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1.20 Hemolytic Anemia IIb
By Dr. Jetly
57
Pathology
Professional
01/21/2012

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Term
What causes TTP?
Definition
deficient activity of vWF-cleaving proteinase (ADAMTS-13)
Term
What does ADAMTS-13 stand for?
Definition
A Disintegrin And Metalloproteinase with Thrombospondin-like domains
Term
What enhances TTP?
Definition
other factors that damage endothelial cells (like ticlopidine, clopidogrel, cyclosporine, oral contraceptives, hypertension, and postpartum)
Term
What happens in TTP?
Definition
fibrin and platelet microthrombi form in the microcirculation throughout the body particularly the kidneys and brain
Term
What is the typical patient population of TTP?
Definition
may occur at any age but is more common in the third decade and 60% is in females
Term
What is the clinical presentation of TTP?
Definition
clinical pentad: MAHA, thrombocytopenia, renal abnormalities, neurologic changes, fever
Term
T/F Most pts with TTP often present with the full clinical pentad.
Definition
false
Term
What causes TTP?
Definition
most cases have no known cause. Some related to HIV, drugs (cyclosporine A and clopidogrel) and hormone replacement therapy. Rarely, familial low levels of ADAMTS-13. Commonly non-familial IgG inhibitor
Term
What causes TTP?
Definition
most cases have no known cause. Some related to HIV, drugs (cyclosporine A and clopidogrel) and hormone replacement therapy. Rarely, familial low levels of ADAMTS-13. Commonly non-familial IgG inhibitor
Term
What is the urinalysis finding of TTP?
Definition
proteinuria
Term
How do you treat TTP?
Definition
plasma infusion and exchange
Term
What is the prognosis of TTP?
Definition
suvival is 80-90% with early diagnosis and treatment with plasma infusion and exchange. 1/3 of survivors have relapse within 10 years, becoming chronic. Mortality rate is 10-20%
Term
What is HUS characterized by?
Definition
acute renal failure (uremia), MAHA, fever, thrombocytopenia
Term
In children, HUS often follows what event?
Definition
a prodromal infectious disease, usually diarrhea (90%)
Term
What causes HUS?
Definition
toxins produced by E coli serotype 0157:H7, or shiga toxins produced by Shigella dysenteriae
Term
How does Shiga toxin cause HUS?
Definition
toxin causes glomerular epithelial cells to release large multimer vWF molecules
Term
What are the urinalysis findings of a pt with HUS?
Definition
uremia
Term
How do you treat HUS?
Definition
dialysis, transfusions, and supportive care
Term
What is the prognosis of HUS?
Definition
with tx reduced mort rate from 50% to 5-10%; residual renal impairment of some degree in 50% of pts
Term
What is DIC?
Definition
activation of the coagulation and fibrinolytic systems; simultaneous formation and destruction of clotting factors that is always a response to an underlying process
Term
What conditions cause DIC?
Definition
severe infections, complications of pregnancy, malignancies, massive tissue trauma, hemorrhagic shock, burns, severe liver disease
Term
What types of pregnancy complications can cause DIC?
Definition
amniotic fluid embolism, premature separation of the placenta, septic abortion, retained dead fetus, retaind products of conception
Term
How do you treat HUS vs. TTP vs. DIC?
Definition
HUS= supportive; TTP= plasmaphoresis, steroids; heparin and blood comp
Term
What percent of patients with severe eclampsia develop HELLP syndrome?
Definition
4-12%
Term
What is the MC presenting symptom of HELLP syndrome?
Definition
RUQ abdominal pain
Term
What is the prognosis of HELLP syndrome.
Definition
most recover within a few days
Term
What are complications of HELLP syndrome?
Definition
hepatic rupture, seizures, blindness, and DIC. Small subset develop severe persistent multisystem disease that requires palsma exchange
Term
What is the criteria for the dx of HELLP syndrome?
Definition
hemolysis (increased LD >600, increased bilirubin),elevated liver enzymes (increased ALT>=72), thrombocytopenia (<100,000)
Term
What abs cause warm autoimmune?
Definition
IgG, anti-Rh
Term
Warm autoimmune hemolytic anemia is optimally active at what temp?
Definition
37C
Term
What other diseases are associated with warm AI hemolytic anemia?
Definition
collagen vascular diasease (most common cause is SLE), lymphoma, drugs
Term
At what temperature is cold AI hemolytic anemia optimally active?
Definition
4C
Term
What antibodies are involved in cold autoimmune?
Definition
IgM, anti-I,i
Term
What diseases are associated with cold AI hemolytic anemia?
Definition
viral and mycoplasma
Term
What is paroxysmal cold hemoglobinuria?
Definition
rare subtype of cold autoimmune hemolytic anemia involving a biphasic IgG, anti-P
Term
What happens to IgG coated RBCs?
Definition
phagocytosed by splenic macrophages (extravascular hemolysis). May see some complement mediated intravascular hemolysis; spherocytes are produced
Term
What happens to IgM coated RBCs?
Definition
extravascular (mainly liver) or intravascular depending on degree of complement activation. RBC agglutination
Term
Describe the PBS of warm IgG AI hemolytic anemia.
Definition
microspherocytes, normocytic to microcytic anemia, reticulocytosis
Term
What CBC values are thrown off by RBC agglutinations d/t cold IgM type hemolytic anemia?
Definition
falsely reduced RBC count, inaccurate Hct, spurious marked elevations in MCV and MCHC
Term
How do you dx AI hemolytic anemia?
Definition
positive direct antihuman globulin test (DAT) aka direct Coomb's test which detects antibodies attached to the pt's RBCs
Term
How does DAT work?
Definition
polyspecific reagent detects RBC coated with IgG or complement C3d. If positive then monospecific reagents (anti-IgG only, anti-IgG + C3d, anti C3d) used
Term
What are the symptoms of AI hemolytic anemia?
Definition
varies in severity and often waxes and wanes; jaundice, hepatosplenomegally, raynaud's phenomenon (in cold types of AIHA),
Term
What is Evans Syndrome?
Definition
AIHA in associated with idiopathic thrombocytopenic purpura
Term
How do you treat AIHA?
Definition
tx underlying disease, discontinue offending drugs, corticosteroids (prednisone)
Term
Lymphoma associatedcold anti RBC antibodies =
Definition
IgM agains anti-i
Term
Mycoplasma associated cold AIHA antibodies=
Definition
IgM antibodies againts anti-I
Term
infectious mononucleosis + AIHA antibodies=
Definition
igM against anti-i
Term
What are the symptoms of cold AIHA?
Definition
chronic hemolytic anemia with periods of jaundice and hemoglobinuria, RBC agglutination may be associated with Raynaud's phenomenon
Term
What is the thermal amplitude test?
Definition
pts plasma reacted with reagent RBCs at different temps; autoantibodies causing agglutination at 30 degrees and above are usually clinically significant
Term
What is the donath landsteiner type antibody?
Definition
IgG, anti-P; biphasic antibody that fixes first components of complement (C1-4) at 4 degrees celcius and the remainder as temperature rises to 25 to 37 degrees celsius. Original description was in the setting of syphilis. Currently most common in children with viral infections (measles, mumps, chicken pox, infectious mono
Term
What is the donath landsteiner test?
Definition
pts serum incubated with test rbcs at 4 degrees for 30 min then at 37 degrees for 30 min and observed for hemolysis. If biphasic hemolysis is present, it is tested against panels of reagent rbcs to determine blood group specificity (usually P)
Term
What are the symptoms of paroxysmal cold hemoglobinuria
Definition
episodes of hemoglobinuria associated with cold exposure; sudden fever, chills, abdominal and back pain, hemoglobinuria, and jaundice characterized acute attacks. Resultant anemia is usually severe (Hb <5)
Term
Whatis the treatment for paroxysmal cold hemoglobinuria?
Definition
keeping the patient warm and transfusing as necessary
Term
How do drugs induce immune hemolytic anemias?
Definition
adsorption of Immune complexes to the RBC membrane (IgG or IgM), adsorption of drug to RBC membrane (IgG), induction of autoantibod by drugs (IgG), non immunologic absorption of immunoglobulins to red cell membrane
Term
What drugs form Ag-Ab complexes that bind partially to RBC membrane, partially to drug?
Definition
quinine, NSAIDS
Term
What drugs bind to RBC membrane and cause IgG antibody to be formed against the drug?
Definition
penicillin
Term
What drugs induce warm type IgG Ab against RBCs?
Definition
alpha methyldopa
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