Lec 7 & 8 Flashcards
problem with intrinsic hemolytic anemia
problem with RBC
problem with extrinsic hemolytic anemia
problem outside RBC
what % of RBC destruction is extravascular (normal conditions)
80%
what % of RBC destruction is intravascular (normal conditions)
10 to 20%
what will happen to unconjugated/indirect bilirubin during excessive extravascular hemolysis
increase
what will happen to urobilinogen during excessive extravascular hemolysis
increase
extravascular hemolysis: end product heme
bilirubin
where does extravascular hemolysis occur in the body
spleen or liver
what will be increased in urine during excessive extravascular hemolysis
urobilinogen
what is present in the blood vessels when RBCs are lysed intravascularly.
free Hb
what does free Hb cause in the urine
hemoglobinuria
T or F: free Hb is not toxic
F
what happens to the kidney when free Hb goes there
it loses iron
what blood protein binds to free Hb
haptoglobin
haptoglobin/hemopexin ____ when intravascular hemolysis increases
decreases
what pathological processes can cause an increase in intravascular hemolysis
sickle cell disease, hemolytic transfusion rxns, and sepsis
what causes gallstones in severe anemia
the super saturation of bile
what is a symptom of hereditary spherocytosis
gallstones
def? decrease urine production
oliguria
def? no urine production
anuria
what causes methemoglobinuria in severe anemia
increased amount of methemoglobin in the blood
describe the retic count, HCT, RBC count in hemolytic anemia
reticulocyteosis, decreased HCT, decreased RBC count
what happens to LDH and HbA1C values during accelerated RBC destruction
increased
what is another name for intravascular hemolysis
fragmentation
what is another name for extravascular hemolysis
macrophage mediated hemolysis
what test help differentiate between intra and extravascular hemolysis
- haptoglobin
- free hb
what is seen on the PBS of intravascular hemolysis
schistocytes
what is seen on the PBS of extravascular hemolysis
spherocytes
disease?
- polychromasia
- spherocytes
- nRBCs
hemolytic anemia
what will hemolytic anemia cause the BM to look like
erythroid hyperplasia
what are the RBC ezymopathies of hemolysic anemia
G6PD def and PK def
what type of hemolytic anemia is hereditary spherocytosis (HS)
chronic
how are spherocytes formed
vertical mmb protein def
how does hereditary spherocytosis get inherited
autosomal dominant
what RBC mmb protein are involved with vertical mmb interactions
- spectrin
- ankyrin
- band 3
what are the clinical features of HS
anemia, jaundice, splenomegaly, and gallstones
HS: ____ MCHC, ____ retic count, direct antiglobin test _____, _____ haptoglobin, ____ LDH, and ____ unconjugated bilirubin test
increased, increased, neg, low, high, increased
immune hemolytic anemia will always have a ____ direct antiglobin test
pos
treatment for HS
splenectomy or blood transfusion
what happens to osmotic fragility in HS
increased
what happens to target cells osmotic fragility
decreased
when spherocytes are present EMA will be _____ because _______ mmb proteins are mutated
decreased, vertical
how is hereditary elliptocytosis (HE) inherited
autosomal dominant
what is the severe form of HE called
hereditary pyropoikilocytosis (HPP)
how are elliptocytes formed
horizontal mmb proteins are mutated
what are the horizontal RBC mmb proteins
cytoskeleton, alpha-spectrin, beta-spectrin, or protein 4.1
symptoms of HE
most people are asymptomatic
how can you tell if someone is autosomal homozygous for HE
100% elliptocytes in PBS
how is HPP inherited
autosomal recessive
what other disease has a similar PBS to HPP
thermal burns
why will MCV be low in HPP
RBC fragments present
RBC from HPP pt will be very sensitive to what
heat
how are stomatocytes formed
defect with pump on RBC mmb
how is hereditary stomatocytosis inherited
autosomal dominant
is paroxysmal nocturnal hemoglobinuria acquired or inherited
acquired
what gene is mutated with paroxysmal nocturnal hemoglobinuria
genes that code for CD55 and CD59
what is the normal function of CD55 and CD59
protect RBC against compliment system
what color urine of paroxysmal nocturnal hemoglobinuria
dark
RBC phenotype of paroxysmal nocturnal hemoglobinuria: normal marker level, little or no complement-mediated hemolysis
RBC I
RBC phenotype of paroxysmal nocturnal hemoglobinuria: partial def of CD55 and CD59, and these cells are relatively resistant to complement-mediated hemolysis
RBC II
RBC phenotype of paroxysmal nocturnal hemoglobinuria: no CD55 and CD59 proteins, highly sensitive to spontaneous lysis by complement
RBC III
how is G6PD def inherited
X linked
what class of G6PD def is the most severe
I
common PBS finding in G6PD def
heinz body
what stain is used for Heinz bodies
supra vital stain
what clinical syndromes cause G6PD def (3)
anti-malarial drugs, infection, fava bean
hemolytic anemia will be ____cytic and ____chromic
normo; normo
what RBC morphology will confirm G6PD def
Heinz bodies
what will the retic count be in G6PD def
very high
what is the function of PK
generates 1 mol of ATP in RBC glycolysis
what happens when people are PK def
their RBCs can not produce enough ATP
what will be seen on the PBS of a pt with PK def
burr cells
what kind of anemia does a PK def cause
chronic hemolytic anemia
how is PK def inherited
autosomal recessive
what is the 2nd most common cause of enzyme-def hemolytic anemia
PK def
what cell is seen in MAHA PBS
schistocytes
what kind of hemolysis is involved in MAHA
mostly extravascular
what are the associated disorders with MAHA
- TTP
- HUS
- DIC
- HELLP
what is the problem with TTP
decrease in plt count
what enzyme is mutated in TTP
ADAMTS13
what is the normal funtion of ADAMTS13
Degrades von willebrand factor
what is the best best treatment for TTP
plasma exchange
what causes HUS
bacterial infection
what is another name for HUS
Hamburger disease
what type of people are most likely to get HUS
children
what is produced in HUS that destroys cells
Shiga toxin
what will be seen in the PBS of HUS
- RBCs fragments
- throbytopenia
what organ is effected in HUS
kidney
what kind of microorganisms is malaria
intracellular
what is the vector of malaria
female anopheles mosquito
what type of malaria lifecycle happens in humans
asexual
(malaria) when the mosquito 1st bites you where does the parasite go
liver
what stage of malaria is in the liver cells
schizont
how do mosquitos get infected with malaria
eats blood with gametocytes
how many blood smears are made for malaria testing
2 (thin and thick)
what type of microorganism is babesia
intracellular
what is the vector of babesia
ixoodes scapularies (tick)
what lab test is used to diagnosis babesia
indirect immunofluorescent Ab assay
what are the 3 types of autoimmune hemolytic anemia
- warm- reactive ab
- cold-reactive ab
- PCH
what is another name for DAT test
direct Coombs test
t or f: warm autoimmune hemolytic anemia is non spherocytic hemolytic anemia
t
what will be seen on the PBS of warm autoimmune hemolytic anemia
- polychromasia
- spherocytes
what temp do warm reactive ab react at
body temp
what type of ab is involved in warm autoimmune hemolytic anemia
IgG
is autoimmune hemolytic anemia DAT pos or neg
pos
what type of hemolysis occurs in autoimmune hemolytic anemia
extravascular
cold autoimmune hemolytic anemia: MCV falsely ___, WBC falsely ___, and RBC falsely ____
high, high, low
what type of cold reactive ab are present in cold autoimmune hemolytic anemia
- monoclonal cold agglutinins
- IgM
autoimmune hemolytic anemia: when does IgM bind to RBCs
during cold temps
what do IgM autoantibodies activate
complement
autoimmune hemolytic anemia: when does IgM ab dissociate
when RBC return to central circulation
autoimmune hemolytic anemia: what will still remain on RBC once IgM ab dissociates
C3b
what does DAT detect
C3b
what type of hemolysis happens in autoimmune hemolytic anemia
extravascular
autoimmune hemolytic anemia: ___ titer at 40 C
high
what is seen on the PBS of autoimmune hemolytic anemia
agglutination
what must be done to a blood sample that is being tested for autoimmune hemolytic anemia
pre warmed
def? acute form of cold reactive hemolytic anemia
paroxysmal cold hemoglobinuria
paroxysmal cold hemoglobinuria is a secondary condition to what
viral infections in kids
what ab is involved in paroxysmal cold hemoglobinuria
anti-P autoantibody
what ab is biphasic
anti-P autoantibody.
when does P ab bind to P ag
cold temps
t or F: when P ab binds to P ag at cold temps it completely activates complement
F
when is complement completely activated in paroxysmal cold hemoglobinuria
when anti- P autoantibody goes to body core
what is another name for anti-p autoantibody
donath-landsteiner ab
what is seen on the PBS of paroxysmal cold hemoglobinuria
erythrophagocytosis
def? a small molecule which, when combined with a larger carrier such as a protein, can elicit the production of antibodies which bind specifically to it
hapten
what kind of drug is penicillin
hapten
what type of drug-induced hemolytic anemia: drug binds to RBC mmb –> ab binds to drug –> RBC is lysed
hapten mechanism
what type of drug-induced hemolytic anemia: combo of drug and Ab causing RBC to lyse
autoimmune mechanism
what is the common autoimmune mechanism drug
methyldopa (hypertension drug)
what type of drug-induced hemolytic anemia: drug changes the structure of protein –> makes a new Ag –> that ag bind to RBC —> RBC lyses
immune complex mechanism
what Is a the common immune complex mechanism drug
anti-malaria drugs
if a hemolytic transfusion rxn is immediate what type of ab is present
IgM
if a hemolytic transfusion rxn is delayed what type of ab is present
IgG
what type of ab is Rh ab
IgG
t or f: Hb doesn’t rise in delayed hemolytic transfusion rxn
T
in HDN the mom will be Rh ___ and the baby will be Rh ____
neg , pos
in HDN what kind of sample will be taken to test for Ab D
serum
what drug is given to moms to prevent HDN
RhoGAM
HDN: high titer signals
Ab D is present
what test is used to test for HDN
kleihauer-betke acid elution
kleihauer-betke acid elution: fetal cells will be ____ color because they contain Hb ___
pink, F
