NCLEX: Hematologic and lymphatic disorders

Treatments

Treatments for hematologic and lymphatic disorders include drug therapy, transfusions, and surgery.

Drug therapy

Drugs used to treat hematologic and lymphatic disorders include:
• hematinics, which fight anemia by increasing the amount of Hb in the blood
• anticoagulants and heparin antagonists, which impede blood clotting
• hemostatics, which arrest blood flow or reduce capillary bleeding
• blood derivatives, which replace blood loss caused by diseases or surgical procedures
• thrombolytic enzymes, which treat thrombotic disorders
• vitamins, which correct deficiencies of vitamins (such as vitamin B12).

Transfusions

Transfusion procedures allow administration of a wide range of blood products. Here are some examples:
• RBC transfusions revive oxygen-starved tissues.
• Leukocyte transfusions combat infections beyond the reach of antibiotics.
• Transfusions of clotting factors, plasma, and platelets help patients with hemophilia live virtually normal lives.

RBC replacement
A patient with severe anemia or acute bleeding that drug or nutritional therapy can’t correct may require transfusion of either whole blood or packed RBCs. A whole blood transfusion replenishes both the volume and oxygen-carrying capacity of the circulatory system by increasing the mass of RBCs.

A paucity of plasma

In contrast, transfusion of packed RBCs—the most common type of transfusion—restores only the oxygen-carrying capacity of the circulatory system because 80% of the plasma has been removed before transfusion. Packed RBCs may also undergo a special washing process to remove WBCs and platelets, decreasing the chance of a reaction in patients who were previously sensitized to transfusions.

Patient preparation
• Become familiar with your facility’s policies and procedures for administering blood products.
• Explain the procedure to the patient, and make sure the patient’s medical record contains a written physician’s order and a signed consent form for the transfusion.

• Verify that the patient has an appropriate, patent peripheral or central venous access site for administration.

Some assembly required

• Assemble the necessary equipment, including a standard blood administration set with an appropriate filter. If needed, flush the venous access with normal saline. Only normal saline may be infused through the same tubing as blood components.
• Obtain the patient’s baseline vital signs, and collect the blood from the blood bank. If the blood isn’t going to be transfused, it must be returned to the blood bank within 30 minutes.
• Inspect the blood product for abnormal color, cloudiness, clots, and excess air. Match the blood to the written order.
• Verify the patient’s identity, using a two-person verification process or a one-person verification process accompanied by automated identification technology (such as bar coding), according to your facility’s policy. Involve the patient in the process if possible. Don’t start the infusion if you find any discrepancies, and notify the blood bank immediately.

Monitoring and aftercare
• Begin the infusion, check the patient’s vital signs, and monitor the patient carefully throughout the transfusion, according to your facility’s policy. Keep in mind that many transfusion reactions occur within the first 15 minutes of starting a transfusion. (See Guide to immediate transfusion reactions.)
• Complete RBC transfusions within 4 hours. If the patient needs multiple units, change the administration tubing after every second unit to help prevent infection.
• If the patient develops an adverse reaction, stop the transfusion at once and notify the physician according to facility policy. Keep the vein open with normal saline, obtain vital signs, and begin appropriate nursing interventions.

Factor replacement
I.V. infusion of deficient clotting factors is a major part of treatment of coagulation disorders. Factor replacement typically corrects clotting factor deficiencies, thereby stopping or preventing hemorrhage. The blood product used depends on the specific disorder being treated.

Cold comfort

Fresh frozen plasma, for instance, helps treat clotting disorders whose causes aren’t known, clotting factor deficiencies resulting from hepatic disease or blood dilution, consumed clotting factors secondary to disseminated intravascular coagulation (DIC), and deficiencies of clotting factors (such as factor V) for which no specific replacement product exists.

For cryin’ out loud

Administration of cryoprecipitate, which forms when fresh frozen plasma thaws slowly, helps treat von Willebrand’s disease, fibrinogen deficiencies, and factor XIII deficiencies.

Eight is really great

Factor VIII (antihemophilic factor) concentrate is the long-term treatment of choice for hemophilia A because it contains a less variable amount of factor VIII than cryoprecipitate. It’s given I.V. to hemophiliac patients who have sustained injuries. It is also used to treat von Willebrand’s disease.

Pooled assets

Prothrombin complex — which contains factors II, VII, IX, and X — is used to treat hemophilia B, severe liver disease, and acquired deficiencies of the factors it contains. However, it carries a high risk of transmitting hepatitis because it’s collected from large pools of donors.

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Patient preparation
• Become familiar with your facility’s policies and procedures for administering blood products.
• Explain the procedure to the patient.

• Assemble the necessary equipment: a standard blood administration set for giving fresh frozen plasma or prothrombin complex, a component syringe or drip set for giving cryoprecipitate, a plastic syringe for I.V. injection of factor VIII, or a plastic syringe and infusion set for I.V. infusion.
• Obtain the plasma fraction from the blood bank or pharmacy. Check the expiration date, and carefully inspect the plasma fraction for cloudiness and turbidity. If you’ll be transfusing fresh frozen plasma, administer it within 4 hours because it doesn’t contain preservatives.
• Take the patient’s vital signs. If an I.V. line isn’t in place, perform venipuncture and infuse normal saline solution at a keep-vein-open r ate.

Monitoring and aftercare
• During and after administration of clotting factors, monitor the patient for signs and symptoms of anaphylaxis, other allergic reactions, and fluid overload.
• Monitor for fever, bleeding, and increased pain or swelling at the transfusion site.
• Closely monitor the patient’s PTT.
• Alert the practitioner if adverse reactions occur or if you suspect bleeding.
• Follow your facility’s protocol for monitoring vital signs.
• Instruct the patient and his family on proper care and use of the patient’s vascular access device.

Home care instructions
The patient or his family can administer factor replacement therapy at home. If home therapy is ordered, cover these topics:
• Demonstrate correct infusion techniques to the patient and his family.
• Tell them to keep factor replacement and infusion equipment readily available and to start treatment immediately if the patient experiences bleeding.
• Teach them to watch for signs and symptoms of anaphylaxis, allergic reactions, and fluid overload. Instruct them to call the doctor immediately if such reactions occur.

Surgery

Surgical removal of the spleen is sometimes done to treat various hematologic disorders.

Splenectomy
The spleen may be removed to reduce the rate of RBC and platelet destruction or to stage Hodgkin’s disease. It’s also done as an emergency procedure to stop hemorrhage after traumatic splenic rupture.

Look Ma, no spleen

Splenectomy is the treatment of choice for such diseases as hereditary spherocytosis and chronic idiopathic thrombocytopenic purpura in patients who don’t respond to steroids or danazol therapy. Besides bleeding and infection, splenectomy can cause other complications, such as pneumonia and atelectasis.

Infection alert

Keep in mind that the spleen’s location close to the diaphragm and the need for a high abdominal incision restrict lung expansion after surgery. Also, splenectomy patients — especially children — are vulnerable to infection because of the spleen’s role in the immune response.

Patient preparation
• Explain to the patient that splenectomy involves removal of the spleen under general anesthesia. Inform him that he can lead a normal life without the spleen, although he’ll be more prone to infection.
• Obtain the results of blood studies, including coagulation tests and complete blood count (CBC), and report them to the practitioner.
• If ordered, transfuse blood to correct anemia or hemorrhagic loss. Similarly, give vitamin K to correct clotting factor deficiencies. Give pneumonia vaccine as ordered.
• Take the patient’s vital signs and perform a baseline respiratory assessment. Note signs and symptoms of respiratory tract infection, such as fever, chills, crackles, rhonchi, and cough. Notify the practitioner if you suspect such infection; he may delay surgery.
• Teach the patient coughing and deep-breathing techniques to help prevent postoperative pulmonary complications.

Monitoring and aftercare
• During the early postoperative period, check closely (especially if the patient has a bleeding disorder) for bleeding from the wound or drain and for signs of internal bleeding, such as hematuria (bloody urine) or hematochezia (bloody feces).
• Know that leukocytosis (an increased WBC count) and thrombocytosis (an increased platelet count) follow splenectomy and may persist for years. Because thrombocytosis may predispose the patient to thromboembolism, help him exercise and walk as soon as possible after surgery. Encourage him to perform coughing and deepbreathing exercises to reduce the risk of pulmonary complications.

• Watch for signs and symptoms of infection, such as fever and sore throat, and monitor the results of hematologic studies. If infection develops, give an antibiotic as prescribed.

Home care instructions
• Inform the patient that he’s at increased risk for infection, and urge him to report signs and symptoms of infection promptly.
• Teach him measures to help prevent infection, such as getting the pneumococcal pneumonia vaccine.

Nursing diagnoses

When caring for patients with hematologic disorders, you’ll typically use several nursing diagnoses. These diagnoses appear here, along with appropriate nursing interventions and rationales.

Fatigue

Related to anemia caused by decreased hematocrit (HCT) and Hb, Fatigue may be associated with sickle cell anemia, pernicious anemia, folic acid and iron deficiency anemias, aplastic or hypoplastic anemias, thalassemias, leukemia, and sideroblastic anemias.

Expected outcomes
• Patient demonstrates that he’s adequately rested by being able to participate in routine daily activities.
• Patient identifies measures to prevent or modify fatigue.
• Patient states that he has increased energy.

Nursing interventions and rationales
• Help the patient avoid unnecessary activity — for example, avoid scheduling two energy-draining procedures on the same day. Consult an occupational therapist for practical suggestions in modifying the home and work environments. Using energy-conserving techniques avoids overexertion and potential for exhaustion.
• Help the patient conserve energy through rest, planning, and setting priorities, to prevent or alleviate fatigue.

Divide and conquer

• Alternate activities with periods of rest. Encourage the patient to engage in activities that he can complete in short periods or divide into several segments. Regular rest periods help decrease fatigue and increase stamina.

• Discuss the effects of fatigue on daily living and personal goals. Explore with the patient the relationship between fatigue and his disorder, to enhance his ability to cope.
• Structure the patient’s environment to encourage compliance with the treatment regimen. For example, devise a daily schedule based on his needs and desires.
• Encourage the patient to eat foods rich in iron and minerals, unless contraindicated, to help prevent anemia and demineralization.
• Provide small, frequent feedings to conserve the patient’s energy and encourage optimal nutrition.
• Establish a regular sleeping pattern. Getting 8 to 10 hours of sleep nightly helps reduce fatigue.
• Avoid highly emotional situations, which worsen fatigue. Encourage the patient to explore feelings and emotions with a supportive counselor, clergy, or other professional to help cope with illness.

Ineffective tissue perfusion

Related to inadequate blood volume or HCT, Ineffective tissue perfusion may be associated with hemophilia, thrombocytopenia, various purpuras, DIC, and von Willebrand’s disease.

Expected outcomes
• Patient’s vital signs are within baseline values.
• Patient’s pulse oximetry value is within normal limits.

Nursing interventions and rationales
• Monitor the patient’s vital signs every 4 hours. Assess for signs and symptoms of both minor bleeding (such as bleeding gums, ecchymoses, epistaxis [nosebleed], and petechiae) and serious bleeding (such as changed mental status, headache, hematemesis [vomiting of blood], hemoptysis [coughing up of blood], hypotension, melena [black, tarry stools], orthostatic changes, and tachycardia). Detecting bleeding early helps control complications.
• Take steps to prevent bleeding. Avoid invasive measures, such as injections, rectal enemas or suppositories, and urinary catheterization. Avoid giving aspirin or aspirin-containing products if possible. Shave the patient with an electric razor only. Give oral care with a soft toothbrush. These measures prevent complications by maintaining skin integrity.

Common hematologic disorders

This section discusses common hematologic disorders, from anemias (such as aplastic anemia and sickle cell anemia) to hemorrhagic disorders (such as hemophilia and thrombocytopenia).

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Aplastic anemia

Aplastic, or hypoplastic, anemia results from injury to or destruction of stem cells in the bone marrow or the bone marrow matrix, causing pancytopenia (deficiency of RBCs, WBCs, and platelets) and bone marrow hypoplasia (underdevelopment).

Alarming mortality

Although commonly used interchangeably with other terms for bone marrow failure, aplastic anemia properly refers to pancytopenia resulting from decreased functional capacity of a hypo plastic, fatty bone marrow. Aplastic anemia with severe pancytopenia carries a mortality of 80% to 90%. Death may result from bleeding or infection.

What causes it
Aplastic anemia may result from:
• adverse drug reactions
• exposure to toxic agents, such as benzene and chloramphenicol
• radiation
• immunologic factors
• severe disease, especially hepatitis
• preleukemia and neoplastic infiltration of bone marrow
• congenital abnormalities
• induced changes in fetal development (suspected as a cause when no consistent genetic history of aplastic anemia exists).

Pathophysiology
Aplastic anemia usually develops when damaged or destroyed stem cells inhibit RBC production. Less commonly, it arises when damaged bone marrow microvasculature creates an unfavorable environment for cell growth and maturation.

What to look for
Clinical features of aplastic anemia vary with the severity of pancytopenia. They commonly develop gradually, and may include:
• pallor, ecchymoses, and petechiae
• retinal hemorrhages

• weakness and fatigue
• alterations in level of consciousness
• bibasilar crackles
• tachycardia
• gallop murmur
• fever
• oral and rectal ulcers
• sore throat
• nausea.

What tests tell you
• RBC tests usually show RBCs of normal size, shape, and color, although larger-than-normal RBCs and RBCs of varying size may be present. Total RBC count is 1 million/μl or less.
• Absolute reticulocyte count is very low.
• Serum iron level is elevated (unless bleeding occurs), but total iron-binding capacity is normal or slightly reduced. Hemosiderin (a blood protein) is present, and tissue iron storage is visible microscopically.
• Platelet and WBC counts fall. A lower platelet count is reflected in abnormal coagulation tests (bleeding time).

Tapped out

• Bone marrow biopsies taken from several sites may yield a “dry tap” or show severely hypocellular or aplastic marrow, with a varying amount of fat, fibrous tissue, or gelatinous replacement; absence of tagged iron and megakaryocytes; and depression of RBC elements.

How it’s treated
Effective treatment must eliminate identifiable causes of anemia and provide vigorous supportive measures, such as packed RBC, platelet, and human leukocyte antigen (HLA)-matched leukocyte transfusions. Even then, recovery may take months. Bone marrow transplantation is the preferred treatment of anemia stemming from severe aplasia and for patients needing constant RBC transfusions.
Patients with low WBC counts may need interventions to avoid infection. They may receive antibiotics, but prophylactic use encourages resistant strains of organisms. Patients with low Hb levels may need oxygen therapy and blood transfusion.

Stimulate, suppress, or complement

Other treatments for aplastic anemia include:
• bone marrow-stimulating agents, such as androgens (controversial)
• immunosuppressants (if the patient doesn’t respond to other therapies)

• colony-stimulating factors, which encourage growth of specific cellular components in patients who have had chemotherapy or radiation therapy; agents include granulocyte colony-stimulating factor, granulocyte-macrophage colony-stimulating factor, and erythropoietic stimulating factor
• alternative and complementary therapies to treat associated fatigue.

What to do
• If the patient’s platelet count is below 20,000/μl, take steps to prevent hemorrhage. For instance, avoid I.M. injections; suggest that the patient use an electric razor and a soft toothbrush; give humidifying oxygen, if ordered, to prevent drying of mucous membranes (dry mucosa may bleed); and promote regular bowel movements through stool softeners and a proper diet. Also, apply pressure to venipuncture sites until bleeding stops. Detect bleeding early by checking for blood in the urine and stools and assessing the skin for petechiae.
• Help prevent infection by performing hand hygiene thoroughly before entering the patient’s room, making sure the patient eats a nutritious diet high in vitamins and proteins to boost his resistance, and encouraging meticulous mouth and perianal care. Make sure routine throat, urine, and blood cultures are done regularly to check for infection.

Keep an eye on it

• Watch for life-threatening hemorrhage, infection, adverse drug reactions, and transfusion reaction.
• Schedule frequent rest periods for a patient with a low Hb level.
• Administer oxygen therapy as needed and ordered.
• If blood transfusions are given, assess for transfusion reaction by checking the patient’s temperature and monitoring him for rash, hives, itching, back pain, restlessness, and shaking chills.
• To prevent aplastic anemia, monitor blood drug levels carefully if the patient is receiving a drug that could cause anemia.
• Evaluate the patient. He should experience fewer infections, his blood cell counts should return to normal, he should breathe easily, and he should no longer experience trauma-induced hemorrhagic episodes. He and his family should demonstrate knowledge of energy-saving strategies.

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