Term
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Definition
| deficient activity of vWF-cleaving proteinase (ADAMTS-13) |
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Term
| What does ADAMTS-13 stand for? |
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Definition
| A Disintegrin And Metalloproteinase with Thrombospondin-like domains |
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Term
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Definition
| other factors that damage endothelial cells (like ticlopidine, clopidogrel, cyclosporine, oral contraceptives, hypertension, and postpartum) |
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Term
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Definition
| fibrin and platelet microthrombi form in the microcirculation throughout the body particularly the kidneys and brain |
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Term
| What is the typical patient population of TTP? |
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Definition
| may occur at any age but is more common in the third decade and 60% is in females |
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Term
| What is the clinical presentation of TTP? |
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Definition
| clinical pentad: MAHA, thrombocytopenia, renal abnormalities, neurologic changes, fever |
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Term
| T/F Most pts with TTP often present with the full clinical pentad. |
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Definition
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Term
|
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 |
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Term
|
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 |
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Term
| What is the urinalysis finding of TTP? |
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Definition
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Term
|
Definition
| plasma infusion and exchange |
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Term
| What is the prognosis of TTP? |
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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% |
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Term
| What is HUS characterized by? |
|
Definition
| acute renal failure (uremia), MAHA, fever, thrombocytopenia |
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Term
| In children, HUS often follows what event? |
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Definition
| a prodromal infectious disease, usually diarrhea (90%) |
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Term
|
Definition
| toxins produced by E coli serotype 0157:H7, or shiga toxins produced by Shigella dysenteriae |
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Term
| How does Shiga toxin cause HUS? |
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Definition
| toxin causes glomerular epithelial cells to release large multimer vWF molecules |
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Term
| What are the urinalysis findings of a pt with HUS? |
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Definition
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Term
|
Definition
| dialysis, transfusions, and supportive care |
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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 |
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Term
|
Definition
| activation of the coagulation and fibrinolytic systems; simultaneous formation and destruction of clotting factors that is always a response to an underlying process |
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Term
| What conditions cause DIC? |
|
Definition
| severe infections, complications of pregnancy, malignancies, massive tissue trauma, hemorrhagic shock, burns, severe liver disease |
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Term
| What types of pregnancy complications can cause DIC? |
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Definition
| amniotic fluid embolism, premature separation of the placenta, septic abortion, retained dead fetus, retaind products of conception |
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Term
| How do you treat HUS vs. TTP vs. DIC? |
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Definition
| HUS= supportive; TTP= plasmaphoresis, steroids; heparin and blood comp |
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Term
| What percent of patients with severe eclampsia develop HELLP syndrome? |
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Definition
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|
Term
| What is the MC presenting symptom of HELLP syndrome? |
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Definition
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|
Term
| What is the prognosis of HELLP syndrome. |
|
Definition
| most recover within a few days |
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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 |
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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) |
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|
Term
| What abs cause warm autoimmune? |
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Definition
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Term
| Warm autoimmune hemolytic anemia is optimally active at what temp? |
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Definition
|
|
Term
| What other diseases are associated with warm AI hemolytic anemia? |
|
Definition
| collagen vascular diasease (most common cause is SLE), lymphoma, drugs |
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Term
| At what temperature is cold AI hemolytic anemia optimally active? |
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Definition
|
|
Term
| What antibodies are involved in cold autoimmune? |
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Definition
|
|
Term
| What diseases are associated with cold AI hemolytic anemia? |
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Definition
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Term
| What is paroxysmal cold hemoglobinuria? |
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Definition
| rare subtype of cold autoimmune hemolytic anemia involving a biphasic IgG, anti-P |
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Term
| What happens to IgG coated RBCs? |
|
Definition
| phagocytosed by splenic macrophages (extravascular hemolysis). May see some complement mediated intravascular hemolysis; spherocytes are produced |
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Term
| What happens to IgM coated RBCs? |
|
Definition
| extravascular (mainly liver) or intravascular depending on degree of complement activation. RBC agglutination |
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|
Term
| Describe the PBS of warm IgG AI hemolytic anemia. |
|
Definition
| microspherocytes, normocytic to microcytic anemia, reticulocytosis |
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|
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 |
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|
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 |
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Term
|
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 |
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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), |
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Term
|
Definition
| AIHA in associated with idiopathic thrombocytopenic purpura |
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Term
|
Definition
| tx underlying disease, discontinue offending drugs, corticosteroids (prednisone) |
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|
Term
| Lymphoma associatedcold anti RBC antibodies = |
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Definition
|
|
Term
| Mycoplasma associated cold AIHA antibodies= |
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Definition
| IgM antibodies againts anti-I |
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|
Term
| infectious mononucleosis + AIHA antibodies= |
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Definition
|
|
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 |
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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 |
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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 |
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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) |
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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) |
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|
Term
| Whatis the treatment for paroxysmal cold hemoglobinuria? |
|
Definition
| keeping the patient warm and transfusing as necessary |
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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 |
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Term
| What drugs form Ag-Ab complexes that bind partially to RBC membrane, partially to drug? |
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Definition
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|
Term
| What drugs bind to RBC membrane and cause IgG antibody to be formed against the drug? |
|
Definition
|
|
Term
| What drugs induce warm type IgG Ab against RBCs? |
|
Definition
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