Hematology

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  • lots of plasma is H2O.\n
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  • almost half blood volume\n
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  • Green is lymphocyte.\nPurple, platelets.\n
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  • reticulocyte - baby RBC with nucleus. \n
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  • remember: many undifferentiated stem cells, can mature into both lymphoid or myeloid. Make both RBC and WBC. \nMyeloid -> monoblast -> monocyte -> macrophage. \nLymph -> B, T, and NKC\n
  • Epo stimulates marrow to make RBCs and Hb production. \n\n
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  • Lab serum feritin low - know there is low iron. Iron hard to absorb.\n
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  • Many disorders show issue with bili.\nLiver failure - elevation of things, RBC’s hemolyze, failure, obstructions. \n
  • RBC described by size and color\nnormal: normocutic/chromic\nPoikilocytosis - variabile shape: target, tear drop, etc.\nAnisocytosis - variation in size\n
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  • MCV - volume\nMCH - amt Hb. \nMCHC - how concentrated the Hb is\nRetic count - helpful for watching tx (put on iron, should see change in month, should increase). No response, worry about bone marrow\n\n
  • small pale cells\ndue to deficiency of iron (infants). Cows milk is much lower. #1 cause of fe efficiency is too much milk. \nDue to loss\n
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  • unable to make Hb, so see small cells, pale,\nTIBC - transferrin, protein carrier. Hi, lots of empty train cars, lots of ability to bind fe. \n
  • pale RBC’s\n
  • Retics in a week, \nNeed to take Fe TIB = constipation --> take cholase, fiber, etc.\n\n\n
  • microcytic and homochromic\n\n
  • Free alpha chains, cause hemoluis\n
  • Mediteranian and Asian descent.\nSee Hb lab not matching presentation. \n
  • Fe in granules around nucleus, waiting to be taken up by Hb. \n
  • WHat to do. \nTHalasemia when HbA is less than 2\n
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  • probably b12 or folate\n
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  • triggared by hypoxia, decreased temperature, sometimes fever due to infection. \nJoint pain, abdominal pain. \n
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  • Scicle celss only leave a short time or can be sequestered in spleen. Can get into an aplastic crisis. \n
  • ishemia, strokes, swelling peripherally, hematuria, \nretinal damag, heart failure, stasis ulser.\n
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  • autosomal recessive. \n
  • inclusion of chromatin -- splenectomy.\n
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  • cause bleeding, anemea, infections.\n
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  • Look in mucus membrane\nSystolic murmur. Depression, weakness. \n
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  • ideopathic, not-malignant.\nTx - phelbotomy.\nCauses Thrombus! Sticky blood. \n
  • sleepy. \n\n
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  • most often will go the bone. \n
  • see malignant plasma cells arise from b cell line, see an increased release on non-specific Ig. \nM protein, associated with Ig.\nBence Jones proteins in urine. \n
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  • there are two paths: intrinsic and extrinsic\n
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  • fibrin is the end product\n
  • Important in DIC tx\n
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  • Ab mediated destruction.\nSometimes is limited, sometimes reoccurs.\n
  • nomal plt ct-200,000\n
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  • Global clotting, followed by global bleeding.\n
  • increased degredation product.\nUnderlying problem is clotting, clinical manifestation is bleeding. \n
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  • Hematology

    1. 1. Hematology Reference: Pathophysiology by Kathryn McCance Mindy Milton, MPA, PA-C July 1, 2010 1Mosby items and derived items © 2006 by Mosby, Inc.
    2. 2. Components of the Blood Plasma: 55-65% of blood Formed elements:  Plasma proteins  Albumin  Globulin  Immune globulins  Clotting factors 2Mosby items and derived items © 2006 by Mosby, Inc.
    3. 3. Composition of the Blood: Plasma 3Mosby items and derived items © 2006 by Mosby, Inc.
    4. 4. Composition of Blood: Formed Elements 4Mosby items and derived items © 2006 by Mosby, Inc.
    5. 5. Structure of the RBC Stroma: innermost layer of lipids and proteins Biconcave Disc - navigation and diffusion Reversible deformability 120 days life span 42-48% of the blood volume 5Mosby items and derived items © 2006 by Mosby, Inc.
    6. 6. RBCs 6Mosby items and derived items © 2006 by Mosby, Inc.
    7. 7. RBCs 7Mosby items and derived items © 2006 by Mosby, Inc.
    8. 8. Peripheral Blood Smear 8Mosby items and derived items © 2006 by Mosby, Inc.
    9. 9. RBC 9Mosby items and derived items © 2006 by Mosby, Inc.
    10. 10. Hemoglobin Structure  Two Alpha chains  Two Beta chains  Single molecule of Heme connected to iron which then can interact with oxygen  Iron-oxygen binding is weak and reversible 10Mosby items and derived items © 2006 by Mosby, Inc.
    11. 11. Structure of Hemoglobin 11Mosby items and derived items © 2006 by Mosby, Inc.
    12. 12. Red Blood Cell Formation Myeloid stem cells in the red bone marrow produce RBCs Takes 5-7 days to mature and form reticulocyte and erythrocyte Nutrients needed for normal red blood cell formation:  Vitamin B12 see in anemias have issue here  Folic Acid  Iron 12Mosby items and derived items © 2006 by Mosby, Inc.
    13. 13. Stages of RBC Formation See reticulocyte have remnants of nucleus. May see more in anemia. <------ Use retic index to see if responding to bone marry tx. 13Mosby items and derived items © 2006 by Mosby, Inc.
    14. 14. Maturation of Formed Elements 14Mosby items and derived items © 2006 by Mosby, Inc.
    15. 15. Regulation of Erythropoesis Erythropoietin is secreted by the kidney in response to tissue hypoxia Increased erythropoietin will increase oxygen carrying capability of the blood Functions:  Stimulates increased cell division rates in erythroblasts  It accelerates Hgb concentration ptsn with chronic hypoxemia - adaptation with increased Epo and RBC’s. 15Mosby items and derived items © 2006 by Mosby, Inc.
    16. 16. Erythropoesis HYPOXIA Erythropoetin < RBC’S <Arterial PO2 16Mosby items and derived items © 2006 by Mosby, Inc.
    17. 17. increased Hb will change size and color of RBC’s Hgb Formation Iron absorbed in duodenum Small amount of absorbed iron is bound to transferrin, a protein transport molecule Remainder is stored in the intestinal epithelial cells and bone marrow as ferritin In the bone marrow the transferrin/iron complex binds with receptor site on erythroblasts Releases iron into erythroblasts and transferrin returned to blood stream Pernicious anemia - loss of intrinsic factor, need B12 from stomach to 17Mosby items and derived items © 2006 by Mosby, Inc. absorb in duodenum.
    18. 18. Iron Cycle Duodenum Iron/transferrin Storage Spleen Storage Liver Storage intestine Storage Macrophages Bone Marrow Increased RBC’S 18Mosby items and derived items © 2006 by Mosby, Inc.
    19. 19. Bilirubin conjugated byIron Cycle the liver to help excretion in the bile. 19Mosby items and derived items © 2006 by Mosby, Inc.
    20. 20. Red Blood Cell Turnover KNOW THIS PICT 20Mosby items and derived items © 2006 by Mosby, Inc.
    21. 21. Definitions NORMOCYTIC  MEGALOBLASTIC NORMOCHROMIC  POIKILOCYTOSIS MICROCYTIC  ANISOCYTOSIS MACROCYTIC Megaloblastic microcytic = B12 defic 21Mosby items and derived items © 2006 by Mosby, Inc.
    22. 22. Peripheral Smear The RBC’s are smaller than normal, increased pallor Increased variation in size (anisocytosis) Increased variation in shape (poikilocytosis) 22Mosby items and derived items © 2006 by Mosby, Inc.
    23. 23. Important Values MCV  87-103 MCH  27-32 MCHC  32-36 RETICULOCYTE  1% COUNT  RETICULOCYTE COUNT x Hct RETICULOCYTE  >3%=bleeding INDEX  <3%=decreased formation 23Mosby items and derived items © 2006 by Mosby, Inc.
    24. 24. Microcytic Hypochromic Anemia Iron Deficiency  ETIOLOGY  Dietary deficiencies of iron  Infants need 1mg/kg/day  Breast fed receive .4mg of iron per quart  Cows milk less than .1mg per quart  Bleeding with increased need  Gastrointestinal and gynecological cancers  Peptic ulcer disease 24Mosby items and derived items © 2006 by Mosby, Inc.
    25. 25. Microcytic Hypochromic Anemia Iron Deficiency  Etiology:  Maternal needs for iron in pregnancy is about 800mg: 300mg for fetus and placenta, and 500mg needed for maternal blood expansion  Rapid expansion of blood volume during second trimester is often manifested by drop in Hgb  Diagnostic data  Microcytosis: decreased MCV , microcytosis 25Mosby items and derived items © 2006 by Mosby, Inc.
    26. 26. Microcytic Hypochromic Anemia Iron Deficiency  Diagnostic data  Hypochromia:  Reduced to very low serum iron  Reduced to very low serum ferritin  High TIBC  In pregnant women the microcytosis and hypochromia may not be as clear but serum ferritin is low 26Mosby items and derived items © 2006 by Mosby, Inc.
    27. 27. Microcytic Hypochromic Anemia The RBCs here are smaller than normal and have an increased zone of central pallor. This is indicative of a hypochromic (less hemoglobin in each RBC) microcytic (smaller size of each RBC) anemia. There is also increased anisocytosis (variation in size) and poikilocytosis (variation in shape). 27Mosby items and derived items © 2006 by Mosby, Inc.
    28. 28. Microcytic Hypochromic Anemia  Iron Deficiency  Response with increased reticulocytosis within one week  Increased Hgb and Hct in one month  Need to continue therapy to increase stores  Serum Ferritin to 50ug/L 28Mosby items and derived items © 2006 by Mosby, Inc.
    29. 29. Microcytic Hypochromic Anemia Thalassemia  Etiology  Hereditary anemias that result from a reduced or absent synthesis of the alpha or beta globulin chains  Occurs primarily in individuals of Mediterranean descent or Asian origin  Decrease in one or more of the globulin chains  Decreased Hgb concentration and increased likelihood of hemolysis 29Mosby items and derived items © 2006 by Mosby, Inc.
    30. 30. Microcytic Hypochromic Anemia Thalassemia  Beta Thalassemia (2 chains)  Beta Thalassemia trait or minor  Microcytosis, hypochromia, mild anemia, elevated Alpha  Diagnosed by elevation of Hgb A2 levels on hemoglobin electrophoresis  Beta Thalassemia Major (Cooley’s)  More severe anemia  Increased cardiovascular burden  Increased cardiac failure - high output cardiac failure. Heart is having to work much harder, so may fail.  Hyperplastic marrow 30Mosby items and derived items © 2006 by Mosby, Inc.
    31. 31. Microcytic Hypochromic Anemia ALPHA Thalassemia  Alpha trait: carrier state with one chain abnormal  Alpha minor: two genes are defective  Clinical Manifestations similar to Beta Thalassemia Minor  Hemoglobin H disease  Three traits are missing  Clinical Manifestations similar to Beta Thalassemia Major  Alpha Thalassemia Major  Hydrops fetalis 31Mosby items and derived items © 2006 by Mosby, Inc.
    32. 32. Microcytic Hypochromic Anemia Sideroblastic Anemia  Insufficient uptake of iron  Altered hemoglobin synthesis  Characteristic ringed sideroblasts in bone marrow  Dimorphism: characteristics of normal and microcytic cells on smear 32Mosby items and derived items © 2006 by Mosby, Inc.
    33. 33. Evaluation of MicrocytosisMicrocytosis/Hypochromia Fe, TIBC, Ferritin < Fe <Fe, <Ferritin, >TIBC Normal HgB Electrophoresis Normal <HgBA2 33Mosby items and derived items © 2006 by Mosby, Inc.
    34. 34. Macrocytic Anemia Etiology  Vitamin B12 deficiency,  Chronic disease due to lack of IF.  Hyperthyroidism  Folic acid deficiency  Drug induced inhibition  Pernicious anemia of folate absorption  Alcoholics, lack of  Increased need with minerals. pregnancy  Gastric atrophy  Dietary deficiency: short gut syndrome, intestinal surgery --> gasric bypass 34Mosby items and derived items © 2006 by Mosby, Inc.
    35. 35. Macrocytic Anemia Diagnostic Data  Macrocytic, megaloblastic anemia  Hyper-segmented PMNs (98%)  Serum Folate  Serum Cobalamin level  Serum Homocysteine and Methylmalonic Acid levels look up some of this stuff. 35Mosby items and derived items © 2006 by Mosby, Inc.
    36. 36. Macrocytic Anemia Hypersegmented PMNs Macrocytosis 36Mosby items and derived items © 2006 by Mosby, Inc.
    37. 37. Macrocytic Anemias Vitamin B12 and Folate Deficiency  Treatment:  1mg of Vitamin B12 per day week 1, 1 mg twice weekly week 2, 1 mg per week for 4 weeks, then 1 mg per month for life.  Oral preparations: 1 mg per day: poor absorption  Folic Acid 200 ug/day (Young adults)  Pregnancy 400ug/day  Folate deficiency 0.5-1mg per day 37Mosby items and derived items © 2006 by Mosby, Inc.
    38. 38. Hemolytic Anemia Spherocytosis  Hereditary disease autosomal dominant trait in individuals of northern European descent  Red blood cells are spheroid in shape with increased fragility  Usually diagnosed before age 10.  Anemia, splenomegaly, jaundice, spherocytes on blood smear 38Mosby items and derived items © 2006 by Mosby, Inc.
    39. 39. Spherocytosis >Cell size > fragility 39Mosby items and derived items © 2006 by Mosby, Inc.
    40. 40. Hemolytic Anemia SICKLE CELL DISEASE  Etiology  Inherited autosomal recessive disease found primarily in African Americans  Abnormal Hemoglobin S (Hb S) in place of normal Hb A due to replacement of glutamic acid with valine on beta chain.  HbS reacts to deoxygenation by stretching the erythrocyte into an elongated sickle shape  Increased hemolysis with ischemia and infarction 40Mosby items and derived items © 2006 by Mosby, Inc.
    41. 41. Hemolytic Anemia Sickle Cell Anemia  Percentage of HbS determines the degrees of sickling with deoxygenation  Sickled cells lose flexibility and cannot change shape as they move through the vascular system  Increased opportunity for obstruction, pain, and organ infarction  Abnormalities of membrane transport may be involved in those with non reversible sickling  Sickling will occur with hypoxia, acidosis, hypovolemia 41Mosby items and derived items © 2006 by Mosby, Inc.
    42. 42. Pathophysiology of Sickle Cell Disease 42Mosby items and derived items © 2006 by Mosby, Inc.
    43. 43. Hemolytic Anemia Sickle Cell Anemia  Clinical Manifestations  Four types of crisis  Vaso-occlusive: sickled cells obstruct peripheral vessels with increased pain, hand and feet swelling, infarction  Aplastic crisis: normal sickled cells have a life span of 10-20 days so without an increase in erythropoesis of 5-8X marked decrease in cells can occur after hemolysis  Sequestration crisis: sequestering of blood in the spleen, especially in young children can decrease blood volume and cause cardiac collapse  Hyper-hemolytic: increased hemolysis in those children with more that one type of abnormality i.e. association of G6PD 43Mosby items and derived items © 2006 by Mosby, Inc.
    44. 44. Clinical Manifestations of Sickle CellDisease 44Mosby items and derived items © 2006 by Mosby, Inc.
    45. 45. Sickle Cell Anemia 45Mosby items and derived items © 2006 by Mosby, Inc.
    46. 46. Sickled RBC 46Mosby items and derived items © 2006 by Mosby, Inc.
    47. 47. Sickle Cell Human Blood (sickle cells = green) (SEM x 6,600) 47Mosby items and derived items © 2006 by Mosby, Inc.
    48. 48. Sickle Cell Anemia  Diagnostic Data  Hemoglobin electrophoresis HbS  Decreased Hct, normocytic, elevated platelet count.  Peripheral smear includes sickled cells, target cells, and Howell-Jolly bodies  Sickle cell test 48Mosby items and derived items © 2006 by Mosby, Inc.
    49. 49. Howell-Jolly Bodies The RBC in the center of the field contains several Howell-Jolly bodies, or inclusions of nuclear chromatin remnants. There is also a nucleated RBC just beneath this RBC. Abnormal and aged RBCs are typically removed by the spleen. The appearance of increased poikilocytosis, anisocytosis, and RBC inclusions suggests that a spleen is not present. 49Mosby items and derived items © 2006 by Mosby, Inc.
    50. 50. Hemolytic Anemia G6PD DEFICIENCY  ETIOLOGY:  Deficiency of G6PD enzyme  Genetic, X linked recessive disorder - comes from mother, goes to son.  Occurrence  Blacks 10%  Asians 5%  Mediterranean 2-25% 50Mosby items and derived items © 2006 by Mosby, Inc.
    51. 51. Hemolytic Anemias G6PD Deficiency  G6Pd is an enzyme that normally enables RBC to respond effectively to injury. In the absence oxidative stressors damage the hemoglobin, interfere with normal enzymatic activity, and damage plasma membranes. Damaged Hgb precipitates in the cell forming Heinz bodies and causing hemolysis  Occurs in response to certain agents such as sulfonamides, fava beans, infection, acidosis, hypoxemia. 51Mosby items and derived items © 2006 by Mosby, Inc.
    52. 52. Hemolytic Anemia G6PD Deficiency  Clinical Manifestations  Pallor  Icterus  Dark urine  Back Pain  All si/sx occur after acute exposure and will clear when exposure is terminated 52Mosby items and derived items © 2006 by Mosby, Inc.
    53. 53. Normocytic Normochromic Anemia Aplastic Anemia  Pancytopenia: reduction or absence of all three types of blood cells  Failure of bone marrow to produce adequate number of blood cells  Etiology  Acquired  Hereditary 53Mosby items and derived items © 2006 by Mosby, Inc.
    54. 54. Normocytic Normochromic Anemia Aplastic Anemia  Hereditary:  Fanconi’s Anemia: characterized by defect in DNA repair  Pancytopenic  Acquired  Idiopathic  Secondary:  Drugs and chemicals  Infections  Radiation 54Mosby items and derived items © 2006 by Mosby, Inc.
    55. 55. Normocytic Normochromic Anemia Aplastic Anemia  Pathophysiology  Autoimmune with involvement of Interferon produced by killer T cells and capable of inhibition hematopoiesis and apoptosis of cells  Clinical Manifestations  Early depend upon which cell line is most significantly involved  Anemia, infections, bleeding 55Mosby items and derived items © 2006 by Mosby, Inc.
    56. 56. Normocytic Normochromic Anemia Post Hemorrhagic Anemia  Acute blood loss  Cells that remain if normal before hemorrhage remain normal in size and character  Stabilization of vascular volume will cause hemodilution  See table 26-5 for clinical manifestations of acute blood loss  Based of severity 56Mosby items and derived items © 2006 by Mosby, Inc.
    57. 57. Anemia of Chronic Disease most puzzling Etiology Not sure why this happens.  Decreased erythrocyte life span **Chronic renal failure  Decreased bone marrow response  Abnormal iron metabolism  Increased production of lactoferrin and apoferritin which compete with transferrin for binding with iron. These two substances increase the storage form of iron but decrease the iron available for erythrocyte formation  Stimulation of cytokines such as interferon that decrease hematopoiesis, decrease response of the bone marrow 57Mosby items and derived items © 2006 by Mosby, Inc.
    58. 58. Anemia of Chronic Disease 58Mosby items and derived items © 2006 by Mosby, Inc.
    59. 59. Clinical Manifestations of Anemia Tachycardia  Pallor Tachypnea  Systolic murmur Movement of fluid  Fatigue from interstitial spaces  Weakness to vascular volume  Dyspnea Increased turbulence of  Dizziness blood flow  Syncope 59Mosby items and derived items © 2006 by Mosby, Inc.
    60. 60. Progression and Manifestations of Anemia review look at co mechanis 60Mosby items and derived items © 2006 by Mosby, Inc.
    61. 61. Polycythemia Definition: increased number of erythrocytes  Types  Relative: increased hematocrit secondary to hemo-concentration  Absolute  Secondary: increased RBCs secondary to hypoxia with increased stimulus for release of erythropoietin 61Mosby items and derived items © 2006 by Mosby, Inc.
    62. 62. Polycythemia Types  Primary  Polycythemia Vera  Non malignant abnormal proliferation of bone marrow stem cells  Normal or below normal erythropoietin  Genetic alterations of stem cells with alterations in all three lines with elevation of red blood cells, white cells, and platelets  Abnormal cell populations develop in response to growth factors, protein phosphorylation, interaction between tumor suppression gene and multiple growth factors 62Mosby items and derived items © 2006 by Mosby, Inc.
    63. 63. Polycythemia Types  Primary: Polycythemia Vera  Clinical Manifestations  Increased absolute numbers of cells  Increased risk of thrombi  Slowed circulation can cause increased drowsiness, delirium, rubor color to hands and feet, engorgement of cerebral and retinal vessels 63Mosby items and derived items © 2006 by Mosby, Inc.
    64. 64. Neutropenia Definition: decreased in circulating neutrophils with clinical signs at less than 2000 Etiology  Infection  Toxins form bacteria.  Antibody mediated  Bone marrow failure  Immune disorders - autoimmune  Anemia due to Folate and/or Vitamin B 12 deficiency 64Mosby items and derived items © 2006 by Mosby, Inc.
    65. 65. Neutrophilia Definition  Increased numbers of neutrophils Etiology  Infection  Leukemia Shift to the left:  Release of immature neutrophils (bands) 65Mosby items and derived items © 2006 by Mosby, Inc.
    66. 66. Neutrophilia The RBCs in the background appear normal. The important finding here is the presence of many PMNs. An elevated WBC count with mainly neutrophils suggests inflammation or infection. A very high WBC count (>50,000) that is not a leukemia is known as a "leukemoid reaction". This reaction can be distinguished from malignant WBCs by the presence of large amounts of leukocyte alkaline phosphatase (LAP) in the normal neutrophils. 66Mosby items and derived items © 2006 by Mosby, Inc.
    67. 67. Leukemia Most common form of childhood cancer  80-85% are ALL  (Acute Lymphoblastic Leukemia) - pretty curable.  15-20% ANLL  (Acute Non Lymphoblastic Leukemia)  Most of these are involving the myeloid cells lines so also AML (Acute Myelogenous Leukemia) 67Mosby items and derived items © 2006 by Mosby, Inc.
    68. 68. Leukemia  Classification  Cell Line  Acute and chronic  Morphologic: FAB system (L1),L2, L3  Immunologic: cell surface markers  Etiology  Genetic Risk  Other inherited diseases such as Down’s Syndrome and Fanconi’s Anemia 68Mosby items and derived items © 2006 by Mosby, Inc.
    69. 69. All hemolysis will cause an increase in uric acidLeukemia Clinical Manifestations  Pallor: decreased # of red blood cells  Fatigue: decreased O2 carrying capability  Bleeding: decreased platelet function - nose bleeds  Fever: infection or increased metabolism secondary to increased number and destruction of white blood cells  Petechiae: decreased platelets  Renal Failure: elevated uric acid levels from increased cell breakdown  Metastatic extramedullary invasion: CNS 69Mosby items and derived items © 2006 by Mosby, Inc.
    70. 70. Acute Lymphocytic Lukemia The WBCs seen here are lymphocytes, but they are blasts--very immature cells with larger nuclei that contain nucleoli. Such lymphocytes are indicative of acute lymphocytic leukemia (ALL). ALL is more common in children than adults. Many cases of ALL in children respond well to treatment, and many are curable. 70Mosby items and derived items © 2006 by Mosby, Inc.
    71. 71. Acute Lymphoblastic Leukemia Cytoplasmic Vacoules 71Mosby items and derived items © 2006 by Mosby, Inc.
    72. 72. Leukemia Types  Chronic myelocytic  Philadelphia chromosome is a diagnostic marker  Not believed to be genetically transmitted but an error in mitosis  Increased splenomegaly  Chronic Lymphoblastic  Abnormality of B cells  Do not mature into plasma cells  Decreased humoral immune system function 72Mosby items and derived items © 2006 by Mosby, Inc.
    73. 73. Philadelphia Chromosome: CML Myeloid cells of CML are also characterized by the Philadelphia chromosome (Ph1) on karyotyping. This is a translocation of a portion of the q arm of chromosome 22 to the q arm of chromosome 9, designated t(9:22). 73Mosby items and derived items © 2006 by Mosby, Inc.
    74. 74. Chronic Lymphocytic Leukemia These mature lymphocytes are increased markedly in number. They are indicative of chronic lymphocytic leukemia, a disease most often seen in older adults. This disease responds poorly to treatment, but it is indolent. 74Mosby items and derived items © 2006 by Mosby, Inc.
    75. 75. Cell Differentiation 75Mosby items and derived items © 2006 by Mosby, Inc.
    76. 76. Hodgkin Lymphoma Definition  Malignancy of a lymph node  Arises in one or a chain of lymph nodes and spreads in contiguous nodes  Characterized by the presence of Reed Sternberg cells  Review the Cotswold Staging System Table 27-6 Clinical manifestations  Fever, wt loss, night sweats, pruritis  Painless enlargement of lymph node most commonly in cervical area 76Mosby items and derived items © 2006 by Mosby, Inc.
    77. 77. Hodgkin Lymphoma 77Mosby items and derived items © 2006 by Mosby, Inc.
    78. 78. Hodgkin Lymphoma Note the large cells with large, pale nuclei containing large purple nucleoli at the arrowheads. These are Reed-Sternberg cells that are indicative of Hodgkins disease. Reed Sternberg 78Mosby items and derived items © 2006 by Mosby, Inc.
    79. 79. Non Hodgkin Lymphoma Definition  Malignant changes within the lymphoid system without the presence of Reed Sternberg cells  Usually involves multiple peripheral nodes  Swelling is painless with indolent growth  Can have abdominal tumors, tumors of testes 79Mosby items and derived items © 2006 by Mosby, Inc.
    80. 80. Multiple Myeloma Neoplastic proliferation of a single clone of a plasma cell involved in production of a specific immunoglobulin. Clinical manifestations: bone pain, weakness, fatigue, anemia secondary to bone marrow crowding with abnormal cells 80Mosby items and derived items © 2006 by Mosby, Inc.
    81. 81. Multiple Myeloma The rounded "punched out" lesions of multiple myeloma appear as lucent areas with this skull radiograph. 81Mosby items and derived items © 2006 by Mosby, Inc.
    82. 82. Multiple Myeloma The skull demonstrates the characteristic rounded "punched out" lesions of multiple myeloma. 82Mosby items and derived items © 2006 by Mosby, Inc.
    83. 83. Hemostasis Vasoconstriction Formation of the platelet plug  Vessel damage  Platelet aggregation and attachment to vessel wall in the presence of calcium  In the presence of ADP released by erythrocytes platelets attach to cell wall  Release chemical mediators: histamine, thromboxane A, and prostacyclin 83Mosby items and derived items © 2006 by Mosby, Inc.
    84. 84. Platelet Plug Formation 84Mosby items and derived items © 2006 by Mosby, Inc.
    85. 85. Platelet Aggregation AdherenceFibrinThreads Fibrin Mesh 85 Mosby items and derived items © 2006 by Mosby, Inc.
    86. 86. Hemostasis Steps Activation of the clotting factors  Prothrombin-PA-Thrombin  Thrombin to Fibrinogen  Platelets > Fibrin Stabilizing Factor > Cross Linking Bonds  Clot Retraction 86Mosby items and derived items © 2006 by Mosby, Inc.
    87. 87. Hemostasis Steps Intrinsic Pathway  Activated when Factor VIII (Hageman Factor) contacts subendothelial tissue after vascular injury Extrinsic pathway  Tissue Thromboplastin is released by damaged endothelial cells and comes into contact with clotting factors 87Mosby items and derived items © 2006 by Mosby, Inc.
    88. 88. Coagulation Cascade review 88Mosby items and derived items © 2006 by Mosby, Inc.
    89. 89. Lysis of Clot - fibrinolytic system Plasminogen activates Plasmin in the presence of t-PA and thrombin Plasmin is a proteolytic enzyme  Splits fibrin and fibrinogen into fibrin degradation products - can be used as tx.  Uses up clotting factors in the process: Factor V, Factor VII 89Mosby items and derived items © 2006 by Mosby, Inc.
    90. 90. Fibrinolytic System know this 90Mosby items and derived items © 2006 by Mosby, Inc.
    91. 91. Immune Thrombocytopenic Purpura (ITP) Pathophysiology  Autoimmune mediated platelet destruction  Recurrent episodes  Usually initially follows a viral infection  Peak onset 20-40 years of age  Platelets are the first line defense against bleeding  Platelet counts below 100,000  IgE attaches to platelet and causes destruction. 91Mosby items and derived items © 2006 by Mosby, Inc.
    92. 92. Immune Thrombocytopenia Purpura Clinical Manifestations  Bruising  Petechiae  Bleeding  Counts below 50,000 with increase risk  Counts below 10,000-15,000 frank bleeding 92Mosby items and derived items © 2006 by Mosby, Inc.
    93. 93. Immune Thrombocytopenia Purpura Differential Diagnosis  Leukemia  Meningococcemia - cause petichei 2 toxic  Drug Induced  Von Willebrand’s Disease - hemophylia Diagnostic Data  CBC with platelet count  aPt, Ptt, INR, bleeding time 93Mosby items and derived items © 2006 by Mosby, Inc.
    94. 94. Immune Thrombocytopenia Purpura Treatment  Referral  Prednisone Infusion  Splenectomy 94Mosby items and derived items © 2006 by Mosby, Inc.
    95. 95. Hemophilia Inherited Hemorrhagic Disease Types  Hemophilia A: Classic - Factor VIII deficiency (most common, X recessive)  Hemophilia B: Christmas Disease - IX deficiency (x linked)  Hemophilia C: Factor XI deficiency (men and women)  von Willebrand Disease: Factor VIII deficiency (Inherited autosomal; will see increase for several days, vs classic). Pathophysiology: lack of the clotting factor impacts the clotting cascade and formation of thrombin 95Mosby items and derived items © 2006 by Mosby, Inc.
    96. 96. Hemophilia Clinical Manifestations  Often not noted during first year of life  Prolonged bleeding  After minor trauma  Hemarthrosis of the joints (can predispose to degenerative disease)  Deep muscle bruising  Joint deformities with contractures (muscle deformaties).  Intracranial hemorrhage and abdominal bleeding  the most feared complication - emergency 96Mosby items and derived items © 2006 by Mosby, Inc.
    97. 97. Disseminated Intravascular Coagulation(DIC) Etiology  Associated with well defined clinical conditions that act as a procoagulant (activates clotting cascade)  Hypoxemia  Acidosis  Shock  Sepsis  Precipitated by  Endothelial damage  Tissue damage  Direct activation of Factor X 97Mosby items and derived items © 2006 by Mosby, Inc.
    98. 98. Disseminated Intravascular Coagulation(DIC) Pathophysiology  Wide spread clotting  Utilization of clotting factors  Increase in levels of thrombin  Activation of the fibrinolytic pathway  Elevation of Plasmin  Increased Fibrin Degradation Products 98Mosby items and derived items © 2006 by Mosby, Inc.
    99. 99. Blood Clot Formation (blood cells, platelets, fibrin clot) (SEM x10,980) 99Mosby items and derived items © 2006 by Mosby, Inc.
    100. 100. Human Red Blood Cells, T-lymphocytes, Platelets and Fibrin Threads (SEM x7,700) 100Mosby items and derived items © 2006 by Mosby, Inc.

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