

A

warthin-starry

174
Q

algorithm for fatty acid oxidation disorder suspicion

A
  1. screen blood & bile for acylcarnitine analysis
  2. frozen sections of liver > ORO, metabolic profile, carnitine studies
  3. fibroblast culture of skin > in vitro enzyme and molecular studies
175
Q

pseudocholinesterase deficiency > delayed metabolism of what drug?

A

succinylcholine

176
Q

what is the most likely antibody implicated in an ABO mismatch HDFN case?

A

probably anti-A,B antibodies bc they are IgG and they can cross the placenta

177
Q

what is the first line treatment for a patient with ITP and a plt count under 30?

A

steroids, long course

IVIg

IV RhIg in Rh+ patients

178
Q

which lymphoma has a universally extremely high Ki that usually exceeds 95%?

179
Q

DLBCL: good prognosis

A

CD10 & Bcl-6 positive

these suggest germinal center origin which is better than activated B-cell subtype

180
Q

most common cytogenetic abnormality in DLBCL

A

most common translocation involves 3q27 (Bcl-6)

181
Q

Assign +/- for CD10, MUM1, and Bcl6 for

DLBCL, ABC

DLBCL, GC

A

ABC: CD10 neg, MUM1 positive

GC: CD10 or Bcl-6 positive, MUM1 negative

182
Q

Brucella is a gram-negative coccobacillus that is positive for 4 biochemical reactions. what are they?

A
  1. urease
  2. nitrate reduction
  3. oxidase
  4. catalase
183
Q

Brucella and Bordetella are both urease & oxidase positive. How do you tell them apart?

A

Bordetella WILL grow on MacConkey

Brucella will NOT grow on MacConkey

184
Q

what’s this

A

brucella mellitensis

185
Q

best method for measuring antithrombin activity

A

chromogenic amidolytic

186
Q

best method for measuring antithrombin antigen levels

A

ELISA

immunodiffusion

latex particle immunoturbidimetric

187
Q

Type I AT deficiency and Type II AT deficiency

which is qual & which is quant?

A

Type I: quantitative antithrombin defect

Type II: qualitative antithrombin defect

188
Q

Type I AT deficiency and Type II AT deficiency

antigen assay & functional assay

low/high/normal in each?

A

type I (quant): Ag assay low, functional assay low [bc you have so little of it that you have less activity]

type II (qual): Ag assay normal, functional assay low

189
Q

assign each of these to a disorder

A
  1. smooth - SLE
  2. centromeric (aka speckled) - CREST/scleroderma
  3. peripheral - SLE
  4. nucleolar - Sjogren’s
190
Q

marginal zone B-cells, mantle zone B-cells, and paracortical T-cells are all positive for X

whereas germinal center B-cells are negative for X but positive for Y

A

X = Bcl-2

Y = Bcl-6

191
Q

simplified

CD10+ lymphoma

A

follicular or DLBCL

192
Q

simplified

CD10-/5+ lymphoma

A

SLL or mantle cell

193
Q

simplified

CD10-/5+/23+

194
Q

simplified

CD10-/5+/23-

A

mantle cell

195
Q

simplified

CD10-/5-

A

MZL (MALT) or LPL

196
Q

simplified

CD15+/30+ lymphoma

197
Q

which Ig antibodies can cause intra and extravascular hemolysis?

A

IgM = intra (multivalent)

IgG = extra (two Fab sites)

198
Q

how does EBV get into a cell?

199
Q

giant platelets should make you think of THESE entities (there are 4)

A

acquired: MDS
inherited: Bernard-Soulier, May-Hegglin, familial macrothrombocytopenia

200
Q

what is the enzyme that transports most (~95%) of the copper from the GI tract to peripheral tissues?

A

ceruloplasmin

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