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Management of the patient with a hemorrhagic disorder is aimed at correction of thereversible defect(s), prevention of hemorrhagic episodes, prompt control of bleedingwhen it occurs, and management of the sequelae of the disease and its therapy.

Platelet Disorders Treatment modalities for platelet disorders are determined bythe type of defect. The thrombocytopenias are primarily

managed acutely with transfusions of platelets to maintainthe minimum level of 10,000 to 0,000!mm" necessary toprevent spontaneous hemorrhage. #orticosteroids are indicatedfor $T%, with titration governed by the severity of hemorrhagicsymptoms."&,"' plenectomy may be necessary inchronic $T% to prevent antiplatelet antibody production andsequestration and removal of antibodylabeled platelets."'%lasma e*change therapy combined with aspirin!dipyridamoleor corticosteroids has recently lowered the mortalityrate for patients with TT% over that previously obtained bytreatment with fresh fro+en plasma (%) infusions.-&,-' Thethrombocytopenia of is/ottldrich syndrome may bemanaged with platelet transfusions, splenectomy, or bone

marrow transplantation." Treatment of bleeding episodes in the patient with the congenitalqualitative platelet defect of 2lan+mann3s thrombastheniais usually not warranted unless hemorrhage is lifethreatening. Therapy has included periodic random platelettransfusions, which carry the ris/ of development of antiplatelet isoantibodies. 4uman leu/ocyte antigen(45)6matched platelets may be required after antibodydevelopment, to reduce the number of platelet transfusionsneeded for hemostasis. $n the absence of satisfactorily compatibleplatelets, blood volume and constituents can be maintainedwith lowantigenicity blood products. %lasmapheresisto remove circulating isoantibodies is held in reserve for cases

of severe thrombasthenia and lifethreatening bleeding.Hemophilias A and B Therapy for hemophilias and 7 is dependent upon the severityof disease, type and site of hemorrhage, and presence orabsence of inhibitors. #ommercially prepared s 8$$$ and $9comple* concentrates, desmopressin acetate, and, to a lessere*tent, cryoprecipitate and % are replacement option dose of 0 :!/g 8$$$ concentratetypically is used to raise the 8$$$ level to '0 to 100;for management of signi0 to>0 mg m5 volume. #ryoprecipitate

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has been used to treat selected patients with a. % contains all coagulation factorsin nearly normalconcentrations and may aid hemorrhage control in a patientwith mild hemophilia 7. $n the averagesi+e patient, one unitof % raises $9 levels by ";. %ostoperative bleeding in mildto moderate 9 de to 'hours for 8$$$ and ' to 10 hours for v.&& $ntranasal sprayapplication of ??8% (timate, ventis 7ehring, Cing of %russia,%) contains 1.> m5 of desmopressin per milliliter,witheach 0.1 m5 pump spray delivering a dose of 1>0 Bg. #hildrenrequire one nostril spray, and adults require two nostril spraysto achieve favorable responseD correction of bleeding occurs inaround E0; of patients with mild to moderate hemophilia and type $ v?.&E Time to pea/ levels is "0 to -0 minutes afterintravenous inFection and E0 to 10 minutes following subcutaneousor intranasal application.&&:nfortunately, ??8% is ineGective in individuals withsevere hemophilia . ??8% trial or test dose response maybe indicated prior to e*tensive surgery, to evaluate the level of drug eGect on assayed 8$$$ activity in the individual patient.??8%, thought to stimulate endogenous release of 8$$$and v from blood vessel endothelial cell storage sites, ishemostatically eGective provided that adequate plasma concentrationsare attained.'0 %rolonged use of ??8% results ine*haustion of 8$$$ storage sites and diminished hemostaticeGectD hence, anti

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HORAL HEALTH CONSIDERATIONS

%latelet de0,000!mm" required for surgical hemostasis,platelet transfusions may be required prior to dentale*tractions or other oral surgical procedures. The therapeuticallye*pected increment in platelet count from infusion of oneunit of platelets is appro*imately 10,000 to 1,000!mm". i*units of platelets are commonly infused at a time. %atients whohave received multiple transfusions may be refractory to randomdonor platelets as a result of alloimmuni+ation. These

individuals may require singledonor apheresis or leu/ocytereducedplatelets. 5ocal hemostatic measures are also important.

 The thrombasthenic patient needing dental e*tractionsmay be successfully treated with the use of hemostatic measuressuch as micro0 to 100; when singlebolus infusion is usedfor outpatient treatment. This provides greater assurance of hemorrhage control, given the problems of possible failure of factor activity to rise as high as e*pected and variable plasmahalflives of ' to 1 hours for 8$$$ and 1' to hours for $9.dditional postoperative factor maintenance may be indicatedfor e*tensive surgery. This can be accomplished by infusion of factor concentrates,??8%, cryoprecipitate, or %, dependingon the patient3s de