Iv immunoglobulin and anti rh d when unresponsive to

 IV immunoglobulin and anti RH D
when unresponsive to
corticosteroids or spleenectomy or
spleenectomy isn’t an option.
 Rituximab to lyse B cell and
reduce immune recognition of
platelets.
 Effectiveness of spleenectomy is
based on 4 factors.
 Thrombopoietin receptor agonist
to increase platelet production.
 Platelet transfusion may cause
further antibody formation.
 Anti-fibrinolytic agent may be
used for severe bleeding.
 Treatment Specific: Thrombotic
thrombocytopenic purpura
 Plasmapheresis is used to
aggressively reverse platelet
consumption by supplying the
10

appropriate vWf and enzyme and
removing large vWf molecules
that bind with platelets.
 Corticosteroids
 Spleenectomy
 Platelet transfusion generally is
contraindicated because this may
lead to new vWf platelet complex
and increased clotting.
 Treatment Specific: Heparin Induced
Thrombocytopenia
 Discontinue heparin and start
direct thrombin inhibitor.
 Warfarin sould be started when
platelet is at least 150,000.
 If clotting is severe,
Plasmapheresis to clear the platelet
aggregating igG from blood,
protamine sulfate, thrombolytic
agents, or surgery to remove clots.
 Platelet transfusion is NOT
effective because it enhance
thromboembolic events.
 Never give heparin or LMWH.
 Treatment Specific: Acquired
thrombocytopenia from Decreased
Platelet Production
 If the precipitating factor is
unknown, the patient may receive
corticosteroid.
 Platelet transfusion is given if life-
threatening hemorrhage develops.
 Hemophilia: X-linked recessive disorder
caused by defective coagulation factor 8
or 9.
 Hemophilia A
 Factor 8 deficiency, recessive,
transmitted by female carriers,
displayed almost exclusively in
men.
 Most common hemophilia.
 Hemophilia B
 Factor 9 (Christmas disease)
deficiency, recessive, transmitted
by female, displayed almost
exclusively in men
 Von Willebrand Disease
 Autosomal dominant, seen in both
genders. (Recessive in severe
form).
 Deficiency of von Willebrand
coagulation protein.
 Factor 8 is bind with von
Willebrand
 Most common congenital bleeding
disorder.
 Treatment
 Prevent and treat bleeding
 Replacement therapy may be
given before surgery and dental
care.
 Desmopressin to stimulate an
increase in factor 8 and VWF. This
drug act on platelets and
endothelial cells to cause the
release of VWF which bind with
factor8, thus increasing their
concentration. IV/IN
 Anti-fibrinolytic therapy inhibits
fibrin Lysis by inhibiting
plasminogen activation in the
fibrin clot, thereby enhancing clot
stability.
 Complications
 Development of
antibodies/inhibitor for factor 8 or
9.
 Transfusion complication.
 Thrombotic episode with use of
factor 9.