Hematologic Disorders & Nursing Priorities Hematology-study of blood and blood forming tissues that includes bone marrow, blood, spleen and lymph system Keith Rischer RN, MA, CEN

Objectives for Today Review pathophysiology related to hematologic cells and blood forming tissues Interpret significance of altered hematologic lab values Review commonly used medications that alter hematologic function Identify the patho, clinical manifestations, diagnostic tests, nursing priorities, and client education in clients with anemia, sickle cell anemia, leukemia, lymphomas, and multiple myeloma. Identify the nursing priorities with blood transfusion and the most common transfusion reactions.

Blood Cells Hematopoesis: Red bone marrow The blood forming tissue that produces the 3 major cell components of blood Erythrocytes Leukocytes Thrombocytes Hematopoesis-blood cell production within bone marrow-produces 100 billion new RBC daily HOW MANY RBC’S IN DROP OF BLOOD?...250 million in average lab tube 10 billion RBC 6 liters of volume=6000cc=60,000 gtts= how many in body?....15,000,000,000,000 hemopoietin, produced by the kidney, and is the hormone that regulates the red blood cell production Bone Marrow-soft material that fills central core of bones Red marrow-actively produces blood cells and found in the flat irregular bones…femur, pelvic, sacrum, sternum, ribs, scapula Bone marrow is stimulated by erythropoetin that influences differentiation of the different blood components Stem Cell- all components derived from this non-differentiated immature blood cell found in the red marrow Why are renal failure patients prone to anemia???

Erythrocytes Function Normal Life span: 120 days Norms Transport of gases (O2 & CO2) Erythropoesis Normal Life span: 120 days Norms Hgb RBC Women: 12-16 g/dl 4.0-5.0 mm3 Men: 13.5-18n g/dl 4.5-6.0 mm3 HCT Women: 38-47% Men: 40-54% In order for erythropoesis to occur need certain vital nutrients-IRON, FOLIC ACID, VIT B12 (cobalmin) B vitamins (B2,6) Regulated by cellular O2 requirements and stimulated by hypoxia Why do smokers have higher incidence of blood clotting that can contribute to MI or embolic CVA??? RBC Comprised of heme (iron compound) and globin (simple protein) Small disc with a bi-concave shape optimal for gas diffusion and can change to a torpedo shape….can squeeze through capilaries that are 1/3 the size of a single RBC HGB is standard to assess adequacy of RBC production in patient Hemolysis done in liver, bone marrow and spleen and causes release of….BILIRUBIN. If liver damaged/failure/cirrhosis…JAUNDICE

Leukocytes Types Granulocytes (Also known as polymorphonuclear leukocytes) Neutrophils Eosinophils Basophils One drop of Blood can contain from 7,000 to 25,000 white Blood cells Leukocytes originate from stem cells within bone marrow 5 different types…classified as either granulocytes or agranulocytes or by function as either phagocytes or immunocytes Granulocytes contain granules within the cytoplasm-granules contain enzymes capable of killing micro-organisms and ingested debris during phagocytosis Primary function is phagocytosis-pacman cells neutrophil - the first line of defense, 100 billion mature neutrophils are released into the body everyday. Neutrophil-The primary phagocytic cells involved in acute bacterial infection responses…migrate to bacterial invasion source and search and destroy bacterial invaders and then die in 1-2 days most common-50-70% of all WBC’s…if >70% consider infection Bands-immature neutrophils-present only with overwhelming sepsis-often referred to LEFT SHIFT Eosinophils-2-4% Engulf antigen-antibody complexes formed during an allergic response; defend against parasitic infections. Elevated in allergic responses Basophils: Cont. granules consisting of heparin, serotonin & histamine which are released during allergic & inflammatory reactions. Basophils-2% or less…function similar to mast cells and are elevated in inflammatory states

Leukocytes Normal Blood Count of all WBC: 4,000-11,000/ul Monocytes agranular) Lymphocytes B cells: mediate the humoral immune response T cells: Mediate cellular immunity Normal Blood Count of all WBC: 4,000-11,000/ul Elderly considerations Monocytes-4-8% -are immature macrophages--Ingest bacteria, dead cells, tissue debris & old or defective RBC’s. When monocytes leave the blood and enter the liver, spleen and lymph nodes they differentiate into macrophages. Lymphocytes-20-40%...are the primary cells of the immune respone-transiently circulate and then reside in lymphoid tissue as mature T cells B cells will see elevated with viral infection as well as immune response >11,000 or <4000 consider infection Granulocytes may live for hours…T lymphocytes may live for years

Thrombocytes (Platelets) Function: Aid in blood clotting Maintain capillary integrity by working as “plugs” to close any openings in the capillary wall. Normal Blood Count: 150,000-400,000 mm3 First response to clotting cascade is platelet aggregation-work as a plug…WHY GIVE ASA W/CP or AMI??? When inactivated are smooth but when activated become entangled

Anemia Mild Moderate Severe Hgb 10-14 g/dl Hgb 6-10 g/dl DEFINITION-reduction in the # of RBC, amt of HGB or HCT Result from dietary deficiencies, genetic disorders-bone marrow disease Gi bleeding most common Mild- asymptomatic Physical appearance:skin, mucous membranes, lips, nail beds and conjunctivae become pale Mod-weakness, fatigue, palpitations, SOB, decreased tol to activity-orthostatic hypotension Thinner blood flows faster through CV system causing hyperdynamic circulatory state that results in increased stroke volume and HR Severe-decr. LOC,dizziness, CP, tachy, SOB…WHY…CO=SVxHR, tachypnea, hypotensive, pallor hypotension & Vasoconstriction diverts blood flow to kidneys activates renin-angiotensin response What lab value will be elevated if kidneys take a hit??? Impaired O2 supply to GI system can cause abd pain, N&V Because RBC’s transport O2, anemias can lead to tissue hypoxia which accounts for many of the clinical manifestations of anemia What are some signs/sx’s of tissue hypoxia? Hypoxia: confusion, SOB,skin pallor- and MM and nailbeds, dizziness, weakness, tachycardia, Decr Hgb and HCT LIFECYCLE CONSIDERATION_ elderly men lose 1 gm HGB because of decreased production of androgens---women do not have this problem

Anemia:Causes Macrocytic Pernicious Anemia (B12 deficiency) Folate deficiency Microcytic Iron deficiency anemia Normocytic Blood loss Sickle cell anemia Not a specific disease; rather a manifestation of a pathological process. Once identified, determine the cause. Fundamental common pathology is reduced oxygen carrying capacity of the blood resulting in tissue hypoxia

Macrocytic Anemia Megaloblastic Anemias: Presence of large RBC’s) Caused by defective DNA synthesis Two common types: 1. Cobalamin (vitamin B12 deficiency) Pernicious anemia =most common cause. 2. Folic acid deficiency Poor nutrition (Anorexia) malabsorption in small bowel ETOH Hemodialysis PATIENT EDUCATION Megaloblastic Anemias -when DNA synthesis impaired-defective RBC maturation results… RBC’s are large, abnormal easily destroyed due to fragile membranes Produced by extra large stem cells that mature to unusually large erythrocytes Hgb content is normal compared to normal RBC Pernicious Anemia- refers to the history of this disease as highly destructive and fatal protein called intrinsic factor is required for Vit B12 absorption-this is secreted by the parietal cells in the gastric mucousa no intrinsic factor secreted due to antibodies directed against parietal cells and or intrinsic factor B12 essential for DNA synthesis of RBC These defective erythrocytes die prematurely causing anemia Other causes of cobalmin deficiency are gastrectomy, gastritis, ETOH abuse… and those on LT H2 blockers such as Pepcid Takes 20-30 years to become symptomatic Early sx vague-but once Hgb decreases to 7-8 becomes symptomatic—weakness, fatigue etc. Will need Vit B12 injections for life—initially weekly then monthly…death as complication now due only to non-compliance Folic acid essential vitamin required for RNA/DNA synthesis in erythrocyte Can only be obtained through diet…400mcg/day FOLIC ACID deficiency-Folic acid required for DNA synthesis to lead to RBC formation Dialysis- folic acid lost during dialysis Foods high in folic acid or supplementation teaching Leafy green veges, liver, citrus fruits, beans, nuts and whole grains

Microcytic Anemia: Iron Deficiency Abnormal-small erythrocytes…decr. Hgb Most common anemia Manifestations Pallor Glossitis fatigue Dietary sources Patient education Most common anemia in world 30% worlds population have this problem….malnutrition. In US 5-10% Most susceptible-children, malnourished, women in reproductive years…WHY? Men need 10 mg daily…women 18mg…pregnant women 30mg Patho-daily blood loss of 2-4cc/daily enough to cause or inadequate dietary of iron Body is able to recycle majority of iron from destroyed RBC-blood loss destroys this balance Only 1 mg of every 10-20 mg ingested is absorbed in duodenum…translates to 5-10 % absorption Iron-supplements if needed-l Ferrous Gluconate 325mg-65mg is elemental iron only Nursing: Teach your patient about dietary sources of Fe, along with how to properly take Fe supplements. Requires acidic environment to promote absorption-therefore take on empty stomach Take with Vit C also enhance absorption Lifespan Consideration Elderly – anemia is commonly unrecognized and/or untreated…mental confusion, memory loss, disorientation may be anemia related Nursing Assessment - thorough history-taking, gathering subjective/objective data Fe supplements teaching… .black stools…why? Constipation, take 1 hour before meals…why? Vit C in cooridination will increase absorption PT EDUCATION-What ? Could be asked of any elderly pt. that would help identify possible undiagnosed anemia (check most recent CBC) Any change in level of activity or energy? Any SOB at rest or with exertion? Assess diet Risky patients-premenopausal-pregnat women, poverty, elderly Education- diet, meds and when to take, need to take iron for 2-3 months after hgb returns to normal to replenish stores PLAY GAME

Normocytic Anemia: Etiology Blood Loss Acute Chronic Extrinsic (acquired) hemolytic anemias – (damage to RBCs due to external factors) Physical factors Acute-what will change first in acute bleed…HR, BP or both What are positive orthostatic BP findings? Body can compensate for low hgb if slow bleed or chronic anemia Tx-replace lost volume with crystaloid such as NS 500-1000cc til responds with stabilization in VS. 2-4 liters not uncommon in emergency…what is the potential problem…can NS carry O2-definitive tx-get T&C and transfuse PRBC’s? Remember: Signs and symptoms are valuable indicators of blood loss. Rx: *Replace blood volume*Oxygen*ID source of hemorrhage*RBCs as needed Chronic-Geriatric men-common to become anemic as they age. (hemorrhoids, heavy menses). Rx: ID source and stop bleeding, Fe supplements. UGI Bleed: black, tarry stools….Why? Damage due to exertion of extreme force on the RBC’s Physical-dialysis, heart-lung bypass machine w/CABG, prosthetic heart valves…even seen with severe atherosclerosis (DM)

ED Case Study 88 yr women w/dk tarry stools last 5 days. c/o weakness, nausea. Pale, cool-initial VS 80-16-124/30….2 hours later 96-20-94/49 Wbc 9.8, hgb 6.9 (was 12.7 2 weeks ago), hct 21.5, plt 176, INR 4.8 (was 2.1 2 weeks ago) Nursing priorities

Sickle Cell Anemia Patho Sickle Cell Crisis Nsg Management Pain control Hydration Patient Education Tx infection Psychosocial Sickle cell-inherited recessive genetic trait-results in abnormal form of hgb on the RBC that elongates and stiffens in response to low O2 levels…fatal most ofetn in mid adulthood due to renal and pulmonary failure A family of genetic disorders affecting African-Americans predominantly. Systemic: RBCs form a sickle shape=clog capillaries resulting in hypoxia of tissues Dominant in African Americans-1:400 births, Hispanic 1:1000-1400 Sickling episodes triggered by low oxygentension in the blood-caused by infection, stress, dehydration and high altitudes….sickled cells cannot pass easily through capillaries and cause micro vascular acclusion which leads to further hypoxia… can last for hours to days Avg lifespanRBC 120 days-sickle 12-15 days…leads to disposition to chronic anemia…hypoxia…sickle cell crisis Sx-severe pain-high doses narcs-one ED pt received up to Dilaudid 16 mg IM in one visit-this is equal to almost 100mg Morphine! Concerns for drug seeking exist Sickle cell crisis-vasoocclusive crisis-impaired blood flow—cellular hypoxia—leads to thrombus development—furthur tissue ischemia—increased pain Pain in back, chest, abd and extremities most common Heart failure, kidney failure due to increased blood viscosity and lack of O2, CVA common complications as well as infection susceptibility because spleen becomes unable to phagocytize foreign substances as it becomes infarcted and dysfunctional by the age of 2-4 years Are usually asymptomatic but are consistently anemic How to assess African American??? Pallor of mucous membranes Nursing Management -O2 to treat hypoxia…Pain control with large doses of Narcotics…IV hydration… Education –understanding of disease process, maintain fluid intake, treat any infection promptly, avoid high altitudes..psychosocial support due to chronic pain/depression refer as needed

Thrombocytopenia Reduction of platelets below normal range Normal = 150,000-400,000 mm3 Etiology: Immune Thrombocytopenic Purpura (ITP) Heparin Bone marrow suppression Critical values 50,000 or less- risk of bleeding <20,000 spontaneous life threatening hemorrhages (brain bleed) <10,000 transfusions recommended Most cases are acquired by dz processes such as spleenmegaly, bone marrow suppression (chemo or infectious) or HEPARIN Immune Thrombocytopenic Purpura (ITP): Abnormal destruction of circulating platelets by the spleen - caused by an autoimmune reaction –platelets coated with antibodies—spleen sees as foreign and destroyed— Normal life of platelets 8-10 days---ITP 1-3 days Heparin induced thrombocytopenia -platelets decrease due to immune response to Heparin… Tx-stop heparin and use alternative anticoagulants such as argatroban or lepirudin Any pt on Heparin IV or subq routinely monitored for platelet decreases-watch closely! Bone marrow suppression-chemo most common Very important #’s: 50,000 or less- risk of bleeding <20,000 spontaneous life threatening hemorrhages (brain bleed) <10,000 transfusions recommended

Clinical Manifestations Petechiae Purpura Ecchymosis Bleeding Purpura (Reddish skin bruise) Ecchymosis (Flat, raised, occ. painful) Prolonged bleeding and internal bleeding *Bleeding can be insidious or acute; external or internal – What are some general symptoms the nurse would be monitoring for? VS changes with routine VS to watch for….what will you look for? Why? Don’t forget neuro changes-decreased LOC, confusion, dizziness

Nursing Management If acute care-Peripheral IV established No ASA products for pain control Prevent/control acute bleeding Platelet transfusions-assess for reaction Steroids-pt. teaching Education-signs of bleeding Nsg care-make sure has at least 20 gu.-preferably 18 guage available and in place. Assess patency IV….what will you assess each shift? Why no ASA? Steroid education?...take with food-immunosuppressive-watch closely for signs of infection and avoid those with known infection…must be tapered and not suddenly stopped or can cause life threatening adrenal crisis (anorexia, nausea, weakness, SOB) What will you teach how to know if internal bleeding-this is also same teaching you will do with Coumadin

Blood Product Administration Minimum 22 g.(blue hub) IV-prefer 20g. (pink) or 18g. (green) Blood tubing with filter-use NS to prime/flush Validate pt., type of blood product, expiration date, blood tag # VS before, 15” after initiation, end of each Infuse PRBC’s over 2 hours (appx 300cc/unit) Refer to p.731 90% hemolytic transfusion reactions-worst life threatening due to giving blood product to wrong pt Asses closely first 15-30” infuse at rate no more than 125cc/hr Transfusion reaction—… … People older than 65 use 43% of all donated Blood. The demand for Blood and Blood products will increase as the population ages. 25% of all Blood products are used to treat cancer patients. One out of every ten people entering a hospital requires Blood. The average liver transplant patient needs 40 units of red Blood cells, 30 units of platelets, 20 bags of Cryoprecipitate, and 25 units of fresh frozen plasma. heart surgery uses, on average, the red Blood cells and platelets provided by from six Blood unit donations. People who have been in car accidents and suffered massive Blood loss can need transfusions of 50 units or more of red Blood cells. The average bone marrow transplant requires the platelets from about 120 donations, and the red Blood cells from about 20 Blood unit donations.

Complications Blood Products Circulatory Overload Acute Hemolytic Reaction Chills, fever, flushing, tachycardia, SOB, hypotension, acute renal failure, shock, cardiac arrest, death Febrile-Nonhemolytic Reaction Sudden onset of chills, fever, temp elevation >1 degree C. headache, anxiety Mild Allergic Reaction Flushing, urticaria, hives Who is at risk?...how can you minimize this potential problem? Hemolytic-due to incompatible blood-antibodies of pt. attaching to incompatible antibodies of unit Febrile-most common-due to sensitization to donor WBC’s, platelets or plasma proteins Mild allergic due to sensitivity to plasma proteins

Nursing Responsibilities STOP transfusion Maintain IV site-disconnect from IV and flush with NS Notify blood bank/MD Recheck ID Monitor VS Treat sx per MD orders Save bag and tubing-send to blood bank