DISORDERS OF THE HEMATOPOETIC SYSTEM

Transcription

1 DISORDERS OF THE HEMATOPOETIC SYSTEM

2 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.

3 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.

4 Structures and Components of the Hematologic System Bone marrow Liver Spleen Blood Red blood cells (erythrocytes) Platelets (thrombocytes) Clotting factors Plasma

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7 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

8 Blood tests Diagnostic Tests and Procedures Red blood cell count Hemoglobin (Hb or Hgb) Hematocrit (Hct) Platelet counts Bone marrow biopsy

9 Important Lab Values RBC million/cubic mm Hemoglobin Male: Gm / 100 ml Female: Gm / 100 ml Platelets (Thrombocytes) 150, ,000 per microliter

10 Learning Goal Reflection List the components of the hematologic system and describe their role in oxygenation and hemostasis.

11 Bone Marrow Aspirated from: Posterior Iliac Crest (most common) Anterior Iliac Tibia Sternum What is Nursing care before, during and after?

12 Learning Goal Reflection Describe tests and procedures used to diagnose disorders of the hematologic/immune system, and nursing considerations for each.

13 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?

14 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 mins after blood started Monitor vitals Document procedure

15 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

16 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

17 Learning Goal Reflection Describe nursing care for patients undergoing common therapeutic measures for disorders of the hematologic system, including blood transfusion.

18 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)

19 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

20 Blood Loss Aplastic Autoimmune Hemolytic Iron Deficiency Pernicious Sickle Cell

21 Results from complete failure of bone marrow Will see Pancytopenia decrease in: - RBC - WBC - Platelets

22 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?

23 Treatment of Aplastic Anemia ID and Treat Cause Transfusions Antibiotics Corticosteroids Bone Marrow Transplant (BMT) Neutropenic Precautions Any others?

24 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

25 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?

26 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

27 B-12 injections IM or SC -cyanocobalamin how to administer? Given q 4 weeks for lifetime

28 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

29 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)

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31 Sickle Cell Anemia Lab studies CBC shows Anemia Sickle cell test or Sickledex HGB electrophoresis Signs and Symptoms: Fatigue Jaundice (Splenomegaly) Cardiomegaly / Tachycardia

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33 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

34 Sickle Cell Disease Severe Pain Pain location varies depending on where circulation is blocked Complications: Thrombus Infection

35 No Cure Symptomatic during crisis IV Fluids Pain Medication Oxygen Antibiotics Blood Transfusions Hydroxyurea

36 Sickle Cell Nursing Diagnosis and Interventions Acute Pain Anxiety Risk for Injury Fluid Volume Deficit Ineffective Therapeutic Regimen - Patient Teaching

37 Learning Goal Reflection Differentiate between iron deficiency anemia, pernicious anemia, aplastic anemia, and sickle cell anemia according to pathophysiology, symptoms, treatment, and nursing management.

38 Coagulation Disorders

39 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)

40 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?

41 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

42 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

43 Nursing Care w/ Bleeding Disorders See Box 32-4 pg. 612 Prevent Injury ideas? Check for S/S bleeding ideas? Safety Measures ideas?

44 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)

45 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

46 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?

47 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

48 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

49 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

50 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

51 Learning Goal Reflection Define and describe the differences between the following bleeding disorders: Thrombocytopenia ITP/TTP Hemophilia DIC

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53 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

54 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

55 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

56 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

57 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

58 Affects Agranulocytes Lymphocytes form antibodies immune response Monocytes Largest WBC Macrophages in tissues Live longer than neutrophils

59 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

60 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

61 Chemo Antibiotics Colony Stimulating Factors Bone Marrow Transplant (BMT) Peripheral Blood Stem Cell Transplant (PBST)

62 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

63 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

64 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

65 Cancer of the lymph system 1 st sx often enlarged lymph nodes Higher incidence in men Etiology unknown 2 Types: Hodgkin s Disease Non-Hodgkin s Lymphoma

66 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

67 Diagnosis Lymph node biopsy Reed-Sternberg cell Bone Marrow Biopsy CT Detect large nodes and metastasis Gallium Scan Radioactive tracer to malignant lymph tissue

68 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

69 Treatment includes: Radiation Therapy Chemotherapy Combination of 2 PBST Nursing Care: Coping w/ SE of radiation Chemo SE Infection Prevention

70 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.

71 Symptoms/Diagnosis/Treatment Same as Hodgkin s except: no pruritis no Reed-Sternberg cell

72 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?

73 Diagnosis: X-ray Bone marrow biopsy Serum Protein Electrophoresis Measures immunoglobulins 24 hour urine protein electrophoresis Look at: BUN/Creatinine Calcium Level Protein Levels

74 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

75 Management: No cure Radiation / Chemo BMT Nursing Care: Maintaining Hydration Pain management Activity / Nutrition Decrease infections

76 Risk for Injury Acute Pain Ineffective Therapeutic Regimen Pt Teaching Plan

77 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.

78 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

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80 Charlie Cobalt 64 yo Has been working in the factory for 35 years and is 1 year away from retirement. He comes in today c/o fatigue and SOB. CBC Dx: RBC 2.5 WBC 2.5 Hgb 8.2 Hct 20 Platlets- 24,000 Aplastic Anemia

81 Tx: Blood Transfusion Bone Marrow replacement AB s Corticosteroids No more factory!

82 Nursing Dx: Potential for infection Activity intolerance Potential for hemorrhage Fear Knowledge deficit

83 Jimmy Sutton 8yo Comes in complaining of severe fatigue, weakness, loss of appetite, and sick. CBC RBC- 3.2 WBC- 40,000 Hgb- 8.4 Platlets- 23,000

84 Dx: Possible ALL Leukemia is a cancer of the blood manufacturing center 2 types: Lymphocytic and Myelogenous 2 types: Acute and Chronic Confirm Dx? Bone marrow biopsy Tx: Chemotherapy Ax for infections (Cloudy urine, chest congestion) due to neutropenia.

85 Nursing Dx: Potential for infection Activity intolerance Impaired mucus membrane Knowledge deficit Fear Potential for hemorrhage (Small needles) Nutrition: < body requirements (Stomatitis)

86 Thomas Wheeling: In a fight after a soccer game. Unable to speak. CT Clean and dress wounds. Labs: HgB- 6.2 HcT Transfuse Blood ASAP Which? How? Signs of Reaction Big fever Flushed/Itching Dyspnea If seen do what?

87 Carlos is a 6 yo boy. He was brought in by his Mother C/O severe pain in his left knee Why? Obtain Hx US? History of Hemophilia How? From Mom (Carrier) to son. Dx: Internal bleeding in joint. Tx: Prevent injury, Infuse clotting factors Nursing Dx: Potential for

88 Kris is a 3 yo female brought in by her mother. Mother reports she fell on tiled floor at home while playing with her brother. Bruising of face and body covered with purpura and petechiae. Abuse? Labs: HgB Hct- 39 Platelets- 50,000 Dx: Thrombocytopenia Tx: Prevent further injury!!! Small needles

89 Sally is a 8 yo with a history of Leukemia. She was brought in by her father with c/o Not feeling well and cloudy urine. She finished her last round of chemo 3 days ago. Labs: WBC: 500 Platlets: 74,000 Dx: Leukemia, Stomatitis, Thrombocytopenia, Urinary infection Tx: Neutorpenic precautions Soft foods Prevent injury AB s Nursing Dx: Potential for.. Impaired oral mucus membranes

90 Carlton is a 28 yo male with a history of sickle cell anemia. Comes in today c/o severe abd. and leg pain. Dx: Sickle Cell Crisis How? Stress on body Dehydration Infection Smoking Tx: Treat the pain Infuse? Hydroxyurea? Treat the cause Nursing Dx: Pain! Knowledge deficit?

91 Michelle is a 17 yo female with c/o severe weakness, fatigue, and numbness and tingling in arms, and cold all the time History: Partial gastrectomy 8 months ago for weight issues Labs: HgB: 8.1 HcT: 32 Dx: Pernicious Anemia Tx: B12 1 x month for life Nursing Dx: Activity intolerance (rest periods)

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