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Management of Clients with Hematologic Disorders NRS 108 Majuvy L. Sulse RN, MSN, CCRN HEMATOLOGIC DISORDERS Disorders associated with Erythrocytes Disorders of bleeding Disorders associated with white blood cells Lymphomas ANEMIA (decreased in number of RBCs) Abnormal & deficient production, blood loss, destruction of RBC Low Hgb ( Causes) H/H, Bone marrow aspiration, Peripheral smear ( Diagnosis) Less 02 carrying capacity Hypoxia Pallor, Fatigue, Palpitation, Low BP, SOB, DOE, MI, Renal failure Management of Anemia Medical: Identify cause Treat cause Relieve symptoms Prevent complications Nursing: Assess Educate Classification of Anemia Based on the size of the RBC Normocytic Microcytic Macrocytic Iron Deficiency Anemia Causes: Inadequate iron supply Chronic blood loss without iron replacement Decreased iron absorption in the intestines Signs/ Symptoms: Hypochromic (low MCH), microcytic ( low MCV) Elevated serum binding capacity Brittle, spoon-shaped nails with longitudinal ridges Cheilosis (painful mouth cracks/ sores) Red shiny tongue Insidious development of fatigue Diagnosis/Treatment Treatment: Identify cause Diagnosis: Laboratory values Gastroscopy Sigmoidoscopy Occult blood in stools Radiographic studies of GI Iron supplement Nutritional/dietary Megaloblastic Anemia Predominance of megaloblasts & lack of normoblasts Includes pernicious anemia, Vit. B12 & Folic acid deficiencies Related to surgery particularly of terminal ileum where B12 is absorbed; vegetarian diet; prolonged exposure to nitrous oxide Related to aging & long term gastritis Related to alcohol malnutrition & malabsorption (Folic acid deficiency) Macrocytic, normochromic RBC Lack of Intrinsic factor Autoimmune response Diagnosis & Treatment: (Megaloblastic Anemia) Schilling test- definitive dx of pernicious anemia Gastric secretion analysis- pH, free HCL, low gastric secretion Diagnosis & Treatment : (Megaloblastic Anemia) ManagementLifelong tx with Vit B12 injection Iron & Folic acid supplement Nutritional/ dietary changes Foods that are rich in folic acid and vitamin B12 include the following: eggs meat poultry milk shellfish fortified cereals Aplastic Anemia Low Hb & pancytopenia Unknown etiology / autoimmune disturbance Direct injury by Myelotoxins- agents causing bone marrow damage when received in large doses Medication induced: Chemo, chloramphenicol, sulfonamides, mephenytoin, quinine Exposure to environmental hazards: Benzene, insecticides, radiation & radioactive materials Infections Hepatitis, miliary TB, EPBV Congenital/hereditary causes Aplastic Anemia-Manifestations & Dx Manifestations Exertional dyspnea, fatigue, pallor, infections Bleeding (nasal,oral, rectal,vaginal) ecchymosis, petechiae, pupura Low platelets (less 30, 000), RBCs, WBCs Dry bone marrow on aspiration Diagnosis Differential count Manifestations History of exposure to myelotoxins Aplastic Anemia Medical Remove causative Idiopathic cause- treat with steroids, hormone therapy Autoimmune cause- bone marrow transplants (younger than 30 or those who have not received transfusions yet Antithymocyte & cyclosporine therapy- skin testing & watch for allergic reactions Blood transfusions Frequent CBCs esp for those on radiation therapy Nursing management Infection control Client education Anemia from Blood Loss Types Acute-trauma, complications from surgery Hypovolemia, hypotension, hypoxemia, weakness, tachycardia, stupor Chronic- bleeding ulcer, hemorrhoids Gradual and vague symptoms as fatigue, pallor, dyspnea Medical/ Nursing Interventions Identify source of bleeding Control through medical or surgical interventions Blood transfusions Hemolytic Anemia Abnormal destruction of RBCs by Intrinsic-defective RBCs, enzyme deficit(G6PD) extrinsic factors- toxins, injury as in prosthetic heart valves Failure of bone marrow to replace destroyed RBCs Sites of hemolysis: Intravascular-circulation Extravascular- macrophages of spleen, liver & bone marrow Findings/ Treatment Findings Normocytic anemia Reticulocytosis as compensatory mechanism Increased RBC fragility Short lifespan Hyperbilirubinemia - blood, urine, stools S/S as in Anemia Treatment Identify/Treat cause IV fluids to flush kidneys NAHCO3 or Na Lactate to alter urine pH (decrease) precipitation in renal tubules Splenectomy Sickle Cell Anemia and Sickle Cell Trait Inherited, autosomal, recessive disorders of HB synthesis resulting in decrease O2 to the tissues (hypoxia) and obstruction of blood vessels Substitution of valine for glutamic acid in B-globin gene Primarily seen in the black population Sickle cell trait is a mild form of the disease and it is the commonest Sickle cell trait is prevalent in Africa Resistant to the parasite that causes malaria Genetically inherited from each parent Inheritance Pathophysiology Sickling of RBC-triggered by Hypoxia- low O2 tension in blood High altitude Emotional/ physical stress Surgery Blood loss Infection-bacterial & viral Dehydration Acidosis Decreased plasma volumeIncreased blood viscosity Hypothermia Stress Pathophysiology Sickled cell-rigid & elongated causes tissue injury (as cells can’t pass through small vessels) results in local hypoxia anemia as more cells are hemolyzed by spleen Initially reversible with re-oxygenation then becomes irreversible due to cell membrane damage from recurrent sickling Sickle Cell crisis Exacerbation of RBC sickling Vaso- occlusion (Vaso-occlusive crises) vasospasm Changes in membrane permeability plasma loss & hemoconcentration development of thrombi, tissue ischemia, infarction, necrosis Shock is a life threatening consequence Frequency, extent, & severity of episode is dependent on percentage of HbS present Causes of Sickle Cell Crisis Vaso - Occlusion Aplastic crisis Hemolytic crisis Sequestration crisis Mixed crisis Types of Sickle Cell Disease Sickle cell anemia Sickle cell Thalassemia Sickle cell HbC- Clinical Manifestations Cardiovascular changes Skin changes Abdominal changes Musculoskeletal changes Central nervous system changes Clinical Manifestation Noticed after 6 months when fetal Hb is no longer present Improper growth related to anemia Retarded growth and delay in sexual maturity Hand- foot syndrome- edema of hands and feet Pain from tissue ischemia-hands, feet , joints Clinical Manifestation Weakness and fatigue-exercise intolerance Jaundice- development of gallstones Pallor-of mucous membrane-grayish cast on skin Priapism-occlusion of penile veins Infarct on the spleen - small and scarred Leg ulcers in about 75% of cases Complications CHF from ischemia & heart enlargement Acute Chest syndromefever, chest pains, cough, dyspnea Pulmonary infarctpulmonary HTN, MI, Cor Pulmonale Blindness from retinal obstruction-hemmorhagedetachment Renal failurehemoconcentration Autosplenectomyshrinking of spleen from repetitious scarring CVA- thrombus formation Osteoporosis/ Osteosclerosis Leg ulcers esp. at ankles-tissue hypoxia Infection- absence of phagocytic activity by spleen Diagnostic findings Peripheral Smear Sickling Test DNA Elevated serum bilirubin levels Skeletal xrays-reveal bone/joint deformities & flattening MRI-check for clots (CVA) Medical/ Nursing Management No cure Supportive interventions: pain relief, Hydration, O2, rest, blood transfusions Patient Teaching to avoid factors that cause crisis Assessment of family understanding of the disease & coping mechanism Links for support Genetic counseling Get Involved Class! What nursing diagnosis should receive the highest priority in a client with sickle cell crisis? A nurse is preparing a teaching plan for a sickle cell client, what should the nurse emphasize on to prevent sickle cell crisis? Polycythemia Vera Excessive production of erythrocytes, leukocytes, platelets due to excessive activation of pluripotent stem cells Manifestations HTN- headache, vertigo, tinnitus, dizziness, visual disturbances CHF- angina- hypervolemia & viscosity Intermittent claudication- thrombophlebitis Polycythemia Vera Stroke- thrombi formation Pruritus- histamine release from basophils Hemmorrhage- vessel rupture from tissue distention Hepatomegaly & Slenomegaly from organ engorgement Plethora- Ruddy complexion Gout—hyper uric acid production from RBC destruction Diagnosis RBC=8-12 million Hgb=18-25 gm Increased platelet count HCT= >54% in men; 49% in women Elevated WBCs with basophilia Increased serum uric acid, B12 Splenomegaly Hyperplastic bone marrow Medical/Nursing Management Goal of treatment is Reduce blood volume & viscosity Phlebotomy to Hct levels of 45-48% (about 300500ml/day) Reduce bone marrow activitymyelosuppressive agents & radioactive phosphorous Hydration therapy with I & O DISORDERS OF BLEEDINGTHROMBOCYTOPENIA Decreased production of platelets below 150,000/uL Manifests as bleeding- skin bruises easily Maybe acquired –food, drugs, infections, aplastic anemia inherited- pancytopenia, hereditary thrombocytopenia Diagnosis/ Management CBC shows low platelet & Hb count Assess for hx of NSAIDS Avoid injury Good oral hygiene & skin care Rectal enemas & suppository-avoid constipation Avoid IM, SC injections, rectal temperatures Apply pressure on any bleeding source Monitor signs of bleeding Immune Thrombocytopenia Purpura( ITP) Autoimmune bleeding disorder Platelets coated with antibodies Destroyed by macrophages in liver & spleen Survival is 1-3 days instead of 8-10 Gradual onset & transient remissions occur Clinical Manifestations Petecchiae- small, flat, pinpoint red Purpura-numerous petecchiae Ecchymosis-large purplish lesions EpistaxisBleeding gums Heavy menses Complication=hemorrhage Diagnosis Platelet Ct<100, 000 Prolonged bleeding time with normal coagulation time Increased capillary fragility Positive platelet antibody test Bone marrow aspirate contains normal or increased megakaryocytes Management Cortecosteroids (prednisone)-suppreses the phagocytic response of Spleenic macrophages, depress antibody formation, reduce capillary permeability & bleeding time IV immunoglobulin (IVIG) Immunosuppressive therapy SplenectomyPlatelet transfusions Hemophilia Characterized by prolonged bleeding after surgical/ dental or small trauma or cuts Types Hemophilia A- factor Vlll-most common-80% cases Hemophilia B- factor lX deficiency-inherited gene Von Willebrand’s disease- deficient Vlll & platelet dysfunction Etiology Sex linked genetic disorder Transmitted by females but males express the disorder Carriers transmit the gene to half their daughters and the disorder to half their sons Males transmit the gene to all their daughters but none to their sons Clinical Manifestations Slow persistent bleeding from cuts/scratches Delayed hemorrhage-hours/days after injury Severe bleeding after dental surgery GI bleed Nosebleed Hematoma Prolonged APTT Treatment Goal is to stop bleeding Increase anti-hemophilic factor-give factor Vlll, lX Prevent complications Support therapy LEUKEMIA DESCRIPTION Malignant exacerbation in the number of leukocytes, usually at an immature stage, in the bone marrow May be acute, with a sudden onset and short duration, or chronic, with a slow onset and persistent symptoms over a period of years Affects the bone marrow, causing anemia, leukopenia, the production of immature cells, thrombocytopenia, and a decline in immunity LEUKEMIA DESCRIPTION The cause is unknown and appears to involve gene damage of cells leading to the transformation of cells from a normal state to a malignant state Risk factors include genetic, viral, immunological, and environmental factors and exposure to radiation, chemicals, and medications CLASSIFICATION OF LEUKEMIA ACUTE LYMPHOCYTIC LEUKEMIA (ALL) Mostly lymphoblasts present in bone marrow Age of onset is less than 15 years CNS manifestation common-leukemic meningitis Normally, the lymphocytes fight infection by making antibodies that attack harmful elements. But, in ALL, the cells are immature and overabundant. They crowd out other blood cells, and may collect in the blood, bone marrow, and lymph tissue. CLASSIFICATION OF LEUKEMIA ACUTE MYELOGENOUS LEUKEMIA (AML) affects the young blood cells (called blasts) that develop into a type of white blood cell (called granulocytes). The main function of granulocytes is to destroy bacteria. The blasts, which do not mature and become too numerous, remain in the bone marrow and blood. Age of onset is between 15 and 39 years Classification CHRONIC MYELOGENOUS LEUKEMIA (CML) Mostly granulocytes present in bone marrow Age of onset is after 50 years of age CML occurs mainly in adults and is rare in children CHRONIC LYMPHOCYTIC LEUKEMIA (CLL) Mostly lymphocytes (B cells) present in bone marrow Age of onset is after 50 years of age, Diagnosis CBC-WBC may be normal, decreased or elevated Decreased platelet Decreased Hb Bone marrow aspiration- increase in marrow cells LEUKEMIA ASSESSMENT Anorexia, fatigue, weakness, weight loss Anemia Bleeding (nosebleeds, gum bleeding, rectal bleeding, hematuria, increased menstrual flow) Petechiae Prolonged bleeding after minor abrasions or lacerations Assessment Elevated temperature Lymphadenopathy and splenomegaly Palpitations, tachycardia, orthostatic hypotension Pallor and dyspnea on exertion Headache Bone pain and joint swelling Leukemia ASSESSMENT Normal, elevated, or reduced white blood cell (WBC) count Decreased hemoglobin and hematocrit levels Decreased platelet count Positive bone marrow biopsy identifying leukemic blast phase cells Leukemia INFECTION A major cause of death in the immunosuppressed client Can occur through autocontamination or cross-contamination Common sites of infection are the skin, respiratory tract, and gastrointestinal (GI) tract Leukemia IMPLEMENTATION: INFECTION Initiate protective isolation procedure-also called Neutropenic Precautions or Reverse isolation Ensure frequent and thorough hand washing Reduce exposure to environmental organisms by eliminating raw fruits and vegetables (low-bacteria) from the diet, fresh flowers from the client’s room, and by not leaving standing water in the client’s room Avoid invasive procedures such as injections, rectal temperatures, and urinary catheterization Leukemia CLIENT EDUCATION: INFECTION Avoid crowds and those with infections Consume a low-bacteria diet and to avoid drinking water that has been standing for longer than 15 minutes Avoid activities that expose the client to infection such as changing a pet’s litterbox or working with houseplants or in the garden Neither the client nor their household contacts should receive immunization with a live virus Leukemia BLEEDING During the period of greatest bone marrow suppression (the nadir), the platelet count may be extremely low, less than 10,000/mm3 (bleeding risk) Examine the client for signs and symptoms of bleeding; examine all body fluids and excrement for the presence of blood Handle the client gently; use caution when taking blood pressures to prevent skin injury Measure abdominal girth, which can provide an indication of internal hemorrhage Provide safety-pad side rails and sharp corners of the bed and furniture Leukemia IMPLEMENTATION: BLEEDING Provide soft foods that are cool to warm in temperature Avoid injections if possible to prevent trauma to the skin and bleeding; apply firm, gentle pressure to a needle stick site for at least 10 minutes Avoid rectal suppositories, enemas, and thermometers If the female client is menstruating, count the number of pads or tampons used Administer blood products as prescribed Leukemia FATIGUE AND NUTRITION Assist the client in selecting a well-balanced diet Provide small, frequent meals (high calorie, highprotein, high-carbohydrate) that require little chewing Assist the client in self-care and mobility activities Allow adequate rest periods during care; do not perform activities unless they are essential Administer blood products for anemia as prescribed Leukemia CHEMOTHERAPY Induction therapy: Aimed at achieving a rapid, complete remission of all manifestations of the disease Consolidation therapy: Administered early in remission with the aim of cure Maintenance therapy: May be prescribed for months or years following successful induction and consolidation therapy; the aim is to maintain remission Leukemia IMPLEMENTATION Administer antibiotic, antibacterial, antiviral, and antifungal medications Blood replacements as needed Prepare the client for transplantation Administer colony-stimulating factors as prescribed Maintain infection and bleeding precautions Leukemia IMPLEMENTATION Provide an adequate diet Provide an activity schedule that will conserve energy Instruct the client in appropriate home care measures Provide psychosocial support and support services for home care Leukemias Acute Combination Chemo Induction Rest- 3 weeks Consolidation Maintenace follows remission- lasts 2-3 years Bone marrow transplant Chronic Treatment based on symptoms Radiation to spleen, nodes Interferon Autologous stem cell transplant Blood transfusions: RBCs, platelets, WBCs HODGKIN’S DISEASE DESCRIPTION A malignancy of the lymph nodes that originates in a single lymph node or a single chain of nodes; metastasis occurs to other adjacent lymph structures and eventually invades nonlymphoid tissue Usually involves lymph nodes, tonsils, spleen, and bone marrow and is characterized by the presence of the Reed-Sternberg cell in the nodes Possible causes include viral infections and previous exposure to alkalyting chemical agents Prognosis is dependent on the stage of the disease Etiology & Pathophysiology Unknown cause Linked to Epstein-Barr Virus Genetics- high among Jews Mechanism of growth & spread- unknown Cancerous cells transforms in lymph and progresses to other nodes or direct infiltration of blood vessels Clinical Manifestations Enlargement of cervical, axillary, inguinal lymph nodes may be painless B symptoms-Weight loss, Fever, night sweats Cough, stridor, dyspnea Hepatomegaly Splenomegaly SVC syndrome from intra thoracic involvement Diagnosis & Staging Peripheral blood analysis-microcytic, hypochromic anemia, leukocytosis Lymph node biopsy-definitive dx Bone marrow examination-staging( l-lV) CXR, CT Scan, radioisotope studiesdefine sites Lymphangiography-assess nodes & vessels Hodgkins Disease IMPLEMENTATION For stage 1 and 2 without mediastinal node involvement, the treatment of choice is extensive external radiation of the involved lymph node regions With more extensive disease, radiation along with multi agent chemotherapy is utilized HODGKIN’S DISEASE Implementation Monitor for side effects related to chemo or radiation Monitor for signs of infection & bleeding Maintain infection and bleeding precautions Discuss possibility of sterility with male client receiving radiation & inform of options related to sperm banks Pain management Client & family support If terminal- ensure client has dignified death NHL NON- HODGKIN’S LYPHOMA Group of malignancies with origins in lymphoid cells affecting all ages Most common occurring cancer & 5th leading cancer causing death Men are more affected than women; survival better for women than in men Incidence higher in whites than other races Painless lymph node enlargement is primary manifestation Symptoms will present on where disease has spread Diagnosis & Treatment Diagnostic tests are same as for Hodgkin’s disease-Lymph node biopsy done Treatment-radiation & chemotherapy Nursing assessment for nodes characteristics, B symptoms appearance Support for client & family Manage risk for infection, bleeding, sexual & reproductive dysfunction Nutritional management Multiple Myeloma Cancer of the plasma cell, an important part of the immune system that produces immunoglobulins (antibodies) to help fight infection and disease. Hypercalcemia, anemia, renal damage, increased susceptibility to bacterial infection, and impaired production of normal immunoglobulin are common clinical manifestations of multiple myeloma. It is often also characterized by diffuse osteoporosis, usually in the pelvis, spine, ribs, and skull. Diagnostics/ Management Diagnostics Radiologic studies- lesions in bones, demineralization, osteoporosis Bone marrow biopsy- immature plasma cells Abnormal immunoglobulin Monoclonal immunoglobulin chains in blood & urine exam Multiple Myeloma Management Chemotherapy, radiation tx Autologous bone marrow transplant Plasmapheresis Hydration, diuretics, phosphate for treatment of Hypercalcemia Pain management Supportive for anemia, leukopenia, thrombocytopenia