Path Winter 1 Hematology

Front 1

warm or cold AB

1. IgG
2. IgM
3. MAC- fast severe intravascular hemolysis
4. opsinization- slow hemolysis

Back 1

IgG: warm
IgM: cold

MAC: cold
Extravascular: warm

Front 2

what are BGA

Back 2

blood group AG- ABO

**carb that is ATTACHED to the membrane via lipid or protein (cant be washed off). seen on RBC and also other tissues and secretions

Front 3

what is the inheritance of BGA

Back 3

bi-allelic and co dominant

**O and Rh are NOT co-dom

Front 4

what is the single more important consideration for transfusion matching

Back 4

ABO blood type

Front 5

what determines if an AG will cause an AB to be formed

Back 5

1. the AG itself
- carbs (ABO) are T cell independent and make IgM
-proteins: stim T cells and make IgG

2. Host HLA

Front 6

what are the consequences of transfusion of incompatible blood, pathogenesis

Back 6

hemolysis! type II HS rxn

RBC with AG + AB -->
1. Compliment: can do AMC and cause immediate severe osmotic lysis OR can do opsinization for hemolysis by spleen
2. Fc Macrophage--> extravascular hemolysis in spleen

Front 7

what are the categories of immune hemolysis

what kind of reaction

Back 7

1. Intravascular: compliment, MAC. severe (immediate osmotic lysis)

2. Extravascular- in the spleen (Fc/macro mediated)

3. alloimmune: lysis of transfused RBC

4. Autoimmune: lysis of self RBC

type II hypersensitivity- ALWAYS. the AG on the RBC is integral and cant be washed away, AB will always kill it

**hemolysis: release of RBC cell membrane and Hg is released

Front 8

how are blood groups determined

Back 8

pts RBC are added to antiserum and agglutination is looked for

**so if there is antiA in the serum and your pts blood clumps the pt is type A, if no clump there is NO A AG

Front 9

what are cold acting AB

Back 9

active in cold temps (Raynauds in cold places)

IgM (carbs make IgM, proteins make IgG)

agglut RBC

fix compliment--> intravascular (MAC) hemolysis, BAD

Front 10

what are warm acting AB

Back 10

active at body temp

IgG (protein)

more common to fix compliment and get opsinization NOT MAC. opsinization --> extravascular hemolysis (spleen). slow hemolysis

Front 11

what makes up a blood group

Back 11

more than just ABO

*RBC, WBC, platelet AG SYSTEMS.
**AG vary in immunogenicity

Front 12

what AG are the most immunogenic

Back 12

1. A
2. B
3. D (Rh)
4. K (kell)

Front 13

does the O blood type have hte enzyme that adds sugar to carbs

Back 13

ya, its inactive though

AB are codominant. not fully expressed at birth

Front 14

what is the AB found in the serum of a person with the following blood type

1. O
2. A
3. B
4. AB

Back 14

1. O- anti A, antiB AB
2. A- anti B AB
3. B: antiA AB
4. AB: none

Front 15

what happens when blood with an AG is transfused into a person who does NOT have that AG

ie a type A person getting type B blood

Back 15

hemolytic transfusion reaction

**a person with type A blood will have antiB AB, these AB will attack the new blood

**ABO incompatibility btwn mom/fetus --> hemolytic disease of newborn
**ABO incompatibility --> hyperacute rejjection of solid organ transplant
**can be FATAL

Front 16

if you have AB to the following what is your blood type

1. anti A
2. Anti B
3. Anti A and anti B
4. no anti AB

Back 16

1. B
2. A
3. O
4. AB

**if you HAVE type A blood you have AB to type B blood!

Front 17

when do you see ABO AB

Back 17

not til you are like 6 mo

they are usually IgM, cold, agglutinating AB that fix compliment and cause hemolysis

decreased AB with old ppl and hypoagammaglobunemia

Front 18

if a person with type B blood gets a type A kidney what can happen

Back 18

ABO incompatibility. hemolytic transfusion

**the person with type B blood has antiA AB, these kill the kidney and we get a hyperacute rejection

**immune hemolysis is a type II hypersentivity reaction

Front 19

what is forward and backward blood typing

Back 19

determiens ABO blood type

Forward: types
Pts RBC are put in anti A and anti B serum. If one tube agglutinates its a + test.

Backward: confirms type
Anti A/B AB are taken from pts serum and added to known RBC of A/B. look for agglutination

Front 20

what is the universal donor? is it ALWAYS safe

Back 20

type O, is has anti A and anti B AB in their serum but have NO A or B AG in its cells
Rh negative


not safe for ppl with atypical AB

Front 21

we said ABO is biallelic

what is the genotype if the phenotype is...

1. A
2. B
3. AB
4. O

Back 21

1. AA AO
2. BB BO
3. AB
4. OO

Front 22

universal donor is
type ___
Rh __

Back 22

O
negative

**cells have NO A,B or Rh AG
**there will be antiAantiB in serum but its a small amt so not clinically significant

Front 23

An 83 y/o female was admitted to the hospital
following a fall on the slick wet floor of the
grocery store. She complained of pain in her
left hip.
• On physical exam with the patient supine, the
left hip was externally rotated with pain on
attempt to internally rotate. There was pain on
palpation over the hip with swelling
An x‐ray revealed a fracture of the left femoral
neck.
Lab tests Patient results Reference range
Hemoglobin (Hb) 9 gm 13.5‐16 g/dL
Hematocrit (Hct) 27% 35‐45%
In preparation for surgery, two units of blood
were ordered to be typed and crossmatched for
transfusion at surgery.
The lab reported that the patient had an
atypical antibody or antibodies and that
compatibility testing would be delayed for
antibody identification.
• The surgeon demanded that his patient receive
blood from a universal donor.
• What is the universal donor for this case?
• What are atypical antibodies

Back 23

hip fractures bleed lots to Hg is low

Typed: ABO determination. put pts RBC in antiA/B serum and then confirmed with pts plasma combined with known RBC

Crossmatched:

Atypical: not A or B. NO universal donor! can cause fatal hemolytic transfusion reaction. determined by a screen!

Front 24

whats an atypical AB

why do you get atypical AB

whats the consequence

how do you know they are present

Back 24

1. any AB to a blood group AG that is not A or B

2. pregnancy or transfusion

3. can cause hemolytic transfusion rxn (fatal sometimes)

4. a screen tells us about atypical AB (mix pts serum with known RBC AG to detect pts AB)

Front 25

what does this describe

a pts serum is mixed with known RBC AG to determine what AB are present in the pts serum

a pts RBC is mixed with anti A and anti B serum

Back 25

screen for atypical AB

forward typing

Front 26

what are the important atypical AB

Back 26

any from the end of the alphabet

Front 27

whats D
whats d

what are the other locus for Rh determination

what is the consequence of ALL Rh system AB

Back 27

D Rh+ (super immunogenic)
d Rh - (neg is the universal donor)

Cc and Ee. these are more common in ppl previously transfused

Consequence: hemolytic transfusion rxn or hemolytic disease of newborn

Front 28

can 2 Rh + parents have Rh - kid

can to Rh neg parents have Rh + kid

Back 28

yes
nope

Rh + "D" is highly immunogenic

Front 29

whats a type
whats a screen

Back 29

ABO blood type and Rh

atypical AB

Front 30

whats Kell

what happnes at first and 2 exposure

Back 30

atypical AB, highly immunogenic

**first exposure makes AB
**2 exposure --> hemolytic transfusion reaction or hemolytic disease of newborn

Front 31

what are some common atypical AB

Back 31

1. Rh
2. Kell- highly immunogenic
3. Duffy- when absent decreased maleria
4. Kidd- serious life threatening reaction

Front 32

whats duffy

Back 32

atypical AB

**when present can lead to hemolytic transfusion reaction or hemolytic disease of newborn

**its the attachment site for maleria, when its absent there is less risk of maleria

Front 33

whats a very serious atypical AB

Back 33

Kidd

**life threatening

Front 34

ok so your pts has atypical AB, does it matter which ones

Back 34

ya A-K will kill you (kidd is serious bad)

L-P is ok, still transfuse if needed

**no universal donor for atypicals

Front 35

what happens if most ppl have a particulat AG but you do not

whats a common example of this

Back 35

k - blood is really rare, most ppl have k

when someone who is k- gets k+ blood the first time its no big deal but another transfustion will cause issues if the person develops an AB

SOLUTION: autologous units, frozen rare units

Front 36

1. what is an atypical AB
2. how do you get them
3. what are consequences
4. what is the universal donor

Back 36

1. AB that is not A or B (kidd, duffy, Kell)
2. transfusion, preggo
3. cause hemolytic transfusion reaction or hemolytic disease of newborn
4. none, do autologous frozen blood

Front 37

what are autoAB

Back 37

when pt has AB against self RBC AG

ex type A blood, typical to have anti B AB, but antiA would be autoAB

**pts seruim is incompatible with ALL cells, including donors. + direct antihuman globin test (DAT)

Front 38

what is going on if a pts serum is incompatible with ALL cells, including donot and has a + DAT

Back 38

AutoAB

**pt developed AB that react to self RBC AG

Front 39

what is warm autoimmune hemolytic anemia

Back 39

+ DAT w/o hemolysis
common
AB reactive at 37*
caused by drugs, SLE, lymphoproliforative disease

Front 40

what is cold agglutinins (autoAB)

Back 40

hemolytic anemia may occur

caused by virus, or lymphoproliformative disease

Front 41

what is a direct and indirect antihuman globulin test aka coombs

Back 41

1. Direct: antihuman globulin (from rabbit) is put into pts cells. if cells agglutinate its +. AB has coated the pts cell in VIVO

*+ direct antihuman globulin test (DAT) is seen with autoAB, autoimmune hemolytic anemia, hemolytic transfusion reaction, hemolytic disease of the newborn

2. Indirect: pt serum is incubated with known AG, cells are washed, antihuman globulin is added. + means there are atypical AB, SCREEN

Front 42

what is a screen

Back 42

indirect coombs test, detects the presence of aytpical AB

Front 43

can donated blood last forever

Back 43

nope, shelf life of 42 days. cells die during storage

lose NO,
release K+,
ammonia,
hemoglobin;
lose 2,3 DPG; (loose O2 capacity)
release inflammatory mediators

Front 44

type and screen before a transfusion or type and cross match

Back 44

Type and screen is cheaper: type- ABO, Rh
Screen- indirect coombs for atypical AB

Cross match: dbl check ABO, donor cells are mixed with recipient serum, if agglut occurs its NOT compatible

** regardless with transfusion DOCUMENT DOCUMENT DOCUMENT, the transfusion cant be with drugs or half saline

Front 45

what are the indications of transfusion of:

1. Packed RBC
2. Frozen RBC
3. PLatelets
4. Fresh Frozen Plasma
5. Cryoprecipitate

Back 45

1. Packed: acute blood loss, chronic anemia (decreased O2 capacity requires transfusion)

2. Frozen: rare blood types, autologous units, weird AB in the recipient

3. Platelets: you really need it, hard to come by. Bleeding and platelet count is <50k. platelet <30k not bleeding. documented platelet prblm in a bleeding pt. CI in TPP, not effective in autoimmunethrombocytopenia

4. FFP: gives coagulation factors. used when PT and PTT are long and pt is bleeding or there is a planned surgery.

5. Cryoprecipitate: has factor 8, fibrinogen and vWF. used when there is bleeding in a pt with these deficits

Front 46

what do you use...

1. a pt who is bleeding and has low platelts and TTP
2. Gunshot wound
3. person is bleeding and has factor 8 deficit
4. person is bleeding and has a long PPT, PT
5. your pts blood is rare bc they have lots of AB
6. your pt has repeated allergic transfusion reactions

Back 46

1. Platelets are CI!!!
2. packed RBC
3. cryoprecipitate: used with factor 8, fibrinogen, vWF deficit
4. fresh frozen plasma (FFP has coagulation factors)
5. frozen RBC
5. Washed RBC- this removes the plasma proteins

Front 47

when do you give washed RBC

Back 47

**keep in mind the integral membrane things arent going anywhere, ie the ABO AG will still be there!

**washed removes PLASMA PROTEINS
- removes IgA
- good for repeated allergic transfusion reactions

Front 48

what transfusable product contains:

1. vWF, factor 8, fibrinogen
2. coagulation factors
3. NO plasma proteins

Back 48

1. cryoprecipitate
2. FFP
3. washed RBC

Front 49

when m ight you use CMV negative blood

Back 49

neonates
immunocomprimised pts
pts who will get a transplant in the future

Front 50

when might you give serum immune globulin

Back 50

this has lots of VIII, and IX in it as well as albumin

**used for tx of autoimmune deficit
**brutons agammaglobunimea
**exposure to infection
**immune thrombocytopenia

Front 51

what is autoimmune thrombocytopenia treated with

Back 51

NOT platelets, this is CI!!!

transfuse with serum immune globulin (this is also done for autoimmune deficits in Ig like brutons (x linked) a gammaglobinemia

Front 52

what do they think might be used to help "bridge the gap" so that a person needing a transfusion can wait for a perfect match

Back 52

polymerized Hg, this will carry O2 til we can get the right blood

**not ready yet, there are lots of bad effects

Front 53

what are some adverse effects of transfusions
(9)

Back 53

1. hypothermia--> V fib (warm blood first!)

2. Electrolyte, acid base disturbance

3. dilutional coagulopathy

4. DEPRESSION OF EPO

5. Poor survival for renal pts

6. Fe overload after multiples

7. circulatory overload

8. Proinflammatory

9. Alloimmunization: risk for ppl with second transfusion or ppl who will get pregnant

Front 54

tell me about transfusion related GvH disease

Back 54

DEADLY!!! T cells from donor kill host,

recall its graft c host so: yello jaundice, red rash and diarrhea!
more common in comprimised pts

**kill T cells with radiation proir to transfusion

Front 55

but doc, are transfusions safe, i dont wanna get AIDS

whats disease transmission like

Back 55

most diseases about 1 in a million *HIV, Hep C, HTLV I/II, Hep B,

CMV- common, most donors have it
, EBV- common

Front 56

list the transfusion reactions that are possible (6)

Back 56

1. febrile non hemolytic
2. allergic: anaphylactic can be fatal
3. Delayed hemolytic
4. Acute Lund Disease- TRALI
5. Acute hemolytic- can be fatal
6. TRIM- transfusion related immunomodulation

Front 57

Patient underwent plasmapheresis for TTP with
volume replacement by fresh frozen plasma.
1 hour after plasma transfusion and 15 minutes
into transfusion of packed RBCs, patient reported
chills and dyspnea. BP was 80/50.
• O2 saturation fell to 49 mm Hg on 100% O2.
• Patient was intubated and maintained on
ventilator. She recovered in 3 days.
• Chest x‐ray showed pulmonary edema.
• No hemolysis.

DIAGNOSIS

Back 57

TRALI

**common cause of serious, immediate, life threatening transfusion reaction. Sudden pulm edems ---> ARDS

**most common with plasma transfusion (FFP)

Front 58

if you transfuse FFP and the person gets pulm edema --> ARDS whats going on

Back 58

transfuaion related acute lung injury; TRALI

**serious, immediate, fatal

**caused by transfused plasma that has AB to recipient WBC (common in preg woman)

**injury to pulm vaqsculature by free radicles

Front 59

if your transfused blood has AB to WBC what can happen

Back 59

TRALI- the donor AB will attack the recipients WBC and we get lung injury

Front 60

whats a febrile non hemolytic reaction

Back 60

reaction to a transfusion

**when you get fever chills, HA, tachycardic, flushing after a transfusion.

**think its caused by cytokines in stored blood

**tx with tylenol

Front 61

what is an allergic transfusion reaction

Back 61

AB against plasma proteins in the donor ex IgA deficit

type I IgE mediated HS reaction

**you get hives, anaphylaxis, tachycardic, wheeze, dyspnea

**treat with antihistamines

Front 62

what is a type I IgE mediated transfusion reaction

Back 62

allergic reaction

Front 63

what causes acute hemolytic transfusion reaction

what are the lab findings

Back 63

immediate hemolysis of transfused RBC bc recipient has AB to donor RBC

ex ABO incompatibility, undetected atypicals, can be fatal

+ DAT test

Front 64

what is the cause and lab finding for delayed hemolytic transfusion reaction

Back 64

happnes 3-10 days after trasfusion as an anamnestic response
- you had a really low AB titer and it will suddenly rise

Front 65

ok so you just had a transfusion adn the injection site is burning, yuo have pain in low back/kidney, and you have pink urine, you have bleeding from IV site, and acute renal failure

whats going on

Back 65

hemolytic transfusion reaction

**if it happens immediate its acute, can also be delayed

**you had AB to the donor blood cells

Front 66

whats the pathogenesis of hemolytic transfusion reactions (we know its bc you have AB to donor RBC AG)

Back 66

you can have eihter intra or extravascular hemolysis

treat by stopping infision, IV fluids, diuretics (mannitol) to spare the kidney, tx DIC

Front 67

Whats TRIM associated with
who gets good, bad outcome
whats the clinical

Back 67

TRIM- transfusion related immunomodulation

**PMN activation adn release of inflammatory cytokines
**better outcome in renal transplant, greater risk of cancer recurrence. can also lead to multiple organ failure

*jaundice, anemia +DAT

Front 68

what are some signs/sx of hemolysis

Back 68

jaundice
anemia
+ DAT test (direct coombs)

Front 69

whats apheresis

Back 69

removal of whole blood from patient or donor and then retranfuse it into donor or patient

Front 70

in what diseases is apheresis used as treatment

Back 70

apheresis is when you take whole blood out and only put certain bits back in

goodpastures
TTP (dont use platelets)
MG
GBS

**you take out the component that contributes to disease state

Front 71

what is hydrops fetalis, what blood things cause it

Back 71

water baby. accumulation of water--> edema in uterine (anasarca, extreme generalized edema)

Caused:
1. non immune fetal anemia: homozygous a thalassemia, aplastic anemia due to parvovirus B19
2. hemolytic disease of newborn
3. twin to twin transfusion
4. infections

Front 72

IF A BABY HAS HOMOZYGOUS A THALASSEMIA, APLASTIC ANEMIA due to parvovirus B19 or hemolytic disease of newborn what can happen

Back 72

hydrops fetalis

Front 73

what happens in immune hydrops fetalis

Back 73

its a hemolytic disease of new born

**fetus inherits Blood Group AG from father. this AG is foerign to the mom and so she maks AB against it and will attack the next baby (typically its the first pregnancy that is affected)

**the problem si with the RED BLOD CELLS

Front 74

what is the most common cause of hemolytic disease of newborn

Back 74

D
Other Rh AG: e E C c

also atypical AB (kell, duffy)
ABO incompatibillty (common, but not serious)

Front 75

using D as the AG explain the pathogenesis of hemolytic disease of the new born

Back 75

d (Rh- mom) is preg with a D (Rh +) babe

D+ cells enter her circulation and she makes AB against it. typically a bigger prob for the SECOND baby bc initially IgM is made and it wont cross placenta, IgG will cross placenta

**ways to enter circulation:
abortion/miscarriage
loss of cytotrophoblast barrier
placental accidents in child birth
anmiocentesis

Front 76

whats the pathogenesis of hemolytic disease of newborn Rh+ babe

Back 76

mom is Rh- but is somehow exposed to Rh+ and makes AB

IgM is made at first but WONT cross the placenta, this is good. first baby not affected

on the 2 pregnancy the Igm --> IgG and WILL cross the placents. moms anti Rh AB kill the RBC of the babe and leads to anemia, Hgb destruction, --> hydrops, jaundice, kernicterus

Front 77

how do we prevent Rh hemolytic disease

is it 100%, does it prevent hemolytic disease caused by other incompatabilities

Back 77

we give Rh Immune globulin (rhogam) at 28 weeks of ALL Rh- moms, 72 hrs b4 delivery of Rh+ babe, and before any abortion or amniocentesis

its like 90% and is ONLY for Rh

**check for rising titer in mom. mom gets a screen on first prenatal visit, if there is an abnormal AB we look for the titer to rise, if titer rises we do amni for biliruben levels. when bili is high we can do an intrauterine transfusion to combat anemia

Front 78

whats the consequence of hemolytic disease of newborn

Back 78

Extramedularry Hematopoiesis: make blood cells in places outside BM (liver, spleen) gives blueberry muffin apprearance

Erythroblastosis fetalis: nucleated RBC in circulation

Hydrops Fetalis (anasarca): lots of edema, heart fialure, liver failure, decreased albumin synthesis

Front 79

whats Rh immune globulin

Back 79

its meds given to Rh - moms who are preg.

given at 28 weeks (trophoblast degeneration)
before delivery of Rh+ babe
before abortion or amniocentesis

**MOST pPL ARE Rh+

Front 80

what do we test for in ALL pregnant women

Back 80

atypical AB

we measure the titer, if it increases it means baby is affected and we do an amnio and check biliruben levels. increased biliruben indicates anemia and is combated with intrauterine transfusion

Front 81

is ABO fetal maternal incompatability common, is it serious

Back 81

common, not serious. Rh is less common and more serious

any AB the mom has are IgM and wont cross placenta so no hemolysis

**nothing prenatally we can do

Front 82

whats the big deal about hemolytic disease of newborn

Back 82

can leads to:

1. Extramedularry hematopoiesis: ie make blood outside of BM, blueberry muffin appearance

2. Erythroblastosis fetalis: nucleated RBC in circulation

3. Hydrops fetalis (anasarca):heart/liver failre

Front 83

whats kernicterus

Back 83

when you have hemolytic disease of newborn you can get liver failure in the babe. this can let uncong biliruben build up and bind to lipids and deposit in brain, this is called kernicterus and can lead to brain damage

**can cause mental retardation, deafness, and other neurological abnormalities

Front 84

what are deposits in the brain that are attached to lipids called, what is it caused by and what does it cause

Back 84

caused by hemolytic disease of the newborn

can lead to brain damage and yellow coloration of brain

Front 85

what are the clinical signs of Hemolytic disease of newborn

Back 85

when mild you get pallor (anemia) and hepatosplenomegaly

when severe you get:
intense jaundice
signs of neurologic involvement
generalized edema (liver failure, no albumin being made)

Front 86

if you have an infant with decreased hgb and HCT and increased uncon biliruben, +DAT and the mom has atypical AB what are you thinking

Back 86

hemolytic disease of newborn

Front 87

what are hte hemotologic causes of hydrops fetalis

Back 87

1. Non immine fetal anemias: homozygous a thal, aplastic anemia due to parvovirus,
2 hemolytic disease of newborn
3. twin, twin transfusion
4. other infections
5. malformations, tumor, metabolic disease

Front 88

does ABO incompatibility increase or decrease risk of having maternal AB that attack the baby

Back 88

ABO INCOMPATABILITY DECREASES RISK!

if a type mismatched baby is also Rh+ and mom is Rh- that mismatched blood type is killed so Rh is less of a risk

Front 89

if Maternal IgG anti D (anti Rh) crosses into the Rh+ fetus what happens

Back 89

Rh + RBC are hemolyzed, this leads to fetal anemia and hyperbilirubenemia

Front 90

DAT is + in the mom or babe

AB screen (indirect coombs) is + in the mom or babe

Back 90

DAT + babe

Indirect Coombs mom

Front 91

why do we see extramedullary hematopoesis in infants attacked by maternal Rh

Back 91

its the response to anemia- make more red blood in liver, spleen etc. this is why we have hepatosplenomegaly

CHF, nutmeg liver

Front 92

what is erythroblastosis fetalis

Back 92

nucleated RBC in circulation

**this term is used interchangably with hemolytic disease of the newborn.

Front 93

what are the consequences of the anemia that is seen in hemolytic disease of newborn

Back 93

1. extramedullary hematopoiesis
2. erythroblastosis fetalis
3. hydrops fetalis

Front 94

if a babe has swollen feet and hands and ascites, hepatosplenomegaly whats the deal

Back 94

fetal hydrops

**erythroblastosis fetalis

**will also have a large pale placenta

Front 95

what are the labs assocaited with hemolytic disease of newborn

Back 95

1. anemia
2. uncong biliruben is high (haptoglobin is low)
3. infant has + DAT
4. + Atypical AB screen in mom
5. ABO- no findings in mother