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hemophilia A patients have a defect in
factor VIII
which hemophilia is X linked and therefore mostly affects males
hemophilia A
hemophilia B is a defect in
factor IX
one dosage of factor VIII will increase factor activity by
2%
one dosage of factor IX will increase factor activity by
1%
what are inhibitors
antibodies to factor VIII and factor IX
sickle cell mutation
glutamic acid is replaced by valine on position 6
what is ischemia
lack of oxygen to tissues
in critical cases of SCD, what therapy is used
exchange transfusion
what blood component is used to reduce the possibility of febrile non hemolytic transfusion reactions
leukoreduced packed RBCs
what is an HPC
hematopoietic progenitor cell, stem cells that will divide and eventually develop into RBCs, WBCs, and platelets
for a stem cell transplant, the donor must be
HLA compatible
all blood units to be transfused to HPC transplant patients shoudl be ___
irradiated
what does irradiation do
it kills T cells in the donor unit that might mount an immune response to the patient
granulocyte transfusions are most effective against ___ infections and less effective against ___ infections
fungal, bacterial
what is passenger lymphocyte syndrome
lymphocytes in a transplanted organ produce anti-A or anti-B and cause mild hemolysis
solid organs SHOULD be
HLA compatible
vitamin K is produced in the
liver
which factors are vitamin K dependent
II, VII, IX, and X
fibrinogen is produced in the
liver
FFP is given to patients with liver disease to correct
coagulation deficiencies
patients who cannot receive FFP are administered
factor VII
erythropoietin is produced in the
kidneys
what is the half life of factor VIII
12 hours
a physician orders an exchange transfusion for a patient with sickle cell disease. what are the anticipated benefits of this type of transfusion
decreased percentage of sickle cells
which technique reduces the risk of hyperhemolysis in sickle cell anemia patients
phenotype matched RBCs
allogeneic HPC transplants require donor and recipient to be
HLA compatible
TA-GVHD is preventable with which of the following methods
irradiation
RBC hemolysis that occurs approximately 7-14 days after transplant of an organ with minor ABO incompatibility is termed
passenger lymphocyte syndrome
which coagulation factor has the shortest half life
factor VII
the process that is used to remove circulating antibody that is causing disease is called
plasmapheresis