Chapter 31 Hematologic Disorders

Overall goals for patients with anemia

(1) assume normal activities of daily living, (2) maintain adequate nutrition, and (3) develop no complications related to anemia.

three reasons for decreased rbc production

(1) decreased hemoglobin synthesis may lead to iron-deficiency anemia, thalassemia, and sideroblastic anemia; (2) defective deoxyribonucleic acid (DNA) synthesis in RBCs (e.g., cobalamin deficiency, folic acid deficiency) may lead to megaloblastic anemias; and (3) diminished availability of erythrocyte precursors may result in aplastic anemia and anemia of chronic disease

if an acute transfusion reaction takes place do the following steps

(1) stop the transfusion; (2) maintain a patent IV line with saline solution; (3) notify the blood bank and the health care provider immediately; (4) recheck identifying tags and numbers; (5) monitor vital signs and urine output; (6) treat symptoms per physician order; (7) save the blood bag and tubing and send them to the blood bank for examination; (8) collect required blood and urine specimens at intervals based on the hospital policy to evaluate for hemolysis; and (9) document on transfusion reaction form and patient chart. The blood bank and laboratory are responsible for identifying the type of reaction.

Anemia of Chronic Disease

(also called anemia of inflammation) can be caused by chronic inflammation, autoimmune and infectious disorders (human immunodeficiency virus [HIV], hepatitis, malaria), HF, or malignant diseases. Bleeding episodes can also contribute to anemia of chronic disease. Anemia of chronic disease is associated with an underproduction of RBCs and mild shortening of RBC survival. The RBCs are usually normocytic, normochromic, and hypoproliferative. The anemia is usually mild, but it can become more severe if the underlying disorder is not treated. This type of anemia, which usually develops after 1 to 2 months of disease activity, has an immune basis. The cytokines released in these conditions, particularly interleukin 6 (IL-6), cause an increased uptake and retention of iron within macrophages (see Fig. 30-3). This leads to a diversion of iron from circulation into storage sites with subsequent limited iron available for erythropoiesis. There is also reduced RBC life span, suppressed production of erythropoietin, and an ineffective bone marrow response to erythropoietin.

Thrombo patient education

1. Notify your health care provider of any manifestations of bleeding. These include the following: • Black, tarry, or bloody bowel movements • Black or bloody vomit, sputum, or urine • Bruising or small red or purple spots on the skin • Bleeding from the mouth or anywhere in the body • Headache or changes in how well you can see • Difficulty talking, sudden weakness of an arm or leg, confusion 2. Ask your health care provider regarding restrictions in your normal activities, such as vigorous exercise, lifting weights. Generally, walking can be done safely and should be done with sturdy shoes or slippers. If you are weak and at risk for falling, get help or supervision when getting out of bed or chair. 3. Do not blow your nose forcefully; gently pat it with a tissue if needed. For a nosebleed, keep your head up and apply firm pressure to the nostrils and bridge of your nose. If bleeding continues, place an ice bag over the bridge of your nose and the nape of your neck. If you are unable to stop a nosebleed after 10 min, call your health care provider. 4. Do not bend down with your head lower than your waist. 5. Prevent constipation by drinking plenty of fluids. Do not strain when having a bowel movement. Your health care provider may prescribe a stool softener. Do not use a suppository, an enema, or a rectal thermometer without the permission of your health care provider. 6. Shave only with an electric razor; do not use blades. 7. Do not pluck your eyebrows or other body hair. 8. Do not puncture your skin, such as getting tattoos or body piercing. 9. Avoid using any medication that can prolong bleeding, such as aspirin. Other medications and herbs can have similar effects. If you are unsure about any medication, ask your health care provider or pharmacist about it in relation to your thrombocytopenia. 10. Use a soft-bristle toothbrush to prevent injuring the gums. Flossing is usually safe if it is done gently using the thin tape floss. Do not use alcohol-based mouthwashes, since they can dry your gums and increase bleeding. 11. Women who are menstruating should keep track of the number of pads that are used per day. When you start using more pads per day than usual or bleed more days, notify your health care provider. Do not use tampons; use sanitary pads only. 12. Ask your health care provider before you have any invasive procedures done, such as a dental cleaning, manicure, or pedicure.

Neutropenia teachining guide

1. WASH YOUR HANDS frequently and make sure those around you wash their hands frequently, particularly if they help with your care. An antibacterial hand gel may also be used. 2. Notify your nurse or health care provider if you have any of the following: • Fever≥100.4°F (38°C)* • Chills or feeling hot • Redness, swelling, discharge, or new pain on or in your body • Changes in urination or bowel movements • Cough, sore throat, mouth sores, or blisters 3. If you are at home, take your temperature as directed and follow instructions on what to do if you have a fever. 4. Avoid crowds and people with colds, flu, or infections. If you are in a public area, wear a mask and use hand sanitizing gel frequently. 5. Avoid uncooked meats, seafood, or eggs and unwashed fruits and vegetables. Ask your health care provider about specific dietary guidelines for you. 6. Bathe or shower daily. A moisturizer may be used to prevent skin from drying and cracking. 7. Brush your teeth with a soft toothbrush four times daily. You may floss once daily if it does not cause excessive pain or bleeding. Avoid alcohol-based mouthwashes. 8. Do not perform gardening or clean up after pets. Feeding and petting your dog or cat are fine as long as you wash your hands well after handling.

Staging of Hodgkin's Lymphoma

31-14 Staging system for Hodgkin's lymphoma and non-Hodgkin's lymphoma. Stage I, involvement of single lymph node (e.g., cervical node). Stage II, involvement of two or more lymph nodes on one side of diaphragm. Stage III, lymph node involvement above and below the diaphragm. Stage IV, involvement outside of diaphragm (e.g., liver, bone marrow). The stage is followed by the letter A (absence) or B (presence) to indicate significant systemic symptoms (e.g., fever, night sweats, weight

Blood refusion reactions

A blood transfusion reaction is an adverse reaction to blood transfusion therapy that can range in severity from mild symptoms to a life-threatening condition. Because complications of transfusion therapy may be significant, judicious evaluation of the patient is required. Blood transfusion reactions can be classified as acute or delayed

Acute interventions for anemia

Acute interventions may include blood or blood product transfusions, drug therapy (e.g., erythropoietin, vitamin supplements), volume replacement, and O2 therapy to stabilize the patient. Dietary and lifestyle changes (described in sections on specific types of anemia) can reverse some anemias so that the patient can return to the former state of health. Assess the patient's knowledge regarding adequate nutritional intake and adherence to safety precautions to prevent falls and injury.

megaloblastic anemia care

Although the disease(pernicious anemia) cannot be prevented, early detection and treatment can lead to reversal of symptoms. Also consider signs and symptoms of other possible megaloblastic anemias and bring them to the attention of the health care provider. The nursing measures presented in Nursing Care Plan 31-1, for the patient with anemia, are appropriate for the patient with cobalamin or folic acid deficiency anemia. In addition to these measures, ensure that injuries are not sustained because of the diminished sensitivity to heat and pain resulting from the neurologic impairment. Protect the patient from falling, burns, and trauma. If heat therapy is required, evaluate the patient's skin at frequent intervals to detect redness. Ongoing care focuses on ensuring good patient adherence with treatment. Carefully assess for neurologic difficulties that were not fully corrected by adequate replacement therapy. Because the potential for gastric cancer may be increased in patients with atrophic gastritis-related pernicious anemia, the patient should have frequent and careful appropriate screenings.

Anemia

Anemia is a deficiency in the number of erythrocytes (red blood cells [RBCs]), the quantity or quality of hemoglobin, and/ or the volume of packed RBCs (hematocrit). It is a prevalent condition with many diverse causes such as blood loss, impaired production of erythrocytes, or increased destruction of erythrocytes

Stages of Chemotherapy

Chemotherapy is often divided into stages: induction, postinduction or postremission, and maintenance.

Clinical manifestations of polycthemia

Circulatory manifestations of polycythemia vera occur because of the hypertension caused by hypervolemia and hyperviscosity. They are often the first manifestations and include subjective complaints of headache, vertigo, dizziness, tinnitus, and visual disturbances. Generalized pruritus (often exacerbated by a hot bath) may be a striking symptom and is related to histamine release from an increased number of basophils. Paresthesias and erythromelalgia (painful burning and redness of the hands and feet) may also be present. In addition, the patient may experience angina, HF, intermittent claudication, and thrombophlebitis, which may be complicated by embolization. These manifestations are caused by blood vessel distention, impaired blood flow, circulatory stasis, thrombosis, and tissue hypoxia caused by the hypervolemia and hyperviscosity. The most common serious acute complication is stroke secondary to thrombosis.

Clinical Manifestations of neutropenia

Clinical manifestations related to infection at these sites include complaints of sore throat and dysphagia, ulcerative lesions of the pharyngeal and buccal mucosa, diarrhea, rectal tenderness, vaginal itching or discharge, shortness of breath, and nonproductive cough. Seriously consider any minor complaint of pain or any other symptom by the patient, and report it to the physician immediately. These can progess very rapidly because of the patient's immune status.

Classifications of megaloblastic anemias

Cobalamin (Vitamin B12) Deficiency • Dietary deficiency • Deficiency of gastric intrinsic factor • Pernicious anemia • Gastrectomy • Intestinal malabsorption • Increased requirement • Chronic alcoholism Folic Acid Deficiency • Dietary deficiency (e.g., leafy green vegetables, citrus fruits) • Malabsorption syndromes • Drugs interfering with absorption or use of folic acid • Methotrexate • Antiseizure drugs (e.g., phenobarbital, phenytoin [Dilantin]) • Increased requirement • Alcohol abuse • Anorexia • Hemodialysis patients (folic acid lost during dialysis) Drug-Induced Suppression of DNA Synthesis • Folate antagonists • Metabolic inhibitors • Alkylating agents Inborn Errors • Defective folate metabolism • Defective transport of cobalamin Erythroleukemia

Collab Care of neutropenia

Diagnostic • History and physical examination • WBC count with differential count • WBC morphology • Hct and Hgb values • Reticulocyte and platelet count • Bone marrow aspiration or biopsy • Cultures of nose, throat, sputum, urine, stool, obvious lesions, blood (as indicated) • Chest x-ray Collaborative Therapy • Identification and removal of cause of neutropenia (if possible) • Identification of site of infection (if present) and causative organism • Antibiotic therapy* • Blood cultures drawn STAT, immediately before antibiotics • Hematopoietic growth factors (G-CSF, GM-CSF, pegfilgrastim [Neulasta]) • Strict hand washing and patient hygiene (skin and oral care) • Single-patient room, positive-pressure or high-efficiency particulate air (HEPA) filtration, depending on risk • Community isolation and home precautions if outpatient

Diagnostic Studies of NHL

Diagnostic studies used for NHL resemble those used for Hodgkin's lymphoma. However, because NHL is more often in extranodal sites, more diagnostic studies may be done, such as an MRI to rule out CNS or bone marrow infiltration, or a barium enema, upper endoscopy, or CT to visualize suspected GI involvement. Clinical staging, as described for Hodgkin's lymphoma, is used to help guide therapy (see Fig. 31-14), but establishment of the precise histologic subtype is extremely important. Lymph node excisional biopsy establishes the cell type and pattern. NHL is classified based on morphologic, genetic, immunophenotypic (cell surface antigens, CD20, CD52), and clinical features. The World Health Organization has categorized more than 30 unique types of NHL. However, clinical studies have verified that different disease categories can be divided into one of two major categories: indolent (low grade) and aggressive (high grade), which is useful, along with gene expression patterns, in determining therapy 52 (Table 31-29). Additional factors, known as the International Prognostic Index (IPI), may be considered for each subtype to help select the appropriate treatment for these patients. Factors considered may include the clinical stage, number of extranodal sites, serum LDH, WBC count, hemoglobin, and patient's age and performance status. Immunologic, cytogenetic, and molecular studies are also useful for making therapeutic decisions and assessing prognosis. Other studies might include blood tests for tumor lysis (see Chapter 16); screening for hepatitis, HIV, and H. pylori; skin biopsies; bone marrow biopsies; and lumbar punctures. The prognosis for NHL is based on the histopathology.

Collabo care for thrombocytopenia

Diagnostic • History and physical examination • Bone marrow aspiration and biopsy • CBC, including platelet count • Specific studies (see Table 31-13) Collaborative Therapy Immune Thrombocytopenic Purpura • Corticosteroids • IV immunoglobulin (IVIG) • Anti-Rho(D) • Rituximab • Splenectomy • Romiplostim (Nplate) • Eltrombopag (Promacta) • Danazol • Immunosuppressives (e.g., cyclosporine, cyclophosphamide [Cytoxan], azathioprine [Imuran], mycophenolate mofetil [CellCept]) • High-dose cyclophosphamide or combination chemotherapy • Platelet transfusions Thrombotic Thrombocytopenic Purpura • Identification and treatment of cause • Plasmapheresis (plasma exchange) • High-dose prednisone • Dextran • Chemotherapy (vincristine [Oncovin], vinblastine [Velban]) • Immunosuppressives (cyclophosphamide, rituximab [Rituxan]) • Splenectomy Heparin-lnduced Thrombocytopenia • Direct thrombin inhibitor (lepirudin [Refludan], argatroban [Acova]) • Indirect thrombin inhibitor (fondaparinux [Arixtra]) • Warfarin (Coumadin) • Plasmapheresis (plasma exchange) • Protamine sulfate • Thrombolytic agents Decreased Platelet Production • Identification and treatment or removal of cause • Corticosteroids • Platelet transfusions

Nursing Management Care

Discuss with the patient the need for diagnostic studies to identify the cause. Reassess the hemoglobin and RBC counts to evaluate the response to therapy. Emphasize adherence with dietary and drug therapy. To replenish the body's iron stores, the patient needs to take iron therapy for 2 to 3 months after the hemoglobin level returns to normal. Patients who require lifelong iron supplementation should be monitored for potential liver problems related to the iron storage.

Causes of neutropenia

Drugs* • Antitumor antibiotics (daunorubicin [Cerubidine], doxorubicin [Adriamycin]) • Alkylating agents (nitrogen mustards, busulfan [Myleran]) • Antimetabolites (methotrexate, 6-mercaptopurine [6-MP]) • Antiinflammatory drugs (phenylbutazone) • Cardiovascular drugs (captopril [Capoten], procainamide [Pronestyl]) • Diuretics (thiazides, furosemide [Lasix]) • Psychotropic and antidepressant agents (clozapine) • Miscellaneous (gold, penicillamine) • Antimicrobial agents (ganciclovir, penicillin G, amphotericin B, vancomycin, trimethoprim/sulfamethoxazole [Bactrim]) Hematologic Disorders • Idiopathic neutropenia • Congenital (cyclic neutropenia) • Aplastic anemia • Fanconi syndrome • Leukemia • Myelodysplastic syndrome Autoimmune Disorders • Systemic lupus erythematosus • Felty syndrome • Rheumatoid arthritis Infections • Viral (e.g., hepatitis, influenza, HIV, measles) • Fulminant bacterial infection (e.g., typhoid fever, miliary tuberculosis) • Parasitic • Rickettsial Others • Severe sepsis • Bone marrow infiltration (e.g., carcinoma, tuberculosis, lymphoma) • Hypersplenism (e.g., portal hypertension, Felty syndrome, storage diseases [e.g., Gaucher disease]) • Nutritional deficiencies (cobalamin, folic acid) • Transfusion reaction • Hemodialysis

ger anemeia continued

For older adults with anemia, about one third have a nutritional type of anemia (e.g., iron, folate, cobalamin). About another third have renal insufficiency and/or chronic inflammation, and the other third have anemia that is unexplained. Cobalamin (vitamin B12 ) and folate deficiency may occur in about 14% of older adults because of pernicious anemia, insufficient dietary intake, and malabsorption caused by low stomach acidity. 4 Multiple co-morbid conditions in older adults increase the likelihood of many types of anemia occurring. Clinical manifestations of anemia in older adults may include pallor, confusion, ataxia, fatigue, worsening angina, and heart failure. 2 Unfortunately, anemia may go unrecognized in older adults because these manifestations may be mistaken for normal aging changes or overlooked because of another health problem. By recognizing signs of anemia, you can play a pivotal role in health assessment and related interventions for older adults.

Objective Data

General Lethargy, apathy, general lymphadenopathy, fever Integumentary Pale skin and mucous membranes; blue, pale white, or icteric sclera; cheilitis (inflammation of the lips); poor skin turgor; brittle, spoonshaped fingernails; jaundice; petechiae; ecchymoses; nose or gingival bleeding; poor healing; dry, brittle, thinning hair Respiratory Tachypnea Cardiovascular Tachycardia, systolic murmur, dysrhythmias; postural hypotension, widened pulse pressure, bruits (especially carotid); intermittent claudication, ankle edema Gastrointestinal Hepatosplenomegaly; glossitis; beefy, red tongue; stomatitis; abdominal distention; anorexia Neurologic Headache, roaring in the ears, confusion, impaired judgment, irritability, ataxia, unsteady gait, paralysis, loss of vibration sense Possible Diagnostic Findings ↓RBCs,↓Hgb;↓Hct;↑or↓reticulocytes, MCV, serum iron, ferritin, folate, or cobalamin (vitamin B12); heme (guaiac)-positive stools; ↓serum erythropoietin level;↑or↓LDH, bilirubin, transferrin

Clinical manifestations of cobalamin deficiency

General manifestations of anemia related to cobalamin deficiency develop because of tissue hypoxia (see Table 31-3). GI manifestations include a sore, red, beefy, and shiny tongue; anorexia, nausea, and vomiting; and abdominal pain. Typical neuromuscular manifestations include weakness, paresthesias of the feet and hands, reduced vibratory and position senses, ataxia, muscle weakness, and impaired thought processes ranging from confusion to dementia. Because cobalamin deficiency-related anemia has an insidious onset, it may take several months for these manifestations to develop.

older men and hemoglobin

Healthy older men have a modest decline in hemoglobin of about 1 g/dL between ages 70 and 88 years, in part because of the decreased production of testosterone.

Nursing Mangement of Leukopenias

IMPLEMENTATION ACUTE INTERVENTION. The nursing role during acute phases of leukemia is extremely challenging because the patient has many physical and psychosocial needs. As with other forms of cancer, the diagnosis of leukemia can evoke great fear and be equated with death. It may be viewed as a hopeless, horrible disease with many painful and undesirable consequences. The treatment and prognosis of each patient with leukemia are driven by many factors, such as age and type of leukemia. Patients are not the same. Therefore it is important that you understand the patient's type of leukemia, the prognosis, the treatment plan, and the goals. By doing this, you can help the patient realize that, although the future may be uncertain, one can have a meaningful quality of life while in remission or with disease control, and that, in some cases, there is reasonable hope for cure. The family also needs help adjusting to the stress of this abrupt onset of serious illness and the losses imposed by the sick role (e.g., dependence, withdrawal, changes in role responsibilities, alterations in body image). The diagnosis of leukemia often brings with it the need to make difficult decisions at a time of profound stress for the patient and family. Patients may have co-morbid conditions that affect treatment decisions. Important nursing interventions include (1) maximizing the patient's physical functioning, (2) teaching patients that acute side effects of treatment are usually temporary, and (3) encouraging patients to discuss their quality-of-life issues. You are an important advocate in helping the patient and family understand the complexities of treatment decisions and manage the side effects and toxicities. A patient may require hospitalization or may need to temporarily relocate to an appropriate treatment center. This situation can lead a patient to feel deserted and isolated at a time when support is most needed. You have contact with a patient many hours a day and can help reverse feelings of abandonment and loneliness by balancing the demanding technical needs with a humanistic, caring approach. The needs of the patient with leukemia are best met by an interdisciplinary team (e.g., psychiatric and oncology clinical nurse specialists, case managers, dietitians, chaplains, and social workers). From a physical care perspective, you are challenged to make astute assessments and plan care to help the patient manage the severe side effects of chemotherapy. The life-threatening results of bone marrow suppression (neutropenia, thrombocytopenia, and anemia) require aggressive nursing interventions. These patients may be at risk for oncologic emergencies such as tumor lysis syndrome, DIC, and leukostasis. Additional complications of chemotherapy may affect the patient's GI tract, nutritional status, skin and mucosa, cardiopulmonary status, liver, kidneys, and neurologic system. (Nursing interventions related to chemotherapy are discussed in Chapter 16.) Review all drugs being administered, including the mechanism of action, purpose, routes of administration, usual doses, potential side effects, safe-handling considerations, and toxic effects. Assess laboratory data reflecting the effects of the drugs and sequelae of the disease. Unlike treatments for solid tumors, chemotherapy is administered to patients with leukemia even if they have severe myelosuppression, since the underlying disorder is causing the problem and will not resolve unless treated. Patient survival and comfort during aggressive chemotherapy are significantly affected by the quality of nursing care. AMBULATORY AND HOME CARE. Ongoing care for the patient with leukemia is necessary to monitor for signs and symptoms of disease control or relapse. For a patient requiring long-term or maintenance chemotherapy, the fatigue of long-term chronic disease management can become arduous and discouraging. Therefore teach the patient and caregiver to understand the importance of continued diligence in disease management and the need for follow-up care. Also teach them about the drugs, self-care measures, and when to seek medical attention. The goals of rehabilitation for long-term survivors of leukemia

Acquired Causes of aplastic anemia

Idiopathic or autoimmune • Chemical agents and toxins (e.g., benzene, insecticides, arsenic, alcohol) • Drugs (e.g., alkylating agents, antiseizure drugs, antimetabolites, antimicrobials, gold) • Radiation • Viral and bacterial infections (e.g., hepatitis, parvovirus.

Nursing Management of Acute Blood Loss

In the case of trauma it may be impossible to prevent the loss of blood. For the postoperative patient, carefully monitor the blood loss from various drainage tubes and dressings and implement appropriate actions. The nursing care for the patient with anemia resulting from acute blood loss most likely includes administration of blood products (described at the end of this chapter). The anemia should begin to correct itself once the source of hemorrhage is identified, blood loss is controlled, and fluid and blood volumes are replaced. There should be no need for longterm treatment of this type of anemia.

Causes of thrombocytopenia

Inherited • Fanconi syndrome (pancytopenia) • Hereditary thrombocytopenia Acquired Immune • Immune thrombocytopenic purpura (ITP) • Neonatal alloimmune thrombocytopenia Nonimmune • Shortened circulation (increased consumption) • Thrombotic thrombocytopenic purpura (TTP) • Disseminated intravascular coagulation (DIC) • Heparin-induced thrombocytopenia (HIT) • Splenomegaly or splenic sequestration • Turbulent blood flow (hemangiomas, abnormal cardiac valves, intraaortic balloon pumps) • Decreased production • Drug-induced marrow suppression • Chemotherapy • Viral infection (hepatitis C virus, HIV, cytomegalovirus) • Bacterial infection (sepsis) • Alcoholism, bone marrow suppression • Myelodysplastic syndrome (MDS) • Myelofibrosis • Aplastic anemia • Hematologic malignancy (leukemias, lymphomas, myeloma) • Solid tumor infiltrating bone marrow • Radiation to the bone Lewis, Sharon L.; Dirksen, Shannon Ruff; Heitkemper, Margaret M.; Bucher, Linda. Medical-Surgical Nursing: Assessment and Management of Clinical Problems, Single Volume (Page 650). Elsevier Health Sciences. Kindle Edition.

Integementary Changes with Anemia

Integumentary changes include pallor, jaundice, and pruritus. Pallor results from reduced amounts of hemoglobin and reduced blood flow to the skin. Jaundice occurs when hemolysis of RBCs results in an increased concentration of serum bilirubin. Pruritus occurs because of increased serum and skin bile salt concentrations. In addition to the skin, the sclera of the eyes and mucous membranes should be evaluated for jaundice because they reflect the integumentary changes more accurately, especially in a dark-skinned individual.

neutropenia

Leukopenia refers to a decrease in the total WBC count (granulocytes, monocytes, and lymphocytes). Granulocytopenia is a deficiency of granulocytes, which include neutrophils, eosinophils, and basophils. The neutrophilic granulocytes, which play a major role in phagocytizing pathogenic microbes, are closely monitored in clinical practice as an indicator of a patient's risk for infection. A reduction in neutrophils is termed neutropenia. (Some clinicians use the terms granulocytopenia and neutropenia interchangeably because the largest constituency of granulocytes is the neutrophils.) The absolute neutrophil count (ANC) is determined by multiplying the total WBC count by the percentage of neutrophils. Neutropenia is defined as ANC less than 1000 cells/µL (1 × 10 9 /L). (Normally, neutrophils range from 2200 to 7700 cells/µL.) Severe neutropenia is defined as an ANC less than 500 cells/µL. In considering the clinical significance of neutropenia, it is important to know whether the decrease in the neutrophil count was gradual or rapid, the degree of neutropenia, and the duration. The faster the drop and the longer the duration, the greater the likelihood of life-threatening infection, sepsis, or death. Other factors and co-morbid conditions, such as being older than 60, having an existing infection, being in the hospital, and having diabetes, can increase the risk of a serious infection. Neutropenia is a clinical consequence that occurs with a variety of conditions or diseases 34,35 (Table 31-22). It can also be an expected effect, a side effect, or an unintentional effect of taking certain drugs. The most common cause of neutropenia is the use of chemotherapy and immunosuppressive therapy in the treatment of malignancies and autoimmune diseases. A term that is often used to describe the lowest point of neutropenia (and other blood cells) in a patient treated with chemotherapy is nadir.

Lymphomas

Lymphomas are malignant neoplasms originating in the bone marrow and lymphatic structures resulting in the proliferation of lymphocytes. Lymphomas are the fifth most common type of cancer in the United States. 49 Two major types of lymphoma are Hodgkin's lymphoma and non-Hodgkin's lymphoma (NHL). A comparison of these two types of lymphoma is presented in

Nursing Management of Aplastic Anemia

Management of aplastic anemia is based on identifying and removing the causative agent (when possible) and providing supportive care until the pancytopenia reverses. Nursing interventions appropriate for the patient with pancytopenia from aplastic anemia are presented in Nursing Care Plan 31-1 for anemia and in eNursing Care Plan 31-1 for thrombocytopenia and eNursing Care Plan 31-2 for neutropenia (available on the website for this chapter). Nursing actions are directed at preventing complications from infection and hemorrhage. The treatment of choice for adults less than 55 years of age who do not respond to the immunosuppressive therapy and who have a human leukocyte antigen (HLA)-matched donor is an HSCT. The best results occur in younger patients who have not had previous blood transfusions. Prior transfusions increase the risk of graft rejection. (HSCT is discussed in Chapter 16.) For older adults without an HLA-matched donor, the treatment of choice is immunosuppression with ATG or cyclosporine or high-dose cyclophosphamide. High-dose corticosteroids may also be used. However, this therapy may be only partially beneficial. Patients who need ongoing supportive blood transfusion should be on an iron-binding agent to prevent iron overload.

Clinical Manifestations of thrombocytopenia

Many patients with thrombocytopenia are usually asymptomatic. The most common symptom is bleeding, usually mucosal or cutaneous. Mucosal bleeding may manifest as epistaxis and gingival bleeding, and large bullous hemorrhages may appear on the buccal mucosa because of the lack of vessel protection by the submucosal tissue.

NHL clincial manifestation

NHLs can originate outside the lymph nodes, the method of spread can be unpredictable, and the majority of patients have widely disseminated disease at the time of diagnosis (Fig. 31-15). The primary clinical manifestation is painless lymph node enlargement. The lymphadenopathy can wax and wane in indolent disease. Because the disease is usually disseminated when it is diagnosed, other symptoms are present depending on where the disease has spread (e.g., hepatomegaly with liver involvement, neurologic symptoms with CNS disease). NHL can also manifest in nonspecific ways, such as an airway obstruction, hyperuricemia and renal failure from tumor lysis syndrome, pericardial tamponade, and GI complaints. Patients with high-grade lymphomas may have lymphadenopathy and constitutional symptoms (B symptoms) such as fever, night sweats, and weight loss. The peripheral blood is usually normal, but some lymphomas manifest in a "leukemic" phase.

Cobalamin (Vitamin B12 ) Deficiency

Normally, a protein termed intrinsic factor (IF) is secreted by the parietal cells of the gastric mucosa. IF is required for cobalamin (extrinsic factor) absorption. (Cobalamin is normally absorbed in the distal ileum.) Therefore if IF is not secreted, cobalamin will not be absorbed.

Etiology Hodgkins Lymphoma

Normally, the lymph nodes are composed of connective tissues that surround a fine mesh of reticular fibers and cells. In Hodgkin's lymphoma the normal structure of lymph nodes is destroyed by hyperplasia of monocytes and macrophages. The main diagnostic feature of Hodgkin's lymphoma is the presence of Reed-Sternberg cells in lymph node biopsy specimens. The disease is believed to arise in a single location (it originates in cervical lymph nodes in 70% of patients) and then spreads along adjacent lymphatics. However, in recurrent disease, it may be more diffuse and not necessarily contiguous. It eventually infiltrates other organs, especially lungs, spleen, and liver.

HIT

One of the risks associated with the broad and increasing use of heparin is the development of the life-threatening condition called heparin-induced thrombocytopenia (HIT), also called heparin-induced thrombocytopenia and thrombosis syndrome (HITTS). Typically, patients develop thrombocytopenia 5 to 10 days after the onset of heparin therapy. HIT should be suspected if the platelet count falls by more than 50% or falls to below 150,000/µL. As many as 5% of patients on heparin therapy develop HIT. 24 The major clinical problem of HIT is venous thrombosis; arterial thrombosis can also develop. Deep vein thromboses and pulmonary emboli most commonly result as a complication of the thromboses. Additional complications may include arterial vascular infarcts resulting in skin necrosis, stroke, and endorgan damage (e.g., kidneys). Symptoms of bleeding are unusual because the platelet count rarely drops below 60,000/µL.

Drug Therapy

Oral iron should be used whenever possible because it is inexpensive and convenient. Many iron preparations are available. When administering iron, consider the following five factors: 1. Iron is absorbed best from the duodenum and proximal jejunum. Therefore enteric-coated or sustained-release capsules, which release iron farther down in the GI tract, are counterproductive and expensive. 2. The daily dose should provide 150 to 200 mg of elemental iron. This can be ingested in three or four daily doses, with each tablet or capsule of the iron preparation containing between 50 and 100 mg of iron (e.g., a 300-mg tablet of ferrous sulfate contains 60 mg of elemental iron). 3. Iron is best absorbed as ferrous sulfate (Fe 2+ ) in an acidic environment. For this reason and to avoid binding the iron with food, iron should be taken about an hour before meals, when the duodenal mucosa is most acidic. Taking iron with vitamin C (ascorbic acid) or orange juice, which contains ascorbic acid, enhances iron absorption. Gastric side effects, however, may necessitate ingesting iron with meals. 4. Undiluted liquid iron may stain the patient's teeth. Therefore it should be diluted and ingested through a straw. 5. GI side effects of iron administration may occur, including heartburn, constipation, and diarrhea. If side effects develop, the dose and type of iron supplement may be adjusted. For example, many individuals who need supplemental iron cannot tolerate ferrous sulfate because of the effects of the sulfate base. However, ferrous gluconate may be an acceptable substitute. Tell patients that the use of iron preparations will cause their stools to become black because the GI tract excretes excess iron. Constipation is common, and the patient should be started on stool softeners and laxatives, if needed, when started on iron. DRUG ALERT: Iron • Some preparations of IV iron have a risk of an allergic reaction, and the patient should be monitored accordingly. • Oral iron should be taken about 1 hr before meals. • Vitamin C (ascorbic acid) enhances iron absorption.

Clinical manifestations of iron def anemia

Pallor is the most common finding, and glossitis (inflammation of the tongue) is the second most common. Another finding is cheilitis (inflammation of the lips). In addition, the patient may report headache, paresthesias, and a burning sensation of the tongue, all of which are caused by lack of iron in the tissues.

Diagnostic hodgkin

Peripheral blood analysis, excisional lymph node biopsy, bone marrow examination, and radiologic studies are important means of evaluating Hodgkin's lymphoma. Abnormalities in the CBC, such as a microcytic hypochromic anemia, are variable and not diagnostic. Leukopenia and thrombocytopenia may develop, but they are usually a consequence of treatment, advanced disease, or superimposed hypersplenism. Other blood studies may show hypoferremia caused by excessive iron uptake by the liver and the spleen, elevated leukocyte alkaline phosphatase from liver and bone involvement, hypercalcemia from bone involvement, and hypoalbuminemia from liver involvement. Radiologic evaluation can help define all sites and determine the clinical stage of the disease. Positron emission tomography (PET) with or without CT scan is used to stage and then assess the response to therapy and to differentiate residual tumor from fibrotic masses after treatment. These scans may show increased uptake (by PET) and masses (by CT) such as mediastinal lymphadenopathy; renal displacement caused by retroperitoneal node enlargement; abdominal lymph node enlargement; and liver, spleen, bone, and brain infiltration.

Polycthemia

Polycythemia is the production and presence of increased numbers of RBCs. The increase in RBCs can be so great that blood circulation is impaired as a result of the increased blood viscosity (hyperviscosity) and volume (hypervolemia).

Polycthemia vera

Polycythemia vera is a chronic myeloproliferative disorder. Therefore not only are RBCs involved, but also WBCs and platelets, leading to increased production of each of these blood cells. The disease develops insidiously and follows a chronic, vacillating course. The median age at diagnosis is 60 years old, with a slight male predominance. In polycythemia vera there is enhanced blood viscosity and blood volume and congestion of organs and tissues with blood. Splenomegaly (which occurs in 90% of patients) and hepatomegaly are common. These patients have hypercoagulopathies that predispose them to clotting.

Polycthemia vera genetic link

Polycythemia vera is associated with mutations in the Janus kinase-2 (JAK2) gene. The JAK2 gene provides instructions for making a protein that promotes proliferation of cells, especially blood cells from hematopoietic stem cells. Polycythemia vera begins with one or more DNA mutations of a single hematopoietic stem cell. Most cases of polycythemia vera are not inherited. This condition is associated with genetic changes that are somatic, which means they are acquired during a person's lifetime and are present only in certain cells.

Polycthemia vera nursing management

Primary polycythemia (polycythemia vera) is not preventable. When acute exacerbations of polycythemia vera develop, you have several responsibilities. Depending on the institution's policies, you may either assist with or perform the phlebotomy. Evaluate fluid intake and output during hydration therapy to avoid fluid overload (which further complicates the circulatory congestion) and underhydration (which can make the blood even more viscous). If myelosuppressive agents are used, administer the drugs as ordered, observe the patient, and teach the patient about medication side effects. Assess the patient's nutritional status, since inadequate food intake can result from GI symptoms of fullness, pain, and dyspepsia. Begin activities and/or medications to decrease thrombus formation. Initiate active or passive leg exercises and ambulation when possible. Because of its chronic nature, polycythemia vera requires ongoing evaluation. Phlebotomy may need to be done every 2 to 3 months, reducing the blood volume by about 500 mL each time. Evaluate the patient for complications.

Blood Transfusions Delegation Decisions

Role of Registered Nurse (RN) • Ensure that an IV line is being used with a large-bore needle, catheter, or cannula, preferably 19 gauge or larger. • Double-check patient identification and blood product identification data with another licensed nurse (consider state nurse practice act and agency policy). • Adjust infusion rate of transfusion according to patient needs. • Evaluate patient for signs of transfusion reactions. • Evaluate for therapeutic effect of blood product (improvement in complete blood count, increased blood pressure, improved patient color, decreased bleeding). • Monitor for signs of circulatory overload (e.g., shortness of breath) if the transfusion must be given rapidly. Role of Licensed Practical/Vocational Nurse (LPN/LVN) • Assist with checking patient identification and blood product identification data with the RN (consider state nurse practice act and agency policy). • Monitor blood transfusion rate (consider state nurse practice act and agency policy). Role of Unlicensed Assistive Personnel (UAP) • Obtain blood products from the blood bank as directed by the RN. • Take vital signs before the transfusion and after the first 15 min.

Safety Alert Blood product administration

SAFETY ALERT • Do not use dextrose solutions or lactated Ringer's solution for administering blood because they will cause RBC hemolysis. • Do not give any additives (including medications) via the same tubing as the blood unless the tubing is first cleared with saline solution.

subjective data

Subjective Data Important Health Information Past health history: Recent blood loss or trauma; chronic liver, endocrine, or renal disease (including dialysis); GI disease (malabsorption syndrome, ulcers, gastritis, or hemorrhoids); inflammatory disorders (especially Crohn's disease); smoking, exposure to radiation or chemical toxins (arsenic, lead, benzenes, copper); infectious diseases (HIV) or recent travel with possible exposure to infection; angina, myocardial infarction; history of falling Medications: Use of vitamin and iron supplements; aspirin, anticoagulants, oral contraceptives, phenobarbital, penicillins, nonsteroidal antiinflammatory drugs, omeprazole, phenacetin, phenytoin (Dilantin), sulfonamides, herbal products Surgery or other treatments: Recent surgery, small bowel resection, gastrectomy, prosthetic heart valves, chemotherapy, radiation therapy Dietary history: General dietary patterns, consumption of alcohol Functional Health Patterns Health perception-health management: Family history of anemia; malaise Nutritional-metabolic: Nausea, vomiting, anorexia, weight loss; dysphagia, dyspepsia, heartburn; night sweats, cold intolerance Elimination: Hematuria, decreased urine output; diarrhea, constipation, flatulence, tarry stools, bloody stools Activity-exercise: Fatigue, muscle weakness and decreased strength; dyspnea, orthopnea, cough, hemoptysis; palpitations; shortness of breath with activity Cognitive-perceptual: Headache; abdominal, chest, and bone pain; painful tongue; paresthesias of feet and hands; pruritus; disturbances in vision, taste, or hearing; vertigo; hypersensitivity to cold; dizziness Sexuality-reproductive: Menorrhagia, metrorrhagia; recent or current

Blood admin

Take the patient's vital signs before beginning the transfusion so that you have a baseline measure. If the patient has abnormal vital signs (e.g., high fever), call the physician to clarify when the blood component may be administered. Administer the blood as soon as it is brought to the patient. Do not refrigerate it on the nursing unit. If the blood is not used within 30 minutes, return it to the blood bank. During the first 15 minutes or 50 mL of blood infusion, remain with the patient. If there are any untoward reactions, they are most likely to occur at this time. The rate of infusion during this period should be no more than 2 mL/minute. Do not infuse PRBCs quickly except in an emergency. Rapid infusion of cold blood may cause the patient to become chilled. If rapid replacement of large amounts of blood is necessary, a blood-warming device may be used. Other blood components, such as fresh frozen plasma and platelets, may be infused over 15 to 30 minutes. Refer to your institution's policy and procedure. After the first 15 minutes, vital signs are usually retaken, and the rate of infusion is determined by the patient's clinical condition and the product being infused. Observe the patient periodically throughout the transfusion (e.g., every 30 minutes) and up to 1 hour after the transfusion. Most patients not in danger of fluid overload can tolerate the infusion of 1 unit of PRBCs over 2 hours. The transfusion should not take more than 4 hours to administer because of the increased risk of bacterial growth in the product once it is out of refrigeration. Blood that is unrefrigerated for 4 hours or longer should not be infused and should be returned to the blood bank.

Collab Care of iron def anemia

The main goal is to treat the underlying disease that is causing reduced intake (e.g., malnutrition, alcoholism) or absorption of iron. In addition, efforts are directed toward replacing iron (Table 31-7). Teach the patient which foods are good sources of iron (see Table 31-5). If nutrition is already adequate, increasing iron intake by dietary means may not be practical. Consequently, oral or occasionally parenteral iron supplements are used. If the iron deficiency is from acute blood loss, the patient may require a transfusion of packed RBCs.

ITP

The most common acquired thrombocytopenia is a syndrome of abnormal destruction of circulating platelets termed immune thrombocytopenic purpura (ITP). It was originally termed idiopathic thrombocytopenic purpura because its cause was unknown. However, it is now known that ITP is primarily an autoimmune disease. In ITP, platelets are coated with antibodies. Although these platelets function normally, when they reach the spleen, the antibodycoated platelets are recognized as foreign and are destroyed by macrophages. Decreased platelet production contributes to ITP.

Pernicious anemia

The most common cause of cobalamin deficiency is pernicious anemia, which is caused by an absence of IF. In pernicious anemia the gastric mucosa is not secreting IF because of either gastric mucosal atrophy or autoimmune destruction of parietal cells. In the autoimmune process antibodies are directed against the gastric parietal cells and/or IF itself. Because parietal cells also secrete hydrochloric acid (HCl), in pernicious anemia there is a decrease in HCl in the stomach. An acid environment in the stomach is required for the secretion of IF. Pernicious anemia is a disease of insidious onset that begins in middle age or later (usually after age 40), with 60 years being the most common age at diagnosis.

Clinical Manifestations of Hodgkins ly

The onset of symptoms in Hodgkin's lymphoma is usually insidious. The initial development is most often enlargement of cervical, axillary, or inguinal lymph nodes (Fig. 31-13); a mediastinal node mass is the second most common location. This lymphadenopathy affects discrete nodes that remain movable and nontender. The enlarged nodes are not painful unless they exert pressure on adjacent nerves. The patient may notice weight loss, fatigue, weakness, fever, chills, tachycardia, or night sweats. A group of initial findings including fever (in excess of 100.4°F [38°C]), drenching night sweats, and weight loss (exceeding 10% in 6 months) are termed B symptoms and correlate with a worse prognosis. After the ingestion of even small amounts of alcohol, individuals with Hodgkin's lymphoma may complain of a rapid onset of pain at the site of disease. The cause for the alcohol-induced pain is unknown. Generalized pruritus without skin lesions may develop. Cough, dyspnea, stridor, and dysphagia may all reflect mediastinal node involvement. In more advanced disease, there may be hepatomegaly and splenomegaly. Anemia results from increased destruction and decreased production of erythrocytes. Other physical signs vary depending on where the disease is located. For example, intrathoracic involvement may lead to superior vena cava syndrome, enlarged retroperitoneal nodes may cause palpable abdominal masses or interfere with renal function, jaundice may occur from liver involvement, spinal cord compression leading to paraplegia may occur with extradural involvement, and bone pain occurs as a result of bone involvement.

Nursing Management of Chronic Blood Loss

The sources of chronic blood loss are similar to those of irondeficiency anemia (e.g., bleeding ulcer, hemorrhoids, menstrual and postmenopausal blood loss). The effects of chronic blood loss are usually related to the depletion of iron stores and considered as iron-deficiency anemia. Management of chronic blood loss anemia involves identifying the source and stopping the bleeding. Supplemental iron may be required.

Thrombocytopenia

Thrombocytopenia is a reduction of platelets below 150,000/µL (150 × 10 9 /L). Acute, severe, or prolonged decreases from this normal range can result in abnormal hemostasis that manifests as prolonged bleeding from minor trauma or spontaneous bleeding without injury.

TTP

Thrombotic thrombocytopenic purpura (TTP) is an uncommon syndrome characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever (in the absence of infection), and renal abnormalities; not all features are present in all patients. Because it is almost always associated with hemolytic-uremic syndrome (HUS), it is often referred to as TTP-HUS. The disease is associated with enhanced aggregation of platelets, which form microthrombi that deposit in arterioles and capillaries. TTP is seen primarily in adults between 20 and 50 years of age, with a slight female predominance. The syndrome may be idiopathic (thought to be due to an autoimmune disorder against ADAMTS13), but it may be due to certain drug toxicities (e.g., chemotherapy, cyclosporine, quinine, oral contraceptives, valacyclovir [Valtrex], clopidogrel [Plavix]), pregnancy or preeclampsia, infection, or a known autoimmune disorder such as systemic lupus erythematosus or scleroderma. TTP is a medical emergency because bleeding and clotting occur simultaneously.

Nursing Management of NHL

Treatment for NHL involves chemotherapy and sometimes radiation therapy (Table 31-30). Ironically, more aggressive lymphomas are more responsive to treatment and more likely to be cured. In contrast, indolent lymphomas have a naturally long course but are difficult to effectively treat. 52 Patients with low-grade (indolent) lymphoma may live 10 years or more without treatment. However, some initial therapies can be well tolerated and have been shown to reduce the time to progression of the disease. Lymphomas that are infectious driven, such as H. pylori gastric lymphomas, may be treated with antibiotic or antiviral therapy. HSCT may have some benefit in certain subtypes of aggressive or refractory lymphomas. 45 Rituximab, a monoclonal antibody against the CD20 antigen on the surface of normal and malignant B cells, is used to treat NHL. Once bound to the cells, rituximab causes lysis and cell death. Numerous chemotherapy combinations have been used to try to overcome the resistant nature of this disease (see Table 31-30). Complete remissions are uncommon, but the majority of patients respond with improvement in symptoms. The nursing care for NHL is similar to that for Hodgkin's lymphoma. It is largely based on managing problems related to the disease (e.g., pain caused by the tumor, spinal cord compression, tumor lysis syndrome), pancytopenia, and other side effects of therapy. However, because NHL can be more extensive and involve specific organs (e.g., CNS, spleen, liver, GI tract, bone marrow), it is important to understand the subtype and extent of the disease. For example, a patient with known involvement of the colon may complain of acute abdominal pain. The patient most likely would have abdominal guarding and an enlarged and tympanic abdomen. This could indicate a bowel perforation and be considered a medical emergency. A patient with a Burkitt's NHL beginning chemotherapy would be at high risk for tumor lysis syndrome and would require frequent laboratory studies and monitoring, as well as strict documentation of intake and output. (Cancer-related therapies and side effects are discussed in Chapter 16.) The patient undergoing external beam radiation therapy has special nursing needs. The skin in the radiation field requires attention. Concepts related to safety issues regarding radiation therapy are important in the plan of care (see Chapter 16). Psychosocial considerations are important. Help the patient and family understand the disease, treatment, and expected and potential side effects. Fertility issues may be of concern in young patients. As in Hodgkin's lymphoma, evaluation of patients with NHL for long-term effects of therapy is important because the delayed consequences of disease and treatment may not become apparent for many years. (Secondary malignancies and delayed effects are discussed in Chapter 16.

Collab Care for polycythemia

Treatment is directed toward reducing blood volume and viscosity and bone marrow activity. Phlebotomy is the mainstay of treatment. The aim of phlebotomy is to reduce the hematocrit and keep it less than 45% to 48%. Generally, at the time of diagnosis 300 to 500 mL of blood may be removed every other day until the hematocrit is reduced to normal levels. An individual managed with repeated phlebotomies eventually becomes deficient in iron, although this effect is rarely symptomatic. Avoid iron supplementation. Hydration therapy is used to reduce the blood's viscosity. Myelosuppressive agents such as hydroxyurea, busulfan (Myleran), and chlorambucil are used. Ruxolitinib (Jakafi), which is a new drug inhibiting the expression of the JAK2 mutation, is used for patients who have polycythemia-related myelofibrosis. Low-dose aspirin is used to prevent clotting. α-Interferon (α-IFN) is of particular use in women of childbearing age or those with intractable pruritus. Anagrelide (Agrylin) may be used to reduce the platelet count and inhibit platelet aggregation. Allopurinol (Zyloprim) may reduce the number of acute gouty attacks.

Nursing Management of Hodkins

Using all of the information from the various diagnostic studies, one can determine a clinical stage of disease 50,51 (Fig. 31-14). The final staging is based on the clinical stage (extent of the disease) and the presence of B symptoms. Treatment depends on the nature and extent of the disease. The nomenclature used in staging involves an A or B classification, depending on whether symptoms are present when the disease is found, and a Roman numeral (I to IV) that reflects the location and extent of the disease. Additional features that may move an early stage (I or II) to an unfavorable prognosis, warranting more aggressive therapy, include an elevated sedimentation rate; age of 45 years or older; male gender; and the presence of a large mediastinal mass and low serum albumin, hemoglobin, and low or high lymphocyte counts. 51 Once the stage of Hodgkin's lymphoma is established, management focuses on selecting a treatment plan. The standard for chemotherapy is the ABVD regimen: doxorubicin (Adriamycin), bleomycin, vinblastine, and dacarbazine. Patients with favorable early-stage disease receive two to four cycles of chemotherapy. Patients with early-stage disease but unfavorable prognostic features (e.g., B symptoms) or intermediate-stage disease are treated with four to six cycles of chemotherapy. Advanced-stage Hodgkin's lymphoma is treated more aggressively using six to eight cycles of chemotherapy. A common regimen is BEACOPP (bleomycin, etoposide, doxorubicin (Adriamycin), cyclophosphamide, vincristine (Oncovin), procarbazine, and prednisone). 51 The role of radiation as a supplement to chemotherapy varies depending on sites of disease and the presence of resistant disease after chemotherapy. Response to therapy is determined by CT and PET scans and other diagnostic tests (e.g., bone marrow biopsy). A variety of chemotherapy regimens and newer agents, such as brentuximab vedotin (Adcetris), are used to treat patients who have relapsed or refractory disease. Ideally, once remission is obtained, a hope for curative option may be intensive chemotherapy with the use of autologous or allogeneic HSCT. The nursing care for Hodgkin's lymphoma is largely based on managing problems related to the disease (e.g., pain caused by a tumor, superior vena cava syndrome), pancytopenia, and other side effects of therapy. Because the survival of patients with Hodgkin's lymphoma depends on their response to treatment, supporting the patient through the consequences of treatment is extremely important. Psychosocial considerations are as important as they are with leukemia. However, the prognosis for Hodgkin's lymphoma is better than that for many forms of cancer or leukemia. The physical, psychologic, social, and spiritual consequences of the patient's disease must be addressed. Fertility issues may be of particular concern because this disease is frequently seen in adolescents and young adults. Help to ensure that these issues have been addressed soon after diagnosis. Evaluation of patients for long-term effects of therapy is important because delayed consequences of disease and treatment may not be apparent for many years. (Secondary malignancies and delayed effects are discussed in Chapter 16.)

hemolytic anemia

a condition caused by the destruction or hemolysis of RBCs at a rate that exceeds production. Hemolysis can occur because of problems intrinsic or extrinsic to the RBCs. Intrinsic hemolytic anemias, which are usually hereditary, result from defects in the RBCs themselves (see Table 31-2). More common are the acquired hemolytic anemias. In this type of anemia the RBCs are normal, but damage is caused by external factors (see Table 31-2). The spleen is the primary site of the destruction of RBCs that are old, defective, or moderately damaged. Fig. 31-2 indicates the sequence of events involved in extravascular hemolysis. The patient with hemolytic anemia has the general manifestations of anemia and specific manifestations related to this type of anemia (see Table 31-3). Jaundice is likely because the increased destruction of RBCs causes an elevation in bilirubin levels. The spleen and liver may enlarge because of their hyperactivity, which is related to macrophage phagocytosis of the defective erythrocytes. In all causes of hemolysis, a major focus of treatment is to maintain renal function. When RBCs are hemolyzed, the hemoglobin molecule is released and filtered by the kidneys. The accumulation of hemoglobin molecules can obstruct the renal tubules and lead to acute tubular necrosis

Folic Acid Defiencieny

also causes megaloblastic anemia. Folic acid is required for DNA synthesis leading to RBC formation and maturation. Common causes of folic acid deficiency are listed in Table 31-8. The clinical manifestations of folic acid deficiency are similar to those of cobalamin deficiency. The disease develops insidiously, and the patient's symptoms may be attributed to other coexisting problems (e.g., cirrhosis, esophageal varices). GI disturbances include dyspepsia and a smooth, beefy red tongue. The absence of neurologic problems is an important diagnostic finding and differentiates folic acid deficiency from cobalamin deficiency. The diagnostic findings for folic acid deficiency are presented in Table 31-6. In addition, the serum folate level is low (normal is 3 to 25 mg/mL [7 to 57 mol/L]) and the serum cobalamin level is normal. Folic acid deficiency is treated by replacement therapy. The usual dosage is 1 mg/day by mouth. In malabsorption states or with chronic alcoholism, up to 5 mg/day may be required. The duration of treatment depends on the reason for the deficiency. Encourage the patient to eat foods containing large amounts of folic acid

Collab care for cobalamin def

anemia. However, instruct the patient on adequate dietary intake to maintain good nutrition (see Table 31-5). Parenteral vitamin B12 (cyanocobalamin or hydroxocobalamin) or intranasal cyanocobalamin (Nascobal, CaloMist) is the treatment of choice. Without cobalamin administration, these individuals will die in 1 to 3 years. A typical treatment schedule consists of 1000 mcg/day of cobalamin IM for 2 weeks and then weekly until the hemoglobin is normal, and then monthly for life. High-dose oral cobalamin and sublingual cobalamin are also available for those in whom GI absorption is intact. As long as supplemental cobalamin is used, the anemia can be reversed. However, if the person has had long-standing neuromuscular complications, they may not be reversible.

Meglobalstic anemias

are a group of disorders caused by impaired DNA synthesis and characterized by the presence of large RBCs. When DNA synthesis is impaired, defective RBC maturation results. The RBCs are large (macrocytic) and abnormal and are referred to as megaloblasts. Macrocytic RBCs are easily destroyed because they have fragile cell membranes. Although the overwhelming majority of megaloblastic anemias result from cobalamin (vitamin B12 ) and folic acid deficiencies, this type of RBC deformity can also occur from suppression of DNA synthesis by drugs, inborn errors of cobalamin and folic acid metabolism, and erythroleukemia (malignant blood disorder characterized by a proliferation of erythropoietic cells in bone marrow)

NHLs

are a heterogeneous group of malignant neoplasms of primarily B-, T-, or natural killer (NK) cell origin affecting all ages. B-cell lymphomas constitute about 85% of all NHLs. They are categorized by the level of differentiation, cell of origin, and rate of cellular proliferation. 26 A variety of clinical presentations and courses are recognized, from indolent (slowly developing) to rapidly progressive disease. NHL is the most commonly occurring hematologic cancer and the fifth leading cause of cancer death. Each year approximately 66,360 new cases of NHL are diagnosed and approximately 19,320 deaths occur.

Secondary Polychtmeia

can be either hypoxia driven or hypoxia independent. In hypoxiadriven secondary polycythemia, hypoxia stimulates erythropoietin (EPO) production in the kidney, which in turn stimulates RBC production. The need for oxygen may result from high altitude, pulmonary disease, cardiovascular disease, alveolar hypoventilation, defective oxygen transport, or tissue hypoxia. EPO levels may return to normal once the hemoglobin is stablilized at a higher level. no splenomagly

Hodgkins lymphoma

cell origin: B lymphoyctes extent of disease: localized to regional but may be more widespread B symtoms: common Extranodal involvement: rare

Non-hodgkins

cell origin: B lymphoyctes 85% T or natural killer lympho 15% extent of disease: disseminated B symtoms: 40% Extranodal involvement: commom

NHLs more info

chemotherapy puts patient at risk for NHL

older men 70 to 88

decrease 1 g/dl and women 0.2 g/dl

nursing management thrombo

if subq injection is needed use small gauge needle and direct pressure for 5 to 10 minutes no IM Monitor all blood cell and coagulation studies Counting sanitary napkins used during menses is another important intervention to detect excess blood loss. Blood loss of 50 mL will completely soak a sanitary napkin. Suppression of menses with hormonal agents may be indicated during predictable periods of thrombocytopenia (e.g., during chemotherapy and HSCT) to reduce blood loss from menses. Closely monitor the platelet count, coagulation studies, hemoglobin, and hematocrit. Together these provide important information regarding potential or actual bleeding. The proper administration of platelet transfusions is an important nursing responsibility. avoid causative agents and avoid trauma/injury

Nursing and Collab Management of neutropenia

include (1) determining the cause of the neutropenia, (2) identifying the offending organisms if an infection has developed, (3) instituting antibiotic therapy, (4) administering hematopoietic growth factors, and (5) implementing protective environmental practices (e.g., strict hand washing) When a febrile episode occurs in a neutropenic patient, antibiotic therapy must be initiated immediately (within 1 hour) even before the determination of a specific causative organism by culture. Administration of broad-spectrum antibiotics is usually by the IV route because of the rapidly lethal effects of infection. However, some oral antibiotics are highly effective and routinely used for prophylaxis against infection in some neutropenic patients. The use of a third- or fourthgeneration cephalosporin (e.g., cefepime [Maxipime], ceftazidime [Ceptaz]), a carbapenem (e.g., imipenem/cilastatin [Primaxin]), or a combination of an aminoglycoside plus an antipseudomonal offers broad-spectrum coverage for initial management. Determine the best means to protect the patient whose own defenses against infection are compromised. Keep the following principles in mind: (1) the patient's normal flora is the most common source of microbial colonization and infection; (2) transmission of organisms from humans most commonly occurs by direct contact with the hands; (3) air, food, water, and equipment provide additional opportunities for infection transmission; and (4) health care providers with transmissible illnesses and other patients with infections can also be sources of infection transmission under certain conditions. Hand washing is the single most important preventive measure to minimize the risk of infection in the neutropenic patient. Strict hand washing by staff and visitors using an antiseptic hand wash, before and after contact, is the major method to prevent transmission of harmful pathogens.

Aplastic Anemia

is a disease in which the patient has peripheral blood pancytopenia (decrease of all blood cell types—RBCs, white blood cells [WBCs], and platelets) and hypocellular bone marrow. The spectrum of the anemia can range from a chronic condition managed with erythropoietin or blood transfusions to a critical condition with hemorrhage and sepsis. The incidence of aplastic anemia is low, affecting approximately 2 of every 1 million persons per year. It has two major categories of disease congenital and acquired

Hodgkins Lymphoma

lymphoma, also called Hodgkin's disease, makes up about 11% of all lymphomas. It is a malignant condition characterized by proliferation of abnormal giant, multinucleated cells, called Reed-Sternberg cells, which are located in lymph nodes. The disease has a bimodal age-specific incidence, occurring most frequently in persons from 15 to 35 years of age and above 50 years of age. In adults, it is twice as prevalent in men as in women. Each year, approximately 9060 new cases of Hodgkin's lymphoma are diagnosed and approximately 1200 deaths occur. However, long-term survival exceeds 85% for all stages.

severities of anemica

mild 10 -12 moderate 10 - 6 sever less than 6

iron deficiency anemia

one of the most common chronic hematologic disorders, is found in 2% to 5% of adult men and postmenopausal women in developed countries. Those most susceptible to iron-deficiency anemia are the very young, those on poor diets, and women in their reproductive years. 5,6 Normally, 1 mg of iron is lost daily through feces, sweat, and urine. may develop as a result of inadequate dietary intake, malabsorption, blood loss, or hemolysis.

Table 31-3

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pg 676

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cardiopulmomary changes with anemia

the heart and lungs to provide adequate amounts of oxygen to the tissues. Cardiac output is maintained by increasing the heart rate and stroke volume. The low viscosity of the blood contributes to the development of systolic murmurs and bruits. In extreme cases or when concomitant heart disease is present, angina pectoris and myocardial infarction (MI) may occur if myocardial O2 needs cannot be met. Heart failure (HF), cardiomegaly, pulmonary and systemic congestion, ascites, and peripheral edema may develop if the heart is overworked for an extended period.

Safety Alerts for neutropenic patients

• A low-grade fever in neutropenic patients is of great significance because it may indicate infection and lead to septic shock and death unless treated promptly. • Neutropenic fever (≥100.4°F [38°C] and a neutrophil count <500/µL) is a medical emergency. • Blood cultures should be drawn STAT and antibiotics started within 1 hr.

Congenital causes of aplastic anemia

• Fanconi syndrome • Congenital dyskeratosis • Amegakaryocytic thrombocytopenia • Shwachman-Diamond syndrome

DRUG ALERT: Rituximab (Rituxan)

• Monitor patient for signs of severe hypersensitivity infusion reactions, especially with first infusion. • Manifestations may include hypotension, bronchospasm, dysrhythmias, angioedema, and cardiogenic shock. • Screen for history of hepatitis because the drug may reactivate hepatitis.

Drugs and Herbal Causes of Thrombocytopenia

• Thiazide diuretics • Alcohol • Chemotherapeutic drugs • Digoxin • Nonsteroidal antiinflammatory drugs: ibuprofen (Advil, Motrin), indomethacin (Indocin), naproxen (Naproxyn, Aleve) • Antibiotics: penicillins, cephalosporins, sulfonamides • Other antiinfectives: rifampin (Rifadin), ganciclovir (Cytovene), amphotericin B • Analgesics: aspirin and aspirin-containing drugs, acetaminophen • Antipsychotic and antiseizure agents: haloperidol (Haldol), valproate (Depakene), lithium • Platelet glycoprotein inhibitors: abciximab (ReoPro), tirofiban (Aggrastat), eptifibatide (Integrilin), clopidogrel (Plavix) • H2-receptor antagonists: cimetidine (Tagamet), ranitidine (Zantac) • Gold compounds: auranofin (Ridaura) • Spices: ginger, cumin, turmeric, cloves • Vitamins: vitamin C, vitamin E • Heparin • Herbs: angelica, bilberry, feverfew, garlic, ginkgo biloba, ginseng • Quinine compounds: tonic water

thrombo nursing diagnosis

•Impaired oral mucous membrane related to low platelet counts and/or effects of pathologic conditions and treatment •Risk for bleeding related to decreased platelets •Deficient knowledge related to lack of information regarding the disease process and treatment goals: (1) have no gross or occult bleeding, (2) maintain vascular integrity, and (3) manage home care to prevent any complications related to an increased risk for bleeding.