4.2 Disorders of Hemostasis Flashcards
Thrombocytopenia
- Low platelet levels (less than 150,000)
- Caused by suppressed platelet production or increased platelet destruction
- Platelets are fragments of megakaryocytes.
Immune Thrombocytopenic Purpura (ITP)
- Autoimmune disease that causes antiplatelet antibodies to destroy platelets. Usually follows after a virus in children or women
Thrombotic Thrombocytopenic Purpura (TTP)
- Rarest thrombocytopenia.
- Hemolytic anemia associated with thrombocytopenia
- Also have neurological issues (fever, malaise, renal disease)
Heparin-Induced Thrombocytopenia and Thrombosis Syndrome (HITTS)
- Risk is lower with low molecular heparin
- Platelets drop around 30% a day which eventually leads to formation of thrombus (increasing risk of DVT, stroke, and cardiac issues)
Heparin Antitode
- Protamine Sulfate
- Reverses anticoagulation of heparin but not development of HITTS (thrombus formation)
Manifestations and Diagnostics of Thrombocytopenia
Manifestations
- Bleeding, hemorrhage, dizziness, weakness
Diagnostics
- Decreased platelets
- Prolonged bleeding
- Coagulation studies are usually normal
- Hep B/C screening (they can cause thrombocytopenia)
Bone marrow aspiration
- If platelet destruction is the cause of thrombocytopenia then marrow shows increased megakaryocytes as the body tries to compensate for lost platelets.
HITTS Intervention (Heparin Induced)
- Discontinue Heparin ASAP
- Begin Thrombin Inhibitor
- Do not use Lovenox or Enoxaparin
- ONCE THIS OCCURS PATIENT CAN NEVER HAVE HEPARIN AGAIN
ITP (Immune) Interventions
- Discontinue medications associated IMMEDIATELY
Steroids (Immunosuppression) - prednisone
- Blocks platelets being destroyed by antibodies
IVIG
- High dose is required and is usually costly
Anti-D (WinRho)
- Increases platelet counts
Splenectomy - Sustains normal platelets 50% of the time
Monoclonal Antibodies - May increase platelet count for up to a year
Thrombopoietin Receptor Antagonist
Platelet Infusion - Tends to be ineffective
Nursing Interventions for Thrombocytopenia
EDUCATION
- Medications, bleeding precautions, methods to decrease bleeding
- Avoid IM/SQ Injections
- Minimize invasive procedures
- Avoid using a straight razor
- Avoid hard toothbrushes
- Avoid suppositories
- Stool Softeners should be used
- Pad count during menstration
Hemophilia
- Deficiency in clotting factors in the intrinsic phase of the coagulation cascade which prevents formation of stable fibrin clots.
Clotting Cascade
- Platelets stick to site of injury which begins the cascade
- Web-like protein encircles platelets to hold them in place (platelet phase)
- Clot forms (coagulation phase)
Hemophilia A - Most common type (lack of Clotting Factor 8)
Hemophilia B (Christmas Disease) - Lack of Clotting Factor 9
Manifestations of Hemophilia
- Related to amount of clotting factor in the blood
- HALLMARK IS PROLONGED BLEEDING AND INABILITY TO FORM CLOTS
- Hemorrhages can occur even with minimal trauma
- Slow persistent bleeding from minor trauma
- Delayed bleed after minor injuries
- Uncontrollable bleed after minor irritation
- Epistaxis (nosebleeds)
- Hemarthrosis (bleeding into joint)
- BLACK TARRY STOOL
Assessments/Diagnostics/Care of Hemophilia
ASSESSMENTS
- Assess for black tarry stools
- Assess for Intracerebral hemorrhage (even small bumps can cause this)
DIAGNOSTICS
- Prothrombin Time (PT) and bleeding time is usually normal
- PTT - Prolonged
- Increased thromboplastin
- Deficient in Factor 8 (A) and Factor 9 (B)
NURSING CARE
- GOAL IS NOT TO CURE, IT IS TO REDUCE BLEEDING/INJURY
- Physical Therapy (to decrease contractures)
- Replacement of clotting factors during bleeding
- NSAID’s for pain
- Treat bleeds, avoid injections
Von Willebrand Disease
- Type of hemophilia
- Most common of inherited bleeding disorders
- Mutation of von Willebrand factor (one of the building blocks of clot formation)
- Symptoms are similar to hemophilia
Treatment
- Replacement of Factor 8
Treatment for Women
- Progesterone, Estrogen and DDAVP (synthetic vasopressin) helps stimulate von willebrand factor.
Disseminated Intravascular Coagulation (DIC)
- Abnormal clotting that initiates in overdrive. Coagulation factors all get used up for these abnormal clots so when a patient does bleed, they can’t form a stable clot
- Bleeding and clotting happen simultaneously
- Both intrinsic and extrinsic activation
AT RISK POPULATIONS
- Infection
- Massive tissue injury
- Obstetric injury
- Vascular and Circulatory Collapse
- Shock
- Transfusion Reactions
- Malignancies
Myelodysplastic Syndromes (MDS)
- Clonal disorder of myeloid stem cell which most commonly causes dysplasia of erythrocytes.
- Cancer cells prevent blood stem cells from maturing into healthy blood cells
- Manifested as macrocytic anemia
- Bone marrow is hypercellular but the cells die before reaching circulation so circulating cells is lower than normal.
Affected Cells Do Not Function Normally
- Neutrophils have diminished ability to destroy bacteria
- Platelets are not able to aggregate as well and are less adhesive
- Increases risk of infection and bleeding
Diagnosed with CBC or Bone Marrow Biopsy
- ONLY CURE IS HEMATOPOIETIC STEM CELL TRANSPLANT
Polycythemia Vera (Myeloproliferative Disorder)
Primary
- Chromosomal mutation that causes increased RBC, WBC, and Platelets
- Causes hypercoagulability which may manifest as splenomegaly or hepatomegaly
Secondary
- HCT becomes extremely elevated due to compensating for hypoxemia, COPD, or HF.
- HYPOXIA STIMULATES RBC PRODUCTION
Symptoms
- Ruddy Complexion
- Splenomegaly
- High BP
- General Pruritis
RISKS
- Thrombosis Complication
- Bleeding from dysfunctional platelets
Erythromelalgia
- Rare condition that affects feet (most common) and hands
- Intense burning in the extremities, severe redness of extremities (erythema) and increased skin temperature.
- Caused by vasodilation
Treatment for Polycythemia Vera
Phlebotomy
- Remove blood to reduce blood viscosity and deplete iron stores. This prevents excessive manufacturing of hemoglobin
Chemotherapy
- Hydroxyurea can be used to suppress marrow function and control blood counts.
- Treat underlying hypertension and hyperlipidemia to reduce risk of thrombosis
Allopurinol
- Prevents gout (painful arthritis)
Aspirin for pain
- Can also reduce risk of thrombosis
Platelet Aggregation Inhibitors
- Anagrelide to inhibit platelet aggregation
Interferon
- Used to control blood counts
