Red Blood Cell
Disorders
DR.Leni Lismayanti, SpPK-K
Dept of Clinical Pathology RSHS/FKUP
Bandung
Reference book:
• Denise M Harmening. Clinical
Hematology and Fundamental of
Hemostasis. 5th edition. Philadelphia:
FA Davis Co, 2009.
RBC Structure & Function
• Areas of RBC metabolism important in
normal RBC survival and function:
 RBC membrane
 Hb structure & function
 RBC metabolic pathway
RBC Membrane
• RBC membran proteins:
- Deformability
- Permeability
• RBC membran lipids:
- Phospholipd
- Glycolipid
- cholesterol
RBC membrane
Abnormality That Can Lead to
a Change in RBC Morphology
Abnormality
RBC Morphology
Cholesterol accumulation in the RBC
membrane (liver disease)
Target cells
Abetalipoproteinemia with cholesterol
accumulation
Acanthocytes
LCAT deficiency with cholesterol
accumulation
Hemolysis with RBC fragmentation
Decreased phosphorylated spectrin or
altered spectrin
Bite cells and spherocytes
Hb Structure & Function
Hb function: delivery and release of oxygen to the
tissue and facilitation of CO2 excretion.
Hb synhesis
depends on 3 prs:
- Adequate Iron
delivery & supply
- Adequate synthesis
of protophorphyrin
- Adequate globin
synthesis
RBC Metabolic Pathway
ATP needs for:
• Hb function
• Membrane integrity & deformability
• RBC volume
• Adequate amounts of reduced pyridine
nucleotides
• Protection of metabolic enzymes
RBC Senescence & Hemolysis
• RBC traveles 200-300 miles during 120
day life span  undergo the process of
senescence (aging)  metabolic &
physical changes.
• 1% RBC taken out (removed) from
circulation by RES/MPS  process:
- Extravascular hemolysis (90%)
- Intravascular hemolysis (5-10%)
Extravascular Hemolysis
Any questions?
RBC Disorders:
1. Numbers:
• Decreased (Anemia)
• Increased (Polycythemia)
2. Structure and function:
• Hemoglobin
• RBC membrane
• Abnormal erythropoiesis  nutritional
RBC disorders  morphologic changes
Anemia
• Inability of the circulating blood pool to
supply the tissue with adequate oxygen
for proper metabolic function.
• Clinically d/ based on: HT, SS, PE, Lab.
• Caused by or associated with
underlying disease.
• Usually associated with decrease Hb &
Hct (Lab: + RBC count).
• Consideration by age, sex and other fact
Classification of Anemia
• Based on Hb level:
• Moderate (7-10), severe (<7).
• BM dynamic:
• Hypoproliferative
• Accelerated destruction
• combination
• Clinically (caused):
• RBC indices (normochromic normocytic)
Categories of anemia by causes
1.
2.
3.
4.
5.
6.
7.
8.
9.
Blood loss
Accelerated destruction
Nutritional deficiency
BM replacement
Infection
Toxicity
Hematopoietic SC arrest/damage
Hereditary/acquired defect
idiopathic
Significance of Anemia and
Compensatory Mechanism
• N: 1% of RBC loss daily  BM produce
(measured by reticulocyte count  0.52.0%).
• Replacement RBC requires:
1. Adequate functioning SC in BM
2. Normal RBC maturation process
3. Ability to release mature RBC from
BM
• Proper HB & RBC production requires:
1. Variety of nutritional factors
2. Normal pathway of Bh synthesis
• In severe anemias  symptoms of
functional impairment of several organs
(+).
• Compensatory mechanism  increase
2,3-DPG levels
2,3-DPG
• Physiologic regulator of:
1. Normal Hb oxygen-carrying capacity
2. Tissue oxygen delivery
• 2,3-DPG (+)  Hb more readily
released oxygen to tissues  depend
on: pH & Oxygen level of arterial blood.
• N individual responds to anemia 
elevated EPO  Recombinant EPO 
Th/ certain anemia.
Lab Tests in
Diagnosis of Anemia
•
•
•
•
•
•
•
Hb
Hct
(RBC Count)
RBC indices
Peripheral Blood Smear
Reticulocyte Count
BM smear/biopsy
Treatment of
anemia:
depends on
etiology
Polycythemia
1. Polycythemia Vera (Myeloproliferative
disorders).
2. Secondary Hypoxic Polycythemia
(Secondary Erythrocytosis).
3. Relative Erythrocytosis.
Manifestations
PV
SE
RE
CLINICAL FEATURES
Cyanosis
Absent
Present
Maybe present
Heart/lung disease
Absent
Present
Absent
Splenomegaly
Present in 75%
Absent
Absent
Hepatomegaly
Present in 35%
Absent
Absent
RBC mass
Increased
Increased
Normal
EPO
Decreased (rarely N)
Increased (rarely N)
Normal
Arterial O2 saturation
Normal
Decreased
Normal
WBC count
Increased in 80%
Normal
Normal
Platelet count
Increased in 50%
Normal
Normal
NRBCs poikilocytes
Often present
Absent
Absent
LAP
Increased in 70%
Normal
Normal
BM
Hypercell; eryth & myelop ↑;
Erythropoiesis ↑
Normal
LAB FEATURES
Megakaryocytes ↑; fibrosis
Serum vit B12
Increased in 75%
Normal
Normal
Culture studies
Autonomous, ery proliferation
EPO dep.col.form
Not applicable
Any questions?
RBC Morphology
Thank You Very Much
for Your Attention