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Disorders of the Hematological System

Disorders of the Hematological System. Blood and Lymph. Two of the body’s most important functions are: Transportation Protection-from foreign invaders Achieved through the circulatory and lymph systems Blood is the primary transportation fluid. Blood Cells. RBC’s, WBC’s, and Platelets

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Disorders of the Hematological System

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  1. Disorders of the Hematological System

  2. Blood and Lymph • Two of the body’s most important functions are: • Transportation • Protection-from foreign invaders • Achieved through the circulatory and lymph systems • Blood is the primary transportation fluid

  3. Blood Cells • RBC’s, WBC’s, and Platelets • Blood cells are made in bone marrow • Other structures involved in the blood cell production are: • Kidneys (Erythropoietin) • Liver (stores iron) • Lymph system (breaks down blood cells, produces WBC)

  4. Red Blood Cell Production (RBC) • Erythropoiesis is the process of making red blood cells • Stimulated by decreased PO2 • The kidneys releases Erythropoietin • Reticulocytes are immature RBC’s • Iron, folic acid, and Vitamin B12 are needed for RBC production • Iron is needed for Hemoglobin production

  5. Red Blood Cells (RBC) • Normal Values • Males 4.7-6.1 • Females 4.2-5.4 • Increased: Dehydration, Polycythemia • Decreased: Anemia, Leukemia, Post-hemorrhage

  6. Hemoglobin (Hgb) • Contained within RBC’s – red pigment Oxygen carrying compound of the RBC • Normal Values • Males 14-18 g/dL • Females 12-16 g/dL • Increased: Dehydration, COPD, Polycythemia • Decreased: Anemia, leukemia, Post-hemorrhage

  7. Hemoglobin • To measure Hgb, RBC’s are broken and Hgb is released and counted • Hemoglobin content is expressed as normochromic or hypochromic anemia • Erythrocytes are classified according to: • Size, shape and color • RBCs size is usually expressed as: macrocytic, microcytic, or normocytic • Average life span of an RBC = 120 days

  8. Hematocrit • A measure of the packed cell volume of RBCs • Expressed as a % of the total blood volume • Normal Values • Males 42-52% • Females 37-47%

  9. Hematocrit • Increased: Polycythemia, dehydration • Decreased: Anemia, leukemia, hemorrhage, fluid overload

  10. White Blood Cells • Have nuclei, are colorless, live from a few days  several years • Primarily involved in body defenses such as destruction of bacteria and viruses • “Differential” – blood count in which the different kinds of WBCs are counted and reported  can discriminate between virus and backteria

  11. Platelets • Also called “thrombocytes • Normal Values 150,000-400,000 cells/microliter – involved with clotting • 5-9 day life span • Increased- Polycythemia, granulocytic leukemia • Decreased- Bleeding, thrombocytopenia, Chemotherapy

  12. Erythrocyte Indices • MCV, MCH, MCHC • Will help determine type of anemia • Iron Deficiency • Folic Acid Deficiency • B12 Deficiency • Definition of Corpuscular: A living cell

  13. Mean Corpuscular Volume (MCV) • Volume (size) of the RBC • Hematocrit divided by RBC Count • Low • RBC = Microcytic (small) • Iron Deficiency Anemia • High • RBC = Macrocytic (large) • B12 • Folic Acid Deficiency Anemia

  14. Mean Corpuscular Hemoglobin (MCH) • Weight of hemoglobin in a RBC • Hemoglobin divided by the total RBC count • Low • Hypochromic (Deficient of hemoglobin) • Iron Deficient Anemia • High • Hyperchromic- Lab error

  15. Mean Corpuscular Hemoglobin Concentration (MCHC) • Concentration of hemoglobin in the RBC • The hemoglobin is divided by the RBC • Low • Hypochromic (deficient of hemoglobin) • Iron Deficiency Anemia • High • Hyperchromic • Lab Error

  16. Blood Types • Genetically determined • Determined by the presence or absence of specific antigens on the outer surface of the RBC • In certain blood types, the antigens on the RBC are accompanied by antibodies in the plasma.

  17. Blood Types • O – Good Donor, Bad Receiver • Contains A and B antibodies at birth • A- Very selective- receives A only • Contains B antibodies at birth • B- Very selective- receives B only • Contains A antibodies at birth • AB – Good Receiver, Bad Donor • Does not contain A or B antibodies

  18. Rh Factor • Located on the surface of RBCs • “positive” = have the factor • “negative” = do not have the factor • When an Rh- person has been exposed to Rh+ blood, antibodies develop within 2 weeks No reaction with this first exposure.

  19. Rh Factor • On any exposure thereafter, the Antibodies will attack Rh positive blood. • “Rh incompatibility” = mother’s antibodies hemolyze the fetal RBCs  rupture and loss of contents of cells

  20. Rh Factor • RhoGAM – intramuscular (or IV) injection • Action: desensitization antibodies • Next baby’s intrautero development without the potential complications associated with Rh incompatibility • Who (include blood type and Rh factor) will need to receive Rhogam? • A women who is Rh Negative and is pregnant (prophylaxis) or has just given birth (baby is Rh positive)

  21. Rh Factor • When is RhoGAM given? • 2 injections • 1. between 20-28wks pregnancy • 2. within 72 hrs. post partum

  22. Who’s the Best? • Which blood type (including Rh factor) is the universal donor? • O negative • Which blood type is the universal recipient? • AB positive

  23. Lymphatic System

  24. LYMPHATIC SYSTEM • A subdivision of the cardiovascular system • Consists of: • Lymph vessels • Lymph fluid • Lymph tissue • Basic Functions: • Maintenance of fluid balance • Production of lymphocytes • Absorption and transportation of lipids from intestine to bloodstream

  25. LYMPHATIC SYSTEM • Lymph is a specialized fluid formed in the tissue spaces and transported by way of lymphatic vessels to eventually  reenter the circulatory system. • Lymphatic Tissue • Lymph nodes • Tonsils • Spleen • Thymus

  26. DISORDERS OF THE HEMATOLOGIC AND LYMPHATIC SYSTEMS

  27. Anemia • Characterized by low RBC count, ↓ hematocrit and hemoglobin • Look at CBC indices to determine RBC health Anemia causes delivery of an insufficient supply of oxygen AND nutrients to the cells • Loss of the O2-carrying element in the blood results in a supply/demand imbalance in vital organs.

  28. Anemia • Causes • Hemorrhage • Impaired production of RBC’s • Increased destruction of RBC’s (hemolysis) • Nutritional deficiencies

  29. Diagnostic Tests: • Coombs’ Test • Hemoglobin Electrophoresis • Schilling • Sickledex • TIBC • Gastric Analysis

  30. Coombs Test • Direct Antiglobulin test – identifies autoantibodies against RBCs • Coombs + = autoimmune hemolytic anemia, hemolytic disease of the newborn, lymphomas, LE, mycoplasma infection, and infectious mononucleosis

  31. Big five DOE Chest pain Palpitations(Tachycardia) Dizziness (Vertigo) Pallor Anorexia Disorientation Dyspepsia Fatigue Headache Insominia General Signs and Symptoms of Anemia

  32. Hypovolemic Shock • May be caused by severe dehydration or extreme blood loss • May require 1000 mL loss before severe s/s • Initial s/s: • Thirst • Weakness • Restlessness • Later s/s • Hypotension • Tachycardia and weak, thready pulse • Pallor and cold, clammy skin • Oliguria • Disorientation • Prostration

  33. Hypovolemic Shock • H&H will not decrease until ~6-8 hours • Treatment • If bleeding source obvious: direct pressure • If not, intervention ASAP • IV fluids (Isotonic/Volume expanders: NS) • O2 • O negative blood transfusion • Platelets • Plasma • Factor 7

  34. Nursing Interventions • Monitor blood and fluid restoration • Identify blood loss sites to control bleeding • VS frequently • I/O – particularly careful monitoring of urine output • Patient teaching

  35. Pernicious Anemia • Caused by autoimmune disease: There is an absence of glycoprotein intrinsic factor that is usually secreted by the stomach and assists with the absorption of Vitamin B12. • Without this factor, Vitamin B12 is unavailable for the maturation of the Red Blood Cell Production.

  36. Subjective Palpitations Nausea Flatulence Indigestion Soreness and burning of the tongue Tingling of the hands and feet Impaired proprioception Objective Smooth and erythematous tongue Gum infection Mental disorientation Personality changes Behavior problems Severe neurological impair from destroyed Spinal Cord Tracts Signs and Symptoms

  37. Diagnostic Tests • Schilling test • Subject will get two doses of vitamin B-12 (cobalamin). The first dose is taken by mouth. The second dose is radioactive and is given as a shot 2-6 hours later. Urine is tested for radioactive B12 • Reveals malabsorption of Vit B12 • Serum Megaloblastic Anemia Profile (serum level of B12) soon to replace Schilling test • Will reveal serum levels of Vit B12 + more • Gastric Analysis – determine pH

  38. Medical Management • Medication • B 12 injections • Folic acid supplement • Iron replacement • Transfused with Packed RBC if severe anemia • CBC every 3-6 months

  39. Iron-deficiency Anemia • Plenty of RBC, normal H &H sometimes, but hypochromic • Causes • Diet • Usually chronic bleeding • Body’s demand exceeds its absorption • Pregnancy • Infants • Young adolescents • Malabsorption of iron as in celiac disease or sprue • Subtotal gastrectomy because iron needs an acid environment to be absorbed.

  40. Iron Deficiency Anemia • Signs and Symptoms • Sore, Swollen Tongue • Cracks in the side of the mouth • Brittle nails • Increased frequency of infections • PICA- Ice, dirt, paint etc. • Restless Leg Syndrome

  41. Iron Deficiency Anemia • Medical Management • Because less than 10% of iron is absorbed in the duodenum, high doses of Iron supplement are needed • Additional Vitamin C has shown to enhance iron absorption • See p. 277 Box 7-2 for food sources of nutrients needed for Erythropoiesis

  42. Z-track Because some patients cannot tolerate oral preparations of iron, Iron can be given IV or IM. The z-track method of giving iron dextran is preferred to prevent staining

  43. Sickle Cell AnemiaEMcrit.org • Genetic --Recessive homozygous • Predominately found in the African and African-American Population • First crisis usually occurs at 10-12 weeks of age • Pathophysiology: After the hemoglobin molecules give up their oxygen, some of them may cluster together and form long, rod-like structures.

  44. Sickle Cell Anemia • A sickle cell is an abnormal, crescent-shaped RBC containing hemoglobin S (a defective hemoglobin molecule) • Sickle Cell crisis – an episode of acute “sickling” of RBCs which causes occlusion and ischemia in distal blood vessels • Sickling clumping or aggregation of these misshapen RBCs which lodge in small vessels

  45. Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis, vaccination against pneumococcus bacteria and folic acid supplementation. Pulmonary Hypertension

  46. Sickle Cell Maintenance • Health maintenance for patients with sickle cell disease • Starts with early diagnosis, preferably in the newborn period • Vaccination against pneumococcus bacteria (infections are a major complication of Sickle-Cell anemia) • Folic acid supplementation • Prevention of infection and dehydration

  47. Medical Management • Supportive • Pain is biggest problem • PCA pump is indicated • Long-term MS therapy with breakthrough pain meds • Rehydration • Packed cells for anemia but clients have problems with iron overload • Probable treatment/cure is stem cell transplant

  48. Aplastic Anemia aka Hypoplastic Anemia • Congenital Aplastic Anemia is caused by a chromosomal alteration • Acquired Aplastic Anemia related to chemical exposures. • Cause of 70% of acquired aplastic anemias remains unknown(idiopathic)

  49. Aplastic Anemia • People are pancytopenic when: • Reduced or absent red blood cells • Reduced or absent white blood cells • Reduced or absent platelets

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