Patient Care and Monitoring

1. Obtain a complete history.

2. In a patient presenting with a bleeding or clotting disorder, an initial evaluation should include bleeding time, prothrombin time (PT), activated partial throm-boplastin time (aPTT), thrombin time, and platelet count.

• Activated partial thromboplastin time: aPTT is performed by adding calcium phospholipids and kaolin to citrated blood and measures the time required for a fibrin clot to form. In this manner, aPTT measures the activity of intrinsic and common pathways. Prolongation of aPTT may be due to a deficiency or inhibitor for factors II, V, VIII, IX, X, XI, and XII. It also may be due to heparin, direct thrombin inhibitors, vitamin K deficiency, liver disease, or lupus anticoagulant.

• Prothrombin time: PT is performed by adding thromboplastin (tissue) factor and calcium to citrate-anticoagulated plasma, recalcifying the plasma, and measuring the clotting time. The major utility of PT is to measure the activity of the vitamin K-dependent factors II, VII, and X. The PT is used in evaluation of liver disease, to monitor warfarin anticoagulant effect, and to assess vitamin K deficiency.

• Thrombin time is an assessment of the time required for the appearance of the fibrin clot after thrombin is added to plasma. It may be affected by thrombin inhibitors or fibrinogen abnormalities. Most commonly, thrombin time is used to monitor fibrinolytic therapy.

• Bleeding time indicates how well platelets interact with blood vessel walls to form blood clots by assessing the length of time to arrest the bleeding after a standardized skin cut. The bleeding time is prolonged in thrombocytopenia, fibrinogen disorders, and collagen defects.

3. After a diagnosis is made, institute specific therapy.

4. Monitor resolution of laboratory and clinical symptoms with treatment.

Immunosuppressants

TTP that fails to respond adequately to PEX can be treated with immunosuppressive agents. Cytotoxic immunosuppressive therapies with potential benefit in refractory TTP include vincristine, cyclophosphamide, azathioprine, rituximab, and the CHOP

combination regimen (cyclophosphamide, doxorubicin, vincristine, and pred-33

nisone)

Outcome Evaluation

Monitor platelet counts, hemoglobin, and LDH.

Abbreviations Introduced in This Chapter

ADAMTS13 A disintegrin and metalloprptease with

DDAVP l-Desamino-8-D-arginine vasopressin

DIC Disseminated intravascular coagulation

ITP Immune thrombocytopenic purpura

IVig Intravenous immunoglobulin

PCC Prothrombin complex concentrate

RICD Recessively inherited coagulation disorders

TT I1 Th rombotic th rombo cy t open ic p urpur a wwvit

^ Self-assessment questions and answers are available at ht-tp://www. mhpharmacotherapy. com/pp.html.

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