Red Blood Cells and Anemias

Jonathan Ben-Ezra, M.D.

Professor of Pathology
MCV Campus of VCU
 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
 Red Blood Cells
Carry Oxygen to
Tissues
Biconcave Disk
Containing
Hemoglobin
Life Span of 120 days
12
4.4-5.9 x 10 RBCs/L
(M)
12
3.8-5.2 x 10 RBCs/L
(F)
 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)
 Hematocrit
Percentage of blood
which is comprised of
RBCs
40-52% (M)
35-47% (F)
 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
Reticulocyte
 MCV (Mean Cell Volume)

80- 100 fl
Microcytic Anemia
Normocytic Anemia
Macrocytic Anemia
 Hypoproliferative Anemia

Microcytic
iron deficiency anemia
thalassemia
sideroblastic anemia
anemia of chronic disease
Macrocytic
Megaloblastic anemia
reticulocytosis
 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)
 Anemia
Easy fatigability
Dyspnea on exertion
Faintness/ Vertigo

Pallor
Rapidly bounding pulse
Dependent edema
Systolic murmurs
 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
 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
 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!!!
 Iron Deficiency Anemia

Hypochromic microcytic anemia

low iron, high transferrin, low ferritin
small ragged RBC precursors
lack of stainable iron
Iron Deficiency Anemia
 Prussian Blue Stain
of Bone Marrow

Iron Present No Iron Present

Therapy of Iron Deficiency
 Anemia of chronic disease

Impaired Fe utilization
low Fe, low transferrin, high ferritin
Increased iron stores
 Megaloblastic Anemia

B12 binds with intrinsic factor, absorbed in

terminal ileum
Schilling test
7-12 year supply
Folate is not stored in body
 Megaloblastic Anemia

Macrocytic anemia with hypersegmented

neutrophils
Neurologic symptoms (dorsal columns)
Nuclear/cytoplasmic asynchrony in BM
Ineffective erythropoiesis
High indirect bilirubin
Very high LDH
Megaloblastic Anemia
 Laboratory Evidence of
Hemolysis

Increased bilirubin
low serum haptoglobin
Hemoglobinemia/ Hemoglobinuria
Hemosiderinuria
Increased LDH
Increased reticulocyte count
 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
Hereditary Speherocytosis
 Enzyme Deficiencies

G6PD
Pyruvate Kinase
 Autoimmune Hemolytic Anemia

Antibody attaching to RBCs

Direct or indirect Coombs test
May be associated with thrombocytopenia
(Evans syndrome)
Idiopathic, drugs, infection
Treatment
treat underlying cause
steroids
Autoimmune Hemolytic Anemia
 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)
Sickle Cell Anemia
 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
 Combination of History and
Smear
24 year old VCU
student with spiking
fevers every day
Recently returned
from trip to Africa