Diseases of the blood

1. BLOOD DISORDERS/DISEASES Diseases of the blood

2. ANEMIATHE MOST COMMON DISORDER OF THE BLOOD LACK OF RBC’S CARRYING OXYGEN • Main classes of etiology include: Excessive blood loss – hemorrhage Excessive blood cell destruction – hemolysis Deficient red blood cell production

3. ANEMIA Diagnosing the type of anemia is based on: Nutrient deficiencies Presence of immature RBC’s Characteristic color and volume of RBC’s Lab values

4. GENERAL SIGNS AND SYMPTOMS (of all anemias) • Pallor or lack of color-especially mucuous membranes • Fatigue • Dizziness • Headaches • Decreased exercise tolerance • Rapid heartbeat • Shortness of breath

5. GENERAL PROGNOSIS(if anemia is untreated) • Heart failure • Cardiovascular collapse • Shock

6. IRON DEFICIENCY ANEMIA(most common cause of anemia) ETIOLOGY: • Increased iron requirements, • Impaired iron absorption • Hemorrhage

7. WHAT DO I NEED TO KNOW ABOUT IRON DEFICIENT ANEMIA? • Iron is needed to synthesize hemoglobin---which is needed to transport oxygen • Iron requirements are greatest from birth to age two

8. WHAT DO I NEED TO KNOW ABOUT IRON DEFICIENT ANEMIA? • Pregnancy requires supplements to ensure fetus gets enough iron • Sudden growth spurts and onset of menstruation are risks for iron deficient anemia

9. WHAT ELSE DO I NEED TO KNOW ABOUT IRON DEFICIENT ANEMIA? Absorption takes place in the GI tract so diseases that affect the mucosa of the GI tract can put your patient at a risk for anemia because they limit the availability of Iron needed to synthesize hemoglobin

10. EXAMPLES OF CAUSES • Chronic diseases that cause inflammation (inflammatory changes can suppress red blood cell synthesis in bone marrow and shorten life of RBC’s) • Removal of the stomach • Chronic disease treatment • Bowel disorders

11. DIAGNOSIS CBC- low H&H Reports of signs and symptoms including: • Extreme fatigue • Pale skin • Weakness • Shortness of breath • Headache • Dizziness or lightheadedness • Cold hands and feet • Irritability • Fast heartbeat • Unusual cravings for non-nutritive substances, such as ice, dirt or starch • Poor appetite, especially in infants and children with iron deficiency anemia • An uncomfortable tingling or crawling feeling in your legs (restless legs syndrome)

12. TREATMENT OF IRON DEFICIENCY ANEMIA • Diet rich in iron • Vitamin supplements with iron • IV Supplements if necessary • NOTE: iron supplements are constipating and oral doses should not be taken on an empty stomach

13. PERNICIOUS ANEMIA Etiology: • Vitamin B12 absorption or intake is deficient or utilization is inadequate • Deficiency in intrinsic factor (a protein in the stomach needed so B12 can be absorbed from the small intestine) • Removal of stomach or the bowel • Abnormal bacterial growth in small intestine • Strict vegetarianism

14. HOW DOES LACK OF B12 OR INTRINSIC FACTOR CAUSE ANEMIA? • The deficiency causes the membranes of immature RBC’s to rupture easily leaving fewer RBC’s to carry oxygen

15. SYMPTOMS OF PERNICIOUS ANEMIA • Nausea, vomiting, burning of the tongue • Neurological disturbances such as numbness, weakness, poor reflexes • GENERAL SYMPTOMS OF ANEMIA • Pallor or lack of color-especially mucuous membranes • Fatigue • Dizziness • Headaches • Decreased exercise tolerance • Rapid heartbeat • Shortness of breath

16. DIAGNOSIS OF PERNICIOUS ANEMIA • Health history to rule out inherited lack of intrinsic factor • Physical exam shows jaundice, enlarged liver, irregular heart rate • CBC abnormal • Bone Marrow aspiration/biopsy(cells that make blood cells will be larger than normal)

17. TREATMENT OF PERNICIOUS ANEMIA • Vitamin B12 supplementation (injectable if it cannot be absorbed) • NOTE: Pernicious Anemia puts you at a higher risk for stomach cancer

18. HEMOLYTIC ANEMIA Etiology: Reduction in RBC’s by conditions that accelerate destruction of RBC’s such as: • Inherited abnormalities such as hemoglobin defects, enzyme defects and membrane defects that impair intrinsic factor physical properties • Infections/immune disorders (HIV, Lupus) • Medications (chemotherapy)

19. SIGNS AND SYMPTOMS OF HEMOLYTIC ANEMIA • Mild may have no signs or symptoms • General s/s of anemia see slide four • Increased serum bilirubin levels from the hemoglobin destruction in destroyed RBC’s • Jaundice, dark urine and feces due to accumulation of bilirubin system

20. DIAGNOSIS OF HEMOLYTIC ANEMIA • Enlarged spleen • CBC Includes H&H • Liver function blood tests • Bone Marrow Aspiration and or biopsy

21. TREATMENTS FOR HEMOLYTIC ANEMIA • Blood transfusions • Medication • Plasmaphoresis • Surgery • Stem Cell transplants

22. Blood Transfusions

23. Blood transfusion reaction • Caused by antibodies forming and person rejecting blood being transfused. • Stop transfusion immediately.

24. MEDICATIONS • Corticosteroids (Prednisone) - limit immune system from making antibodies(proteins) against RBC’s • Rituximab, Cyclosporine – drugs that suppress your immune system

25. PLASMAPHORESIS • A procedure that removes antibodies from the blood using a needle inserted into a vein. The plasma is separated from the rest of the blood and then donor plasma is put back into the blood.

26. SURGERY • Spleenectomy – removal of spleen. An enlarged or diseased spleen may remove more RBC’s than normal. Removing it can help reduce high rates of RBC destruction Note : a healthy spleen helps fight infection and filters out old or damaged blood cells.

27. APLASTIC ANEMIA • bone marrow doesn’t make enough new blood cells because stem cells are damaged (Also caused bone marrow failure) • Can be acquired or inherited

28. ACQUIRED APLASTIC ANEMIA ETIOLOGY • Exposure to toxins—pesticides • Radiation and chemotherapy • Antibiotics like chloramphenicol • Hepatitis, HIV,CMV, Eptstein-Barr virus • Lupus, rheumatoid arthritis

29. SIGNS AND SYMPTOMS OF APLASTIC ANEMIA • General signs and symptoms: • Pallor or lack of color-especially mucuous membranes • Fatigue • Dizziness • Headaches • Decreased exercise tolerance • Rapid heartbeat • Shortness of breath • Decreased RBC’s, WBC’s and platelets • Nausea • Skin rashes

30. DIAGNOSIS OF APLASTIC ANEMIA • History and physical • CBC • Reticulocyte count (# of young blood cells) • Bone marrow aspiration/biopsy

31. TREATMENT OF APLASTIC ANEMIA • Removal of known cause if possible • Blood transfusions • Blood and marrow stem cell transplants • Medications

32. Blood and marrow stem cell transplants

33. MEDICATIONS • Stimulate bone marrow-Erythropoieten and colony stimulating factors • Suppress immune system Antihymocyte globulin (ATG), cyclosporine and methylprednisone – all three given together

34. SICKLE CELL ANEMIA • Blood cells are crescent shaped instead of round, because of abnormal hemoglobin. • It is thready and cells are stiff and sticky. • Sickled cells block blood flow, causing pain, infection and organ damage.

37. WHAT CAUSES THE ANEMIA? • After 10-20 days sickle cells die. Bone marrow can’t make new RBC’s fast enough to replace the dying ones

38. FACTS ABOUT SICKLE CELL ANEMIA • It is inherited-one sickle gene from each parent • People who have the disease are born with it • If sickle cell gene from one parent and normal gene from other parent you have sickle cell trait. • Most common in African Americans

39. SIGNS AND SYMPTOMS OF SICKLE CELL DISEASE • General anemia s/s from slide four • Acute Pain throughout body is called sickle cell crisis • Chronic pain in bones • Multiple organ failure • Infections and dehydration can contribute to a sickle cell crisis

40. DIAGNOSIS • Blood testing at newborn screening. • Amniotic fluid sampling before birth.

41. TREATMENT • If in sickle cell crisis, treatment involves pain management with NSAIDS and or opiates, fluids and oxygen if levels are low. • Hydroxyurea – medication that causes body to make fetal hemoglobin which helps blood cells from sickling and improves anemia • Blood transfusions

42. COMPLICATIONS • Stroke • Eye damage • Multiple organ failure • Leg ulcers • Gall stones • Priapism (painful erection in males)

43. EMERGING TRENDS • Research on blood and marrow stem cell transplants and gene therapy is ongoing. • New medications: Decitabine – prompts body to make Fetal hemoglobin Adenosine A2a receptor agonists – may reduce pain related complications

44. Disseminated Intravascular Coagulation (DIC) This disease is an activation of blood clotting mechanisms that occurs in response to various other diseases. Small blood clots form throughout the body, followed by a disruption in the coagulation process and then abnormal bleeding from several places within the body. This may be followed by multiple organ failure and death if not treated immediately. ETIOLOGY/CAUSES: Cancer Obstetric: Abruptioplacentae, pre-eclampsia, embolism Massive tissue injury from trauma, burns, surgery Infections: Sepsis

45. Signs and symptoms • Bleeding into intravascular spaces due to destruction of platelets and clotting factors. • Widespread hemmorage • Extensive bruising • Renal failure • Shock

46. Diagnosis • Blood test s • Prolonged PT, & PTT • Severe thrombocytopenia • Fragmented RBC’s • Physical exam

47. Treatment • Reverse underlying cause • Platelet transfusions

48. Hemophilia • An inheirited clotting disorder due to a deficiency of clotting factors • X linked recessive disorder primarily affects males • The body is not able to control blood clotting or coagulation when a blood vessel is broken • Hemophilia A is deficient in Factor VIII • Hemophilia B is deficient in Factor IX

49. Signs and symptoms • Internal and external bleeding episodes • Most common internal bleed is into the joint. If bleeding into joints, can cause joint fibrosis • Intracranial hemorrhage often cause of death

50. Diagnosis • Genetic testing • PT/PTT • Platelet count • H&H