Thomas Day 15 Dit Part 2

Front 1

anti- A and B antibodies are what type?

when should rhogam be given?

Back 1

A and B - IgM

rhogam- at delivery of first baby

Front 2

which coagulation factor is responsible for initiation of both coagulation and kallikrein pathways?

Back 2

12 (intrinsic pathway, kallikrein)

Front 3

antithrombin III action

Back 3

inhibits thrombin activation of fibrinogen-->fibrin

Front 4

what is rate limiting step in coagulation cascade?

Back 4

X-->Xa

(where extrinsic and intrinsic come together)

Front 5

crosslinking of fibrin is what type of bond?

Back 5

covalent linking process

Front 6

IV citrate binds what substance and is used for what?

Back 6

binds calcium, which is necessary for many steps of the coagulation cascade. therefore decreases coagulation

Front 7

Enoxaprin mech and how different?

Back 7

low molecular weight heparin that works more on Xa, and can be given subq

Front 8

HIT causes what state and what drugs used to correct

Back 8

causes low platelet, hypercoagulable state

Lepirudin, bivalirudin, argatroban used (direct thrombin inhibitors)

Front 9

pneumonic for warfarin clotting factors

Back 9

diSCo started in 1972

proteins C and S
II, VII, IX, X (1 is 10)

Front 10

how is warfarin metabolized?

Back 10

by p450 (so can be affected by inducers and inhibitors)

Front 11

what are 2 kallikrein functions, and what activates it?

Back 11

activates the fibrinolytic pathway resulting in plasminogen-->plasmin

activates bradykinin (acts like prostaglandin, vasodilates, increases pain, cough specific)

activated by XIIa (just like intrinsic coagulation cascade)

Front 12

vWF helps aggregation of what (2)
binds what factor

Back 12

binds platelets to vessel wall collagen and other platelets (defiiency causes increased bleeding time)

Factor VIII (deficiency looks like Hemophilia A)

Front 13

what on platelets binds vWF?
other platelets?

Back 13

vWF- gpIb

platelets- IIb-IIIa to fibrinogen

Front 14

what is mechanism of decreased platelet aggregation for:
infliximab?
aspirin?
clopidogrel/ticlopidene?

Back 14

infliximab- ab to gpIIb/IIIa

aspirin- inhibits cyclooxygenase, so platelet can't release TXA2

clopidogrel/ticlopidine = irreversibly bind ADP receptor on patelets

Front 15

2 conditions acanthocytes seen in

Back 15

spiky red cells

abetalipoproteinemia
liver disease

Front 16

what is cause of basohpilic stippling and main condition seen in

Back 16

inhibit breakdown of rRNA, so is aggregrated ribosome RNA

Lead poisoning

Front 17

Macro ovalocyte seen in

Back 17

megaloblastic anemia

Front 18

target cells seen in (4)

Back 18

HALT

HbC
asplenia
Liver Disease
Thalassemias

Front 19

2 conditions Heinz bodies seen in, and what is cause of formation

Back 19

alpha-thalassemia
G6PD

oxidation to ferric state of Hb, aggregates

Front 20

"spoon nails" seen in what

Back 20

iron deficiency

Front 21

beta thal population?
what is difference in presentation of beta thal minor and major?

Back 21

Mediterranean

minor (heterozygote)- usually asymptomatic

beta thal major- severe anemia requiring transfusions

Front 22

what are the 2 symptomatic alpha thalassemias

Back 22

HbH (3 hits) --> 4 beta chains

Hb Barts (4 hits)--> gamma 4, hydrops fetalis in utero

Front 23

Mutations in B-thalassemia affect what regions of DNA

Back 23

splicing and promoter regions

Front 24

having a HBA2 (2 delta chains) >3.5 % indicates

Back 24

diagnostic of beta thal minor

Front 25

HbS/beta thal presents as

Back 25

mild to moderate sickle cell

Front 26

LEAD poisoning characteristics

Back 26

Lead lines (on gingiva, epiphyses of kids)
Encephalopathy
Abdominal Colic and Anemia (microcytic, from ALA dehydratase and ferrochelatase inhibition)
Drops (foot and wrist)

Front 27

4 causes of Microcytic Anemias

Back 27

iron deficiency
thalassemia
lead poisoning
heme synthesis problems (sideroblastic anemia, porphyrias)

Front 28

what is problem in sideroblastic anemia, 2 causes, rx

Back 28

defect in heme synthesis
(the basophilic stippling is iron that is trapped inside mitochondria in cell)

causes: alcohol, lead

rx- B6

Front 29

what synonymous with transferrin? what does % transferrin saturation indicate?

Back 29

transferrin- TIBC (amount of stuff available to transport iron around body)

%transferrin saturation= serum fe/TIBC- how much transferrin is being utilized
(used to distinguish iron def. anemia and AOCD)

Front 30

what iron-related lab value increases in pregnancy?

Back 30

transferrin (TIBC)

Front 31

if megaloblastic anemia does not correct with B12 or folate, what is cause? rx?

Back 31

orotic aciduria

must give uridine

Front 32

what substance can be increased in both folate and B12 megaloblastic anemia?

Back 32

homocysteine

only B12 has methylmalonyl acid increase though

Front 33

is increased haptoglobin intravascular or extravascular hemolysis?

Back 33

intravascular (mechanical destruction, paroxysmal nocturnal hemoglobinuria)

Front 34

bone marrow with adipocytes?

can be iatrogenically caused by

Back 34

aplastic anemia

radiation

Front 35

microcytic anemias are due to problems making ?

macrocytic anemias are due to problems making ?

Back 35

microcytic- problems making hemoglobin (either globin, iron, or heme)

macrocytic are due to defects making DNA synthesis

Front 36

positive osmotic fragility test

Back 36

hereditary spherocytosis

Front 37

2 specific renal complications in sickle cell

Back 37

renal papillary necrosis (because decreased 02, medullary infarct- causing microscopic hematuria)

Front 38

HbC is what?

Back 38

glu-lysine instead of valine (HbS)

can have HbSC which is milder sickle cell crisis

Front 39

what is sickle cell mutation

Back 39

point mutation at position 6 glutamine-->valine

Front 40

Paroxysmal nocturnal hemoglobinuria has increased wat in urine

Back 40

hemosiderin

Front 41

Normocytic anemia in HIV patient, drug responsible

Back 41

zidovudine

Front 42

Warm agglutination hemolytic anemia: what type of antibody?
1 autoimmune condition
1 disease
1 drug
1 type of infection

Back 42

IgG "Warm weather is GGGreat!"

SLE
CLL
alpha methyldopa
viruses

Front 43

Cold agglutination hemolytic anemia: what type of antibody
2 infectious causes
1 disease

Back 43

IgM (makes MAC complexes to lyse)

mycoplasma pneumoniae
EBV mono
malignancies

Front 44

direct coomb's tests for?

indirect coomb's test used for?

Back 44

Direct- to detect already bound antibodies on pt serum (add ab that binds to already bound abs)

indirect- screening (use pt serum and add normal RBCs to see if bind)

Front 45

2 infections that can cause hemolytic anemias

Back 45

malaria, babesia

Front 46

acute abdominal pain, psychosis, neuropathy, microcytic anemia?
deficiency?
rx?

Back 46

Acute Intermittent Porphyria

porphobilinogen deaminase
"a poor bilinogen can only acutely make porphyias and then go back to work"

glucose and heme

Front 47

what is rate limiting step in porphyrin synthesis and its cofactor?

2 starting materials

Back 47

gamma-aminolevulinic acid synthase

from glycine and succinyl-coA

B6

Front 48

uroporphyrin
porphobilinogen
gamma-ALA

which of these is common between the porphyrias and which are built up in only 1 porphyria?

Back 48

both
uroporphyrin

Acute intermittent porphyria
protoporphyrin
gamma-ALA

Front 49

Lead poisoning
inhibits what 2 enzymes, what builds up (think robots)

Back 49

ALA dehydratase
ferrochelatase

protoporphyrin
"a PROTOtype roboPORPHYRIN made of lead"

Front 50

microcytic anemia and photosensitvie blisters?
deficiency?

Back 50

Porphyria cutanea tarda

Uroporphyinogen decarboxylase
"a CUTe eURO porphyrinogen can get by easy and get sunburned lying at the beach

Front 51

uroporphyrinogen-1-synthase is deficiency in what?

Back 51

acute intermittent porpyhria (aka porphobiliogen deaminase)

Front 52

think of homeless man for what disease?

Back 52

Porphyria cutanea tarda
(sun burnt blisters, dark skin (hyperpigmentation), hypertrichosis (excess facial hair), Hep C and alcoholism

Front 53

what are changes in BT, Platelet count, pT, pTT for:
ITP
TTP/HUS

Back 53

decreased platelets and increased Bleeding Time for both,
but normal pT and pTT (because PLATELET DEFECTS!)

Front 54

Glanzmann's vs Bernard Soulier's thrombocytopenia defect?

change in BT, Platelet count, pT, pTT for each?

Back 54

Bernard Soulier- gpIb
DECREASED PC, increased BT

Glanzmanns - gpIIb/IIIa
NORMAL PC, increased BT

pT and pTT NORMAL FOR BOTH because platelet disorders!

Front 55

2 diseases that have mech of abciximab, and how tell difference?

Back 55

Glanzmann's Thrombocytopenia (decreased gpIIb/IIIa expression, but normal platelet count)

Idiopathic Thrombocytopenic Purpura (ab to gpIIb/IIIa- natural abciximab)
-here have platelet destruction, so will hace decreased platelets and increased megakaryocytes

Front 56

TTP and HUS:
what is triad of HUS (think about name)
TTP add what 2 things?
defect of TTP

Back 56

HUS- hemolysis, uremia, thrombocytopenia

TTP- + fever and neurologic symptoms

TTP defect- ADAMTS13 (defect in vWF metalloprotease, decreased vWF multimer degredation)

schistocytes

Front 57

in chronic renal disease, what bleeding abnormalities are noted (BT vs. pT and pTT)

Back 57

BT increases (platelet dysfxn), but pT and pTT normal

Front 58

inheritance of von WIllebrands

Back 58

Autosomal Dominant

Front 59

increased inherited thrombosis causes:
-gene mutation in 3' region
-reduced increase in pTT after heparin
-increased Factor V

Back 59

prothrombin mutation
antithrombin III deficiency
Factor V leiden (can't be broken down by protein C)

Front 60

(+) ristocetin test
and bleeding disorder

Back 60

von Willebrands disease

Front 61

fatigue, anemia, + HAM test

Back 61

Paroxysmal Nocturnal Hemoglobinuria (intrinsic hemolysis)

Front 62

B symptoms, mediastinal lymphadenopathy, CD15 and CD30 positive cancer is what?

who gets it?
prognosis correlated to

Back 62

Hodgkins lymphoma

bimodal (young and old, non-hodgkins fills middle)

Reed-Sternburg/lymphocyte ratio

Front 63

Hodgkins with collagen banding in lymph node is what subtype

Back 63

nodular sclerosing (best prognosis, most common)

Front 64

t(14:18) translocation, what is gene associated and condition

Back 64

Follicular B cell lymphoma

BCL-2!!! (anti-apoptosis)

Front 65

what is most common non-Hodgkin's lymphoma

Back 65

Diffuse Large B cell lymphoma (not burkitts!)

Front 66

PAS is positive for what leukemia and negative for what leukemia?

Back 66

+ for ALL
- for AML

Front 67

which leukemia is CD 13/33(+) ?

Back 67

AML

Front 68

what cancer is terminal deoxynucleotide transferase positive?

Back 68

ALL (tdt+, PAS positive as well)

has good prognosis, bone pain is common

Front 69

what % blasts must be present for ALL/AML

Back 69

20%

Front 70

what condition do you see hemolytic anemia and smudge cells in

Back 70

CLL

can progress to blast crisis- ALL

in adults over 50

Front 71

how differentiate from leukemoid reaction?

CML crisis can progress to

Back 71

high leukocytes but low leukocyte alkaline phosphatase

AML (80%) or ALL

Front 72

4 conditions "fried egg appearance" seen

Back 72

oligodendroglioma
seminoma
Multiple myeloma
koilocytes

Front 73

Multiple Myeloma:
what seen on X-ray
electrophoresis:
urinalysis
UPEP
Blood smear
immune function
casts

Back 73

x-ray- punched out lesions
electrophoreis- M spike
urinalysis- eosinophilic casts of bence jones proteins,
UPEP- bence jones proteins (will not see in regular urinalysis)
blood smear- roleaux formation
immune- increased susceptibility to infections

can also get light chain amyloidosis

Front 74

CRAB for MM

Back 74

hyperCalcemia
renal insufficiency
Anemia
Bone lesions/back pain

Front 75

Waldenstrom's macroglobulinemia can be differentiated from IgM MM because it does not have

Back 75

lytic lesions

Front 76

Plasmacytomas are a type of _____? extramedulary type likes to go where

Back 76

Multiple Myeloma

like to go to head and neck (likes nose)

Front 77

how does multiple myeloma cause lytic lesions

Back 77

makes local cytokines that activate osteoclasts and inhibit osteoblasts

Front 78

birbeck granules indiacte what

Back 78

Histiocytosis X (tennis racket looking things)

after watching american histiocyte X, i wanted to do something uplifting like play tennis

Front 79

headache, hepatosplenomegaly, hyperviscosity, increase in all numbers of RBCs, platelets, WBCs? what is genetic mutation?
seen in what other 2 conditions?

Back 79

polycythemia vera

JAK2 mutation, also seen in myelofibrosis and essential thrombocytosis

Front 80

Most common leukemia in kids?
most common leukemia in adults?
most common lymphoma in adults?

Back 80

kid leukemia- ALL

adult leukemia- CLL

adult lymphoma- Hodgkin's lymphoma

Front 81

Intestinal T-Cell lymphoma associated with

Back 81

long term celiac

Front 82

myelodysplastic and myeloproliferative disorders can progress to

Back 82

AML

Front 83

Numerous basophils, splenomegaly, and negative for leukocyte alkaline phosphatase

Back 83

CML

Front 84

3 associations of marginal cell MALT lymphoma

Back 84

H. Pylori
hashimoto's thyroiditis
Sjogren's syndrome

Front 85

Hodgkins age distribution vs non-hodgkins age disribution

Back 85

Hodgkins- young and old (bimodal)

Non-hodgkins lymphomas (burkitts, diffuse large cell, mantle, follicular)

Front 86

which coombs identifies autoimmune hemolytic anemia? non-autoimmune hemolytic anemia

Back 86

autoimmune- direct