Heme onc Flashcards
(201 cards)
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
0
Q
Burkitt’s translocation
A
8:14, c-myc
1
Q
CLL presentation
A
often asympto maybe autoimmune hemolytic anemia
2
Q
HbC mutation
A
glutamic acid to lysine at residue 6 of B globin
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
3
Q
8:14 translocation
A
burkitts, cmyc
4
Q
drug that prevents mast cell degranulation
A
cromylyn sodium
4
Q
lead poisoning kids vs. adults
A
succimer for kids dimercaprol and EDTA for adults
5
Q
RBC membrane prot–>sequester HCO3
A
HCO3/Cl antiporter
5
Q
adult T cell lymphoma pres
A
cutaneous japan, w afr, caribbean *aggressive
6
Q
costimulatory signal for T cells
A
CD28
6
Q
what activates/inactivates bradykinin
A
activates: kallikrein inactivates: ACE
6
Q
mantle cell lymphoma translocation
A
11:14 cyclin D1
6
Q
imatinib toxicity
A
fluid retention
7
Q
trastuzumab mech
A
antiHER-2 (HER2 = TK)
8
Q
eosinphils make arylsulfatase purpose
A
limit reaction after mast cell degranulation
8
Q
1-2 allele a-globin deletion->?
A
nothing really, insignificant
9
Q
pharm cause of B12 deficiency
A
longterm PPI
10
Q
heparin-induced thrombocytopenia mech
A
IgG against heparin-pt factor 4-> activate pts->thrombosis and thrombocytopenia
11
Q
tPA works by?
A
activating plasmin which cleaves fibrin mesh
12
Q
T cell marker
A
CD3
13
Q
thrombomodulin mech/fxn
A
activate protein c–>put brakes on thrombus formation
14
Q
MTX vs. 5FU and leucovorin rescue
A
MTX myelosuppression: leucovorin reverses 5-FU not fixed
15
Q
ferrochelatase reaction
A
adds Fe to protoporphyrin to form heme
15
reed sternberg cells and hodgkin's dx
necessary, not sufficient
16
CML vs. infection lab difference
infxn response: incr. leukocyte alkaline phosphatase CML: decr.!
17
waldenstorm's macroglobulinemia vs. MM
no lytic bone lesions (may still see hyperviscosity sx)
17
hairy cell leukemia tx
cladribine (adenosine analog) (clad in a hair shirt)
18
antithrombin deficiency labs
incr. in PTT decreases w/heparin administration (heparin activates antithrombin)
19
G6PD rbc appearance
heinz (denatured Hb) and bite
19
packed RBCs are used for?
acute blood loss, severe anemia
20
platelet plug formation\* stop and draw
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
21
pt life span
8-10 d
21
intravascular hemolysis labs
\*haptoglobin decr incr. LDH Hb in urine
21
nitrosureas uses
crosses CNS!
21
bevacizumab mech
antiVEGF
22
eptifibatide mech
GPIIb/IIIa inhibitor
23
bivalirudin mech
hirudin derivative from leeches inhibits thrombin heparin alternative for HIT pts
24
2 pathologies underlying howell-jolly bodies
asplenia/hyposplenia \*after naphthalene ingestion!
25
what activates antithrombin?
heparin
25
cryoprecipitate used for?
treat coag deficiencies involving fibrinogen and factor VIII (contains fibrinogen, VIII, XIII, vWF, fibronectin)
27
vWF receptor
Gp1b
28
pulmonary fibrosis chemo drug
busulfan/bleomycin
29
aspirin effect on coagulation
blocks cox--\>decr. txa2 (pro aggregation)
29
pancytopenia BM appearance
nl cells, fatty infiltrate = dry BM tap
29
von willebrand's disease tx
DDAVP (desmopressin) -\>release vWF stores in endothelium
30
CML translocation
9:22 (philadelphia, bcr-abl)
30
langerhans cell histiocytosis presentation
kids, lytic bone lesions, skin rash
32
clopidogrel mech
irrev. blocks pt adp receptor expression!!
33
mycosis fungoides/sezary syndrome pres and marker
indolent, patches, nodules CD4
33
heparin key SE and reversal
osteoporosis protamine sulfate reverses (+ charge, binds - heparin)
34
B thal major presentation
homozygote absent B: blood transfusion marrow expansion: crew cut and chipmunk incr. HbF (a2y2)
34
M3 AML presentation
often w/DIC
35
vinblastine SE
vinblastine blasts bone marrow
36
Reed sternberg cell markers
CD30 and 15
36
low molecular weight heparin advantages
longer half life, better bioavailability subcutaneous administration w/o lab monitoring
38
target cell underlying pathology
HbC disease asplenia liver thalassemia HALT
39
what accumulates in acute intermittent porphyria
porphobilinogen dALA (also in lead) uroporphyrin (in urine)
40
macrophage surface marker
CD14
40
nitrosureas SE
CNS toxicity (of course, you use it because it crosses BBB)
41
anticoag during pregnancy
heparin: does not cross placenta
42
tirfiban mech
GP IIb/IIIa inhibitor
42
cyclophosphamide mech
x link DNA at guanine N-7 liver activates
44
poikilocytosis means
cells of diff shapes
45
vincristine SE
neurotox
46
Von Willebrand's disease dx
ristocetin cofactor assay
46
most common hodgkin's type
nodular sclerosing
47
alteplase mech
thrombolytic (all the eplases)
48
sickle cell xray
crew cut like in thalassemias
50
WBC highly phagocytic for ag:ab complexes
eosinophil
51
vemurafenib mech
small molecule inhibitor of B-Raf kinase w/V600E mutation for metastatic melanoma
52
burkitt's histo
starry sky (lymphos w/interspersed macros)
53
follicular lymphoma translocation
14;18 bcl-2
54
4 allele a-globin deleiton--\>
hydrops fetalis, y4 Hb (barts)
55
3 proaggregation of pt factors
TXA2 (from pts) decr. blood flow incr. aggregation
56
a-thalassemia defect
a-globin deletions!
57
acute intermittent porphyria tx
glucose and heme (inhibit ala synthase, rate limiting)
57
imatinib mech
bcr-abl TK Inhibitor
59
acanthocyte/spur cell associated pathology
liver disease or abetalipoproteinemia
60
pyruvate kinase deficiency RBCs and pres
decr. PK--\>decr. ATP--\>\*rigid RBCs hemolytic anemia (extravasc) in newborn\*
61
warm agglutinin association
SLE, CLL, drugs (a-methyldopa)
62
AML translocation
15:17: M3 subtype :)
63
cromolyn sodium mechanism
prevent mast cell degranulation
65
Bernard-Soulier syndrome deficiency
Gp1b (pt receptor for vWF)
66
fibrinogen receptor
GpIIb-IIIa--\>pt aggregation
67
vWF labs
incr. BT, PTT may increase (from not carrying/protecting VIII)
67
calcium changes on blood transfusion?
hypOCa risk (citrate chelates)
68
chemodrug--\>acoustic nv damage
cisplatin/carboplatin (w/nephrotox)
69
cyclphosphamide se
hemorrhagic cystitis (mesna rescue binds toxic metabolite) ifosfamide too!
70
bernard-soulier syndrome labs, mechanism
decr. Gp1b-\>decr. pt-VWF adhesion decr. plug formation incr. bleed time, decr. pt count
72
neutrophil granule contents
specific: alk phos, collagenase, lysozyme, lactoferrin azurophilic: (aka lysozomes) acid phosphatase, peroxidase, B-glucoronidase
73
cardiotoxic chemo drugs
doxorubicin, trastuzumab
74
hemorrhagic cystitis chemo drug
cyclophosphamide/ifosfamide
75
glanzmann's thrombasthenia deficiency
GpIIb/IIIa
76
heterozygote B thalassemia pres
usually asympto incr. HbA2 (\>3.5%) B-thal minor
77
busulfan uses
CML and \*\*get rid of pt BM pre txpl
77
tamoxifen vs. raloxifene toxicity
tamoxifen--\>endometrial CA, hot flashes raloxifene: just relax (endometrial antag)
79
pt granule contents
dense granules: ADP, Ca a granules: vWF, fibrinogen
80
4 antiaggregation of pt factors
PGI2 NO (both from endos) incr. blood flow decr. pt aggregation
81
abciximab mech
block GpIIb/IIIa
82
pt pool stored where
spleen (1/3 of it)
83
fresh frozen plasma used for?
increasing coag factors DIC cirrhosis warfarin OD
85
factor V Leiden mutation--\>?
factor 5 resistant to protein C inactivation
87
4 causes of non-megaloblastic macrocytic anemia
DNA synth unimpaired liver dis EtOH reticulocytosis drugs
87
TTP labs, deficiency, pres
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
aspirin OD--\> alk/acid?
metabolic acidosis respiratory alkalosis
88
ALL markers
TdT+ and CALLA+
90
PNH mutation pres labs tx
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
AML histo
auer rods: peroxidase + release of auer rods-\>DIC
93
vitamin K activation path
epoxide reductase activates it
94
cold agglutinin association
mycoplasma pneumonia, mono, CLL (CLL also warm associated)
95
langerhans histocytosis cell msarkers
s-100 + (neural crest) CD1a birbeck granules (tennis rackets)
97
porphyria cutanea tarda presentation
blistering cutaneous photosensitivity \*most common porphyria!
98
why are sickle cell babies asympto?
more HbF
100
basophilic stippling associated pathology
TAL thalassemia anemia chronic disease lead
101
CML presentation
incr. neutro, metamyelocytes, basos splenomegaly may--\>AML/ALL
102
thrombolytics mech
eventually incr. plasminogen to plasmin plasmin cleaves thrombin and fibrin clots nl pt ct, incr. pt, ptt
104
K changes on transfusion?
hyperK risk (RBCs may lyse in old blood units)
105
6MP key toxicity
xanthine oxidase inhibits so allopurinol increases toxicity (like BM, GI, liver)
106
vWF fxn
carry/protect VIII
107
reversible sideroblastic anemia causes
EtOH Lead isoniazid
108
prothrombin gene mutation--\>disorder? pres?
mutate 3' untranslated-\>incr! prothrombin production, increased levels and venous clots
109
schistocyte associated pathology
DIC TTP/HUS mechanical (i.e. valve)
110
worst prognosis hodgkin's type
lymphocyte mixed or depleted
112
antithrombin inactivates what
II, VII, IX, X, XI, XII
113
macrophages activated by? (cytokine)
IFNy
115
Heinz bodies are composed of?
denatured Hb due to Hb sulfhydryl groups -\>bite cells
117
BT incr. if?
Pt plug formation off (pt abnormalities)
119
acute intermittent prophyria affects what enzyme
prophobilinogen deaminase (acute intermittent PORPHyria)
120
CML tx
imatinib (small molecule inhibitor of bcr-abl TK)
121
sickle cell tx
hydroxyurea: -\>HbF!! and BM txpl
122
hydroxyurea mechanism for chemotx
inhibit ribonucleotide reductase decr. synth (S specific)
124
B12 vs. folate deficiency homocysteine and methylmalonic acid
folate: incr. homocysteine, nl methylmalonic B12: incr. both (BBBoth)
125
Busulfan SE
pulmonary fibrosis hyperpigmentation
126
warfarin SEs
teratogen (crosses placenta) skin/tissue necrosis (esp w/prot c/s deficiency) bleed intxns
128
ticlopidine mech
irrev. block of pt adp receptor expression!!
128
rituximab mech
anti CD20 (bcell)
129
lead poisoning biochem mech
inhibit ferrochelatase and ALA dehydratase (leaD) inhibit rRNA degredation--\>basophilic stippling
129
what accumulates in porphyria cutanea tarda
uroporphyrin (-\>tea urine)
129
DIC labs
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
lepirudin mech
hirudin derivative, from leeches inhibit thrombin heparin alternative for HIT pts
131
bleomycin SE
lung fibrosis! skin not so much myelo :)
133
ITP labs, defect
anti-GpIIb/IIIa abs--\>spleen eats pt-ab complexes decr. pt, incr. bt incr. megakaryocytes (to replace pts)
134
protein C mech
protein C, activated by thrombomodulin on endos w/protein S it inactivates V and VIII
135
cisplatin/carboplatin key toxicity
nephro and acoustic nerve damage amifostine and chloride diuresis prevent nephrotoxicity!
136
Multiple myeloma associated sx
CRAB (hyperCa, renal, anemia, bone lesions/back pain
138
hereditary spherocytosis findings
splenomegaly \*aplastic crisis i.e. from parvovirus
139
HbS mutation
glu--\>val (vs. lys for HbC) at position 6
140
trastuzumab toxicity
cardiotoxicity
142
3 allele a-globin deletion-\>?
HbH disease, B4 hemoglobine = HbH
143
ALL translocation
12:21 is a good one to have: better prognosis :)
144
extravascular hemolysis labs
incr. LDH incr. UCB--\>jaundice
144
busulfan mech
NDA alkylator
146
thromboplastin aka and fxn
aka tissue factor activates factor 7
148
CLL blood smear
smudge cells
149
dactinomycin mech
DNA intercalation
151
primary anemia lab value change in pregnancy/OCP
incr. transferrin/TIBC -\>decr. % sat, other labs nl
152
3 key myelosuppression chemo drugs
5-FU and 6-MP methotrexate (leucovorin)
153
dipyridamole mech
PDEIII inhibitor incr. cAMP in pts inhibit aggregation vasodilate
155
which has more constitutional sx: hodgkin's or non
hodgkin's
156
sideroblastic anemia mutation
d-ala Synthase (Sideroblastic) x-linked
158
bite cell underlying
G6PD deficiency
159
etopiside/teniposide mech
inhibit topoisomerase II--\>DNA degadation
161
ADP binding on pts--\>?
GpIIb/IIIa insertion on surface
162
ALL spreads to?
CNS and testes
163
cilostazol mech
PDEIII inhibitor--\>incr. cAMP in pts inhibits aggregation vasodilates
164
ticlodipine toxicity
neutropenia
165
ALL imaging
may see mediastinal mass (thymic infiltration)
167
14;18
follicular lymphoma bcl-2 activation
168
direct coombs: looking for?
pt Ig bound to pt's RBCs
169
asian vs. african alpha thal
asian: cis: spontaneous abortions african: trans
169
porphyria cutanea tarda enzyme
uroporphyinogen decarboxylase
170
peripheral neuropathy chemo drug
vincristine
172
MGUS vs. MM
asymptomatic MM 1-2% to MM/yr
173
doxorubicin mech
makes free rads and DNA intercalation
174
B thal mutations
splice sites and promotors--\>decr. synthesis
175
multiple myeloma rbc smears
rouleaux
177
what prevents doxorubicin cardiotoxicity
dexrazoxane iron chelator
178
thrombolytic OD treatment
aminocaproic acid (inhibits fibrinolysis)
179
burkitt's, sporadic form, body location
pelvis or abd (vs. jaw in africa)
180
B thal population
mediterranean
181
follicular lymphoma prognosis
indolent so hard to cure
182
pt transfusions are used for?
stopping significant bleeding
184
9:22 translocation
CML (bcr-abl)
185
RS cells and hodgkin's prognosis
strong response against: better prognosis
186
bradykinin--\>?
vasodilate permeability pain
187
vincristine/vinblastine vs. paclitaxel/...taxol mech
vincristin: blocks polymerization paclitaxel: stabilizes
188
low molecular weight heparin disadvantage
not as reversible
189
what accumulates in lead poisoning
protoporphyrin and dALA in the blood
190
tamoxifen/raloxifene mech
SERM estrogen antag in breast agonist in bone
191
anisocytosis means
cells of diff size
192
11:14 translocation
mantle cell, cyclin D1
193
orotic aciduria mutation, tx, pres
UMP synthase mutation (makes uridine from orotic acid) megaloblastic anemia in kids, B12/folate do not correct + glossitis tx: uridine monophosphate (bypass)
194
fibrinogen receptor
GpIIb/IIIa
195
mantle cell lymphoma prognosis
bad
196
glanzmann's thrombasthenia labs, defect
decr. GpIIb/IIIa--\>defective aggregation smear: no pt clumping long BT, nl pt ct
197
bleomycin mech
free rads break DNA
198
mantle cell lymphoma cell marker
CD5
199
best prognosis hodgkin's type
nodular sclerosing/lymphocyte richg
200
symptoms of acute intermittent porphyria
5Ps painful abd portwine urine polyneuropathy psych precipitated by drugs
201
sideroblastic anemia tx
B6 (alaSynthase cofactor)
