Apheresis - Plasmapheresis Flashcards
(37 cards)
What are the subtypes of Guillain-Barre Syndrome?
Classic is demyelinating (AIDP)
ASMAN/AMAN (axonal damage)
Miller-Fischer (ophthalmoplegia and ataxia)
What is the treatment of Guillain-Barre Syndrome?
Respiratory support, TPE and/or IVIG (equivalent). Steroids are not helpful.
What defines CIDP?
2+ months of chronic symptoms consistent with AIDP (motor/sensory loss).
What is the treatment of CIDP?
TPE is first-line if severe. IVIG is comparably good.
How is peripheral neuropathy with monoclonal gammopathy treated?
Because they are usually IgM-mediated, they should respond to plasma exchange. Exception: POEMS does not respond.
What serologies are seen in Myasthenia Gravis?
Most cases have an anti-AChR, some have anti-MuSK, and some are seronegative.
How is myasthenia gravis treated?
Symptomatic treatment (stigmines), immunosuppression, thymectomy (many a/w malignant thymoma), TPE or IVIG (equivalent)
How does LEMS compare to MG?
LEMS patients may often present with pain, but not diplopia/dysarthria. TPE is somewhat less effective.
What antibodies are implicated in LEMS?
Antibodies against presynaptic antigens, mostly P/Q/N voltage-gated calcium channels.
Stiff-person syndrome
Caused by antibody to Glutamic Acid Decarboxylase, maybe Gephyrin. Extremely poorly described.
MS/NMO
Not antibody-mediated, though NMO does have anti-aquaporin antibodies. NMO is better indication than MS, but reserve TPE for severe cases.
How do the microthrombi in TTP differ from those in DIC?
TTP microthrombi generally lack fibrin and inflammatory cells.
What are some causes of atypical hemolytic uremic syndrome?
Abnormalities of Factor H, Factor I, or CD46 (inborn deficiency vs acquired inihibitors)
What are some secondary causes of thrombotic microangiopathies?
Infection (incl. HIV), CVDs (SLE), drugs (ticlopidine, quinine), pregnancy…
What are the consequences of major incompatible ABO mismatched HSCT?
Hemolysis of red cells in product.
Delayed engraftment.
Pure red cell aplasia (destruction of normoblasts in product)
What are the consequences of minor incompatible ABO mismatched HSCT?
Hemolysis of patient red cells, passenger lymphocyte syndrome
How can apheresis be helpful pre-hSCT-transplant?
In major incompatibility, reduces isohemagglutinins. In minor incompatibility (RCX!), can “convert” patient’s type temporarily.
What is the role of TPE in aplastic anemia?
Basically no role (not even in ASFA), despite some cases being immune-mediated (others: drug, virus, acquired clonal)
What autoimmune hemolytic anemias should respond to TPE?
Cold hemolytic anemias moreso than warms, due to IgM more often than IgG (exception: PCH)
What is the role of TPE in ITP?
Not great; ancient and scant data. Would not recommend.
What is the role of TPE in hemophila?
Can reduce high-strength (>5 BU) inhibitors, but not really favored.
What are the three types of cryoglobulinemias?
Type I: IgG or IgM monoclonal
Type II: IgM (mono) or IgG (poly); HCV-assoc
Type III: Polyclonal, associated with infection
At what serum viscosity should patients become symptomatic?
Above 4-6x viscosity of water.
How should TPE be timed with rituximab?
Always done before rituximab, both to not remove antibody and because of temporary IgM worsening with rituximab infusion.
