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Undeleted > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Heme synthesis precursors

A

succinyl-Coa and glycine

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2
Q

General cause of microcytic/hypochromic anemia

A

Hb synthesis impairment

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3
Q

General cause of macrocytic/normochromic anemia

A

impaired DNA synthesis

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4
Q

Cyclic flu-like sx
seizures
hemoglobinuria
sausage shaped organisms of microscopy

A 
Plasmodium falciparum (malaria)
worst type
P vivax and P. ovale cause relapsing sx
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5
Q

Tx for malaria

A

chloroquine for nonsevere
quinine/quinidine for severe
primaquine for vivax/ovale (liver hypnozoite stage)
Doxycycline for prophylaxis
All drugs use caution with G6PD deficiency or pregnancy

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6
Q

Pear shaped trophoziotes
maltese cross seen
hemolytic anemia

A

Babesiosis

caused by black legged tick (ixodes scapularis), same is B. burgdorferi (Lyme’s)

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7
Q

Hx of African travel
chancre
pruritis/fever
progresses into seizures/somnolence

A

Trypanosoma brucei, african sleeping sickness
tsetse fly vector
hemolytic and CNS stages
tx with suramin for hemolytic, melarsoprol for CNS

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8
Q

unilateral painless periorbital edema in a child
anorexia and lesion of inflammation
chronic cardio and GI pathology

A

Trypanosomia cruzi (Chagas disease)
Romana’s sign and chagoma seen
vector is reduviid (kissing) bugs

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9
Q

Dx for Trypanosomiasosis

A

Giemsa stain smear

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10
Q

Ulcerating lesions that spontaneously heal
Lesions on nasal mucosa with erosion
skin blackens/systemic infection
Tx with Na+ stibogluconate

A

Leishmaniasis
L. braziliensis causes erosion on face
L. donovani causes black poison/Kalaazar (visceral necrosis)
Vector is sandflies

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11
Q

Intrinsic clotting pathway

A

Factor XII, XI, IX, X (in that order)

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12
Q

Extrinsic clotting pathway

A

Factor VII to X

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13
Q

Common clotting pathway

A

Factor X & V, thrombin, fibrin (in that order)

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14
Q

Stimulators of clotting pathways

A

intrinsic-kallikrein/kininogen

extrinsic-trauma to vasculature

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15
Q

Clot formation pathway (from thrombin)

A

thrombin cleaves fibrinogen to fibrin
fibrin aggregates with platelets (via GP IIb/IIIa)
factor XIIIa cross links to hard clot (via Lysine covalent bond to N-terminus)

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16
Q

Endogenous inhibits of clotting

A

thrombomodulin (activates protein C/binds thrombin)
heparan sulfate (activates ATIII)
prostacycline (inhibits platelet aggregation)
Protein C inactiveas factors Va and VIIIa

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17
Q

Factor VIII deficiency

hemarthrosis

A

Hemophilia A

X linked recessive

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18
Q

Factor IX deficiency

hemarthrosis

A

Hemophilia B

X linked recessive

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19
Q

AD inheritance
decreased factor VIII delivery
mucous membrane bleeding

A

von Willebrand disease

normal platelet counts

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20
Q

ineffective protein C/S coagulation inactivation
AD
hypercoaguability

A

Factor V Leiden mutation

factor V resistant to protein S/C inactivation

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21
Q

Levels of this protein decrease with active intravascular hemolysis

A

Haptoglobin
bind free Hb
protects kidney from Hb

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22
Q

AR
bronze skin color
diabetes sx
dx with hepatocellular carcinoma

A

Hemochromatosis
defect in HFE gene (chromosome 6)
excessive iron storage

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23
Q

Levels of molecule are eleveated in multiple myeloma

A

gamma-globulin

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24
Q

Petechial rash starts from limbs and moves to trunk

tx with doxycycline

A

Rickettsia rickettsii
dog/wood tick vector
Rocky Mountain spotted fever
liver/brain/skin sx

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25
Q

Mite vector/mice reservoir

papule at bite site, crusts over

A

Rickettsia akari

Rickettsial pox

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26
Q

body lice vector/flying squirrel

pink macules on upper trunk, spread outward

A

Rickettsia prowazekii
epidemic typhus
Brill-Zinsser Disease (recurrence years later)

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27
Q

Flea vector
U.S. Gulf coast
rash on chest and abd

A

Rickettsia typhi
endemic typhus
low mortality

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28
Q

Chiggers/mites/rodents vector
scab at bite site
flat maculopapular rash

A

Orientia tsutsugamushi
scrub typhus
Asia and SW pacific

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29
Q

Infection of WBC’s
tick vector
rash with thrombocytopenia

A

Ehrlichiosis
Monocyte-E. chaffeensis
Granulocyte-Anaplasma phagocytophilum
morulae on Giemsa stain

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30
Q

Tick vector
US NE/Wisconsin
tx with doxycycline

A

Borrelia burgdorferi
bulls eye rash
migratory arthralgias

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31
Q

Recent trip to Hawaii for surfing
invasion of CSF and blood
recurring sx

A 
Leptospira interrogans
animal urine is cause
Weil Syndrome (liver/blood/CNS)
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32
Q

Poor hygiene
bone pain in back/legs
recurs every 5 days

A

Bartonella quintana
trench fever
tx with doxy

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33
Q

Trip to South America
anemia
Sandfly vector
blood filled nodules in skin

A

Bartonella bacilliformis
oroya fever/carrion disease
infects RBCs

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34
Q

Low serium Fe and ferritin
increased TIBC
GI bleed

A

iron deficiency anemia

microcytic/hypochromic

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35
Q

Microcytic anemia

esophageal webs

A

Plummer-Vinson syndrome

also see atrophic glossitis

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36
Q

Hypersegmented neutrophils (5+nuclei)
vision loss
loss of sensory/motor

A

Pernicious anemia, megaloblastic (B12 deficiency)

macrocytic/normochromic

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37
Q

AR
defect in DNA repair
aplastic anemia

A

Fanconi’s syndrome

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38
Q

Assc with aplastic anemia

A

thymoma

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39
Q

Jaundice/anemia
increased LDH and reticulocytes
decreased Hb and haptoglobin

A

Hemolytic anemia

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40
Q

increased osmotic fragility
round RBCs
splenomegaly

A

Hereditary spherocytosis
mutated spectrin/ankyrin/band 3 or 4.2 protein
AD in whites

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41
Q

Aplastic crisis with hereditary spherocytosis assc

A

parvovirus B19

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42
Q

Bite and spherocyte RBCs
Heinz bodies in RBCs
AR in blacks/mediterannean

A

G6PD deficiency
lack of NADPH to reduce GS
causes oxidative damage to RBCs

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43
Q 
Chronic hemolytic anemia
AR in blacks
vessel occlusions
splenomegaly
gallstones
A

Sicke Cell disease
Glutamate to Valine at pos 6 on beta globin
Heterozygote malarial resistance

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44
Q

Bug risks for Sickle Cell

A

encapsulated organism infection
aplastic crisis with parvovirus B19
osteomyelolitis with salmonella

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45
Q 
Hb Bart (gamma tetramers) in blood
early death
A

Alpha-thalassemia (hydrops fetalis)

all 4 genes deleted

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46
Q

IgG against RBC Ags
spherocytes/splenomegaly
direct Coomb’s test (+)

A

Warm autoimmune hemolytic anemia

hapten or drug + RBC=new Ag

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47
Q

IgM against RBC’s
worse in winter
assc with MGUS

A

Cold autoimmune hemolytic anemia
pentamers bind in cold periphery, release when returning to body core
Causes complement/hemolysis

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48
Q

RBCs lack CD55 and CD59
chronic hemolysis
hypercoaguable

A

Paroxysmal noctural hemoglbinuria
CD55/CD59 usually prevents complement
lysis by endogenous complement

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49
Q

depletion of coagulation factors
concurrent clotting and fibrinolysis
has promyelocytic leukemia

A

Disseminated intravascular coagulation
assc with infection/malignancy
uncontrolled fibrinolytic &clotting system activation

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50
Q

Schistocytes
neuro/kidney sx
lg bruises

A

Thrombotic thrombocytopenic purpura
Abs or defect of ADAMTS13
pentad of sx FAT RN

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51
Q

AR
increased bleeding
platelet aggregations in blood

A

Bernard Soulier syndrome
defect in IbIX on platelet
cannot bind subendothelium

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52
Q

AR
increased bleeding
normal platelet morphology

A

Glanzmann Thrombasthenia
defect in IIb/IIIa
platelets cannot bind fibrinogen

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53
Q

Risk of thienopyridines

A

can cause TTP (especially ticlopidine)
inhibit ADP/platelet aggregation
ticlopidine/clopidogrel/prasugrel

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54
Q

Clopidogrel drawback

A

poor responders due to being a prodrug

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55
Q

Prasugrel contraindications

A

over 75
TIA or CVA hx
less than 60kg

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56
Q

Only nonthienopyridine ADP antagonist

A

ticagrelor

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57
Q

GP IIb/IIIa inhibitors

A

abciximab
eptifibatide
tirofiban
they prevent fibrinogen platelet aggregation

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58
Q

Phosphodiesterase inhibitors for anticoagulation

A

dipyridamole
cilostazol
for tx of ambulatory claudication

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59
Q

Warfarin effects

A

increased INR/PT

stops factors II, VII, IX, X, C and S protein synthesis

60
Q

Need before warfarin therapy in thrombotic pt

A

start heparin first

prevents paradoxical hypercoagulability

61
Q

Tx for warfarin overdose

A

Vit K and frozen plasma

62
Q

Side effect caused by protein C and S deficiency with warfarin

A

skin necrosis

63
Q

Unfractionated heparin MOA/risk

A

complexes with antithrombin III/thrombin
can cause heparin induced thrombocytopenia
tx with direct thrombin inhibitors

64
Q

Tx for heparin overdose

A

protamine sulfate

65
Q

Heparin induced thrombocytopenia

A

caused by IgG+heparin+PF4 complex
causes platelet aggregation
tx with direct thrombin inhibitor

66
Q

Direct thrombin inhibitors

A

lepirudin/bivalirudin/argatroban/dabigatran

dabigatran is only oral one

67
Q

Factor Xa inhibitors

A

fondaparinux/rivaraxaban/apixaban

no antidote

68
Q

Fondaparinux

A

factor Xa inhibitor
binds antithrombin III
used for DVT/PE

69
Q

Rivaraxaban/apixaban

A 
factor Xa inhibitors
do not bind antithrombin III
used for tx of A fib to reduce stroke risk
both need renal dose adjustment
need to anticoagulate when taking off
70
Q

Anticoagulation/platelet drugs needing renal adjustment dose

A

LMWH
dabigatran (direct thrombin inhibitor)
rivaroxaban/apixaban (Xa inhibitors)

71
Q

Mismatch blood type rxn

A

type II hypersensitivity

72
Q

MHC I and II genes

A

MHC I- A,B,C

MHC II- DP, DQ, DR

73
Q

Hyperacute graft rejection

A

preexisting host Abs to graft Ags
type II HS
complement activated

74
Q

Matching needed for transplants:
heart
liver
bone marrow

A

heart-blood type, MHC II if possible
liver-blood type
bone marrow-HLA and ABO typing needed

75
Q

Definitions:
autograft
isograft/syngeneic
allograft

A

autograft-from self
isograft-from identical twin
allograft-from same species

76
Q

Cause of ABO blood type production

A

gut colonization of bacteria
A&B- IgM
O-IgG

77
Q

Ability of A and B blood types to agglutinate

A

IgM larger than IgG of O type
spans zeta-potential
allows agglutination b/t RBC’s

78
Q

Rh Ag

A

D is most antigenic
on chromosome 1
nonagglutinating IgG
causes extravascular hemolysis

79
Q

ABO blood typing methods

A

forward type- detect Ags

Reverse type- detect Abs

80
Q

Pt has transplant and is not infected by a bug, which is most likely?

A

CMV

Hep B/C

81
Q

Low back pain
hematuria
increased IgM/IgG

A

acute hemolytic rxn

factor XII/kinin activated clotting

82
Q

Contents of platelet granules:
alpha
dense
lysosomes

A

alpha-vWF/fibrinogen/factor V/PAI-1 (platelet adhesion)
dense-ADP/5HT/Ca2+ (platelet aggregation)
lysosomes-acid hydrolases

83
Q

What prevents fibrinolysis endogenously?

A

PAI-1 via t-PA inhibition

alpha2-antiplasmin via plasmin

84
Q

What activates plasmin?

A

t-PA converts plasminogen to plasmin

plasmin then degrade fibrin clots

85
Q

Increased PT and aPTT
(+) D-dimer
bleeding/microvascular thrombi

A

Disseminated intravascular coagulation

decreased platelets and fibrinogen

86
Q

Lg multimers of vWF bound to platelets
schistocytes
normal PT and aPTT

A

thrombotic thrombocytopenic purpura

ADAMTS-13 defect (normally degrades vWF multimers)

87
Q

Mucocutaneous bleeding
AR
no clotting

A

Glanzmann thrombasthenia

abn GP IIb/IIIa, no platelet aggregation

88
Q

Giant platelet aggregates in blood
thrombocytopenia
AR
increased bleeding

A

Bernard-Soulier syndrome
abn GP Ib/IX-V or vWF receptor
not platelet adhesion

89
Q

Lab seen with beta-thalassemia

A

compensatory increase in alpha and gamma globulins (fetal Hb)

90
Q

Pt with vaso-occlusive events
splenomegaly
pt tx with hydroxyurea, how does it improve the condition?

A

for Sickle Cell, hydroxyurea increases gamma globin gene production
gamma globin does not sickle and will not cause vaso-occlusions

91
Q

Increased LDH/AST
(+) direct Coombs test
schistocytes/spheroctyes seen

A

Warm autoimmune hemolytic anemia

IgG mediated

92
Q

Pt has anemia following a URT infection

gets worse in the cold

A

Cold autoimmune hemolytic anemia

Igm mediated

93
Q

AR
jaundice seen in 5 day y/o child
european descent

A

Pyruvate kinase deficiency

from decreased ATP production in RBC’s

94
Q

Loss of fine touch/vibration/proprioception
alcoholic
anemia

A

B12 deficiency anemia
megaloblastic anemia
pernicious anemia can cause
alcohol/ileum problems can cause

95
Q 
Normal RBCs
anemia
increased reticulocytes
AD
(-) Coombs test
A

Hereditary spherocytosis
defect in ankyrin/spectrin/band 3.1
(+) osmotic fragility test

96
Q

Howell-Jolly bodies are seen in RBC’s. What does that signify?

A

Pt had a splenectomy

97
Q

Hypercoaguablility in young white male

G1691A gene mutation

A

Factor V Leiden

factor V cannot be inactivated by protein C or S

98
Q

Prothrombin gene mutation effect

A

increased levels of prothrombin
hypercoaguable
due to 3’ UTR gene mutation (increased transcription)

99
Q 
Livedo reticularis (arterial clots)
TIA's
A

Antiphospholipid syndrome

anticoagulant and cardiolipin Abs

100
Q

Tx for acute MI/massive PE/limb DVT/acute stroke within 90 mins of onset

A

thrombolytics (t-PA/streptokinase)
activate plasmin
must be followed by UFH within 24hrs

101
Q

Location of lymphoma

A

tumor mass in LN

102
Q

Leukemoid reaction

A

response to infection

increasedWBC/left shit/leukocyte alkaline phosphatase

103
Q

Hodgkin’s lymphoma characteristics

A 
single group of nodes (contiguous spread)
Reed-Sternberg cells
bimodal (20's and over 60)
assc with EBV
B sx (night sweat/wt loss)
104
Q

Non Hodgkin’s lymphoma presentation

A

multiple peripheral LN involved
extranodal involvement
B cells
20-40 y/o

105
Q

Reed-Sternberg cell description

A

tumor giant cell
binucleate/bilobed (mirror images)
CD30 and CD15 (+)
B cell origin

106
Q

African child
jaw lesion
Starry sky appearance

A

Burkitt’s lymphoma
t(8:14) causing c-myc promoted by Ig heavy chain
assc with EBV

107
Q

Mantle Cell lymphoma

A

t(11:14)
cyclin D1 and Ig heavy chain
CD5 (+)

108
Q

Follicular lymphoma

A

t(14:18)
bcl-2 and Ig heavy chain
bcl-2 inhitis apoptosis
indolent course

109
Q

Adult T cell lymphoma

A

caused by HTLV-1
in Japan/Africa/Caribbean
cutaneous lesions

110
Q

Mycosis fungoides/Sezary syndrome

A

adults with cutaneous patches/nodules
CD4 (+)
indolent

111
Q 
Fried egg appearance
lg amounts of IgG or IgA
lytic bone lesions
renal failure
anemia
A 
Multiple myeloma (from marrow)
M spike of protein
Bence Jones protein (light chain) damages kidney
Rouleaux formation (stacked RBC's)
clock face chromatin
112
Q

Monoclonal gammopathy of undetermined significance

A

MGUS
increased M spike (excess Ig’s made)
precursor to multiple myeloma (1%/per progress)

113
Q

Acute lymphoblastic leukemia/lymphoma

A 
children
TdT (+) and CALLA (+)
increased lymphocytes
t(12:21) better prognosis
can spread to CNS
114
Q

Chronic lymphocytic leukemia (small lymphocytic lymphoma)

A

over 60 y/o
smudge cells on peripheral blood smear
autoimmune hemolytic anemia
SLL has less lymphocytosis in periphery

115
Q

Hairy cell leukemia

A

in adults
mature B cell tumor
hair like projections on cells
stains TRAP (+)

116
Q

Acute myelogenous leukemia

A 
over 65 y/o
Auer rods
increased myeloblasts in periphery
t(15:17)
responds to retinoic acid
DIC is common presentation with M3 AML
117
Q

Chronic myelogenous leukemia

A 
30-60 yrs
Philadelphia chromosome t(9:22)
bcrl-abl fusion protein
blast crisis, progresses to AML/ALL
splenomegaly
responds to imatinib (tyrosine kinase inhibitor)
118
Q

Auer rods

A

peroxidase (+) inclusions in granulocytes/myeloblasts
seen in M3 AML
can be released and cause DIC

119
Q

CML translocation

A

Philadelphia chromosome
t(9:22)
bcr-abl

120
Q

Burkitt’s lymphoma translocation

A

t(8:14)

c-myc activation

121
Q

Mantle cell lymphoma translocation

A

t(11:14)

cyclin D1 activation

122
Q

Follicular lymphoma translocation

A

t(14:18)

bcl-2 activation

123
Q

M3 type of AML translocation

A

t(15:17)

responds to all trans retinoic acid (Vit A)

124
Q

Child
lytic bone lesions
skin rash
Birbeck granules (tennis rackets on EM)

A

Langerhans cell histiocytosis
dendritic cell disorder
cannot stimulate T lymphoctyes with Ag presenting
cells express S-100 (neural crest) and CD1a

125
Q

Polycythemia vera

A

abn clone of HSCs with JAK2 receptors active
proliferate without EPO
type of myeloproliferative disorder

126
Q

Essential thrombocytosis

A

uncontrolled

proliferation of megakaryocytes

127
Q

MOA of methotrexate

A

inhibits dihydrofolate reductase

can cause mucositis

128
Q

MOA of 5-FU

A

inhibits thymidylate synthase

no dTMP, and therefor no DNA synthesis

129
Q

MOA of 6-Merrcaptopurine/azathioprine/6-TG

A

purine analogs

activated by HGPRT

130
Q

MOA of dactinomycin

A

intercalates in DNA

131
Q

MOA of doxorubicin

A

generates free radicals
intercalates in DNA
causes breaks

132
Q

MOA of bleomycin

A

induces free radical formation

causes DNA breaks

133
Q

MOA of cyclophosphamide

A

X link DNA at guanine N-7

activated by the liver

134
Q

Use of nitrosoureas

A

for CNS tumors

135
Q

MOA of busulfan

A

alkylates DNA

for CML

136
Q

MOA of vinblastine/vincristine

A

bind tubulin in M phase

blocks polymerization

137
Q

Side effects of vinblastine/vincristine

A

vincristine-neurotoxicity

vinblastine-myelosuppression

138
Q

MOA of cisplatin

A

cross links DNA

139
Q

MOA of etoposide/teniposide

A

inhibits topoisomerase II

increased DNA degradation

140
Q

MOA of hydroxyurea

A

inhibits ribonucleotide reductase
decreased DNA synthesis
also for Sickle cell (increased HbF)

141
Q

MOA of prednisone

A

triggers apoptosis

142
Q

MOA of Tamoxifen

A

receptor antagonist in breast/agonist in bone

blocks binding of estrogen

143
Q

MOA of trastuzumab

A

Ab against HER-2 (c-erbB2), a tyrosine kinase

for HER-2 breast cancer

144
Q

MOA of imatinib

A

Philadelphia chromosome bcr-abl tyrosine kinase inhibitor

for CML

145
Q

MOA of rituximab

A

Ab against CD20 in B cell neoplasms

146
Q

MOA of bevacizumab

A

Ab against VEGF

inhibits angiogenesis of tumors

Undeleted (71 decks)

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