2. immunoglobinopathies
Dr Sara Amjad
immunoglobinopathies

3. Clinical significance of albumin
Prevention from crossing BBB
Hypoalbuminemia
Malnutrition
Nephrotic syndrome
Cirrhosis of liver

4. Colloid osmotic pressure
Low albumin, causing edema.

5. Albuminuria
Microalbuminuria
Therapeutical use of albumin

6. Clinical significance of α antitrypsin
α antitrypsin deficiency has been implicated in two diseases
Emphysema
α1 AT deficiency liver disease

7. Emphysema Abnormal distension of lungs by air
5 percent of emphysema cases are due to deficiency of α1 AT
Associated with lung infections
Increase in activity of macrophages to release elastase

8. α1 AT deficiency liver disease
Accumulation of a mutant α1 AT which aggregates to form polymers
Polymer cause liver damage
Accumulation of collagen resulting in fibrosis

9. α1 Fetoglobulin
Presence of α1 Fetoglobulin is usually diagnostically in determining presence of hepatocelluar carcinoma

10. Clinical significance of Haptoglobin
Anemia is associated with decrease plasma concentration of haptoglobin

11. Transferrin
Deficiency
PCM
Cirrhosis of liver

12. Clinical significance of Ceruloplasmin: Wilson,s disease
Failure to synthesize ceruloplasmin or an impairment in the binding capacity of copper
Copper is free in the plasma which easily enters tissue

13. Manifestations
Copper deposition in abnormal amounts in liver and brain
Low level of ceruloplasmin in plasma with increase excretion
Copper deposition in kidney causes renal damage
Intestinal absorption of copper is very high

14. Multiple myeloma Plasma cell cancer Abnormal Ig production
malignancy of a single cone of plasma cell
Over production of abnormal immunoglobulin's
Decreased immunity
Recurrent infections

15. Electrophoretic pattern of multiple myeloma
Sharp distant band
M band between beta and gamma globulin
Diminished synthesis of gamma globulin

17. Bence Jones proteins Light chain of immunoglobulin are in excess
Renal tubes damage
Amyloidosis
Deposition of light chains in tissues

19. Genetic deficiency of plasma proteins
Analbuminaemia
Complete absence
Defect in synthesis
Bruton,s agammaglobulinaemia
Inherited, X linked
Differentiation of beta lymphocytes to plasma cells is defective

20. Afibrinogenaemia Inherited disorder Defect in fibrinogen formation
Uncontrollable hemorrhages

22. Plasma Proteins