1. Red Blood Cell Disorders
DR.Leni Lismayanti, SpPK-K
Dept of Clinical Pathology RSHS/FKUP Bandung

2. Reference book:
Denise M Harmening. Clinical Hematology and Fundamental of Hemostasis. 5th edition. Philadelphia: FA Davis Co, 2009.

3. RBC Structure & Function
Areas of RBC metabolism important in normal RBC survival and function:
RBC membrane
Hb structure & function
RBC metabolic pathway

4. RBC Membrane RBC membran proteins: Deformability Permeability
RBC membran lipids:
Phospholipd
Glycolipid
cholesterol

5. RBC membrane

6. Abnormality That Can Lead to a Change in 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

7. 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

8. RBC Metabolic Pathway ATP needs for: Hb function
Membrane integrity & deformability
RBC volume
Adequate amounts of reduced pyridine nucleotides
Protection of metabolic enzymes

9. RBC Senescence & Hemolysis
RBC traveles 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%)

10. Extravascular Hemolysis

12. Any questions?

13. RBC Disorders: RBC disorders  morphologic changes Numbers:
Decreased (Anemia)
Increased (Polycythemia)
Structure and function:
Hemoglobin
RBC membrane
Abnormal erythropoiesis  nutritional
RBC disorders  morphologic changes

14. 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

15. Classification of Anemia
Based on Hb level:
Moderate (7-10), severe (<7).
BM dynamic:
Hypoproliferative
Accelerated destruction
combination
Clinically (caused):
RBC indices (normochromic normocytic)

16. Categories of anemia by causes
Blood loss
Accelerated destruction
Nutritional deficiency
BM replacement
Infection
Toxicity
Hematopoietic SC arrest/damage
Hereditary/acquired defect
idiopathic

17. Significance of Anemia and Compensatory Mechanism
N: 1% of RBC loss daily  BM produce (measured by reticulocyte count  %).
Replacement RBC requires:
Adequate functioning SC in BM
Normal RBC maturation process
Ability to release mature RBC from BM

18. Proper HB & RBC production requires:
Variety of nutritional factors
Normal pathway of Bh synthesis
In severe anemias  symptoms of functional impairment of several organs (+).
Compensatory mechanism  increase 2,3-DPG levels

19. 2,3-DPG Physiologic regulator of: Normal Hb oxygen-carrying capacity
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.

20. Lab Tests in Diagnosis of AnemiaHb
Hct
(RBC Count)
RBC indices
Peripheral Blood Smear
Reticulocyte Count
BM smear/biopsy
Treatment of anemia: depends on etiology

22. Polycythemia Polycythemia Vera (Myeloproliferative disorders).
Secondary Hypoxic Polycythemia (Secondary Erythrocytosis).
Relative Erythrocytosis.

23. Manifestations PV SE RE
CLINICAL FEATURES
Cyanosis
Absent
Present
Maybe present
Heart/lung disease
Splenomegaly
Present in 75%
Hepatomegaly
Present in 35%
LAB FEATURES
RBC mass
Increased
Normal
EPO
Decreased (rarely N)
Increased (rarely N)
Arterial O2 saturation
Decreased
WBC count
Increased in 80%
Platelet count
Increased in 50%
NRBCs poikilocytes
Often present
LAP
Increased in 70% BM
Hypercell; eryth & myelop ↑;
Erythropoiesis ↑
Megakaryocytes ↑; fibrosis
Serum vit B12
Increased in 75%
Culture studies
Autonomous, ery proliferation
EPO dep.col.form
Not applicable

24. Any questions?

25. RBC Morphology

34. Thank You Very Much
for Your Attention