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Heme onc > Heme onc > Flashcards

Heme onc Flashcards

0
Q

protein C or S deficiency–> mech? pres?

A

can’t inactivate V and VIII incr. thrombotic skin necrosis and hemorrhage post warfarin administration

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0
Q

Burkitt’s translocation

A

8:14, c-myc

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1
Q

CLL presentation

A

often asympto maybe autoimmune hemolytic anemia

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2
Q

HbC mutation

A

glutamic acid to lysine at residue 6 of B globin

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2
Q

heme synthesis AA source, cofactor, rate limiting, inhibitors

A

gly + B6 ala synthase : rate (affected in x-linked sideroblastic anemias) glucose and heme inhibit

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3
Q

8:14 translocation

A

burkitts, cmyc

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4
Q

drug that prevents mast cell degranulation

A

cromylyn sodium

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4
Q

lead poisoning kids vs. adults

A

succimer for kids dimercaprol and EDTA for adults

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5
Q

RBC membrane prot–>sequester HCO3

A

HCO3/Cl antiporter

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5
Q

adult T cell lymphoma pres

A

cutaneous japan, w afr, caribbean *aggressive

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6
Q

costimulatory signal for T cells

A

CD28

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6
Q

what activates/inactivates bradykinin

A

activates: kallikrein inactivates: ACE

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6
Q

mantle cell lymphoma translocation

A

11:14 cyclin D1

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6
Q

imatinib toxicity

A

fluid retention

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7
Q

trastuzumab mech

A

antiHER-2 (HER2 = TK)

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8
Q

eosinphils make arylsulfatase purpose

A

limit reaction after mast cell degranulation

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8
Q

1-2 allele a-globin deletion->?

A

nothing really, insignificant

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9
Q

pharm cause of B12 deficiency

A

longterm PPI

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10
Q

heparin-induced thrombocytopenia mech

A

IgG against heparin-pt factor 4-> activate pts->thrombosis and thrombocytopenia

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11
Q

tPA works by?

A

activating plasmin which cleaves fibrin mesh

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12
Q

T cell marker

A

CD3

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13
Q

thrombomodulin mech/fxn

A

activate protein c–>put brakes on thrombus formation

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14
Q

MTX vs. 5FU and leucovorin rescue

A

MTX myelosuppression: leucovorin reverses 5-FU not fixed

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15
Q

ferrochelatase reaction

A

adds Fe to protoporphyrin to form heme

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15
Q

reed sternberg cells and hodgkin’s dx

A

necessary, not sufficient

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16
Q

CML vs. infection lab difference

A

infxn response: incr. leukocyte alkaline phosphatase CML: decr.!

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17
Q

waldenstorm’s macroglobulinemia vs. MM

A

no lytic bone lesions (may still see hyperviscosity sx)

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17
Q

hairy cell leukemia tx

A

cladribine (adenosine analog) (clad in a hair shirt)

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18
Q

antithrombin deficiency labs

A

incr. in PTT decreases w/heparin administration (heparin activates antithrombin)

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19
Q

G6PD rbc appearance

A

heinz (denatured Hb) and bite

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19
Q

packed RBCs are used for?

A

acute blood loss, severe anemia

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20
Q

platelet plug formation* stop and draw

A

vWF binds exposed collagen pts bind vWF w/GpIb pts release ADP and Ca ADP–>pts adhere ADP binding induces GpIIb/IIIa on pts fibrinogen binds GpIIb/IIIa linked pts

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21
Q

pt life span

A

8-10 d

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21
Q

intravascular hemolysis labs

A

*haptoglobin decr incr. LDH Hb in urine

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21
Q

nitrosureas uses

A

crosses CNS!

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21
Q

bevacizumab mech

A

antiVEGF

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22
Q

eptifibatide mech

A

GPIIb/IIIa inhibitor

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23
Q

bivalirudin mech

A

hirudin derivative from leeches inhibits thrombin heparin alternative for HIT pts

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24
Q

2 pathologies underlying howell-jolly bodies

A

asplenia/hyposplenia *after naphthalene ingestion!

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25
Q

what activates antithrombin?

A

heparin

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25
Q

cryoprecipitate used for?

A

treat coag deficiencies involving fibrinogen and factor VIII (contains fibrinogen, VIII, XIII, vWF, fibronectin)

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27
Q

vWF receptor

A

Gp1b

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28
Q

pulmonary fibrosis chemo drug

A

busulfan/bleomycin

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29
Q

aspirin effect on coagulation

A

blocks cox–>decr. txa2 (pro aggregation)

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29
Q

pancytopenia BM appearance

A

nl cells, fatty infiltrate = dry BM tap

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29
Q

von willebrand’s disease tx

A

DDAVP (desmopressin) ->release vWF stores in endothelium

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30
Q

CML translocation

A

9:22 (philadelphia, bcr-abl)

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30
Q

langerhans cell histiocytosis presentation

A

kids, lytic bone lesions, skin rash

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32
Q

clopidogrel mech

A

irrev. blocks pt adp receptor expression!!

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33
Q

mycosis fungoides/sezary syndrome pres and marker

A

indolent, patches, nodules CD4

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33
Q

heparin key SE and reversal

A

osteoporosis protamine sulfate reverses (+ charge, binds - heparin)

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34
Q

B thal major presentation

A

homozygote absent B: blood transfusion marrow expansion: crew cut and chipmunk incr. HbF (a2y2)

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34
Q

M3 AML presentation

A

often w/DIC

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35
Q

vinblastine SE

A

vinblastine blasts bone marrow

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36
Q

Reed sternberg cell markers

A

CD30 and 15

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36
Q

low molecular weight heparin advantages

A

longer half life, better bioavailability subcutaneous administration w/o lab monitoring

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38
Q

target cell underlying pathology

A

HbC disease asplenia liver thalassemia HALT

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39
Q

what accumulates in acute intermittent porphyria

A

porphobilinogen dALA (also in lead) uroporphyrin (in urine)

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40
Q

macrophage surface marker

A

CD14

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40
Q

nitrosureas SE

A

CNS toxicity (of course, you use it because it crosses BBB)

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41
Q

anticoag during pregnancy

A

heparin: does not cross placenta

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42
Q

tirfiban mech

A

GP IIb/IIIa inhibitor

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42
Q

cyclophosphamide mech

A

x link DNA at guanine N-7 liver activates

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44
Q

poikilocytosis means

A

cells of diff shapes

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45
Q

vincristine SE

A

neurotox

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46
Q

Von Willebrand’s disease dx

A

ristocetin cofactor assay

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46
Q

most common hodgkin’s type

A

nodular sclerosing

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47
Q

alteplase mech

A

thrombolytic (all the eplases)

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48
Q

sickle cell xray

A

crew cut like in thalassemias

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50
Q

WBC highly phagocytic for ag:ab complexes

A

eosinophil

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51
Q

vemurafenib mech

A

small molecule inhibitor of B-Raf kinase w/V600E mutation for metastatic melanoma

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52
Q

burkitt’s histo

A

starry sky (lymphos w/interspersed macros)

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53
Q

follicular lymphoma translocation

A

14;18 bcl-2

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54
Q

4 allele a-globin deleiton–>

A

hydrops fetalis, y4 Hb (barts)

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55
Q

3 proaggregation of pt factors

A

TXA2 (from pts) decr. blood flow incr. aggregation

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56
Q

a-thalassemia defect

A

a-globin deletions!

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57
Q

acute intermittent porphyria tx

A

glucose and heme (inhibit ala synthase, rate limiting)

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57
Q

imatinib mech

A

bcr-abl TK Inhibitor

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59
Q

acanthocyte/spur cell associated pathology

A

liver disease or abetalipoproteinemia

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60
Q

pyruvate kinase deficiency RBCs and pres

A

decr. PK–>decr. ATP–>*rigid RBCs hemolytic anemia (extravasc) in newborn*

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61
Q

warm agglutinin association

A

SLE, CLL, drugs (a-methyldopa)

62
Q

AML translocation

A

15:17: M3 subtype :)

63
Q

cromolyn sodium mechanism

A

prevent mast cell degranulation

65
Q

Bernard-Soulier syndrome deficiency

A

Gp1b (pt receptor for vWF)

66
Q

fibrinogen receptor

A

GpIIb-IIIa–>pt aggregation

67
Q

vWF labs

A

incr. BT, PTT may increase (from not carrying/protecting VIII)

67
Q

calcium changes on blood transfusion?

A

hypOCa risk (citrate chelates)

68
Q

chemodrug–>acoustic nv damage

A

cisplatin/carboplatin (w/nephrotox)

69
Q

cyclphosphamide se

A

hemorrhagic cystitis (mesna rescue binds toxic metabolite) ifosfamide too!

70
Q

bernard-soulier syndrome labs, mechanism

A

decr. Gp1b->decr. pt-VWF adhesion decr. plug formation incr. bleed time, decr. pt count

72
Q

neutrophil granule contents

A

specific: alk phos, collagenase, lysozyme, lactoferrin azurophilic: (aka lysozomes) acid phosphatase, peroxidase, B-glucoronidase

73
Q

cardiotoxic chemo drugs

A

doxorubicin, trastuzumab

74
Q

hemorrhagic cystitis chemo drug

A

cyclophosphamide/ifosfamide

75
Q

glanzmann’s thrombasthenia deficiency

A

GpIIb/IIIa

76
Q

heterozygote B thalassemia pres

A

usually asympto incr. HbA2 (>3.5%) B-thal minor

77
Q

busulfan uses

A

CML and **get rid of pt BM pre txpl

77
Q

tamoxifen vs. raloxifene toxicity

A

tamoxifen–>endometrial CA, hot flashes raloxifene: just relax (endometrial antag)

79
Q

pt granule contents

A

dense granules: ADP, Ca a granules: vWF, fibrinogen

80
Q

4 antiaggregation of pt factors

A

PGI2 NO (both from endos) incr. blood flow decr. pt aggregation

81
Q

abciximab mech

A

block GpIIb/IIIa

82
Q

pt pool stored where

A

spleen (1/3 of it)

83
Q

fresh frozen plasma used for?

A

increasing coag factors DIC cirrhosis warfarin OD

85
Q

factor V Leiden mutation–>?

A

factor 5 resistant to protein C inactivation

87
Q

4 causes of non-megaloblastic macrocytic anemia

A

DNA synth unimpaired liver dis EtOH reticulocytosis drugs

87
Q

TTP labs, deficiency, pres

A

deficient ADAMTS13 (vWF metalloprotease): decr. vWF multimer degradation incr. pt aggregation and thrombosis, decr. pt survival incr. BT, decr. PT labs: schistocytes (microangiopathic), incr. LDH sx: neuro, renal, fever, thrombocytopenia, microangiopathic hemolytic

87
Q

aspirin OD–> alk/acid?

A

metabolic acidosis respiratory alkalosis

88
Q

ALL markers

A

TdT+ and CALLA+

90
Q

PNH mutation pres labs tx

A

mutation: impaired DAF or GPI anchor–>incr. compl on RBCs acquired in stem cells pres: hemolytic anemia, pancytopenia, venous thrombosis labs: CD55/59 - RBCs** tx: eculizumab (binds C5)

91
Q

AML histo

A

auer rods: peroxidase + release of auer rods->DIC

93
Q

vitamin K activation path

A

epoxide reductase activates it

94
Q

cold agglutinin association

A

mycoplasma pneumonia, mono, CLL (CLL also warm associated)

95
Q

langerhans histocytosis cell msarkers

A

s-100 + (neural crest) CD1a birbeck granules (tennis rackets)

97
Q

porphyria cutanea tarda presentation

A

blistering cutaneous photosensitivity *most common porphyria!

98
Q

why are sickle cell babies asympto?

A

more HbF

100
Q

basophilic stippling associated pathology

A

TAL thalassemia anemia chronic disease lead

101
Q

CML presentation

A

incr. neutro, metamyelocytes, basos splenomegaly may–>AML/ALL

102
Q

thrombolytics mech

A

eventually incr. plasminogen to plasmin plasmin cleaves thrombin and fibrin clots nl pt ct, incr. pt, ptt

104
Q

K changes on transfusion?

A

hyperK risk (RBCs may lyse in old blood units)

105
Q

6MP key toxicity

A

xanthine oxidase inhibits so allopurinol increases toxicity (like BM, GI, liver)

106
Q

vWF fxn

A

carry/protect VIII

107
Q

reversible sideroblastic anemia causes

A

EtOH Lead isoniazid

108
Q

prothrombin gene mutation–>disorder? pres?

A

mutate 3’ untranslated->incr! prothrombin production, increased levels and venous clots

109
Q

schistocyte associated pathology

A

DIC TTP/HUS mechanical (i.e. valve)

110
Q

worst prognosis hodgkin’s type

A

lymphocyte mixed or depleted

112
Q

antithrombin inactivates what

A

II, VII, IX, X, XI, XII

113
Q

macrophages activated by? (cytokine)

A

IFNy

115
Q

Heinz bodies are composed of?

A

denatured Hb due to Hb sulfhydryl groups ->bite cells

117
Q

BT incr. if?

A

Pt plug formation off (pt abnormalities)

119
Q

acute intermittent prophyria affects what enzyme

A

prophobilinogen deaminase (acute intermittent PORPHyria)

120
Q

CML tx

A

imatinib (small molecule inhibitor of bcr-abl TK)

121
Q

sickle cell tx

A

hydroxyurea: ->HbF!! and BM txpl

122
Q

hydroxyurea mechanism for chemotx

A

inhibit ribonucleotide reductase decr. synth (S specific)

124
Q

B12 vs. folate deficiency homocysteine and methylmalonic acid

A

folate: incr. homocysteine, nl methylmalonic B12: incr. both (BBBoth)

125
Q

Busulfan SE

A

pulmonary fibrosis hyperpigmentation

126
Q

warfarin SEs

A

teratogen (crosses placenta) skin/tissue necrosis (esp w/prot c/s deficiency) bleed intxns

128
Q

ticlopidine mech

A

irrev. block of pt adp receptor expression!!

128
Q

rituximab mech

A

anti CD20 (bcell)

129
Q

lead poisoning biochem mech

A

inhibit ferrochelatase and ALA dehydratase (leaD) inhibit rRNA degredation–>basophilic stippling

129
Q

what accumulates in porphyria cutanea tarda

A

uroporphyrin (->tea urine)

129
Q

DIC labs

A

incr. bt, decr. pts incr. PT and PTT (used up factors) incr. d dimers (fibrin split product) decr. fibrinogen (used up) decr. V and VIII

130
Q

lepirudin mech

A

hirudin derivative, from leeches inhibit thrombin heparin alternative for HIT pts

131
Q

bleomycin SE

A

lung fibrosis! skin not so much myelo :)

133
Q

ITP labs, defect

A

anti-GpIIb/IIIa abs–>spleen eats pt-ab complexes decr. pt, incr. bt incr. megakaryocytes (to replace pts)

134
Q

protein C mech

A

protein C, activated by thrombomodulin on endos w/protein S it inactivates V and VIII

135
Q

cisplatin/carboplatin key toxicity

A

nephro and acoustic nerve damage amifostine and chloride diuresis prevent nephrotoxicity!

136
Q

Multiple myeloma associated sx

A

CRAB (hyperCa, renal, anemia, bone lesions/back pain

138
Q

hereditary spherocytosis findings

A

splenomegaly *aplastic crisis i.e. from parvovirus

139
Q

HbS mutation

A

glu–>val (vs. lys for HbC) at position 6

140
Q

trastuzumab toxicity

A

cardiotoxicity

142
Q

3 allele a-globin deletion->?

A

HbH disease, B4 hemoglobine = HbH

143
Q

ALL translocation

A

12:21 is a good one to have: better prognosis :)

144
Q

extravascular hemolysis labs

A

incr. LDH incr. UCB–>jaundice

144
Q

busulfan mech

A

NDA alkylator

146
Q

thromboplastin aka and fxn

A

aka tissue factor activates factor 7

148
Q

CLL blood smear

A

smudge cells

149
Q

dactinomycin mech

A

DNA intercalation

151
Q

primary anemia lab value change in pregnancy/OCP

A

incr. transferrin/TIBC ->decr. % sat, other labs nl

152
Q

3 key myelosuppression chemo drugs

A

5-FU and 6-MP methotrexate (leucovorin)

153
Q

dipyridamole mech

A

PDEIII inhibitor incr. cAMP in pts inhibit aggregation vasodilate

155
Q

which has more constitutional sx: hodgkin’s or non

A

hodgkin’s

156
Q

sideroblastic anemia mutation

A

d-ala Synthase (Sideroblastic) x-linked

158
Q

bite cell underlying

A

G6PD deficiency

159
Q

etopiside/teniposide mech

A

inhibit topoisomerase II–>DNA degadation

161
Q

ADP binding on pts–>?

A

GpIIb/IIIa insertion on surface

162
Q

ALL spreads to?

A

CNS and testes

163
Q

cilostazol mech

A

PDEIII inhibitor–>incr. cAMP in pts inhibits aggregation vasodilates

164
Q

ticlodipine toxicity

A

neutropenia

165
Q

ALL imaging

A

may see mediastinal mass (thymic infiltration)

167
Q

14;18

A

follicular lymphoma bcl-2 activation

168
Q

direct coombs: looking for?

A

pt Ig bound to pt’s RBCs

169
Q

asian vs. african alpha thal

A

asian: cis: spontaneous abortions african: trans

169
Q

porphyria cutanea tarda enzyme

A

uroporphyinogen decarboxylase

170
Q

peripheral neuropathy chemo drug

A

vincristine

172
Q

MGUS vs. MM

A

asymptomatic MM 1-2% to MM/yr

173
Q

doxorubicin mech

A

makes free rads and DNA intercalation

174
Q

B thal mutations

A

splice sites and promotors–>decr. synthesis

175
Q

multiple myeloma rbc smears

A

rouleaux

177
Q

what prevents doxorubicin cardiotoxicity

A

dexrazoxane iron chelator

178
Q

thrombolytic OD treatment

A

aminocaproic acid (inhibits fibrinolysis)

179
Q

burkitt’s, sporadic form, body location

A

pelvis or abd (vs. jaw in africa)

180
Q

B thal population

A

mediterranean

181
Q

follicular lymphoma prognosis

A

indolent so hard to cure

182
Q

pt transfusions are used for?

A

stopping significant bleeding

184
Q

9:22 translocation

A

CML (bcr-abl)

185
Q

RS cells and hodgkin’s prognosis

A

strong response against: better prognosis

186
Q

bradykinin–>?

A

vasodilate permeability pain

187
Q

vincristine/vinblastine vs. paclitaxel/…taxol mech

A

vincristin: blocks polymerization paclitaxel: stabilizes

188
Q

low molecular weight heparin disadvantage

A

not as reversible

189
Q

what accumulates in lead poisoning

A

protoporphyrin and dALA in the blood

190
Q

tamoxifen/raloxifene mech

A

SERM estrogen antag in breast agonist in bone

191
Q

anisocytosis means

A

cells of diff size

192
Q

11:14 translocation

A

mantle cell, cyclin D1

193
Q

orotic aciduria mutation, tx, pres

A

UMP synthase mutation (makes uridine from orotic acid) megaloblastic anemia in kids, B12/folate do not correct + glossitis tx: uridine monophosphate (bypass)

194
Q

fibrinogen receptor

A

GpIIb/IIIa

195
Q

mantle cell lymphoma prognosis

A

bad

196
Q

glanzmann’s thrombasthenia labs, defect

A

decr. GpIIb/IIIa–>defective aggregation smear: no pt clumping long BT, nl pt ct

197
Q

bleomycin mech

A

free rads break DNA

198
Q

mantle cell lymphoma cell marker

A

CD5

199
Q

best prognosis hodgkin’s type

A

nodular sclerosing/lymphocyte richg

200
Q

symptoms of acute intermittent porphyria

A

5Ps painful abd portwine urine polyneuropathy psych precipitated by drugs

201
Q

sideroblastic anemia tx

A

B6 (alaSynthase cofactor)

Heme onc (1 decks)

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