Heme Exam 3

when to give fresh whole blood

massive hemorrhage

cardiace surgery with hemorrhage

when to give pRBCs

when you want to quickly correct anemia

how much does pRBC raise hgb

1g/dL

when to give FFP

TTP

when you need clotting factors

massive burns

immunodeficiency

multiple clotting factor deficiency

when to give cyroprecipitate

DIC

hemophilia A

VWD

when to give platelets

very low platelet count

not in TTP

types of transfusion reactions

hemolytic transfusion reactions

leukoagglutination reactions

hypersensitivity reaction

infection risk

transfusion related acute lung injury

transfusion associated graft versus host disease

acute hemolytic transfusion reaction onset

immediate

acute hemolytic transfusion reaction degree of reaction

severe

what is acute hemolytic transfusion reaction due to

ABO incompatibility from mislabeled specimen

acute hemolytic transfusion reaction caused by what

hemolysis is intravascular and IgM mediated

RBCs rupture inside blood vessels

acute hemolytic transfusion reaction S/S

fever

chills

HA

backache

more severe → dyspnea, hypotension, CV compromise

acute hemolytic transfusion reaction labs

low hgb

DIC and/or acute kidney injury may be present

acute hemolytic transfusion reaction management

stop transfusion

recheck product label

repeat type and cross

hydrate to protect kidneys

delayed hemolytic transfusion reaction onset

5-10 days post transfusion

delayed hemolytic transfusion reaction caused by what

usually due to minor RBC Ag

delayed hemolytic transfusion reaction what is happening

hemolysis is extravascular and IgG mediated

RBCs destroyed via phagocytosis by macrophages in liver, spleen, lymph nodes

delayed hemolytic transfusion reaction SS

mild at most

possibly asymptomatic

delayed hemolytic transfusion reaction labs

hgb low

total and indirect bilirubin will increase

delayed hemolytic transfusion reaction management

nothing

acetaminophen

water

hydrate

leukoagglutination reactions

reaction to Ag on donor leukocytes in donor blood products in patients who have received previosu transfusions or have been pregnant→ have been sensitized

nonhemolytic

what kind of transfusions most likely cause leukoagglutination reactions

platelet transfusions

leukoagglutination reactions presentation

fever/chills within 12 hrs

leukoagglutination reactions treatments

acetaminophen and diphenhydramine or corticosteroids

what reduces the risk of leukoagglutination reactions

leukoreduced blood products → for those who have had those reactions before

hypersensitivity reactions

occurs from allogenic plasma protein exposure in the transfused product

at risk for hypersensitivity reactions

pts with IgA deficiency

hypersensitivity reactions presentation

urticaria

bronchospasms

anaphylaxis

prevention of hypersensitivity reactions

washed or frozen pRBC for future transfusions

bacterial contamination is most prevalent in what blood product

platelets→ they cant be refridgerated

gram + transfusion contamination

causes fever and bacteremia

usually not sepsis

gram - contamination of transfusion products

often fatal

sepsis

syphilis in blood products

T. pallidum

spirochete

what is screened for in blood products in the US

Hep B

hep C

HIV

west nile virus

transfusion risk for hep B/C, and HIV

B- 1/200,000

C- 1/1.5 mill

HIV- ½ mil

transfusion related acute lung injury

pulmonary edema onset following transfusion

when is TRALI common

in critically ill patients and surgical patients

donor has antileukocyte antibodies → after preg, after transfusion, after organ transplant

how often does TRALI occur

1/5000 patients

why cant some people not donate plasma and platelets

they could have antileukocyte Ab

treament for TRALI

supportive care

transfusion associated graft vs host disease

lymphocytes form transfused blood products engraft in the recipient and attack the recipients tissues

skin, GI, and bone marrow

transfusion associated graft vs host disease how often

rare but fatal when happens

whos at risk for transfusion associated graft vs host disease

in immunocompromised patients receiving non-irradiated blood products that are not a complete HLA match

transfusion associated graft vs host disease SS

fever

rash

GI

hepatitis

lymphadenopathy

profound pancytopenia

transfusion associated graft vs host disease treatment

prevention

general immunodeficiency

inability or reduced ability of the body to fight off infections and cancers

primary immunodeficiency

most present in children

1/4000

an inherited or genetic defect in the immune system that makes a person more susceptible to infections.

seoncdary immunodeficiency

caused by something

illnesses - HIV, cancers, aplastic anemia

medications - steroids, anti-rheumatic drugs

lifestyle choices - alcohol, drugs, and smoking

malnutrition

aging

toxins

management of general immunodeficiency

boost immune system

aggressively treat infections

selective IgA deficiency

most common primary immunodeficiency syndrome

low/none IgA

family tendency

selective IgA deficiency presentation

mostly asymptomati

often seen in concert with the allergic triad

also seen with autoimmune disease

allergic triad

• Atopic dermatitis (eczema)

• Allergic rhinitis (hay fever)

• Asthma

IgA protects what

mucosal surfaces

selective IgA deficiency treatment

no specific treatment

treat infections as they occur

when to consider a selective IgA deficiency diagnosis

if a patient has an anaphylactic reaction to blood products

secondary to IgA Abs

common variable immunodeficiency syndrome

CVID

immune system in chaos

increased susceptibility to infections, autoimmune d/o, and malignancies

most common symptomatic immunodeficiency d/o

common variable immunodeficiency syndrome occurs in who

generally adolescence to young adult

typically sporadic but may be familial

common variable immunodeficiency syndrome infections

recurrent sinusitis, bronchititis, otitis

recurrent pneumonia and complications

bronchiesctasis common

GI infections and autoimmune d/o

what causes more serious infections in common variable immunodeficiency syndrome

encapsulated bacteria

meningitis

sepsis

common variable immunodeficiency syndrome associations

autoimmune disease in 50% of patients → especially cytopenias

cancers → B cell carcinomas (lymphoma) and GI cancers

common variable immunodeficiency syndrome diagnosis

order Ig panel

all have to present

• history of severe recurrent infections

• >/= 2 Ig low

• no other explanation for recurrent infections/ low Ig levels

• insufficient response to vaccines

common variable immunodeficiency syndrome management

Ig therapy

aggressive and early antibiotic use

referral to clinical immunologist/allergist

Ig therapy for common variable immunodeficiency syndrome

subcutaneous IgG replacement therapy → IgG-RT

weight based injections weekly/monthly

autoimmune hemolytic anemia

AIHA

acquired condition where autoantibodies react with pts own RBC and cause hemolysis

subtypes of autoimmune hemolytic anemia

warm

cold

warm autoimmune hemolytic anemia

IgG autoantibody

extravascular hemolysis

occurs at body temperature

more common

cold autoimmune hemolytic anemia

IgM auto antibody

extravascular hemolysis

complement mediated

in response to cold temperature exposure

primary warm autoimmune hemolytic anemia

50% of cases

secondary warm autoimmune hemolytic anemia

other autoimmune disorders

CLL

lymphomas

infections

medications

primary cold autoimmune hemolytic anemia

majority of cases

secondary cold autoimmune hemolytic anemia

mycoplasma pneumonia

mononucleosis

varicella

CLL

warm autoimmune hemolytic anemia presentation

fatigue

dyspnea

jaundice

icterus

splenomegaly

maybe cardio

maybe neuro

rapid in onset

cold autoimmune hemolytic anemia presentation

fatiuge

jaundice

icterus

numbness

mottling of cold exposed areas

hemoglobinuria

low back pain

no severe

autoimmune hemolytic anemia labs

CBC

• H/H low

• MCV elevated

CMP

• bilirubin elevated

LDH elevated

haptoglobin low

specific autoimmune hemolytic anemia tests

coombs test

coombs test

an antiglobulin test to detect potential presence of Ab against RBC Ag

when to order coombs test

when hemolysis is present

eval in transfusion reactions

direct coombs test

DAT

looks for IgG Ab or complement on RBCs

indirect coombs test

looks for free Ab in pt serum

warm autoimmune hemolytic anemia labs

hemolysis

variable anemia

peripheral smear- reticulocytes, spherocytes

coombs test-

• direct: positive for IgG, ± for complement

• indirect: ±

cold autoimmune hemolytic anemia labs

hemolysis

anemia is typically mild

peripheral smear: reticulocytes

coombs test

• direct: positive for complement only

• indirect: negative

warm autoimmune hemolytic anemia treatment

stabilize pt

transfuse if anemia is severe

glucocorticoids

maybe rituximab

folic acid

eval for underlying cause

splenectomy if other tx fail

cold autoimmune hemolytic anemia treatment

if mild or no symptoms no treatment is needed

if treating

• rituximab

• eval for underlying cause

• future transfusions performed thorugh a warmer

• pt education → avoid cold temps

types of imaging modalities

X-ray

skeletal survey

ultrasound

computerized tomography

magnetic resonance imaging

nuclear imagining- PET/CT

bone scan

X ray pros

availability

can be performed at the bedside

less costly than CT/MRI

less radiation than CT

helpful for assessing bony abnormalities

X-ray cons

radiation exposure

less detail provided than CT/MRI

cannot visualize soft tissues well

X ray indications

musculoskeletal assessment

chest

abdominal

post operative/monitoring

musculoskeletal X ray indications

fractures

dislocations

bone tumors

osteoarthritis

chest x ray indications

pneumonia

pneumonothorax

heart failure

COPD

rib factures

lung nodules/masses

abdominal x ray indications

bowl obstruction

perforations

foreign bodies

gall stones- meh

kidney stones - meh

post operative monitoring x ray indications

tube placement

fracture healing

hardware/prosthetics

ultrasound

transducer transmits sound waves to tissues that are echoed back and picked up by the US machine generating an image

US pros

great bedside tool

no radiation

US cons

user dependent

artifact

some areas inaccessible

US indications

prenatal evaluation

can help with procedures → IV, central line placement

heart and lung evaluation

trauma

abdominal pain assessment → nephrolithiases and cholecystitis

many more

US guided IV placement

placement is confirmed by visualization of the needle within the target vessel

short and long axis approaches

computed tomography scan

uses series of X ray images that are processed to create cross sectional images/slices

axial, sagittal, and coronal sections

CT pros

quick

efficient screening

highly sensitive for acute hemorrhage

CT cons

costly

radiation exposure

CT contrast

non contrasted versus iodinated contrasted studies