Hematologic Disorders PDF

Hematologic Disorders  Objectives  Relate principles of anatomy and physiology to the nursing care of individuals with hematologic and immunologic health problems.  Integrate and relate the pathophysiologic changes of normal blood cells to common clinical assessments...

Hematologic Disorders  Objectives  Relate principles of anatomy and physiology to the nursing care of individuals with hematologic and immunologic health problems.  Integrate and relate the pathophysiologic changes of normal blood cells to common clinical assessments in the adult patient.  Objectives  Relate principles of anatomy and physiology to the nursing care of individuals with hematologic and immunologic health problems.  Integrate and relate the pathophysiologic changes of normal blood cells to common clinical assessments in the adult patient.  Objectives  Develop nursing interventions to assist with the adult with alteratations in hematological and immunological function.  Discuss major care concerns for patients and families undergoing hematological and immunological treatments.  Coagulation Cascade  To control bleeding  One has to happen first before the other can begin  initiated by triggering event  Landslide effect  Two pathways › Intrinsic (slower) (scab) › Extrinsic (faster)  Clotting factors are activated sequentially  Regardless of triggering event the outcome is formation of a fibrin clot and coagulation  Fibrin clot to stop bleeding Hematologic Assessment: Common Lab Tests Complete blood count Prothrombin time (PT) Partial Thromboplastin Time (PTT) (CBC), which measure the International Normalized Ratio (INR) including: (PT)- measure how well someone is clotting White blood cell count To evaluate bleeding and clotting disorders and to (WBC) monitor anticoagulation (anticlotting) therapies Red blood cell count Used to diagnose bleeding disorders and measures (RBC) therapeutic effectiveness in certain medications (ex. Platelet count Blood thinner) Red blood cell indices (measurements) Hematocrit red blood cell volume (HCT) Average red blood cell size (MCV) Hemoglobin MCV= mean corpuscular volume concentration (HB). Hemoglobin amount per red blood cell (MCH) Oxygen-carrying MCH=mean corpuscular hemoglobin protein in red blood cells. The amount of hemoglobin relative to the size of the cell (hemoglobin concentration) per red blood cell Differential white blood (MCHC) count How much hemoglobin is inside relative to the When you get a WBC actual size of cell count, you usually get MCHC= mean corpuscular hemoglobin a differential concentration Number of different types of WBCs Can reflect nutritional status  ABSOLUTE NEUTROPHIL COUNT (ANC)  Reflects the number of mature WBC’s › Chemotherapy will suppress some of the immune system which will destroy white blood cells= negative effect on immune status,  so you want to know how many mature white blood cells they have = ANC  Needs to be above 3,000 (Neutrophils + Bands) X Total WBC = ANC neutrophils= white blood cells Bands= immature white blood cells  Hemolytic Anemia  Means body is destroying red blood cells  Abnormal or premature destruction of RBC’s  Intrinsic (Sickle Cell); or a result of infection, chemical, an immune response or radiation  A hemolytic crisis can be the result of extreme physiologic or emotional stress  Can be triggered by anything Diagnosing Hemolytic Anemia  Decreased H&H  Decrease Haptoglobin  A marker of hemolysis  Elevated bilirubin, LDH, cold agglutinin, and reticulocyte counts  Signs and Symptoms: › Fever › Headache › Joint pain › Abdominal pain  H&H= hemoglobin and hematocrit  Hemolysis- red blood cell destruction  LDH- liver enzyme a marker to show how severe damage is. Elevated when liver is working to process the destroyed red blood cells  Reticulocyte counts- immature red blood cells  Hepatoglobin- a protein made by the liver to get rid of hemoglobin outside red blood cells  Hematocrit- % by volume of red blood cells in blood  Bilirubin- a substance produced by the breakdown of red blood cells › When body destroys red blood cells, it sends to the liver to be processed and get rid of- bilirubin is a side product  Signs and Symptoms  Splenomegaly (spleen enlargement)  Hepatomegaly (liver enlargement)  Shortness of breath  Chills  Decreased urinary output  Lymphadenopathy › Lymph node enlargement Treatment of Hemolytic Anemia  Supportive  Discontinuation therapy for shock of causative  IVIG Factor  Transfusion  Volume  Erythrocytaphere Replacement › Certain drugs sis (RBC Exchange)  Oxygen Therapy › Take old red blood  Immunotherapy cells and › Immune system exchange for is reason for healthier ones destroying red   Usually involves Aplastic Anemia Pancytopenia › Pancytopenia- all  Results from an cells within bone inability of blood is effected erythrocyte- › Attack part of the producing organs bone marrow that makes these cells (specifically the  Causes: bone marrow) to produce RBC’s › Antineoplastic or antimicrobial agents › Immunological disorder   Diagnosing Aplastic Anemia  Decreased: › H&H › WBC › Platelets › Reticulocyte count  Because bone marrow is directly affected  Prolonged Bleeding Time has to be diagnosed with Bone Marrow Aspiration › Depression of erythroid elements  Signs and Symptoms of  Fever and Aplastic Anemia infection  Weakness that worsens (specially if issue not being resolved)  Bleeding  Dizziness  Dyspnea › Difficulty breathing/ shortness of breath  Oral ulcerations Management of  Bone Marrow Transplantation Aplastic Anemia › If unable to manage, this is one of the end points  Antibiotic Therapy  Determine the  Steroid Therapy cause  Granulocyte Transfusion › So it can be › A type of white blood cell discontinued › Almost all have reactions  Androgen Therapy  Transfusion with › Stimulates renal packed RBC’s or erythropoietin production frozen plasma  A certain hormone given to stimulate red blood cell  transfusion of  Hemoglobin which will stimulate bone marrow for cell platelets growth  Psychosocial  Leukemia: Basic Physiology  Malignant transformation of the stem cells causing an abnormal proliferation of a specific type of leukocyte stem cell- a cell that doesn’t know what it wants to be  Proliferation shuts down normal bone marrow production  Results in anemia, thrombocytopenia, and leukopenia of the unaffected WBC types › Thrombocytopenia- platelet reduction › Leukopenia- white blood cell reduction  Many immature white blood cells in bone marrow so then bone marrow is full and Leukemia cannot make the things it needs to Group of malignant disorders involving abnormal overproduction of a specific WBC type – Usually at an immature state – In the bone marrow May be acute or chronic Categorized by the specific maturational pathway from which the abnormal cell arose – Lymphocytic/ lymphoblastic – Myelocytic/myelogenous  Leukemia: Acute and Chronic  Acute Lymphocytic Leukemia:  Chronic › Malignant proliferation of WBC Leukemia: precursors (blasts) › Malignant › Accumulate in the proliferation of abnormal bone marrow, body immature WBCs tissue and blood vessels preventing › Prevent normal WBC maturation of maturation WBCs, thus  Immature white blood preventing cells accumulate in normal function bone marrow and  Diagnosing Leukemia  Acute: › Fever  Chronic: › Diffuse petechia › Fatigue  Little › Anorexia hemorrhages › Weight loss under the skin  Increased WBC › Bleeding  Decreased Hgb › Bruising › Anorexia (hemoglobin),  Neutrophils and Increased WBC, platelets uric acid, PT and PTT  Management of Leukemia  Chemotherapy  Radiation (chronic)  Blood Product Support  Bone Marrow Transplantation  Psychosocial Support  Leukemia  INFECTION and HEMORRHAGE are the causes of death  Etiology › Many genetic and environmental factors › Involving gene damage to the cells  May be viral pathogenesis  Can be secondary to bone marrow damage from radiation  Leukemia  Lab assessment › Decreased H&H › Decreased platelets › Altered WBC (low, normal, elevated: usually 20,000 to 100,000 › Bone marrow aspiration/biopsy identifies types  Drug therapy Leukemia › Intensive combination chemotherapy  Peripheral Blood › Major side effects  Bone marrow Stem Cell depression Transplant (PBSCT)  you are looking to shut down bone marrow  Increases vulnerability to infection  if vulnerable to infections, they have to be treated with Antibiotics, antifungals, antivirals  Malignant Lymphoma  Cancer of the lymph nodes  Malignancies characterized by a proliferation of committed lymphocytes rather than stem cell precursors  Can be secondary to: › Solid tumors- particularly affecting lymph nodes and spleen › Types › Hodgkin’s › Non-Hodgkin’s  Hodgkin’s Lymphoma disease of young people Incidence:  Assessment – Peaks in mid to late 20’s – Males < 50 more › Enlarged painless effected than males >50 node or nodes Causes  (sore throat) – Probably viral or › Fever chemical › Malaise – Usually originates in a  Feeling yucky single or chain of lymph nodes › Night sweats – Reed-Sternberg cells (characteristic marker) – Look for those in biopsy to verify hodgkins  Hodgkin’s Lymphoma Diagnosis: Treatment Biopsy reveals R- – Stage 1-2 S(reed-Sternberg) – Extensive radiation cells Staging is Crucial – Stage 3-4 At earlier stage, one of – More extensive the most curable – Radiation with forms of cancers aggressive multi- Treatment is based on agent chemotherapy extent of disease – Nursing Prognosis management One of most curable focuses on side forms of cancer effects  Non-Hodgkin’s Lymphoma disease of older people Cancers of the lymph tissue that are not Hodgkin’s Most occur in older adults Causes unknown – Suspect exposure to virus, viral radiation, chemical Prognosis: – Dependent on cell type – Ranges from excellent to poor – Older people do not have the same physiologic reserve as they did when they were younger Treatment and nursing care similar to Hodgkin’s lymphoma  Autoimmune Thrombocytopenic Purpura Cause- autoimmune Patho- body makes an anti-platelet antibody that destroys platelets Anti-platelet antibody is abnormal destruction of platelets, body will attack itself What manifestations would you expect? Diagnosis: platelet count < 20,000 Normal platelet count is 150,000 Collaborative care (treatment) – Identify cause if possible and treat – Provide safe environment – Because people bleed easier – Immunosuppressants – IVIG – Splenectomy (last resort)  Clotting Factor Disorders  Most are congenitally transmitted gene abnormalities of one clotting factor › Hemophilia A & B › von Willebrand’s  A hereditary Hemophilia bleeding disorder characterized by a deficiency of one or more clotting factors most often VIII and IX  Individuals affected are usually males with females as asymptomatic carriers  Carried by women and passed on to  Diagnosing Hemophilia  By using blood  Bruising tests  Joint pain and  Prolonged PTT, swelling from bleeding time hemathrosis and activated › Hemathrosis- clotting time bleeding in joints  Normal platelet,  Bleeding PT, thrombin  Shock resulting time and from severe fibrinogen level bleeding  Low activity of  Management of Hemoplilia  Factor transfusion (#1)  Transfusion of FFP › FFP- fresh frozen plasma (has blood clotting factors) › Cryoprecipitate  Specific form of plasma that has concentration of clotting factors  Desmopressin (DDAVP)  Aminocaproicacid (amicar) › medication  Psychosocial support  Anemia of Chronic Disease Renal Dysfunction  Anemia of renal disease is an  Treatment anemia resulting primarily  Epoetin (EPO, from deficient erythropoietin Procrit, (EPO) Epogen) › kidneys are not making enough which stimulates bone marrow to make red blood cells › Treatment includes measures to correct the underlying disorder  Doesn’t work if you have end-stage renal disease › Supplementation with EPO and sometimes iron.  Who Can Get Sickle Cell Disease?  ANYONE can get Sickle Cell Disease.  It is most common in the following ancestries: o African o Central or South American o Cuban o Indian o Saudi Arabian o Mediterranean o Hispanics  Vasocclusion In Sickle Cell Anemia  Without enough blood flow= organ damage  Causes acute and chronic organ damage  Acute complications › Sickle cell vaso-occlusive pain crisis  clogs up blood vessels › Hepatic crisis (liver) › Splenic crisis (spleen) › Priapism  An erection that wont go down  Chronic organ damage › Stroke › Chronic lung disease with pulmonary hypertension › Renal failure › Avascular necrosis of bone  Don’t have blood supply so bones will dissolve Sickle Cell Crisis  Precipitating factors › Hypoxia › Acidosis › Fever › Infection › Dehydration › Exposure to cold › High Altitudes Sickle Cell Anemia Painful Events: Management Principles  Three corner stones of life- airway, breathing, cirulation  O2 administration  Correct fluid/electrolyte abnormalities; use hypotonic fluid and limit volume to avoid overhydration  Treat any underlying illness  Opioid analgesics (morphine) (meperidine is not recommended)  Blood transfusion is not indicated for an uncomplicated pain episode  Incentive spirometry should be used during waking hours  Nursing Actions for a Patient who is Anemic or Suffered Blood Loss  Administer oxygen as prescribed  Administer blood products as prescribed (transfusion)  Administer erythropoietin as prescribed  Allow for rest between periods of activity › Pooped out because they do not have breath cells carrying oxygen  Elevate the head of the bed during episodes of shortness of breath  Provide extra blankets if the patient feels cool  Teach the patient/family about underlying pathophysiology and how to manage the symptoms of anemia Practice Question A 32-year-old client with a history of sickle cell anemia is admitted to the hospital during a sickle cell crisis. The physician orders all of these interventions. Which order will you implement first? a) Give morphine sulfate 4-8 mg IV every hour as needed b) Start a large-gauge IV line and infuse normal saline at 200 mL/hour c) Immunize with Pneumovax and Haemophilus influenza vaccines d) Administer oxygen at an Fi02 of 100% per non-rebreather mask Practice Question When the nurse is implementing bleeding precautions for a patient with thrombocytopenia, the following should be included (SELECT ALL THAT APPLY): a) The use of an electric razor b) Discontinuation of aspirin c) Limitation of needle sticks d) Restriction of visitors