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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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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 Stainof 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