N300 Hemolytic Disorders

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cbishop
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N300 Hemolytic Disorders
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2011-02-09 00:41:20
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Hemolytic Disorders
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Hemolytic Disorders
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  1. Pancytopenia
    when entire CBC is suppressed, marked decrease in the number of RBCs, WBCs, and platelets
  2. When is the Hemoglobin (Hb) value reduced?
    in cases of anemia, hemorrhage, and states of hemodilution.

    So, if Hb is increased, it can be found in polycythemia or in states of hemoconcentration-- which can be developed from volume depletion
  3. What is the HCT?
    • represents the percentage of RBCs compared with the total blood volume.
    • Reductions adn elevations of hct are seen in teh same conditions that raise adn lower Hb. HCT= 3xHb
  4. What does a WBC over 11,000 mean?
    associated with indection, inflammation, tissue injury or death, and malignancies (leukemia or lymphoma). Doesn't tell degree of illness but can help with etilogy
  5. What does a WBC of less than 4,000 mean?
    • Leukopenia
    • associated with bone marrow depression, severe of chronis illness, and some types of leukemia
  6. What is the WBC differential count?
    • measures the % of each type of leukocyte
    • provides clues to determine the cause
  7. What is a "shift to the left?"
    • when infections are severe, the body releases more granulocytes from the bone marrow. To meet the increased demand, mant young, immature polymorphonuclear neutrophils are released into circulation.
    • Since we write in lab notes the WBCs in order of maturity, with the immature ones on the left of the sheet. So.... the existence of many immature cells will be termed a "shift to the left".
  8. neutropenia
    • absolute neutrophil count is less than 1000 cells/mul
    • severe is less than 500 cells
  9. What is the normal platelet count?
    150,000 to 400,000
  10. What is thrombocytopenia?
    neutrophil count of below 100,000

    • bleed may occur.
    • Spontaneous hemmorrhage occurs when it is below 20,000
  11. What is Thrombocytosis and when does it occur?
    • excessive platelets
    • occurs with inflammation adn some malignant disorders
    • Excessive clotting is a likely complication
  12. What is anemia?
    deficiency in the # of RBCs, the quality of Hg, and/or the volume of packed RBCs (HCT).
  13. What is thalassemia?
    a group of diseases that have an autosomal recessive genetic basis involving inadequate productino of normal hemoglobin
  14. What is pernicious anemia?
    a disease in which the astric mucosa is not secreting IF beacuse of antibodies being directed against the gastric parietal cells and/or If itself. Causes Megaloblastic anemia
  15. What is megaloblastic anemia?
    • group of disorders caused by impaired DNA synthesis. Characterized by the presence of large RBCs (megaloblasts)
    • Majority result in teh deficiency of Cobalmin (vit B12) and folic acid
  16. What does the lack of B12 do?
    • Alters the structure and disrupts the function of the peripheral nerves, spinal cord and brain.
    • Impairs cellular division and maturation esp in rapidly proliferating RBCs.
  17. What is IF?
    • Intrinsic factor
    • Protein secreted by the patietal cells of teh gastric mucosa
    • IF is required for colbalmin absorption in the small intestine
  18. What happens when you have a folic acid deficiency?
    You can get megaloblastic anemia. Folic acid is required for DNA synthesis-- RBC formatino and maturation
  19. What is aplastic anemia?
    • results from a decreased production of bone marrow elements.
    • Pancytopenia
    • Hypocellular bone marrow
  20. What is pancytopenia and what does it cause?
    • decrease of all blood cell types-- RBCs, WBCs, and platelets.
    • Causes Aplastic anemia
  21. What is hypocellular bone marrow and what does it cause?
    • marrow replaces with fat
    • Causes Aplastic anemia
  22. What is acute blood loss caused by?
    • trauma, stroke, adn surgery-- a result of sudden hemorrhage
    • Blood volume and RBCs usually diminished
  23. Sources of chronic blood loss are.....
    bleeding ulcer, hemorrhoids, menstrual and post menopausal blood loss
  24. What is hemolytic anemia?
    destruction or hemolysis of RBSs at a rate that exceeds production.
  25. What is intrinsic hemolytic anemia?
    • result from defects in the RBCs themselves caused by abnormal hemoglobin (e.g. sickle cells), enzyme deficienciesthat alter glycolysis, or RBC membrane abnormalities
    • *usually hereditary
  26. What is are extrinsic hemolytic anemias?
    These are acquired. in this type, the patient's RBC's are normal, but damage is caused by external factors such as trapping of cells within the sinuses of the liver or spleen, antibody-mediated destruction, toxins, or mechanical injury
  27. What is SCD?
    • Sickle Cell Disease
    • Group of inherited, autosomal recessive disorders.
    • Chronic form of hemolytic anemia
  28. What race is most likely to have SCD?
    • 8% of African americans are heterozygous (carriers),
    • 1% are homozygous (have the disease)
  29. What is Hydroxyurea
    • An anti-sickling agent
    • Erythropoietin in pt unresponsive to this
  30. What is are the 3 causes of acquired hemolytic anemia?
    • 1. physical factors
    • 2. immune reactions
    • 3. infectious agents and toxins
  31. What is hemochromatosis?
    autosomal recessive disease characterized by increased intestional iron absorption and, as a result, increased tissue iron depostition. The most common genetic disorder among whites
  32. What is polycythemia?
    Increase in teh number of circulating RBCs and concentration of Hgb
  33. What is primary polycythemia?
    neoplastic stem cells disorder increase production of RBCs, granulocytes, and platelets
  34. What is secondary polycythemia?
    most common out of the two, abnormal increase in erythropoietin causeing excessive RBC production
  35. What is Erthromyalgia?
    Burning sensation of the fingers and toes
  36. What kind of polycythemia can occur with hypoxia?
    Secondary
  37. What is Thrombocytopenia?
    • a decrease in the # of circulating plateles
    • Lecture= <100,000
    • Book= <150,000
  38. What is the circulating life of platelets?
    8-10 days
  39. What are the 3 causes of Thrombocytopenia?
    • 1. Dec. production (inherited or acquired)
    • 2. Inc. destruction (immune system defect called ITP or caused by infection or drug induced) acute form found in children. Chronic form found in women 20-50
    • 3. Inc. Consumption
  40. What is hemophilia?
    group of hereditary sex-linked clotting factor disorders characterized by prolonged coagulation times resulting in excessive bleeding
  41. What is Hemophilia A?
    • deficiency in factor VII (necessary for stabilization of fibrin clots)
    • Most common form (80% of all cases)
  42. What is hemophilia B?
    deficiency in factor IX (vitamin dependent beta globulin essential in stage one of intrinsic coagulation system as an influence on teh amount of thromboplatin available)
  43. What is Von Wilebrand's disease?
    deficiency of von Willebrand factor which is necessary for factor VII activity and platelet adhesion.
  44. What is DIC?
    disseminated intravascular coagulation

    • abnormal initiation and acceleration of clotting adn simultaneous hemorrhage
    • Paradoxical bleeding occurs as a result of consumption of clotting factors and platelets
  45. What is the cause of DIC
    Always an underlying condition
  46. What is Leukopenia?
    a decrease in the total WBC count (granulocytes, monocytes, and lymphocytes)
  47. What is Granulocytopenia?
    a deficiency of granulocytes (neutrophils, eosinophils, and basophils)
  48. What is neutropenia?
    • reduction in neutrophils-- some use this interchangeably with granulocytopenia.
    • Neutrophils are monitored closely becuase they play a major role in phagocytizing pathogenic microbes-- indicates a patients risk for infection.
  49. What is the normal range for neutrophils?
    4000-11000
  50. What is the level for neutropenia?
    Severe neutropenia?
    • <2000
    • <500
  51. What is Leukemia?
    • •Malignant disorder of blood forming tissues of the bone marrow, spleen, and lymph system
    • characterized by unregulated proliferation of WBCs and their precursors.
  52. What is ALL
    –Acute lymphocytic leukemia (ALL)

    •Peak ages 2-4

    • •Immature granulcytes
    • proliferate and accumulate in marrow
  53. What is CLL?
    • •More common in men peaks between
    • ages 50-70

    • •Abnormal and incompetent
    • lymphocytes proliferate and accumulate in lymph nodes and spleen
  54. What is AML?
    • •Al age groups but peak is around
    • age 60

    • •Uncontrolled proliferation of myeloblasts
    • which are precursors to granulocytes and accumulate in bone marrow
  55. What is CML?
    •Uncommon under age 20

    • •Uncontrolled proliferation of
    • granulocytes resulting in increased circulating blast cells

    • •Marrow expands into long bones,
    • and spleen
  56. What are Malignant Lymphomas?
    • Lymphomas are a group of
    • malignant neoplasms that affect the lymphatic system resulting in the proliferation of lymphocytes

    Hodgkin's and Non-Hodgkins
  57. What are characteristic's of Hodgkin's lymphoma?
    •More common in men and Whites

    • •Peaks 15-35 years of age &
    • 55-70 years of age

    • •Possible causes (Epstein Barr
    • virus/familial pattern, exposure to toxins)

    • •Originates in lymph node and
    • infiltrates the spleen, lung and liver

    •Reed-Sternberg cell present
  58. What are characteristics of Non-Hodgkin's lymphoma?
    •Most common form of lymphoma

    •Affects adults 50-70 years old

    •More common in men and whites

    • •Link to viral infections, immune
    • disorders, genetic abnormalities, exposure to chemicals, infection with
    • H-pylori

    •Reed-Sternberg cells are absent

    • •Lymphoma originates outside lymph
    • and infiltrates the lymph nodes

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