Heme/Onc Flashcards
1
Q
Poikilocytosis
A
varying shapes of RBCs
2
Q
dense vs alpha granules in platelets
A
dense - ADP, Ca
alpha - vWF, fibrinogen
3
Q
vWF receptor
A
Gp1b
4
Q
fibrinogen receptor
A
Gp2b/3a
5
Q
hypersegmented PMNs seen in what condition
A
b12/folate deficiency
6
Q
cell surface marker for macrophages
A
CD14
7
Q
Hemophilia A and B - what factors are deficient?
A
A - 8
B - 9
8
Q
vit K dependent clotting factors
A
2,7,9,10, protein C and S
9
Q
action of proteins C and S
A
activated protein C cleaves and inactivates 5a and 8a
protein S is a cofactor for C
10
Q
2 conditions in which acanthocytes are present
A
liver disease
abetalipoproteinemia
11
Q
causes of basophilic stipling of RBCs (3)
A
thalassemias, anemia of chronic dz, lead poisoning
12
Q
bite cells
A
G6PD def
13
Q
pathologic cell in sideroblastic anemia
A
RINGED sideroblast
14
Q
target cells seen in (4)
A
HbC dz, asplenia, liver dz, thalassemia
15
Q
Heinz bodies
A
oxidized iron > denatured hgb precipitates and damages membrane
seen in G6PD def, and heinz-like inclusions seen in alpha thalassemia
16
Q
Howell Jolly bodies
A
basophilic inclusions of nuclear remnants
seen in asplenia or functional hyposplenia or w/ naphthalene ingestion
17
Q
iron panel in iron def anemia
A
dec iron, inc TIBC, dec ferritin
18
Q
Plummer Vinson syndrome
A
iron def anemia, esophageal webs, atrophic glossitis
19
Q
of gene deletions and presentation in alpha thalassemia
A
1-2 deletion - subclinical anemia
3 deletions - HbH dz, excess beta globin forms beta tetramers (HbH)
4 deletions - excess gamma globin > HbBarts > hydrops fetalis > stillbirth
20
Q
beta thalassemia major sx
A
severe anemia requiring transfusions (possibility for 2ndary hemochromatosis)
marrow expansion > skeletal deformities
chipmunk facies
increased HbF
21
Q
HbS/Beta thalassemia heterozygotes - severity of disease
A
mild to moderate sickle cell depending on amt of beta production
22
Q
lead poisoning - type of anemia caused? signs/sx?
A
sideroblastic due to dec heme production LEAD: lead lines on gingivae (Burton's lines) encephalopathy / erythrocyte basophilic stipling abdominal colic / anemia drops - wrist and foot drop
23
Q
tx for lead poisoning
A
dimercaprol and EDTA, succimer for kids
24
Q
causes of sideroblastic anemia - 1 hereditary, 3 reversible
A
hereditary - XL defect in delta-ALA synthase
reversible - ETOH, lead, INH
25
iron panel in sideroblastic anemia
inc iron, normal TIBC, inc ferritin
26
tx of sideroblastic anemia
pyridoxine (B6)
27
labs for megaloblastic anemia to distinguish folate from B12 def
folate - inc homocysteine, nl methylmalonic acid (MMA)
| B12 - inc homocysteine and MMA
28
neuro sx of b12 def
```
subacute combined degeneration:
peripheral neuropathy
posterior columns - vibration / proprioception
lateral CST - spasticity
dementia
```
29
lab findings for intravascular vs extravascular hemolysis
intra - dec haptoglobin, inc LDH, hemoglobinuria
| extra - inc LDH, inc unconj bili
30
iron panel for anemia of chronic dz
dec iron, dec TIBC, inc ferritin
31
hereditary spherocytosis is generally associated with mutation in ___, which needs to interact with ____ & _____ to make the correct shape
ankyrin, spectrin, band 3
32
mutations in ____ will result in acanthocytic hereditary spherocytosis, while mutations in ____ result in pincered HS
beta spectrin
| band 3
33
hereditary spherocytosis and elliptocytosis - defect in vertical or horizontal interactions?
spherocytosis - vertical
| elliptocytosis - horizontal
34
G6PD def - inheritance? PBS signs?
XL
| Heinz bodies and bite cells
35
pyruvate kinase def - inheritance? pathogenesis? findings?
AR
defect in pyruvate kinase > dec ATP > rigid RBCs
hemolytic anemia in newborn
36
paroxysmal nocturnal hemoglobinuria - cause
inc complement mediated RBC lysis due to acquired mutation in HSC
37
paroxysmal nocturnal hemoglobinuria - triad of findings
hemolytic anemia, pancytopenia, venous thrombosis
38
tx of PNH
eculizumab
39
what precipitates sickling in sickle cell?
low O2 or dehydration
40
complications of sickle cell (5)
aplastic crises w/ infxn (parvo B19 esp)
autosplenectomy / splenic sequestration crisis
salmonella osteomyelitis
pain crises - dactylitis, acute chest syndrome, avascular necrosis
renal papillary necrosis and microhematuria
41
enzymes inhibited by lead poisoning
ferrochelatase and ALA dehydratase
42
acute intermittent porphyria - affected enzyme
porphobilinogen deaminase
43
acute intermittent porphyria - sx and tx
```
5 Ps:
painful abd
port wine colored urine
polyneuropathy
psych disturbanes
precipitated by drugs
```
tx - glucose and heme
44
MC type of porphyria
porphyria cutanea tarda
45
porphyria cutanea tarda - affected enzyme and sx
uroporphyrinogen decarboxylase
| blistering cutaneous photosensitivity
46
Bernard Soulier syndrome
dec Gp1b > defect in platelet to vWF adhesion
47
Glanzmann's thrombasthenia
dec Gp2b/3a > defect in platelet aggregation
48
ITP - pathogenesis
anti-Gp2b/3a antibodies > splenic macrophages eat platelet/ab complexes > less thrombocytopenia
49
TTP - pathogenesis
def of ADAMSTS 13 (vWF metalloprotease) > dec degradation of vWF multimers > inc platelet aggregation > dec platelet survival
50
TTP - pentad of sx
```
neuro sx
renal sx
fever
thombocytopenia
microangiopathic hemolytic anemia
```
51
MC hereditary bleeding disorder
von willebrand's
52
von willebrand dz inheritance
AD
53
tx for von willebrand dz
DDAVP - releases vWF stored in endothelium
54
causes of DIC
Sepsis, Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion
STOP Making New Thrombi
55
factor V leiden
V that is resistant to degradation by activated protein C > MC cause of inherited hypercoagulability in whites
56
what is cryoprecipitate useful for
factor deficiencies involving fibrinogen and factor 8
57
hodgkin's vs NHL - location/spread? age? which is more assoc w/ EBV? constitutional sx?
hodgkin - localized w/ contiguous spread
2 peaks - young adult and >55
assoc w/ EBV
B sx common (fever, night sweats, wt loss)
NHL - multiple sites w/ noncontiguous spread
20-40 yo
fewer constitutional sx
58
main cell of hodgkin's
Reed Sternberg cell
59
form of hodgkin's w/ best prognosis
nodular sclerosing (also most common)
60
"starry sky" appearance
burkitt's
61
burkitt's translocation and gene
t(8;14), c-myc
62
MC adult NHL
diffuse large B cell lymphoma
63
mantle cell lymphoma translocation
t(11;14)
64
follicular lymphoma translocation and gene
t(14;18), bcl-2
65
mycosis fungiodes / sezary syndrome
T cell NHL w/ cutaneous manifestations
66
MC primary tumor arising from bone in elderly
multiple myeloma
67
Waldenstrom's macroglobulinemia
M spike of IgM, hyperviscosity sx but no bone lesions
68
findings in multiple myeloma (6)
```
inc susceptibility to infxn
primary amyloidosis
punched out lytic bone lesions
M spike on protein electrophoresis
Ig light chains in urine - Bence Jones protein
Rouleaux formation
```
69
sx of multiple myeloma (4)
```
CRAB -
hyperCalcemia
Renal insufficiency
Anemia
Bone lytic lesions/Back pain
```
70
good prognosis tranlsocation for ALL
t(12;21)
71
smudge cells
CLL
72
hairy cell leukemia - who gets it, special stain
adults/elderly
| TRAP - tartrate resistant acid phosphatase
73
AML M3 - genetics stuff, presentation/PBS finding, tx
Acute promyelocytic leukemia
t(15;17)
auer rods, presents w/ DIC
tx w/ all trans retinoic acid
74
CML - genetics stuff, tx
t(9;22) philadelphia chromosome, bcr-abl
| tx w/ imatinib
75
Langerhans cell histiocytosis - presentation, microscopic finding
```
child w/ lytic bone lesions and skin rash
Birbeck granules ("tennis rackets" on EM)
```
76
gene mutation assoc with several myeloproliferative disorders
JAK2
77
heparin mech
cofactor in activation of antithrombin, dec thrombin and factor 10a
78
antidote for heparin
protamine sulfate
79
coag tests to follow for heparin and warfarin
heparin - PTT
| warfarin - PT (INR)
80
lower molec weight heparins act more on
factor Xa
81
mech for heparin induced thrombocytopenia
IgG against heparin bound to platelet factor 4 > activation of platelets > thrombosis and thrombocytopenia
82
alternative to heparin for HIT patients
lepirudin, bivalirudin (derived from anticoag found in leeches
83
alteplase is
tPA
84
antidote for thrombolytics
aminocaproic acid (inhibs fibrinolysis)
85
sx of aspirin overdose
resp alkalosis and metab acidosis
86
clopidogrel, ticlopidine, prasgurel, ticagrelor - drug class
ADP receptor inhibitors (irreversible)
87
cilostazol, dipyridamole - drug class, use, SEs
PDE3 inhib
claudication, coronary vasodilation, prevention of stroke/TIA, angina prophylaxis
nausea, HA, facial flushing, hypotension, abd pain
88
abciximab, eptifibatide, tirofiban - class, use
Gp2b/3a inhibitors
| acute coronary syndromes, percutaneous transluminal coronary angioplasty
89
methotrexate acts on what enyzme
dihydrofolate reductase
90
methotrexate tox
**myelosuppression
fatty change in liver
mucositis
teratogen
91
rescue drug for methotrexate induced myelosuppression
leucovorin
92
5FU - class, tox
antimetabolite
**myelosuppression
photosensitivity
93
cytarabine - class, tox
antimetabolite
leukopenia, thrombocytopenia, megaloblastic anemia
94
azathioprine / 6MP - class, tox
antimetabolite
**Myelosuppression
GI/liver tox
can't take w/ allopurinol
95
dactinomycin - class, general use, tox
antitumor abx
used for childhood tumors
myelosuppression
96
doxorubicin/adriamycin - class, tox
antitumor abx
**cardiotox
myelosuppression, alopecia
97
bleomycin - class, tox
antitumor abx
**pulm fibrosis
skin change, some myelosuppression
98
cyclophosphamid/ifosfamide - class, tox
alkylating agent
**hemorrhagic cystitis (prevent w/ mesna)
99
nitrosoureas - class, tox
alklyating agent
cns tox (dizziness, ataxia)
100
busulfan - class, tox
alkylating agent
**pulm fibrosis
101
vinca alkaloids - class, tox
MT inhibitor
vincristine- **peripheral neuropathy
vinblastine - blasts bone marrow
102
taxanes - class, tox
MT inhibitor
myelosuppression
103
cisplatin/carboplatin - mech, tox
cross link DNA
**nephro and acoustic nerve tox
104
etoposide/teniposide - mech, tox
inhibit topoisomerase II > DNA degradation
myelosuppression, GI irritation, alopecia
105
hydroxyurea - mech, tox
inhibits ribonucleotide reductase > dec DNA synth
myelosuppression, GI upset
106
tamoxifen/raloxifene - mech, tox
SERMs (selective estrogen receptor modulators)
tamoxifen - inc risk of endometrial cancer
107
trastuzumab (Herceptin) - mech, tox
ab against HER2
**cardiotox
108
imatinib (Gleevec) - mech, tox
bcr abl tyrosine kinase inhibitor
fluid retention
109
rituximab - mech, tox
anti CD20
NHL, RA
110
vemurafenib - mech, use
inhibitor of B-Raf kinase
| used in metastatic melanoma
111
bevacizumab - mech, use
ab against VEGF
| solid tumors
