Platelet, Clotting, & Plasma Disorders PDF

**I. The Blood and Lymphatic System: Coagulation Disorders** - **Etiology and Pathophysiology:** - Causes of coagulation disorders: - **Trauma or vessel damage**. - **Vessel inadequacy**. - **Disturbance in platelet function or clotting factors**....

**I. The Blood and Lymphatic System: Coagulation Disorders** - **Etiology and Pathophysiology:** - Causes of coagulation disorders: - **Trauma or vessel damage**. - **Vessel inadequacy**. - **Disturbance in platelet function or clotting factors**. - **Liver disease**. - Normal blood coagulation: - **Clotting cascade** is formed by three separate chain reactions involving platelets, prothrombin, thrombin, and fibrin; tissue factor or factor III; and Factors I-XIII. - **Steps in the clotting mechanism**: - First, a hemostatic plug forms, followed by blood clotting. - Second, vasoconstriction inhibits capillary leakage. - Hematoma compression provides pressure. - The body lowers arterial blood pressure in response to these mechanisms. - Disruptions can be congenital or acquired, possibly secondary to disease or medication. - **Clinical Manifestations:** - **Skin and mucous membrane manifestations**: - **Petechiae** and **ecchymosis**. - **Epistaxis** (nosebleeds) and **gingival bleeding** (gum bleeding). - **Circulatory hypovolemia**: - **Hypotension**. - **Pallor**. - **Cool, clammy skin**. - **Tachycardia**. - **GI tract bleeding** with abdominal pain in the flank caused by internal bleeding. - **CNS involvement**: - Altered responses. - Malaise. - Loss of consciousness (LOC). - Speech changes. - **Assessment:** - **Subjective Data**: - History of bleeding after surgery or dental procedures. - Exposure to toxic or hazardous agents. - Radiation exposure. - Headache. - Extremity pain and numbness. - Use of medications such as aspirin. - **Objective Data**: - Pain on abdomen palpation, including liver and spleen tenderness or enlargement. - Skin and mucous membranes may have petechiae, ecchymosis. - Occasional hematomas. - Blood in emesis and stool. - Joint pain. - **Diagnostic Tests**: - **CBC (Complete Blood Count)**: - Low results in **RBCs, platelets, and Hgb**. - Altered coagulation time. - Bone marrow studies may show abnormal cells. - **Medical Management**: - **Correct underlying cause**. - Replacement transfusions may be ordered. - Anticoagulation therapy may be considered a possible cause of the disorder. - Examples include: Heparin, Warfarin (Coumadin), Enoxaparin (Lovenox). - Prevention and treatment of infections and complications. - **Nursing Interventions**: - Accurate reporting of signs and symptoms and nursing observations. - Monitor vital signs for **hypovolemia or hypovolemic shock**. - Move the patient gently to prevent tissue trauma. - Monitor IV infusions and transfusions. - Tapering of anticoagulation therapy as directed by the healthcare provider. **II. Bleeding Disorders** - **Thrombocytopenia** - **Etiology/Pathophysiology**: - A deficiency in the number of circulating platelets or a change in platelet function that alters coagulation. - Platelet count is reduced to fewer than **150,000 to 450,000/mm3**. - Causes: - Decreased production: Aplastic anemia, leukemia, tumors, and chemotherapy. - Decreased platelet survival: Antibody destruction (possibly autoimmune), infection, or viral invasion. - Altered platelet function/increased platelet consumption (DIC). - Platelet sequestration in the spleen. - Most common cause is thrombocytopenia purpura: - Usually idiopathic (ITP). - Acute form is common in children. - Chronic form is most common in women. - May be drug-induced. - **Clinical Manifestations**: - **Petechiae** (occur only in platelet disorders). - **Ecchymosis**. - Severity correlates with platelet count. - Significant bleeding can occur with platelet count \< 20,000/mm3. - Spontaneous bleeding can occur with platelet count \< 5,000/mm3. - **Assessment**: - **Subjective**: - Recent viral infection. - Current use of medications. - Extent of alcohol ingestion. - History of bleeding tendencies. - **Objective**: - Petechiae and ecchymosis. - Epistaxis and gingival bleeding. - Signs of increased intracranial pressure caused by cerebral hemorrhage. - GI tract bleeding: Lower GI (bright red blood), Upper GI (tarry stools/melena). - **Diagnostic Tests**: - Complete blood count: shows decreased platelets. - Peripheral blood smear: identify abnormalities in cell lines. - Bleeding time, PT, PTT, INR. - Bone marrow aspiration: identifies immature platelets or primary bone marrow abnormality. - **Medical Management**: - Corticosteroid therapy. - Intravenous gamma globulin or immunosuppressive drugs (for autoimmune suppression). - Transfusion of platelets (maintain platelets \> 50,000/mm3). - Transfusion of fresh frozen plasma (to replace clotting factors). - Plasmapheresis: removes antibodies that degrade platelets. - Splenectomy for chronic conditions (platelets are sequestered in the spleen). - **Nursing Interventions**: - Monitor medications for toxicity. - Monitor/prevent infection. - Monitor blood, plasma, and platelet infusion for reactions. - Patient teaching on the disease process and causative agents to assist with self-care. - Instruction on specific signs and symptoms as well as preventative measures. - Avoid trauma (sports with high injury risk). - Use of stool softeners and high-fiber diet. - Soft toothbrush. - Gentle nose blowing. - Importance of notifying the physician of bleeding signs and symptoms. - **Hemophilia** - **Etiology/Pathophysiology**: - Hereditary clotting factor defect characterized by a decrease or lack of clotting factors. - X-linked hereditary trait that affects mainly males (females are carriers). - Hemophilia A (most common, 85%): Deficiency of Factor VIII, which is essential for the conversion of prothrombin to thrombin. - Hemophilia B (Christmas Disease): Deficiency of Factor IX, which results in a lack of thromboplastin. - A decrease in prothrombin activators occurs due to decreased clotting factors, therefore a thrombus cannot form. - **Clinical Manifestations**: - Internal or external hemorrhage with large ecchymosis in tissues. Muscles may show deformity and joints become immobile (ankylosed). - **Hemarthrosis** (bleeding into joints- ankles, knees, and elbows) is a hallmark of severe disease. - Pain, erythema, and fever with hemarthrosis. - Excessive bleeding from small cuts and dental procedures may cause fatal hemorrhages. - **Assessment**: - **Subjective**: - Patient and family history of ecchymosis and hemorrhage. - Pain associated with joint movement. - **Objective**: - Presence of blood in subcutaneous tissues, urine, or stool. - Edematous or immobile joints. - **Diagnostic Tests**: - Deficiency or absence of factors VIII and/or IX. - Serum blood tests reveal a normal platelet count, bleeding time, prothrombin time (PT), and INR. - **Partial thromboplastin time (PTT) is prolonged**. - Hemoglobin/hematocrit may be normal or decreased depending on bleeding. - **Medical Management**: - Minimize bleeding and relieve pain. - Transfusion or administration of factors VIII, cryoprecipitate, and IX. - Prophylactic or to control hemorrhage. - Concerns over viral transmission with blood products. - Use of recombinant (genetically engineered) Factor VIII. - **Nursing Interventions**: - Control hemorrhage in emergency situations using pressure and cold. - Give reassurance to the patient. - Monitor transfusion of factor VIII concentrate. - Provide genetic counseling. - Patient teaching: - Understanding of avoiding injury and avoiding aspirin. - Understanding of emergency care: immobilization/pressure, ice, contacting the physician. - Obtain and wear a medical alert tag. - Teach avoidance of obesity. - **Von Willebrand\'s Disease** - Inherited bleeding disorder with abnormally slow coagulation and spontaneous episodes of GI bleeding, epistaxis, and gingival bleeding caused by a mild deficiency of factor VIII. - Common during pregnancy, menorrhagia, and after surgery or trauma. - Increased bleeding during times of infection, surgery, or pregnancy. - **Treatment**: Cryoprecipitate transfusion containing factor VIII, recombinant factor VIII, fibrinogen, and/or fresh plasma. Desmopressin (DDAVP) is a treatment choice for mild hemophilia. - Prognosis is usually good with early diagnosis. - **Disseminated Intravascular Coagulation (DIC)** - **Etiology/Pathophysiology**: - Grave coagulopathy resulting from overstimulation of clotting and anti-clotting processes in response to disease or injury. Mortality reaches 80-90%. - Secondary process with overstimulation of normal clotting (thrombosis) and anti-clotting (fibrinolysis) due to a primary medical process. - Massive stimulation of the clotting cascade results in clots in the microvasculature. - Clotting factors are depleted, leading to thrombosis where clots aren\'t needed, and an inability to produce clots where they are. - Microvascular clotting causes decreased perfusion in organs, which may cause end-organ damage. - Consumption of clotting factors and fibrinolysis causes stable clots to degrade and an inability to create needed clots. - Precipitating causes: - Obstetrical issues. - Neoplastic causes. - Hematological causes. - Trauma. - Other causes: acute infection/sepsis, anaphylaxis, cirrhosis, glomerulonephritis, hepatitis, purpura, shock, systemic lupus erythematosus, ASA poisoning. - **Clinical Manifestations**: - Bleeding is noted from 3 unrelated sites (may be occult or profuse): mucous membranes, venipunctures, GI and urinary tract, orifices, and lungs resulting in hemoptysis and dyspnea. - Diaphoresis with cold and mottled digits. - **Assessment**: - **Subjective**: - Bleeding complaints. - Indications of end-organ damage. - Complaints of bone and joint pain. - Complaint of visual changes. - **Objective**: - Occult blood or obvious bleeding. - Purpura on chest and abdomen. - Petechiae of skin and mucosa. - GI bleeding, abdominal tenderness. - Hematuria. - Pulmonary embolism, pulmonary edema. - Hypotension, tachycardia, decreased or absent peripheral pulses. - Restlessness, confusion, seizures, or coma may be present. - **Diagnostic Test**: - DIC panel: Prolonged PT/PTT, positive D-dimer. - Decreased fibrinogen and decreased clotting factors. - **Medical Management**: - Correct the underlying cause, stop the abnormal coagulation, and control bleeding. - Treat underlying hypoxemia, acidosis. - Treat underlying infection. - Remove inciting trigger. - Volume replacement: crystalloids. - Transfusion (PRBCs, platelets, FFP), and cryoprecipitate (concentrated fibrinogen) to replace losses. - Vitamin K promotes liver synthesis of clotting factors. - Heparin therapy (low dose) prevents thrombosis (controversial). - Aminocaproic acid: fibrinolysis inhibitor. - Anti-thrombin III: thrombosis inhibitor. - **Nursing Interventions**: - Monitor for thrombosis and fibrinolysis. - Control bleeding and report to physician. - Monitor for end-organ damage. - Monitor labs for hemoglobin, hematocrit, and clotting times. - Maintain circulating volume. - Monitor vital signs and administration of heparin, transfusion and cryoprecipitate. - Maintain urine output \> 30ml/hr. - Protect from bleeding and trauma. - Support and reassurance to patient. - Monitor in a quiet, non-stressful environment. - Padded side rails and cotton swabs for mouth care. - Use blood pressure monitoring infrequently to avoid subcutaneous bleeding. **III. Multiple Myeloma** - **Etiology/Pathophysiology**: - Malignant neoplastic disease of the bone marrow. - Neoplastic plasma cells build up and produce one or more tumors. - Tumors destroy bone. - **Monoclonal protein (M protein or paraprotein)** - Immunoglobulin produced by myeloma cells (present in blood and/or urine). - Helpful marker to monitor extent of the disease. - **Patient Population** - Older patients with back pain and elevated serum protein should be evaluated for multiple myeloma. - Over 40 years old. - Peak incidence around 65 years old. - Affects more men than women. - **Clinical Manifestations**: - Single or multiple bone marrow tumors. - Bone destruction with dissemination into lymph nodes, liver, spleen, and kidneys. - Skeletal symptoms -- ribs, spine, pelvis. - Osteolytic lesions -- skull, vertebrae, ribs. - Patient\'s complaints: Bone pain (increases with movement), 30% develop pathologic fractures. - Infection, anemia, increased bleeding. - Hypercalcemia and renal problems. - Hyperuricemia. - High protein levels. - **Assessment**: - **Subjective Data**: - Complaints of pain (especially skeletal). - Location of pain. - **Objective Data**: - Facial expressions (signs of increased pain with movement). - Ability to perform ADLs. - Increased body temperature. - Increased potential for bleeding. - Changes in characteristics of urine. - Effectiveness of medication administration. - **Diagnostic Tests**: - Radiographic studies. - Bone marrow biopsy. - Labs (urine and blood). - **Medical Management**: - Chemotherapy (Vincristine, Doxorubicin, Bendamustine). - Corticosteroids. - Immunomodulating drugs. - Proteasome inhibitors. - Analgesics. - Orthopedic supports. - Localized radiation. - May require IV fluids when hospitalized. - **Nursing Interventions and Patient Teaching**: - Pain relief. - Prevent infection. - Protect from injury. - Encourage ambulation. - Encourage hydration (Fluid intake goal: 3-4 L/day, urinary output: 1.5 to 2L/day). - Attend to psychosocial, emotional, spiritual needs. - Teach patient how to avoid traumatic bone injury and infection. - Discuss importance of hydration. - Review pain control modalities available. - Address patient's understanding of the disease. - **Prognosis**: - Dependent on: stage when diagnosed, effectiveness of medical treatment, comorbidities. - Life expectancy: - Stage I: 62 months. - Stage II: 44 months. - Stage III: 20 months. - Relapse: 9 months.