BB 9: AIHA

for immune hemolytic anemias, the DAT will always have a ____ result.

positive

retic count ____ in both compensated and uncompensated anemia.

increase

compensated or uncompensated anemia: macrocytosis and spherocytosis?

uncompensated

for warm autoimmune disease, the DAT is usually positive for ____ only, but C3d may also be present.

IgG

a DHTR can cause a positive ____ DAT.

IgG

for cold autoimmune disease, the DAT is usually positive for only ____.

C3d

which category of immune hemolytic anemia describes the following:

• HTR

• HDFN

alloimmune

which category of immune hemolytic anemia describes the following:

• CAIHA (20-30%)

  • benign

  • pathological (CAD/CHD and PCH)

• WAIHA (70-80%)

• mixed type - both warm and cold auto Abs present (7-8%)

autoimmune

which category of immune hemolytic anemia describes the following:

• drug absorption

• immune complex

• membrane modification

• warm auto-Ab (alpha-methyldopa [aldomet], daratumumab)

drug induced

to differentiate auto Abs and allo Abs, perform ____ techniques.

adsorption

which adsorption technique is described below:

• no recent transfusion (within the last months) or BM transplant

• auto Abs are removed using patient RBCs, so allo Abs can be identified

• after the patient’s RBC are incubated, the adsorbed serum is tested with panel cells to ID the allo Ab (if present)

autoadsorption

allogenic adsorption may also be referred to as ____ adsorption.

differential

which adsorption technique is described below:

• if recently transfused

• use selected cell types with patient’s serum

  • R₁R_1, R₂R_2, rr

  • plus one K-, one Jk^a-, and one Jk^b-

differential

benign or pathological Abs: thermal reactivity range of <20-24C?

benign

benign or pathological Abs: thermal reactivity range of <30C?

pathological

benign or pathological Abs: titer @4C <64?

benign

benign or pathological Abs: titer @4C >1000?

pathological

benign or pathological Abs: common specificity anti-I or anti-IH?

benign

benign or pathological Abs: common specificity anti-I?

pathological

benign or pathological Abs: capable of binding C3 in vitro with a DAT grading of 0-1+ due to C3?

benign

benign or pathological Abs: capable of binding C3 in vivo with a DAT grading of 2-4+ due to C3?

pathological

what is the most common type associated with cold reactive auto Abs?

CAD

which form of CAD is secondary to lymphoproliferative diseases or Mycoplasma pneumoniae infections?

acute

which form of CAD is seen in elderly and may result in Raynaud’s Disease/Syndrome and hemoglobinuria if exposed to extreme cold temperature?

chronic

what condition is often characterized by RT agglutination of EDTA cells to the extent the sample appears clotted, if Ab is high titered?

CAD

what refers to typical signs of discolored fingers and bluish finger tips?

Raynaud’s syndrome

for individuals with CAIHA/CAD/CHD, the ____ AHG reagent should be used because the antibody is usually IgM.

monospecific

elution is required for DAT for individuals with CAIHA/CAD/CHD. true or false?

false

elution is not required for DAT for individuals with CAIHA/CAD/CHD because there is no ____ present.

IgG

cold auto-adsorption test cannot be performed on recently transfused patients. true or false?

true

____ antibodies include:

• anti-I usually

• anti-i sometimes

• anti-Pr rarely

CAD

what is the least common AIHA?

PCH

____ occurs most often in young children with viral infection.

PCH

____ is originally seen in adults with tertiary syphilis, but it is now rarely seen since syphilis is treatable.

PCH

the PCH antibody is ____ with anti-P specificity, biphasic hemolysin.

IgG

the antibody for the condition ____ is also known as the Donath-Landsteiner Ab.

PCH

PCH has the auto Ab is IgG which acts as a cold agglutinin and has ____ specificity.

anti-P

in the condition ____, the Ab is a biphasic hemolysin where IgG Ab binds in the cold and fixes C’ while at warm temperatures, IgG dissociates and C’ remains so only C’ is detected.

PCH

it is not necessary to transfuse P negative for PCH patients. true or false?

true

the eluate result for PCH is ____ since C’ coat cells.

negative

what AIHA is the most common type and most difficult problem encountered in the transfusion service?

WAIHA

warm or cold auto: splenomegaly?

warm

warm or cold auto: hepatomegaly?

warm

warm or cold auto: adenopathy?

warm

warm or cold auto: no organomegaly?

cold

warm or cold auto: IgG present with small amount of C3 potentially be present?

warm

warm or cold auto: acrocyanosis and raynaud’s syndrome?

cold

a common scenario of ____ is that all cells in the ABS and Ab ID panel including to autocontrol are positive at the AHG phase.

WAIHA

____ auto Abs react best with LISS, PEG, and enzymes.

warm

for WAIHA, to determine if DAT is + due to an allo Ab, auto Ab, or drug induced, perform ____ studies.

elution

____ elution refers to the suspension of autologous cells in albumin or saline with constant agitation of the sample at 56C water bath for approximately 10min.

mechanical

what is the common reagent used for elution procedure for WAIHA?

ZZAP

what reagent that contains Dithiothreitol (DTT), a sulfhydryl reagent, and proteolytic enzyme (e.g., ficin or papain)?

ZZAP

what reagent dissociates both IgG and C’ from RBCs so phenotyping is allowed to be accomplished and can enhance adsorption properties of the RBC?

ZZAP

a warm auto adsorption can be performed if the patient was transfused over three months ago. true or false?

true

____ adsorption for WAIHA should be performed under these conditions:

• if patient’s RBCs are not available for auto adsorption

• if the patient has been transfused recently

• if the patient’s pre-transfusion RBCs are not available for auto adsorption

allogenic

the following cells are used for ____ adsorption for WAIHA:

• R1R1

• R2R2

• rr

• one cell K negative

• one cell Jka negative

• another cell Jkb negative

• use ZZAP to denature other important antigens

allogenic

it is best to avoid transfusing a ____ individual when they are asymptomatic.

WAIHA

____ auto antibodies refers to a combination of cold and warm autoimmune Abs.

mixed

the DAT for ____ auto antibodies will detect both IgG and C3d.

mixed

for drug induced immune hemolytic anemia, a classic result for eluate testing is no reactivity is seen. true or false?

true

the following are the 4 theoretical mechanisms for ____ immune hemolytic anemia:

• drug adsorption (HAPTEN)

• immune complex formation (innocent bystander)

• membrane modification (nonimmunologic protein adsorption) NON IMMUNE = STICKY

• autoantibody production (WAIHA-like)

drug induced

drug induced hemolytic anemia by drug adsorption may be caused by the drug ____

• drug + RBCs → drug-RBC → stimulates production of anti-drug-RBC

• anti-drug-drug-RBC complex + C’ → lysis

• DAT is often negative (Abs are IgM)

penicillin

drug induced hemolytic anemia by immune complex (innocent bystander) may be caused by the drug ____

• antibody to drug + drug → complex + C’ → RBC lysis

• positive DAT with polyspecific AHG

quinidine

drug induced hemolytic anemia by membrane modification (nonimmune protein adsorption) may be caused by the drug ____

• drug + RBC → membrane changes → nonspecific adsorption of proteins to RBCs (sensitized) → RBCs destroyed by spleen

• positive DAT

cephalosporins

drug induced hemolytic anemia by auto IgG Ab formation (mimics WAIHA) may be caused by the drug ____

• certain prescription drugs can cause the formation of a warm auto Ab

• mechanism is unknown

• other drugs may alter T cell function

• positive DAT (polyspecific and IgG)

aldomet

the following describes the mechanism of ____ for drug induced hemolytic anemia:

• drug adsorbed onto RBC in vivo and becomes a hapten

• if patient develops Abs to penicillin, they will bind to penicillin on RBC

• hemolysis occurs extravascularly

drug adsorption

the following drugs are ____ forming:

• penicillin

• methicillin

• nafcillin

• tetracycline

• erythromycin

• cephalothin

• carbromal

• cefazolin

• cefamandole

hapten

the following describes ____ formation:

• drug binds with Ab in vivo in the plasma

• → immune drug Ab complex binds to RBC and initiates C’ activation

• → leads to intravascular hemolysis

• → after RBC hemolyzed, drug antibody complex dissociates and attaches to new RBC

• → small amounts of drug can result in acute hemolysis

immune complex

____ (synthetic penicillin), second- and third- generation cephalosporins, and anti-ceftriaxone have been implicated.

piperacillin

the following are the classic drugs that cause ____ formation:

• quinine

• quinidine

• cephalosporins

immune complex

the following are other drugs that cause ____ formation:

• acetaminophen

• hydralazine

• methotrexate

immune complex

which drug induced hemolytic anemia mechanism is associated with the following:

• the modification causes RBC membrane to adsorb proteins (albumin IgA, IgG, IgM, and C’ components) in a non-specific and non-immunologic manner

  • RBC membrane becomes sticky

• eluates are nonreactive because there is no drug Ab present

membrane modification

the follow drugs (anti-neoplastic agents) may cause ____ of RBCs:

• cephalothin

• cisplatin

• diglycoaldehyde

• suramin

membrane modification

what drug refers to an anti cancer drug (monoclonal Ab)?

daratumumab

what drug refers to an anti-hypertensive drug that interferes with suppressor T cell function that leads to elaboration of auto Abs by B cells?

alpha methyldopa

what is another term for alpha methyldopa?

aldomet

levodopa is an anti-____ drug.

hypertensive

what drug refers to a cardiac arrhythmic drug?

procainamide

what drug refers to a nonsteroidal anti-inflammatory drug?

mefenamic acid

what drug alters the function of T suppressor cells, thereby, upsetting the immune system and allowing the production of auto antibodies?

aldomet

quinidine and phenacetin are associated with which mechanism that leads to the development of drug related antibodies?

immune complex

quinidine and phenacetin are associated with which Ig class?

IgM or IgG

penicillins, streptomycin, and cephalosporin are associated with which mechanism that leads to the development of drug related antibodies?

drug adsorption

penicillins, streptomycin, and cephalosporin are associated with which Ig class?

IgG

cephalosporins (alone) are associated with which mechanism that leads to the development of drug related antibodies?

membrane modification

cephalosporins are associated with which Ig class?

many plasma proteins

aldomet isassociated with which mechanism that leads to the development of drug related antibodies?

methyldopa induced

aldomet is associated with which Ig class?

IgG

which mechanism that leads to the development of drug related antibodies have a positive eluate result due to warm auto antibodies identical to those found in WAIHA?

methyldopa induced

which mechanism that leads to the development of drug related antibodies often causes acute intravascular hemolysis?

immune complex

which mechanism that leads to the development of drug related antibodies often causes extravascular hemolysis?

drug adsorption

which mechanism that leads to the development of drug related antibodies develops no hemolysis?

membrane modification

which mechanism that leads to the development of drug related antibodies have 0-8% of patients develop a hemolytic anemia that mimics a warm AIHA?

methyldopa induced