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Hematologic Disorder

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The hematological system is composed of what 3 parts ? blood, bone marrow, lymphatic system
What are the cellular components of blood? erythrocytes, leukocytes, thrombocytes
What substances constitute the fluid portion of blood? Plasma Protein (albumin), electrolytes, coagulation factors, antibodies
Define hematopoiesis The production of all types of blood cells including formation, development, and differentiation of blood cells.
Hematopoietic organs : Bone marrow All of the formed elements of blood are believed to develop in the bone marrow
Lymphatic system: Lymph nodes Lymph nodes regulate the maturation of lymphocytes
Lymphatic system: liver and spleen deal with cell removal
What is the principle component of mature RBCs? Hemoglobin
What is the function of that component (mature RBCs)? carries oxygen
What hormone is important to the production of RBCs Erythropoietin
What other substances are required for normal erythrocyte production? Iron, protein, folate, vitamin B12
What are normal lab values related to RBCs? 3.5 for RBCs. 13-18 for hemoglobin, depending on sex
What do normal RBCs look like under microscope Biconcave disc
Anemia definition an abnormality in RBC number, structure or function which results in hypoxia because of inadequate transport of oxygen to the body’s tissues
What are the three main causes of anemia? blood loss impaired production increased destruction
What is the most common site of bleeding? GI tract
Acute blood loss causes trauma, blood vessel rupture
Chronic blood loss causes gastritis, hemorrhoids, menses
DNA synthesis problems in RBC prodution is caused by B12, Folate deficiency
b12 folate deficiency results in overlarge RBCs- megalocytic
Hgb synthesis in RBC production is caused by Iron deficiency, thalassemia, sideroblastic anemia
Decreased production of RBCs aplastic anemia, leukemia & myelodysplasia, chronic disease
Increased destruction of RBCS : INTRINSIC Sickle cell anemia (more later), enzyme or membrane problems
Increased destruction of RBCs: EXTRINSIC Trauma =>Hemolysis, auto-antibodies, toxins, infections (malaria!)
What does the Coombs test do? looks for certain antibodies in the blood
What are the Hgb levels for Mild Anemia? Symptoms? (Hgb 10-14): usually asymptomatic
What are the Hgb levels for Moderate Anemia? Symptoms? (Hgb 6-10):Dyspnea, palpitations, diaphoresis, chronic fatigue
What are the Hgb levels for Severe Anemia? Symptoms? (Hgb<6): pallor, activity intolerance, dizziness, multiple systems affected
What are some medical management techniques for Anemia? Identify and address cause Iron or vitamin therapy Repair of hemorrhaging sites Splenectomy Remove toxins Stem cell transplant for aplastic or leukemic anemia Corticosteroids for autoimmune disorders
What are some techniques to relieve the symptoms of anemia? Oxygen, transfusion, erythropoietin (Epoietin)
What assessment should you do for nursing care of Anemia? complete health history including diet, family history
What are the nursing diagnoses for Anemia? Activity intolerance, fatigue Expected Outcomes: improved activity tolerance, Self ADLs
What are some interventions for nursing care of Anemia? Education: explain causes of anemia, importance of adherence to medication and/or diet to patient and family
Why is evaluation in nursing care of Anemia important? reassess regularly to monitor progress toward outcomes
What type of cells might you have with Iron deficiency? Chronic, hypochromic, microcytic
What are the causes of Iron deficiency Anemia? Inadequate iron intake Impaired absorption of iron Increase in body’s need for iron Impaired synthesis of iron
What is the treatment/management of Iron deficiency Anemia? Diet and Iron supplementation Teach risk of gastric irritation=>take with food; coping with constipation=>fiber Avoid coffee & tea with iron
What are type of blood cells might you see with Vitamin B12 deficiency? Macrocytic, defective RBCs
What are the causes of Vitamin B12 deficiency? Lack of intrinsic factor (pernicious anemia) OR Failure to absorb vitamin B12 from intestinal tract (partial gastrectomy) OR Vegetarian diet (B12 comes from animal sources)
Vitamin B12 deficiency is also called Pernicious anemia if caused by lack of intrinsic factor in the gut
What testing is done for Vitamin B12 deficiency? review blood smear and RBC indices, Schilling Test for intrinsic factor adequacy
What is the treatment for Vitamin B12 deficiency? Cobalamin injections for life Oral or IV iron supplements
What do red blood cells look like with folic acid deficiency? megaloblastic (macrocytic) on blood smear
What are the causes of Folic Acid deficiency? Dietary deficiency of leafy greens and citrus Malabsorption syndromes Alcohol abuse and eating disorders Certain drugs (Oral contraceptives, anticonvulsants, methotrexate)
What is the treatment for Folic Acid deficiency? Oral folic acid until blood profile improves or underlying cause corrected Multivitamins
What is Sickle Cell Disease? Genetic disorder: normal adult hemoglobin replaced by mutant sickle hemoglobin
What is the pathophysiology of Sickle Cell? When O2 decreased, hgb becomes viscous and forms gel & crystals inside RBCs, cells form sickle shape: Sickle Cell Anemia (SCA)
Sickle Cell disease is most common in what population? African Americans in US (8% carry gene)
What are the genetics behind getting Sickle Cell disease? Autosomal recessive inheritance of two defective genes for hemoglobin synthesis = disease
What are the genetics behind getting Sickle Cell trait? One defective gene = trait: usually asymptomatic carrier. People with just the trait usually do well unless there is sickle cell crisis
How do sickle cells affect perfusion? stiff rigid and inflexible so they get stuck and affects perfusion. Also causes pain because extremities are hypoxic
What happens when there is obstruction due to sickled cells? Obstruction caused by sickled RBC’s Vaso-occlusion “pain crisis” due to local hypoxia Enlarged spleen from capture of damaged cells Kidney, liver, bone, CNS & cardiac problems
What happens when there is increased destruction of RBCs? Chronic fatigue and pallor
What is the most common cause of death in Sickle Cell? Acute Chest Syndrome: chest pain, fever, cough, tachypnea, elev. WBC and pulmonary infiltrate=>ARDS=>death
What are the medical management interventions of SCA? Prevention of sickling Treat sickle cell crisis (medical emergency ) Pain management with iv Morphine via PCA, then prn Rest, DVT prophylaxis Heat application Decrease oxygen demand
What is a supportive treatment of SCA? Folic acid, oxygen
What is a complementary treatment of SCA? TENS, hypnosis, acupuncture
What is a specific approved medicine to treat SCA? Hydroxyurea only FDA approved agent
What are some possible future curative treatments of SCA? Gene therapy research – alter fetal hemoglobin BMT offers future hope
What are some nursing interventions for SCA? Educate patient & family on avoiding sickle crisis, Instruct on dietary & activity management, Avoid high altitudes, dehydration Call physician if fever, vomiting or diarrhea Advise genetic counseling for young adults who carry gene Counsel women with
Which of the following tests will be most useful in determining whether a client has pernicious anemia? Schilling test
Ms. S, is admitted to the hospital because of a sickle cell crisis. Which nursing action is most appropriate to include in the plan of care? Encourage large amounts of fluid daily.
During hospital admission, the nurse learns that a teenage patient Junie is a vegetarian. The nurse will monitor Junie for: B12 deficiency anemia.
What are some disorders of Hemostasis? Platelet defects/ deficiencies Coagulation Factor deficiencies DIC
Quick review of Hemostasis 1)Platelets: cell fragments, respond to vascular injury by becoming sticky to form plug & secreting thromboxane, a vasoconstrictor 2)Coagulation cascade initiated, forming fibrin clot 3)TPA forms plasmin which breaks down clot
Immune Thrombocytopenic Purpura (ITP) Auto antibodies bind to plts; removed by spleen & liver Clinical: bruising, petechiae, purpura
Heparin Induced Thrombocytopenia (HIT) Suspect when plt count drops in patient receiving heparin therapy, can occur in 1 day! Anti-plt antibody formation induced by heparin sulfate Paradox: prone to thrombus formation, not bleeding!
What are some examples of Hemorrhagic Fever viruses? Ebola, Dengue, Marburg, Lassa
What are hemorrhagic fever caused by? animal reservoir, arthropod vector
What are the clinical manifestations of HFV? bleeding from all orifices, purpura, capillary leak, shock, liver failure
Which disorder is the loss of balance between clot formation and lysis called Disseminated Intravascular Coagulation
What are the causes of DIC? Infection (septicemia, typhoid, viral, parasitic) Tissue coag factors enter circulation Vascular damage Stagnant blood flow
What is the pathophysiology behind DIC? Pathophysiology : Coag cascade precipitated, forming clots throughout body and depleting platelets, prothrombin and coag factors, while activiating fibrinolysis.
How do you diagnose DIC? based on: Prolonged PT, PTT fibrinogen & plt count diminished Increased fibrinogen degradation product (FDP) and d-dimer
What are the manifestations of DIC? Petechiae, purpura, bleeding from all orifices, shock and coma. Mortality 80% in severe cases
What is the treatment for DIC? correct underlying cause. Replace blood components. monitor lab, VS & bleeding. Apply pressure to bleeding sites. Reposition carefully. Emotional support for pt and family
What is the clotting factor deficiency for Hemophilia A? factor VIII deficiency, X-linked
What is the clotting factor deficiency for Hemophilia B? factor IX deficiency, X-linked
What is the clotting factor deficiency for von Willebrand Disease ? vW factor, Autosomal dominant
Why is Hemophilia less common in females? they need a bad “x” from both parents but some are not genetic transmitters so it wouldn’t make a different in that case
The 2 hemophilia disorders that are x-linked are Hemophilia A and B
How cases of hemophilia are new mutations 30%
What testing is done for Hemophilia? prolonged PTT, factor assay (all other labs would be normal)
What types of bleed can you have with hemophilia? hemarthrosis- joints are swollen with blood- with any trauma – painful and deforming Epistaxis- nose bleed- can be fatal! Bleeding of gums, GI tract, hematuria Muscle hemorrhage Soft tissue: purpura
Leading cause of death in hemophiliacs Cerebral hemorrhage
What is the treatment for Hemophilia? Replacement of missing clotting factor (protein) Available as powdered factor concentrate Reconstituted and infused Repeat q 12 hours until bleeding stops
Treatment for hemophilia? Fresh frozen plasma, cryoprecipitate in emergencies (contain factor but less pure than factor concentrate) DDAVP / Stimate Antifibrinolytic Agents Supportive measures: pack ice around joints, aspirate blood from joint, ROM exercises to prevent deformi
Mr. H, who has hemophilia, has a knee injury at home and calls the clinic asking for advice. Which action should the nurse recommend that he take before coming to the clinic? immobilize the knee using an elastic bandage
Disseminated intravascular coagulation (DIC) is characterized by which of the following clinical manifestations? Prolonged PT and PTT (; low platelet count (<100,000); reduced fibrinogen; and prolonged clotting times)
Name the two classes and 5 types of WBCs Agranular (lymphocytes/monocytes), Granular (basophils, neutrophils, eosinophils)
Define leukocytosis a white blood cell count (the leukocyte count) above the normal range in the blood.It is frequently a sign of an inflammatory response, most commonly the result of infection
Define lymphocystosis is an increase in the number or proportion of lymphocytes in the blood.
Define leukopenia ) is a decrease in the number of white blood cells (leukocytes) found in the blood, which places individuals at increased risk of infection.
Define neutropenia subtype of leukopenia, refers to a decrease in the number of circulating neutrophil granulocytes, the most abundant white blood cells.
Define lymphopenia is the condition of having an abnormally low level of lymphocytes in the blood. Lymphocytes are a white blood cell with important functions in the immune system
What is agranulocytosis? Acute, life-threatening blood disorder Profound neutropenia (define) Rare; more common in women than men
What is the most common cause of agranulocytosis? Drug or chemical toxicity (chemo, dilantin, tranquilizers, antibiotics)
Die within a week from septicemia if what disorder isn't treated? Agranulocytosis
Diagnosing of Lymphoma, Leukemia, Multiple Myeloma history, CBC raise suspicion Bone Marrow biopsy is definitive
What is the purpose of a bone marrow biopsy? obtain liquid marrow specimen and bone core for evaluation by pathology
What site is used for the bone marrow biopsy? superior inferior iliac crest
What are the 5 nursing responsibilities during bone marrow biopsy Ensure supplies available in patient room Provide education to patient, evaluate understanding Administer analgesics & sedatives as ordered Assist pt to lateral decubitus position Standby assist to MD at bedside
How long should patient lie flat after bone marrow? pt should lie flat 1 hour to prevent hemorrhage
What is multiple myeloma ? Malignancy of plasma B lymphocyte
Multiple myeloma is more common in what gender and what race? Men 2x more likely than women and African Americans 2x more than Caucasians
What assessment findings do you see with multiple myeloma? Bones are painful and very fragile, can cause pathological fractures – fracture that wouldn’t normal occur
What is the cure for Multiple Myeloma? No cure! Chemotherapy: Melphalan & prednisone 4-7 days, repeat at 4 week intervals; 50-60% response rate Thalidomide & lenalidomide approved (neuropathy common) BMT with Hi-dose chemo has 80% response rate
What is the nursing care for MM? Manage bone pain, nausea Small frequent feedings, stool softeners, push fluids Monitor I & O, daily weights, blood studies Care in transferring patient to avoid fractures Monitor mental status Instruct family on home safety to prevent injury
What is Leukemia ? Cancer of WBC precursors
What are the possible causes of leukemia? Benzene exposure (usually from tobacco) High dose radiation exposure Certain chemotherapies for other cancer
What is the most common form of leukemia in children? ALL ( Acute Lymphocytic leukemia)
What is the most common overall leukemia? CLL (Chronic Lymphotic leukemia)
What is the most deadly form of leukemia? AML (Acute Myelocytic Leukemia)
Hodgkin's vs. Non-Hodgkin's Hodgkin’s (most common in young and older adults; associated with Epstein-Barr virus; 90% five year survival rate) Non-Hodgkin’s (Highly variable aggressiveness; 5 year survival rate 69% overall, 85% in children)
Which nursing action would NOT be appropriate when planning care of a client hospitalized with multiple myeloma? Increase dairy product intake or provide calcium supplement.
Neutropenic precautions? Maintain clean skin and oral mucosa Teach patient and visitors about hand hygiene, reinforce often! Screen visitors for flu or cold symptoms No visits from persons who had active vaccinations recently Monitor for low-grade fevers or mental status chan
What is the most effective method of preventing infection in a client with neutropenia? Enforce strict hand-washing for everyone coming into contact with the client
Created by: UARN85