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DISORDERS OF THE HEMATOPOETIC SYSTEM Student Learning Goals At the completion of this unit, the student will be able to: List the components of the hematologic system and describe their role in oxygenation and hemostasis. Identify data to be collected when assessing a patient with a disorder of the hematologic and/or immune system. Describe tests and procedures used to diagnose disorders of the hematologic/immune system, and nursing considerations for each. Describe nursing care for patients undergoing common therapeutic measures for disorders of the hematologic system, including blood transfusion. Differentiate between iron deficiency anemia, pernicious anemia, aplastic anemia, and sickle cell anemia according to pathophysiology, symptoms, treatment, and nursing management. Student Learning Goals Define the major aspects of diagnosis, treatment and nursing management of the patient with Leukemia, Hodgkin's disease, Non-Hodgkin’s Lymphoma and Multiple Myeloma. Define and describe the differences between the following bleeding disorders: – – – – Thrombocytopenia ITP/TTP Hemophilia DIC Assist in planning nursing care for a patient with a disorder of the hematologic/Immune system. – Risk for Injury with Low RBCs – Risk for Injury from Bleeding – Risk for Injury from Infecction Define, spell correctly and pronounce all words on Unit III Word List. Structures and Components of the Hematologic System – Bone marrow – Liver – Spleen – Blood – Red blood cells (erythrocytes) – Platelets (thrombocytes) – Clotting factors – Plasma Age-Related Changes Bone marrow becomes less productive Hematologic function not affected unless a person is unusually stressed with trauma, a chronic illness, or treatment for cancer In conditions necessitating a higher production of blood cells, bone marrow usually responds to the increased demand, given time Diagnostic Tests and Procedures Blood tests – Red blood cell count – Hemoglobin (Hb or Hgb) – Hematocrit (Hct) – Platelet counts Bone marrow biopsy Important Lab Values RBC – 4.5 – 5 million/cubic mm Hemoglobin – Male: 13.5 – 18 Gm / 100 ml – Female: 12.1 – 16 Gm / 100 ml Platelets (Thrombocytes) – 150,000 – 400,000 per microliter Learning Goal Reflection List the components of the hematologic system and describe their role in oxygenation and hemostasis. Bone Marrow Aspirated from: Posterior Iliac Crest (most common) Anterior Iliac Tibia Sternum What is Nursing care before, during and after? Learning Goal Reflection Describe tests and procedures used to diagnose disorders of the hematologic/immune system, and nursing considerations for each. Type / Cross Match Recipient and donor must be of same Type (ABO) & Rh Factor – What is Rh factor and why do we care – Universal Donor? – Universal Recipient? Is donor blood safe? Hung on separate IV line Normal Saline (0.9% Sodium Chloride) only! Special filter to IV line that screens clots Larger bore IV catheter – 20 gauge minimum Must be hung ASAP – timing important Requires 2 licensed people at bedside Get baseline vitals Stay with pt. for 10-20 mins after blood started Monitor vitals Document procedure Blood Product Transfusion Reactions to blood transfusions – Four main types Hemolytic Anaphylactic Febrile Circulatory overload – Symptoms Back or chest pain, fever, chills, decreased blood pressure, urticaria, wheezing, dyspnea, or coughing during the transfusion Blood Product Transfusion Reactions to blood transfusions – Interventions Stop transfusion immediately; keep intravenous line open with normal saline Immediately notify physician, nursing supervisor, blood bank Be prepared to administer oxygen, epinephrine, Solu-Cortef, furosemide (Lasix), antipyretics as prescribed by physician Save the unused portion of the blood bag for the blood bank Be prepared to collect blood and urine samples from the patient for evaluation Learning Goal Reflection Describe nursing care for patients undergoing common therapeutic measures for disorders of the hematologic system, including blood transfusion. Too many RBC’s - Polycythemia Vera Too few RBC’s - Anemia What is Anemia: – Disease / Symptom – Condition of low red blood cells (RBC) or low hemoglobin (HgB) Anemia Symptoms & Diagnosis Symptoms: – Pallor / hypersensitive to cold – why? – Extreme Fatigue – why? – Tachycardia – why? – SOB – why? Diagnosis – – – – Eval of RBC and HgB Further lab studies to determine type of anemia Schillings Test Bone Marrow Biopsy Blood Loss Aplastic Autoimmune Hemolytic Iron Deficiency Pernicious Sickle Cell Results from complete failure of bone marrow Will see Pancytopenia decrease in: - RBC - WBC - Platelets Aplastic Anemia Causes: – Many Unknown – Toxic Chemicals Look at work history – Drugs Good history and previous drug use – Radiation Exposure? Additional S & S: – Frequent Infections – why? – Prolonged or spontaneous bleeding – why? Treatment of Aplastic Anemia ID and Treat Cause Transfusions Antibiotics Corticosteroids Bone Marrow Transplant (BMT) Neutropenic Precautions Any others? Autoimmune Hemolytic Anemia Blood cells destroyed once they are released into circulation – See jaundice with destruction of RBC’s Causes: – Infections, drug reactions, certain cancers Treatment: – ID cause; transfusions; corticosteroids Iron Deficiency Anemia Results from diet low in iron or poor absorption of iron Treatment: – Iron Supplements How to administer Side Effects – Incorporating Iron rich foods into diet What foods are iron rich? Pernicious Anemia Results when a person does not absorb B12 from stomach – B12 needed for iron absorption, RBC maturation and protection of nerve fibers Additional S&S: – Weakness – Sore Tongue – Numbness of hands and feet B-12 injections IM or SC -cyanocobalamin how to administer? Given q 4 weeks for lifetime Genetic blood disease Sickle-shaped RBC’s Crescent shaped, rigid, and inflexible Shape change d/t presence of abnormal hemoglobin S – reduced O2 carrying capacity Sickle Cells: – Are Destroyed easier: - live only 6-20 days - normal is 120 days – Tend to jam capillaries and decreased blood flow Sickle Cell Anemia Almost exclusive to AF/AM Autosomal recessive gene – a person must inherit the gene from both the mother and the father to actually have the disease 8% of AF AM carry genetic trait Sickle cell trait - have 25 – 50% HGB S (AS) Sickle cell disease - have >80% HGB S (SS) Sickle Cell Anemia Lab studies – CBC shows Anemia – Sickle cell test or Sickledex – HGB electrophoresis Signs and Symptoms: – Fatigue – Jaundice (Splenomegaly) – Cardiomegaly / Tachycardia Occurs when sickled cells become stuck in a larger blood vessel - Obstructing Blood Flow - Causing Severe Pain- Why? Crisis Triggers: – – – – – Dehydration Infection Overexertion Cold Weather Changes Excess ETOH / Smoking Sickle Cell Disease Severe Pain –Pain location varies depending on where circulation is blocked Complications: –Thrombus –Infection No Cure Symptomatic during crisis IV Fluids Pain Medication Oxygen Antibiotics Blood Transfusions Hydroxyurea Sickle Cell Nursing Diagnosis and Interventions Acute Pain Anxiety Risk for Injury Fluid Volume Deficit Ineffective Therapeutic Regimen - Patient Teaching Learning Goal Reflection Differentiate between iron deficiency anemia, pernicious anemia, aplastic anemia, and sickle cell anemia according to pathophysiology, symptoms, treatment, and nursing management. Coagulation Disorders Thrombocytopenia Too few platelets circulating in the blood Why? – Not enough platelets being made in bone marrow – Too many platelets are being destroyed in circulation Major cause for bone suppression: – chemotherapy – radiation therapy Causes for platelets being either destroyed or consumed are: – idiopathic thrombocytopenic purpura (ITP) – thrombotic thrombocytopenic purpura (TTP) Thrombocytopenia Symtoms: – petechiae and purpura – gingival bleeding – epistaxis (nosebleeds) – any other unusual or prolonged bleeding Treatment: – treat or stop the causative factor – What is Neupogen? When would it be used? Idiopathic Thrombocytopenic Purpura (ITP) IgG mistakenly helps destroy patient’s platelets Drugs that induce ITP: sulfonamides, thiazide diuretics, chlorpropamide, quinidine, and gold. Patients with HIV are at increased risk for developing ITP Treatment: – – – – steroids intravenous immune globulin (IVIG) Splenectomy – maybe/Why? Immunosuppressive therapy with cytotoxic drugs Thrombotic Thrombocytopenic Purpura (TTP) Exaggerated immunologic response to blood vessel injury – results in extensive clot formation and decreased blood flow to the site Patients critically ill – develop fever, thrombocytopenia, hemolytic anemia, renal impairment, and neurologic symptoms Treatment – plasmapheresis – steroids, antiplatelet agents (e.g., aspirin, dipyridamole), splenectomy, or all three Nursing Care w/ Bleeding Disorders See Box 32-4 pg. 612 – Prevent Injury – ideas? – Check for S/S bleeding – ideas? – Safety Measures – ideas? Disseminated Intravascular Coagulation (DIC) A hypercoagulable state Overstimulation of normal coagulation cascade – simultaneous thrombosis and hemorrhage Always secondary to another pathologic process: – overwhelming sepsis, shock, major trauma, crush injuries, burns, cancer, acute tumor lysis syndrome, or obstetric complications (abruptio placentae, fetal demise) D.I.C. cont’d Coagulation occurs at so many sites Eventually all available platelets and clotting factors are depleted Uncontrolled hemorrhage results Blood tests that help diagnose DIC include: – prothrombin time, partial thromboplastin time, fibrinogen, thrombin time, fibrin split products level, and D-dimers D.I.C. cont’d Treatment – Blood component replacement therapy ? – Heparin to interrupt the DIC cycle and allow the body to replenish platelets and clotting factors Controversial Nursing care? – Box 32-4 – is there a theme? HEMOPHILIA Genetic disease: affected person lacks some blood clotting factors normally found in plasma – X-linked recessive genetic condition – Women inherit and pass on to son – From grandfather to grandson through mother Blood slow to coagulate Platelets OK Clotting intrinsic factor affected Hemophilia A - Factor VIII Hemophilia B – Factor IX Hemophilia Signs and symptoms – Uncontrollable bleeding is the hallmark of hemophilia Occurs after trauma; however, also spontaneously for no clear reason Commonly, bleeding occurs into the joints, causing swelling and severe pain Also can occur into the skin; from the mouth, gums, and lips; and from the gastrointestinal tract Prolonged oozing from minor injuries Hemophilia cont’d Medical diagnosis – Measuring factors VIII and IX in the blood – Partial thromboplastin time Medical treatment – No cure; treatment is symptomatic – Physician prescribes transfusions of fresh frozen plasma or cryoprecipitate, or both – Red blood cell transfusions – Intravenous morphine – Physicians try quickly to transition from IV opioids to oral opioids to nonopioid pain relievers as crisis resolves Hemophilia cont’d Assessment – For bleeding and pain; note what measures have stopped the bleeding and relieved pain in the past – Monitor vital signs and urine output Interventions – Risk for Injury – Acute Pain – Ineffective Therapeutic Regimen Management Learning Goal Reflection Define and describe the differences between the following bleeding disorders: – Thrombocytopenia – ITP/TTP – Hemophilia – DIC Let’s look at the WBC Aka: Leukocytes 4,500 – 10,000 per microliter 5 Major types – Each combats different microorganisms Identify and destroy foreign antigens and proteins by ingesting them – This process destroys the WBC – Short life span Malignant disease Cancer of the white blood cells: – bone marrow produces too many immature white blood cells Immature white blood cells leave patient at great risk for life-threatening infections Overabundance of immature WBC’s – Results in decreased RBC’s and Platelets Leukemia Most common CA in childhood Also affects adults Fatal without treatment Types of Leukemia –Myelogenous –Lymphocytic Further divided by how rapidly they progress: Acute Chronic Diagnosis of Leukemia –Presenting Symptoms Pancytopenia sx. –CBC w/ differential Most useful initial test Shows increased and immature leukocytes –Bone Marrow Biopsy To identify specific type Affects Granulocytes: – Eosinophils detoxify foreign proteins in allergic reactions & fights parasites – Basophils inflammatory response – Neutrophils fight bacterial infections, most numerous WBC Easily destroyed, rapidly replaced Affects Agranulocytes –Lymphocytes form antibodies immune response –Monocytes Largest WBC Macrophages in tissues Live longer than neutrophils ALL – Acute Lymphocytic Leukemia – Most frequently affects children – 2 – 6 years most frequent age – With tx. Generally good prognosis AML – Acute Myelogenous Leukemia – Most common in adults – With tx, generally poor prognosis Symptoms of Acute Leukemia – Sx of Neutropenia Fevers and night sweats – Sx of Anemia Fatigue, paleness, tachycardia, and tachypnea – Sx of Thrombocytopenia Petechiae or purpura, epistaxis, gingival bleeding, melena, or menorrhagia – Weight loss and swollen lymph nodes – Bone Pain Chemo Antibiotics Colony Stimulating Factors Bone Marrow Transplant (BMT) Peripheral Blood Stem Cell Transplant (PBST) CLL - Chronic Lymphocytic Leukemia CML - Chronic Myelogenous Leukemia – Occur most often in adults – WBC counts slowly increase over years – Usually controlled with oral chemo Gleevec – Life expectancy 2 – 10 years Symptoms and Diagnosis same as for Acute but slow onset – What are the Sx? – How is it diagnosed? Treatment – Oral Chemo – Antibiotics – Interferon – BMT/PBST Leukemia - Nursing Diagnosis/Intervention Risk for Injury – Infection/Thrombocytopenia/Anemia Fatigue Impaired Oral Mucous Membrane Imbalanced Nutrition less than body requirements Anxiety Ineffective Self Help Management Cancer of the lymph system 1st sx often enlarged lymph nodes Higher incidence in men Etiology unknown 2 Types: –Hodgkin’s Disease –Non-Hodgkin’s Lymphoma Most common in 20’s and 50’s More common in men Symptoms: – Painless enlargement of lymph nodes – Persistent fatigue – Generalized pruritis – Chills, fever that come and go – Soaking Night Sweats – Anorexia and Unexplained Weight Loss – Pain r/t pressure from enlarged lymph nodes Diagnosis – Lymph node biopsy Reed-Sternberg cell – Bone Marrow Biopsy – CT Detect large nodes and metastasis – Gallium Scan Radioactive tracer to malignant lymph tissue Treatment depends on: – The type of Hodgkin lymphoma – The stage (where the disease has spread) – Whether the tumor is more than 4 inches (10 cm) wide – Age and other medical issues – Other factors, including weight loss, night sweats, and fever Treatment includes: – Radiation Therapy – Chemotherapy – Combination of 2 – PBST Nursing Care: – Coping w/ SE of radiation – Chemo SE – Infection Prevention Group of lymphatic neoplasms More common than Hodgkin’s Non-Hodgkin lymphomas begin when a type of white blood cell, called a T cell or B cell, becomes abnormal. – he cell divides again and again, making more and more abnormal cells. Symptoms/Diagnosis/Treatment Same as Hodgkin’s except: no pruritis no Reed-Sternberg cell Cancer of plasma cells in bone marrow – Plasma cell is type of WBC High levels of immunoglobulins – Plasma proteins Bones weaken – As cells grow and break down bone Pancytopenia – What is this? Diagnosis: – X-ray – Bone marrow biopsy – Serum Protein Electrophoresis Measures immunoglobulins – 24 hour urine protein electrophoresis – Look at: BUN/Creatinine Calcium Level Protein Levels Common symptoms may include: – Bone pain, often in the back or ribs – Broken bones – Weakness or fatigue – Weight loss – Repeated infections – Frequent infections and fevers – Feeling very thirsty – Frequent urination Management: – No cure – Radiation / Chemo – BMT – Nursing Care: Maintaining Hydration Pain management Activity / Nutrition Decrease infections Risk for Injury Acute Pain Ineffective Therapeutic Regimen –Pt Teaching Plan Learning Goal Reflection Define the major aspects of diagnosis, treatment and nursing management of the patient with: – Leukemia – Hodgkin's disease – Non-Hodgkin’s Lymphoma – Multiple Myeloma. Final (Conceptual) Learning Goal Assist in planning nursing care for a patient with a disorder of the hematologic/Immune system. – Risk for Injury with Low RBCs – Risk for Injury from Bleeding – Risk for Injury from Infecction