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Red Blood Cells and Anemias

Blood. Red Blood Cells (RBCs)White Blood Cells (WBCs)PlateletsPlasmaRBCs, WBCs, and Platelets Produced in Bone MarrowApproximately 5L of blood in adult. Red Blood Cells. Carry Oxygen to TissuesBiconcave Disk Containing HemoglobinLife Span of 120 days4.4-5.9 x 1012 RBCs/L (M)3.8-5.2 x 1012 R

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Red Blood Cells and Anemias

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    1. Red Blood Cells and Anemias Jonathan Ben-Ezra, M.D. Professor of Pathology MCV Campus of VCU

    2. Blood Red Blood Cells (RBCs) White Blood Cells (WBCs) Platelets Plasma RBCs, WBCs, and Platelets Produced in Bone Marrow Approximately 5L of blood in adult

    3. Red Blood Cells Carry Oxygen to Tissues Biconcave Disk Containing Hemoglobin Life Span of 120 days 4.4-5.9 x 1012 RBCs/L (M) 3.8-5.2 x 1012 RBCs/L (F)

    4. Hemoglobin Oxygen Carrying Molecule Tetramer of 2 alpha chains and two “beta” chains (?2 ?2) attached to Heme pocket Heme comprised of protoporphyrin ring and iron 13-18 g/dl (M) 12-16 g/dl (F)

    5. Hematocrit Percentage of blood which is comprised of RBCs 40-52% (M) 35-47% (F)

    6. Anemia Reduction in Oxygen Carrying Capacity of Blood Not enough production by Bone Marrow Too much peripheral destruction (hemolytic anemia) Reticulocyte count (0.8- 2.5%) cell which still has mRNA

    7. Reticulocyte

    8. MCV (Mean Cell Volume) 80- 100 fl Microcytic Anemia Normocytic Anemia Macrocytic Anemia

    9. Hypoproliferative Anemia Microcytic iron deficiency anemia thalassemia sideroblastic anemia anemia of chronic disease Macrocytic Megaloblastic anemia reticulocytosis

    10. Hemolytic Anemia Hemoglobinopathy (e.g. sickle cell) Membrane Abnormality (e.g. hereditary spherocyosis) Enzyme Defect (e.g. G6PD deficiency) Autoimmune hemolytic anemia Trauma (e.g. DIC, TTP) Infection (e.g. malaria)

    11. Anemia Easy fatigability Dyspnea on exertion Faintness/ Vertigo Pallor Rapidly bounding pulse Dependent edema Systolic murmurs

    12. Iron Deficiency Anemia Iron absorbed in GI tract, transported by transferrin to BM Iron absorbed by pinocytosis by RBC precursors After 120 days, RBC phagocytosed in spleen iron is reused hemoglobin converted to bilirubin

    14. Iron Deficiency Increased Need Early childhood and adolescence (growth spurts) Pregnancy (extra 3.8 mg/day over baseline) Lactation Poor Intake/Absorption Milk baby Achlorhydria Inflammatory bowel disease

    15. Iron Deficiency Menstruating women GI bleeding (most common pathologic cause) Tissue loss Urinary Loss Iatrogenic IRON DEFICIENCY IN A MALE ALWAYS NEEDS TO BE WORKED UP!!!

    16. Iron Deficiency Anemia Hypochromic microcytic anemia low iron, high transferrin, low ferritin small ragged RBC precursors lack of stainable iron

    17. Iron Deficiency Anemia

    18. Prussian Blue Stain of Bone Marrow

    19. Therapy of Iron Deficiency

    20. Anemia of chronic disease Impaired Fe utilization low Fe, low transferrin, high ferritin Increased iron stores

    21. Megaloblastic Anemia B12 binds with intrinsic factor, absorbed in terminal ileum Schilling test 7-12 year supply Folate is not stored in body

    22. Megaloblastic Anemia Macrocytic anemia with hypersegmented neutrophils Neurologic symptoms (dorsal columns) Nuclear/cytoplasmic asynchrony in BM Ineffective erythropoiesis High indirect bilirubin Very high LDH

    23. Megaloblastic Anemia

    24. Laboratory Evidence of Hemolysis Increased bilirubin low serum haptoglobin Hemoglobinemia/ Hemoglobinuria Hemosiderinuria Increased LDH Increased reticulocyte count

    25. Hereditary Spherocytosis Defect of spectrin-Protein 4.1 interaction or in ankyrin decreased deformability sluggish transversing of splenic cords Clinical triad of anemia, splenomegaly, and jaundice Spherocytes on smear Osmotic fragility test

    26. Hereditary Speherocytosis

    27. Enzyme Deficiencies G6PD Pyruvate Kinase

    28. Autoimmune Hemolytic Anemia Antibody attaching to RBCs Direct or indirect Coombs test May be associated with thrombocytopenia (Evan’s syndrome) Idiopathic, drugs, infection Treatment treat underlying cause steroids

    29. Autoimmune Hemolytic Anemia

    30. Sickle Cell Anemia Autosomal recessive ?-hemoglobinopathy Symptomatic at 6 months Irreversible sickling upon deoxygenation pain crises infarcts of spleen (asplenia), kidneys, brain Hemolytic vs. aplastic crisis (Parvovirus B19)

    31. Sickle Cell Anemia

    32. Thalassemia Imbalance of globin chain production ?- vs. ?-thalassemia anemia due to both decreased production and increased hemolysis in spleen ?- thalassemia has decreased/absent Hgb A, increased Hgb F, and increased Hgb A2 Treatment- hypertransfusion with chelation, splenectomy, bone marrow transplantation

    33. Combination of History and Smear 24 year old VCU student with spiking fevers every day Recently returned from trip to Africa

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