Patients with immune thrombocytopenia purpura (ITP), treat with _____
IVIG (intravenous immune globulin)
Patients with von Willebrand disease and hemophilia A, treat with ____
Desmopressin
When a rapid increase in platelet count is required in patients with ITP, what is the recommended treatment regimen?
IVIG with corticosteroid use
IVIG is purified Ig____ products manufactured from pooled ______ plasma.
IgG
Human
Conditions for which IVIG has an established therapeutic role as immunomodulation therapy:
ITP
Guillain-Barre syndrome
Myasthenia gravis
MOA of IVIG in ITP:
Not fully understood, but appears to be due to competitive inhibition of Fc receptors on phagocytic cells, creating reticuloendothelial system blockade
How is IVIG dosed?
Weight-based
Total dose can be split into doses given every day for 2-3 days
ADR for IVIG:
ADR can occur soon after infusion and can include HA, flushing, chills, myalgia, wheezing tachy cardia, lower back pain, nausea nausea and hypotension
What can prevent IVIG infusion reaction?
Premedicating patient with antihistamine or IV hydrocortisone
T/F: IVIG is given by a short IV infusion over several hours. The adult fluid volume per dose is usually in the range of 750-1500 ml
True
What is the black box warning for IVIG?
Renal failure likely due to high sucrose content
IgA deficient patient there could be anaphylaxis reactions
IVIG can be used as replacement therapy in which condition?
IgG deficiency and immunologic disease like myasthenia gravis
T/F: Desmopressin is a synthetic arginine vasopressin (ADH) derivative used in hematology because it increase serum levels of von Willebrand factor and clotting factor VIII: aka ADH
True
MOA of desmopressin?
V2 receptor Stimulator on endothelial cells causes cell to release vWF
Increase water absorption
Besides desmopressin, what are other treatment methods for vWD and hemophilia A?
vWF concentrates containing all vWF multimers
recombinant vWF
Route of administration for desmopressin?
Nasally
IV
Desmopressin ____ water absorption, and its affect on renal is_____
Increase
Hyponatremia, with CNS effects
T/F: desmopressin can cause hyponatremia with CNS adverse reactions
True; this drug has a high risk of decreasing serum osmolarity
How to prevent desmopressin hyponatremia?
fluid restriction. Fluid intake should be limited to a minimum from one hour before administration until at least 24 hours after administration
T/F: Desmopressin nasal spray can treat hemophilia A and B but injection only treats hemophilia A
False; both only treat hemophilia A, no affect on hemophilia B no matter the route.
This is because desmopressin causes increased release of vWF which plays a role in stabilizing factor VIII not IX, which is seen in hemophilia B
T/F: desmopressin will not increase the Factor VIII activity high enough to treat bleeding episodes seen in SEVERE hemophilia A
True
High does desmopressin is used to treat _______, while lower dose of desmopressin is used for _____
High- hematological : vWD and hemophilia A
Lower dose – ADH replacement seen in diabetes insipidous
