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Hematological System Alterations Diane L. McLean, RN, BSN, MSN Nursing 202 1 Learning Objectives: • Define terms associated with selected hematological alterations. • Describe selected hematological alterations. • Describe the pathophysiology for elected hematological alterations. • Describe the role of the nurse in providing care for patients experiencing selected hematological alterations. 2 Learning Objectives… • Explain health promotion related to selected hematological alterations. • Interpret clinical manifestations of selected hematological alterations. • Interpret diagnostic tests for selected hematological alterations. • Describe the pharmacological agents and/or treatments for selected hematological alterations. 3 Learning Objectives… • Evaluate nutritional considerations for patients experiencing selected hematological alterations. • Identify expected outcomes of treatment modalities for patients experiencing selected hematological alterations. • Evaluate psychosocial needs of patients, families, and/or support systems. 4 Learning Objectives… • Use critical thinking to manage nursing care for culturally diverse patients experiencing selected hematological alterations. • Evaluate expected outcomes of nursing care for patients experiencing select hematological alterations. • Assess the impact of selected hematological alterations on maternal and pediatric patients. 5 Anatomy and Physiology of Liver • The largest organ in the body • Produces bile, which emulsifies fat and stimulates peristalsis • Conveys bile to the duodenum at the sphincter of Oddi through the common bile duct • Metabolizes carbohydrates, fats and proteins • Synthesizes coagulation factors VII, IX, X and prothrombin • Stores Vitamin A, D, B12, and Iron • Detoxifies chemicals • Excretes bilirubin • Receives dual blood supply from portal vein and hepatic artery 6 Accessory Organs of Blood Formation •Spleen •Liver 7 Anatomy and Physiology of Spleen • Small hand-sized organ in front of the left kidney, behind the stomach The largest lymphatic organ Filters blood Traps formed particles Destroys bacteria Serves as blood reservoir Forms lymphocytes and monocytes 8 Splenectomy • • • • Used to treat: Idiopathic thrombocytopenic purpura Immune system destroys platelets ITP is sometimes called immune thrombocytopenic purpura. • ITP occurs when certain immune system cells produce antibodies against platelets. Platelets help your blood clot by clumping together to plug small holes in damaged blood vessels. • The antibodies attach to the platelets. The spleen destroys the platelets that carry the antibodies. • Spleen out…platelets remain in the blood stream 9 Splenectomy… • Small percent develop an accessory (extra) spleen…requiring removal • More successful in younger <40 • >65 higher complications and fatality rate • More difficult time recovering from pneumonia, hospital-based infections, meningitis, etc. 10 Splenectomy • 11 Introduction to Blood 12 13 Blood Components 14 Blood Components… • Plasma – a straw colored liquid portion of the blood in which the cells and platelets are suspended; makes up approximately 50-55% of a blood consists of water, amino acids, proteins, carbohydrate, lipids, vitamins, hormones, electrolytes and cellular wastes • Serum – is essentially the same as plasma only without the fibrinogen and clotting factors • The volume of blood is approximately 8% of total body weight 15 Blood Plasma Makes up 55% of blood volume Part of extracellular fluid (like ISF except 7% protein instead of 2%) Has 3 types of plasma proteins: ALBUMIN - to increase osmotic pressure GLOBULINS - to transport & for antibodies FIBRINOGEN - basic for blood clotting 16 Plasma 17 Normal Types of Blood Cells • • • • • • • RBC’s Basophils (WBC’s) Neutrophils (WBC’s) Monocytes (WBC) Eosinophils (WBC) B- Lymphocytes Platelet (Thrombocytes) 18 Anatomy and Physiology Erythrocytes: red blood cells (RBCs) Erythropoietin stimulates production O2 transport Thrombocytes: platelets Thrombopoietin stimulates production Clotting Leukocytes: white blood cells (WBCs) Fight infection and antigens Granular and agranular 19 20 Stem Cell Family Pressure 21 RBC’s Biconcave, disc shaped cells also known as erythrocytes The production of red blood cells are called erythropoiesis Erythropoiesis is when RBC’s are being formed within the bone marrow as erythroblasts, they mature and turn into normoblasts and form reticulocytes and they mature within the blood or spleen and become erythrocytes 22 23 Hematopoiesis – Formation of blood cells • Primary site is bone marrow • RBCs - 175 billion per day • Neutrophils - 70 billion per day • Platelets - 175 billion per day • Bone marrow will respond to inc. need with more production 24 Hemoglobin • Hemoglobin is contained within the red blood cells and is the oxygen carrying unit of red blood cells; it also carries CO2 to the lungs • The destruction of RBC’s is called culling which live approximately 120 days 25 The Path of a RBC 26 27 Cellular Components of Blood • ERYTHROCYTES - largest part; anuclear, biconcave disks; designed to carry hemoglobin; produced in red bone marrow; survive about 120 days • PLATELETS – non-nucleated cell fragments; help in homeostasis & coagulation; normall ct 150,000400,000; life span 10 days* • LEUKOCYTES - help fight infection & mediate the immune response 28 Leukocytes – White Blood Cells (WBCs) • NEUTROPHILS- first to arrive at site of injury; phagocytosis; *55-70%; life 2-4hrs* • EOSINOPHILS- regulate hypersensitivity reactions; seen in allergic reactions *1-4% • BASOPHILS- release heparin & histamine from granules into tissue*0.5-1% • MONOCYTES (Macrophages)phagocytosis; stimulate maturation of T cells *2-8%; stimulate inflammatory process* 29 Neutropenia • Abnormal low count of neutrophils • Adults: < 1700 / microliter of blood • Children varies with age VULNERABLE to infectious diseases • Severe neutropenia – < 500 cells per microliter of blood 30 Lymphocytes • ACTIVE IN IMMUNE RESPONSE • COMPOSED OF: B-cells - produce antibodies T-cells - mediate immunologic responses • MAKES UP 20-40% OF WBCs 31 Erythrocytes or Red Blood Cells (RBCs) • Size – Denoted by “CYTIC” • Normocytic • Microcytic • Macrocytic • Density – From amount of hemoglobin – Denoted by “CHROMIC” – Normochromic – Hypochromic – Hyperchromic 32 Erythropoiesis • Must have adequate space & perfusion in the bone marrow • Must have essential substrates like IRON, FOLIC ACID, VITAMIN B12 • Erythropoietin from the kidney stimulates it. 33 Bone Marrow • The bone marrow is the site of hematopoiesis or blood cell formation. 34 Bone Marrow Aspiration • Taken from posterior iliac crest most often • Patient needs explanation & signed consent form • Local anesthesia used for skin & SQ but not bone itself • Sharp pain during actual aspiration though brief • Watch for bleeding & infection 35 Bone Marrow Sites • Sites for bone marrow aspiration may include: –Sternum –Iliac Crest –Tibia 36 Bone Marrow Biopsy 37 Video • www.youtube.com • Bone Marrow Aspiration & Biopsy • 12-29-08 csmcd 38 • • • • • • • • • • • • • • Frequently Used Laboratory Tests in Hematology CBC RBC’s Hgb Hct MCV MCHC Reticulocyte Count WBC’s Platelets PT INR PTT Fibrinogen D- Dimer Coomb’s Test (used for blood typing )use direct Coombs 39 Complete Blood Count • RBC - RED BLOOD COUNT- actual count of circulating RBCs in 1 mm of blood M 4.5 – 6.0 ; F 4.0 – 5.5 million/mm3 • HCT - HEMATOCRIT - volume of packed RBCs in 100 mls of blood; expressed as %; nl M 40 – 50 %; F 37-47% • HGB - HEMOGLOBIN - protein substance that gives blood its color & is composed of iron that is an oxygen carrier; nl M 1418g/dl; F 12-16g/dl 40 Major Blood disorders and Lab Findings • Anemia – ↓ in Hgb & Hct • Polycythemia – ↑ in Hgb. & Hct. • Leukopenia & Neutropenia – ↓ in WBC & neutrophil count • Leukocytosis – ↑ in WBC • Thrombocytosis – ↑ in platelet count • Thrombocytopenia – ↓ in platelet count 41 Reticulocyte Count (Retic Count) • Normal - 0.5%-1.5 % of total RBCs • Measures the marrow’s production of erythrocytes ability to respond to anemia & make RBCs • Increased: – hemolytic anemia, – hemorrhage • • • • Decreased: pernicious folic acid iron def., Aplastic anemia 42 Coagulation Studies • • • • Bleeding Time 1-9 min Prothrombin Time (PT) 11 – 12.5 sec Partial thromboplastin time (PTT) 60 – 70 sec Activated Partial thromboplastin Time (APTT) – quicker to perform 60 – 70 sec • INR: 2.0 – 3.0 (1.5-2.5) • Fibrinogen • *** desired therapeutic ranges for anticoagulant = 1.5 to 2.0 times normal values 43 Erythrocyte Disorders • Loss of O2 carrying capacity of the blood due to low numbers or cells that don’t function adequately • Can categorize: – Decreased production – Increased destruction – Blood loss 44 What does Increased RBCs indicate?? 45 Defective RBC Production • Iron Deficiency • Vitamin B6 deficiency (megablastic) • Decreased erythropoietin production • Cancer/Inflammation 46 Bleeding (from RBC) Loss • Bleeding from the GI tract, Menorrhagia, epistaxis 47 Hemolytic (From destruction of RBCs) – Altered erythropoiesis • Sickle Cell Anemia • Thalassemia – Autoimmune Anemia – Drug Induced Anemia – Mechanical Heart Valve Related Anemia 48 Clinical Manifestations of Anemia • Severity of the anemia • Speed with which the anemia has developed - more rapid > severity • Duration of the anemia • Metabolic requirements of the individual - more active may be more symptomatic • Other disorders or disabilities 49 Physical Assessment Findings • SKIN: – pallor or icteric – petechiae or ecchymosis – tongue may be smooth • CHEST: – SOB or DOE – tachycardia, irregular rhythm, murmurs & gallops – orthostatic hypotension, • GI - may have large liver or spleen • CNS - impaired cerebral or peripheral nerve function; chronic def. B12 can cause irreversible neurologic degeneration 50 Assessment with Anemia… • Weakness, fatigue, malaise, pallor common • May see jaundice with megaloblastic & hemolytic anemia • Tongue may be smooth & red with iron def. or beefy red & sore with megaloblastic anemia • Mouth corners may be ulcerated (angular cheilosis) • Person may crave ice, starch, or dirt (pica) • Nails may be brittle, ridged, or concave • Nutritional assessment • Cardiac status for tachycardia, palpitations, dyspnea, dizziness, orthopnea, 51 Signs & Symptoms of Anemia 52 Assessment & Diagnoses • GI system: – – – – nausea & vomiting & diarrhea melena anorexia glossitis • Menstrual periods and pregnancy • Neuro Exam : – – – – emphasis on paresthesia ataxia poor coordination confusion 53 Assessment & Diagnoses… • Activity intolerance RT weakness, fatigue and malaise • Altered nutrition RT inadequate intake of essential nutrients • Altered tissue perfusion RT inadequate blood volume or hct • Noncompliance with prescribed therapy 54 Iron Deficiency Anemia • RBCs contain decreased amounts of hemoglobin due to decreased iron supply • RBCs are microcytic & hypochromic • Can result from: blood loss, ↑body demands, malabsorption, or dietary deficits • Peculiar S&S : glossitis, pagohagia, stomatitis, cheilosis • Treat with iron by mouth or injection 55 Hemochromatosis • Too much iron being absorbed from the GI tract • Primary: specific genetic problem – Most common genetic disorder in US – 1 of every 200 to 300 Americans • Secondary: caused by diseases – Thalassemia – Sideroblastic anemia – Large number of blood transfusions 56 Hemochromatosis… • Men more than women • Caucasians of Western European descent • Men ages 30 -50 • Women ages > 50 • Can occur age 20 • Familiar 57 Hemochromatosis… • Symptoms – Abdominal pain – Fatigue – Darkening of skin color (bronzing) – Joint pain – Loss of body hair – Weight loss – Liver and spleen swelling Confirmed with liver biopsy 58 Hemochromatosis… • Treatment – Phlebotomy: • ½ liter of blood removed each week until the body iron level is normal • May take months/years to accomplish • Specific diet to reduce iron from diet • Prohibits alcohol • Avoid iron cookware, raw seafood Overtime: liver scarring/damage. Extra iron builds up in other body tissues 59 Iron Administration • ORAL SUPPLEMENTS: – stools may be green or black – Take with ascorbic acid to inc. absorption – May need to drink with straw to prevent staining of teeth • BEST ABSORBED ON EMPTY STOMACH* • Take with or after meals to avoid GI upset-not with antacid • Intramuscular injection –should use Z-track method with needle change and 0.5ml of air to prevent leakage of med 60 Iron Supplement • Take Iron on an empty stomach because Iron absorption is reduced with food especially dairy products • Increase vitamin C to enhance absorption • Explain to pt that stool will be dark in appearance • Review Z track for Iron administration 61 Z track • Draw the medication up into the syringe using aseptic technique • Add 0.25 mL of air to the syringe • Discard the needle used to draw up the medication • Place a new needle 22 G – 2 – 3 inch long on the syringe • Make certain the needle is not in bright light • Select the dorsal gluteal ONLY • Once the site is selected, pull the sq tissue sideways away from the muscle (z track) • Cleanse the site and insert into the muscle tissue • Aspirate to determine placement • If blood is aspirated, discard and begin again • Inject the medication slowly 62 Iron Sources • • • • • • • • Organ meats - liver, red meats Kidney beans Iron-fortified cereals Dark, green leafy vegetables, legumes Whole grains Blackstrap molasses Dried fruit (raisins) Foods cooked in iron pans 63 Nursing Interventions • Managing fatigue • Maintaining adequate nutrition - well balanced diet • Maintaining adequate perfusion acute blood loss may require IV replacement with fluids or transfusions, oxygen & close monitoring - NOTE TACHYCARDIA • Complying with prescribed therapy understand medication regimen • Monitor for complications - CHF & neuro 64 Folic Acid Deficiency Anemia • Causes megaloblastic anemia • Similar to B12 deficiency without the nervous system manifestations • Caused by: Poor nutrition (esp. lacking leafy green veg., liver, citrus fruits, dried beans, nuts) as seen in alcoholics Malabsorption syndromes like Crohn’s . Drugs that inhibit folic acid metabolism like methotrexate, anticonvulsants, & oral contraceptives 65 Folic Acid Foods • • • • • • Liver Organ Meats Eggs Cabbage Broccoli Brussels sprouts 66 Vitamin B12 Deficiency Anemia • PERNICIOUS ANEMIA- caused by a deficiency of adequate intrinsic factor (IF) which is essential for the intestinal absorption of vitamin B12 • Macrocytic RBCs ; do Schilling test • S&S: smooth sore tongue, numbness & tingling • of hands & feet • Tx with B12 (cyanocobalamin) injections IM • OTHER CAUSES- inadequate dietary intake or poor absorption of vitamin B12 • Vitamin B12 foods: milk, eggs, meat, liver, OJ, spinach 67 Assess tongue carefully for different types of anemia’s 68 B12 foods • • • • • • • Liver Organ Meats Dried Beans Nuts Green Leafy Vegetables Citrus Fruits Brewer’s Yeast 69 • • • • • • Nursing Assessment: Vitamin B¹² Deficiency Chief complaint and past medical history Family history Review of systems Functional assessment Physical examination Signs of hematologic abnormalities: – Petechiae – Purpura – Ecchymosis 70 Anemias of Renal Disease • End stage renal disease: – shorten RBC life – cause a deficiency in erythropoietin • Dialysis patients: – lose blood in dialyzer & become iron deficient – possibly folic acid deficient • May need to take recombinant erythropoietin (Epogen) & oral iron supplements 71 Anemia of Chronic Diseases • Extremely common • RBCs may look normal • TIBC ↓, Fe ↓ – – – – infections cancer inflammatory disease malnourished state • Not understood but does not respond to Fe • Treatment: packed RBCs 72 Aplastic Anemia • Results from a deficiency of circulating erythrocytes resulting from arrested development of RBC’s within the bone marrow 73 Aplastic Anemia • Common causes: – Exposure to myelotoxic agents • • • • • • Radiation Benzene Alkylating agents Antimetabolites Sulfonamides Insecticides – Viral infection (unproven) • Epstein-Barr virus • Hepatitis B • Cytomegalovirus 74 Aplastic Anemia • Caused by bone marrow malfunction • Three problems together: – Erythrocytopenia, – Leukopenia – Thrombocytopenia • May present with: – Fatigue – pallor, – DOE • Treatment of choice is bone marrow transplant replaces the defective stem cells or removal of spleen that may be destroying normal RBCs or suppressing their development 75 Megaloblastic anemia • Characterized by macrocytic red cells • Main problem caused from defective DNA synthesis found in vitamin B12 or folic acid deficiency 76 Megaloblastic anemia… • Deficiency of Vitamin B12 – Poor intake of foods containing B12 • Vegetarian diets • Diets lacking dairy products • Problems of small intestine – – – – Small bowel resection Diverticulitis Tapeworm Overgrowth of intestinal bacteria leads to poor absorption of B12 77 Hemolytic Anemia • May be Inherited or Acquired • 200 disorders are known • Due to increased RBC destruction missing enzymes • RBC survival may be < 15-20 days • I. Sickle Cell Anemia • II. G-6-PD Deficiency Anemia III. Autoimmune Hemolytic Anemia • IV. Extrinsic Factors 78 Inherited Hemolytic Anemia • Sickle Cell • Thalassemia • G-6-PD 79 Acquired Hemolytic Anemia • • • • • • Liver disease Uremia Trauma Mechanical Heart Valve Infection DIC 80 Blood Loss Anemia • ACUTE : trauma to blood vessels or internal hemorrhage • Degree may be hard to tell since lowest hct may not be reached till 24hrs later & retic ct does not rise for 3-5day • May see low BP, inc. HR, weakness, cool skin & light headed • CHRONIC : gastrointestinal & menstrual bleeding • Associated with Fe deficiency so microcytic & hypochromic • ID source to stop & treatment by iron supplements 81 Sickle Cell anemia 82 Sickle Cell Anemia • Inherited autosomal recessive disorder of hemoglobin • Primarily persons of African an Eastern Mediterranean descent • Appears after 4 months old • Hemoglobin S (HgbS) replaces all or part of normal hemoglobin, which causes the RBCs to sickle when oxygen is released into the tissues 83 Sickle Cell Anemia… • HgbS less than normal lifespan: 40 days • Leads to chronic anemia • Crisis is characterized by severe pain that occurs when blood flow is obstructed • Treatment: hydration, oxygen, pain medication, and administration of Hydroxyurea 84 85 Pregnancy… • Autosomal recessive inheritance • Both parents must pass the defective form of the gene for a child to be affected • If only one parent passes the gene, that child will have the sickle cell trait • Two people with sickle cell trait: – 25% having unaffected child – 50% having a child who is a carrier – 25% having a child with sickle cell anemia 86 Clinical Manifestations of Sickle Cell Anemia • Vaso-occlusive crisis: the classic sign – Severe abdominal pain – Fever – Painful edematous hands & feet – Arthralgia – Leg ulcers (adolescents) – Delayed growth – Frequent infections – Anemia – CVA (↑ risk with dehydration) 87 Complications of Sickle Cell Anemia • • • • • • • • Stroke Acute chest syndrome Pulmonary hypertension Organ damage Blindness Skin ulcers Gallstones Priapism 88 Care of the Patient in sickle Cell Crisis • Oxygen therapy • Drug therapy –48 hours of IV analgesics • Hydrate the patient with normal saline and oral fluids without caffeine • Transfusion therapy • Do not raise knee gatch of the bed • Keep room temperature at or above 72 degrees 89 Drug therapy • • • • Morphine & hydromorphine(Dilaudid) Opioid addiction rare Avoid IM (absorption impaired) Moderate pain – NSAIDs – Complementary therapies • • • • • Warm room Distraction Relaxation Aroma therapy Therapeutic touch 90 Drug Therapy… • Hydroxyurea is a chemotherapy agent that has been shown to increase hemoglobin F levels thereby decreasing the permanent formation of sickled cells – Suppresses bone marrow function – Increased incidences of leukemia – Drug toxicity – Causes birth defects • Folic Acid Replacement • Treat infection promptly with Antibiotics (Haemophilus Influenzae) 91 Treatments…. • Bone marrow transplant only potential cure: hard to find donor • Blood transfusion: excess amount of iron – Deferasirox (Exjade) • Heavy metal chelating agent • Stem cell transplant – Long hospital stay – Life-threatening complications 92 Experimental treatments • Gene therapy – Insert normal gene into bone marrow for normal hemoglobin – Turning off defective gene while reactivating another gene that prevents sickle cells from forming • Nitric oxide – Prevent sickle cells from clumping together • Drugs to boost fetal hemoglobin production 93 Women’s Health Considerations • Pregnancy in women with sickle cell disease may be life threatening. Patients who have damage to vital organs are advised against becoming pregnant. The use of oral contraceptives is controversial, because Ocs may increase clot formation, especially among smokers, predisposing them to crises. The risks versus benefits must be examined for each patient 94 Thalassemia • Group of hereditary disorders associated with defective hemoglobin chain synthesis • Found in people of Mediterranean, Africa and Southeast Asian • Characterized by Hypochromia (abnormal decrease in the Hgb of RBC’s) and extreme microcytosis (smaller than normal RBC’s) and destruction of blood • Males and females • Lifespan: 20-30 years 95 Thalassemia… • There are two types: – Alpha (microytic ) • Stillborn • Bone deformities of face • Growth failure – Beta (can be fatal)…Cooley’s Anemia – Chelation therapy is used (too much iron that is removed after chronic transfusions) • Normal at birth develops severe anemia during 1st year of life – Blood transfusion/ folate supplements – Bone marrow transplant helps children 96 97 98 Alpha thalassemia (Hemoglobin Bart's) 99 Glucose-6-Phosphate Dehyrogenase Deficiency • Hereditary condition in which RBCs break down when body is exposed to certain drugs/stress of infection • Most common human enzyme deficit • “Favism”: allergic to fava beans • Common black males 100 G-6-PD… • Affected / episode triggered by certain medications: – ASA – Antimalarial – Nitrofurantoin – NSAIDS – Sulfa – Common: mothballs • Spontaneous recovery from a hemolytic episode is the usual outcome • Prevention: avoid triggers • **Benefit: confers a resistance to malaria** 101 Hereditary Spherocytosis • Type of hemolytic anemia • Characterized by abnormal permeability of the RBC membrane • Causes spherical shape and causes RBC’s to be destroyed prematurely and sent to the spleen • Spleen usually has to be removed 102 Red Blood Cell Disorders • Polycythemia Vera: production of too many red blood cells • Anemias: deficiency of red blood cells or hemoglobin caused by – Blood loss – Iron deficient diet – Vitamin B12 deficiency (pernicious anemia) – Bone marrow failure (hemolytic anemia) – Genetic abnormalities 103 Polycythemia Vera • Dark color of the skin 104 Polycythemia Vera –Cancer of RBCs • Malignant disease • Disease with a sustained increase in hemoglobin or hematocrit • Massive production of RBCs…hallmark • Excessive leukocyte production…hallmark • Excessive production of platelets…hallmark • Plethoric (dark, facial appearance from the blood is so oxygenated • Medical Treatment – If untreated may die within 2 yrs • Men > women; rare < 40 years of age 105 Symptoms • • • • • • • • • Breathing difficulty when lying down Dizziness Excessive bleeding Fullness in the left upper abdomen (due to enlarged spleen) Headache Itchiness, especially after a warm bath Red coloring, especially of the face Shortness of breath Symptoms of phlebitis 106 Polycythemia Vera Treatment • Phlebotomies (2-5 times/week) • Therapy aimed to prevent clot formation – Aspirin 107 Treatment • Reduce the thickness of the blood and prevent bleeding and clotting. • Phlebotomy is used to decrease blood thickness. One unit of blood (about 1 pint) is removed weekly until the hematocrit level is less than 45 (males) or 42 (females). Then therapy is continued as needed. • Chemotherapy (specifically Hydroxyurea) may be given to reduce the number of red blood cells made by the bone marrow. Interferon may also be given to lower blood counts. A medicine called anagrelide may be given to lower platelet counts. 108 Patient Education /guide for Polycythemia Vera • Drink at least 3 Liters of liquids a day • Avoid tight or constrictive clothing • Wear gloves when outdoors in temperatures lower than 50 degrees F • Take anticoagulant as ordered • Wear support hose • Elevate feet whenever possible • Use electric razors • Use a soft bristled toothbrush • Do not floss between teeth 109 77-year old female 110 WBC and Immune Disorders • Immunocompromised patient – Defect in WBC or immune physiology 111 Immunocompromise • • • • Defects in WBCs or immune physiology Lack of normal defenses May be asymptomatic Infection is leading cause of death 112 Immunocompromise… • Symptoms of infection often absent • Fever may be only sign of infection • Pain without signs of inflammation is also a cue • Anergy may be noted with the use of skin tests 113 Laboratory Analysis • • • • Leukopenia Low CD4 counts Decreased immunoglobulins No response to antigen skin test 114 Causes of Leukopenia • Primary cause: – – – – – – – – – – extensive neutrophil use reduction in the number of neutrophils. chemotherapy radiation therapy leukemia Myelofibrosis aplastic anemia, influenza Hodgkin's lymphoma Folate deficiencies, psittacosis and sepsis Other causes are deficiency in certain minerals, such as copper and zinc. 115 Medical Management • Reverse the cause – Cell replacement – Bone marrow transplant – Immunoglobulins • Treat infections • Nutrition 116 Nursing Management • • • • • • Isolation Hygiene Aseptic technique for procedures Good assessment for cues of infection Maintain skin integrity Optimum nutritional support 117 Neutropenia • Absolute neutrophil count less than 1000 • Causes – Inadequate production – Excessive destruction • High risk of infection 118 Neutropenia Risks • Overwhelming infection • Radiation therapy • Chemicals and drugs – Antibiotics – Chemotherapy • Disease states 119 Assessment of Neutropenia • Classic symptoms of infection often absent • Areas of heavy bacteria at risk for infection (mouth, perineum, IV sites, catheter) 120 121 122 Neutropenia: Medical Management • • • • Prevent and treat infection Reverse cause Colony-stimulating factors Prophylactic antibiotics 123 Neutropenia: Nursing Management • Similar to that used for immunocompromised patient 124 Malignant WBC Disorders Leukemia Lymphoma Multiple myeloma 125 Leukemia • Malignant neoplasm of the blood-forming organs • Abnormal over production of immature forms of any of the leukocytes • Interference with normal blood production that results on decreased number of RBCs and platelets 126 Leukemia… • Exact cause: unknown • Precipitating factors: – Highest in children 3 to 4 years – Declines until age 35 then steady ↑ 127 Assessment Findings • • • • • • Petechiae Ecchymosis Enlarged Lymph nodes Joint pain Hematemesis Diagnostics show decreased RBC’s and platelets, immature WBC’s and bleeding time • Bone marrow biopsy shows large number of immature leukocytes 128 Leukemia… • Acute leukemia – Bone marrow cells cannot mature properly – Immature cells continue to reproduce and build up • Chronic leukemia – Cells can mature partly-not completely – These cells are not normal – Survive longer – But cells continue to build up and crowd out normal cells 129 Medical Management • Radiation • Chemotherapy • Transfusion therapy of platelets, packed RBC’s whole blood • Antipyretics • Analgesics 130 Leukemia Interventions • • • • • • Infection: major cause of death Sepsis common complication Auto-contamination Cross-contamination Drug therapy Hematopoietic stem cell transplantation 131 Review • Page 903 –Chart 42-6 –Need to know the differences 132 Acute Myeloid Leukemia (AML) • Group of disorders characterized by replacement of the bone marrow with abnormal immature cells of myeloid • Nonspecific symptoms: – Anorexia – Malaise – Irritability – Weight loss – Fever – Bone pain 133 AML… • Most common adults > 65 years • Males > female • Previous childhood ALL • Causes: – DNA mutations – Radiation, exposure to certain chemicals and some chemotherapy drugs may play a role. 134 Acute Myeloid Leukemia (AML)… • Treatment –Bone marrow transplant –Chemotherapy –50% cured/remission 135 Acute Myeloid Leukemia (AML)… 136 Chronic Myelogenous Leukemia • Rare in children • Uncommon in people < 20 years • Philadelphia Chromosome found in 9095% • Chromosomes switch (9 & 22) and combine creating a new gene: BCR/ABL (which is found in virtually all patients with CML) 137 CML… • Mostly males • Causes: – High levels radiation – Atomic bomb survivors • Symptoms: – Tiredness – SOB with activities – Pale looking skin – Enlarged spleen – Night sweats – Weight loss 138 CML… • 3 phases: – Chronic • Slowing progressing – Accelerated phase • Spleen enlargement • 6-12 months – Blast phase • Aggressive acute leukemia Are not cured! Remission long periods 139 Acute Lymphocytic Leukemia (ALL) • Malignant disease of bone marrow • Most common Leukemia in children • Physical signs: – Pallor – Cardiac flow murmur – Fever/signs of infection – Petechiae of LE’s – Rashes from infiltration of the skin with leukemic cells – Prognosis: 80% of children >years or longer 140 Chronic Lymphocytic Leukemia (CLL) • Cancer of blood and bone marrow • Progression slow/affects WBC’s • Rare symptoms in early phase – Enlarged but painless lymph nodes – Fever – weight loss/loss of appetite – Lethargy/fatigue – Night sweats – Pain/fullness below ribs left side 141 Chronic Lymphocytic Leukemia (CLL)… • Causes: damage to the DNA of developing cells in the bone marrow • Researchers: discovered people with CLL have chromosome deletions. • Affect older adults ≥ 60 /Caucasians • Not inherited/few risk factors – Herbicides – insecticides 142 Hodgkin Disease • • • • • Malignancy of the lymphoid system Painless Lymphadenopathy Cause unknown Prognosis good: 5 year 90% Diagnosis: excision of node for biopsy – Reed-Sternberg cells 143 Hodgkins Disease… • More common in men and has two peaks 15-35 years of age and 55-75 years of age; incidence is higher in Caucasians than in African Americans • The cause of the disease is unknown although several factors have been identified to contribute to the development of the disease including Epstein Barr virus (EBV) and exposure to toxins 144 Hodgkin's… • • • • • • 1% of all cancers USA 20-30 years of age….50 or greater 6th most common cancer in USA males 5th in woman Steadily increasing Actually a group of >30 types of cancers 145 Staging of disease • Stage l – Single lymph node region/site • Stage ll – Two or more lymph nodes on same side of the diaphragm or site • Stage lll – Lymph node areas on both sides of the diaphragm to one extralymphatic organ, the spleen, or both • Stage lV – Diffuse one or more extralymphatic organ, with or without lymph node involvement 146 Non-Hodgkins Lymphoma • Non Hodgkin's Lymphoma is type of cancer than originates from lymphoid tissue that are not diagnosed as Hodgkin's Lymphoma • Treatment: – chemotherapy – radiation – multiagent chemotherapy 147 Non-Hodgkins Lymphoma… • Symptoms: – Swollen lymph nodes in your neck, armpit or groin – Abdominal pain or swelling – Chest pain, coughing or trouble breathing – Fatigue – Fever – Night sweats – Weight loss 148 Multiple Myeloma • Multiple myeloma: cancer of your plasma cells, a type of white blood cell present in bone marrow. • Plasma cells normally make proteins called antibodies to help fight infections. • In multiple myeloma, a group of plasma cells (myeloma cells) becomes cancerous and multiplies, raising the number of plasma cells to a higher than normal level. • Since these cells normally make proteins (antibodies), the level of abnormal proteins in your blood also may go up. • Health problems caused by multiple myeloma can affect your bones, immune system, kidneys and red blood cell count. 149 Multiple Myeloma… • • • • • • Rare in people younger than 60 High American Black Males Incurable “Spike” pattern of plasma proteins IgG, IgA Treatment varies 150 Multiple Myeloma… • 4 Common Major Problems – Ca++ high levels – Kidney failure – Anemia-related fatigue – Bone damage and fracture CRAB 151 Multiple Myeloma… • Symptoms: – Common bone pain • • • • Back Pelvis Ribs Skull – Repeated infections – obesity 152 Thrombocytopenia • Decreased platelets – <100,000µl • Risk for bleeding • Treated with platelet transfusion 153 Care…Thrombocytopenia • • • • • • • • • • Handle the patient gently Use a sheet to life patient Avoid IM injections Apply firm pressure to all sticks Do not perform rectal temps, give enema’s & tell to avoid intercourse Teach to use electric razors Soft bristled tooth brushed Avoid dental work Encourage not to blow nose Check all urine and stool for blood 154 Thrombocytopenia… 155 Idiopathic Thrombocytopenic Purpura (ITP) • • • • • • Low number of platelets Cause unknown Purpura : skin or mucous membrane Petechiae: red/purple dots Nosebleeds, gums bleed, heavy menses ? Immune system might be the cause: your immune system attacks and destroys its own platelets: for an unknown reason 156 Idiopathic Thrombocytopenic Purpura (ITP)… • Risk factors: – Women 2X > males – Age: common > 60 years – Recent viral infection • Bleeding rare with ITP • Complications arise from treatment – Corticosteroids/surgery Children: follows viral (mumps/flu) Clears on its own within 2-8 weeks with or without treatment 157 Thrombotic Thrombocytopenic Purpura • TTP • Rare disorder: platelets clump together abnormally in the capillaries & too few platelets remain in circulation • Inappropriate clotting yet blood fails to clot properly when trauma occurs • Treatment: plasmaphersis, fresh frozen plasma, aspirin, immunosuppressive therapy 158 Vitamin K deficiencies • Proper liver function and bile production are critical for formation of vitamin K 159 Hyperhomocystinemia • Homocysteine – causes endothelial dysfunction…leads to smooth muscle growth = atherosclerosis • Inherited/acquired early stage of renal failure/renal transplantation/vitamin B12 deficiency • High levels = risk factor for ischemic stroke • Hyperhomocystinemia = Independent risk factor for vascular disease 160 Bleeding Disorders • Abnormality in stages of clotting – Vasoconstriction – Creation of platelet plug – Development of clot – Fibrinolysis • Inherited or acquired • Common in renal, hepatic, and gastrointestinal disorders; malnutrition 161 Tests Measuring Bleeding and Coagulation • CBC – Hemoglobin – Hematocrit • Fibrinogen • Prothrombin time • International normalized ratio • Partial thromboplastin time • Platelet agglutination/aggregation 162 Nursing Diagnoses • • • • • Risk for bleeding Pain Deficient fluid volume Altered self-image Altered tissue perfusion 163 Coagulation Disorders • Thrombocytopenia – Idiopathic (ITP) – Thrombotic Thrombocytopenic Purpura (TTP) • Hemophilia – Genetic disorder – Absence of Factor VIII – Treated with replacement of missing clotting factor 164 Von Willebrands Disease • The disease is caused by a deficiency of factor VII 165 Hemophilia • Hemophilia comprises two hereditary bleeding disorders resulting from deficiencies of specific clotting factors • Hemophilia A results from a deficiency of factor VIII • Hemophilia B (Christmas Disease) is a deficiency of factor IX • Hemophilia is an X recessive trait • Hemophilia A is a disease affecting males, non of whose sons will have the gene for Hemophilia and all whose daughters will be carriers 166 Medical Management: Bleeding • • • • • • • • • Whole blood Packed RBCs Leukocyte-poor RBCs Platelets Cryoprecipitate Albumin Granulocytes Plasma protein Fresh frozen plasma 167 DIC • DIC is a syndrome characterized by abnormal initiation and acceleration of clotting and simultaneous hemorrhage • Risk Factors for DIC include venomous snakebite, sepsis, trauma, obstetric complications, hypoxia, tissue necrosis, drug reaction, extensive burns, prosthetic devices 168 Pathophysiology of DIC • Initiating event: procoagulants • Stimulation of intrinsic or extrinsic pathway • Clots in microvasculature • Consumption of clotting factors • Fibrinolysis • FDPs: potent anticoagulants 169 Etiology of DIC • Infection • Trauma (e.g., burns, crush) • Obstetric conditions (e.g., Abruptio, amniotic fluid embolus, retained dead fetus) • Hematologic disorders • Oncologic disorders • Other: shock or sepsis, acute respiratory distress syndrome 170 Assessment of DIC • Evidence of occult bleeding • Signs of platelet deficiency – Petechiae – Ecchymosis 171 Clinical Manifestations of DIC • Integumentary – pallor, purpura, ecchymosis, hematomas, gingival bleeding, bleeding from puncture sites • GI – Hemoptysis, Melena, Occult Blood, Abdominal Distention, abdominal pain • Respiratory – dyspnea, tachypnea, orthopnea, decreased breath sounds, CP • Cardiac – decreased pulses, tachycardia, venous distention • Genitourinary – hematuria, 172 Laboratory Diagnosis: DIC • • • • • • • Decreased platelets Decreased fibrinogen Prolonged PT, aPTT, thrombin time Elevated FDP or FSP Increased D-dimer Decrease in coagulating factors Decrease in hemoglobin and hematocrit 173 Treatment of DIC • Correct underlying cause • Administer blood and components – Platelets – Fresh frozen plasma – Cryoprecipitate – Packed RBCs • Stop abnormal coagulation – Heparin: controversial when experiencing more hemorrhage than thrombosis 174 Nursing Management of DIC • • • • • • Assess and prevent Frequent laboratory analysis Administration of blood products Assess circulation Relieve pain Assess for complications: shock, multisystem organ failure 175 Transfusion Therapy • • • • • • • • • Assess lab values Verify MD orders Assess baseline vital signs Need IV access with #20 Jelco or greater Obtain blood product from blood bank Check with two RN’s Use only normal saline because of hemolysis Blood must be hung within 15 minutes CANNOT hang longer than 4 hours because of bacterial growth • Remain with patient during first 15-30 minutes of infusion • Administer blood using the appropriate filter 176 177 Possible Diseases from Transfusions • • • • • Hepatitis B, C AIDS CMV (Cytomegalovirus) Graft-Verus-Host Disease Crutzfeldt-Jacob Disease (fatal) – Mad Cow Disease – is a rare, degenerative, invariably fatal brain disorder – Unknown, inherited or disease is transmitted by exposure to brain or nervous system tissue 178 Platelet Transfusions • Platelets are administered to patients with platelet counts below 20,000 mm and to patients with thrombocytopenia • Platelets are administered • rapidly since they are fragile • Standard sets are not used 179 Plasma Transfusion • Freezing preserves the clotting factor • FFP – or fresh frozen plasma is infused immediately after thawing while the clotting factors are still viable • ABO compatibility is required for transfusion of plasma 180 Cryoprecipitate • Cryoprecipitate is a product derived from plasma • Clotting factors VIII von Willebrands factor, fibrinectin, and fibrinogen are precipitated from pooled plasma to produce cryoprecipitate 181 Transfusion Reactions • Can occur when whole blood, PRBCs, platelets, or FFP are given • 4 types of reactions can occur: – Hemolytic – Anaphylactic – Febrile – Circulatory overload 182 Hemolytic Transfusion Reaction • Caused by blood type or RH incompatibility • These complexes can destroy the transfused cells and initiate inflammatory responses in the recipients blood vessel walls and organs • S/S include apprehension, HA, CP, low back pain, tachycardia, tachypnea, hypotension, hemoglobinuria 183 Allergic Transfusion Reaction • Allergic reaction are most often seen in patients with a history of allergy • They may have urticaria, itching, bronchospasms or occasionally anaphylaxis • Onset usually occurs within 24 hours after transfusion • Can receive “buffy coat” or washed cell 184 Febrile Transfusion Reaction • Febrile Transfusion reaction occur most commonly in the patient with anti-WBC antibodies, a situation seen after multiple transfusions • S/S – sensation of cold, tachycardia, fever, hypotension, tachypnea • Leukocyte filters may also be used to trap WBC’s 185 Bacterial Transfusion Reaction • Bacterial transfusion reaction are seen after transfusion of contaminated blood • Usually a gram negative organism • S/S – fever, chills, shock, tachycardia, hypotension 186 Circulatory Overload • Circulatory overload can occur when a blood product is administered too quickly • Usually associated with whole blood • S/S – hypertension, bounding pulse, distended jugular veins, restlessness, confusion 187 Transfusion-Associated GraftVersus-Host Disease • Seen in patients who are immunocompromised • Involved the donor attacking the host tissues 188