Hemolytic anemia, Introduction, Characteristic features, sickle cell anemia and sideroblastic anemia in detail

1. Hemolytic Anemia
Dr. Subhash Thakur
Clinical Oncologist
MD (PGIMER, Chandigarh)
MBBS 3rd Year Lecture, 21/04/2021 @ CMC,
Bharatpur, Nepal

2. Content
• Introduction to hemolysis
• Characteristics of Hemolytic Anemia
• Classification
• Sickle Cell Anemia
• Etiology
• Pathophysiology
• Clinical Features
• Investigation
• Treatment
• Hereditary Spherocytosis
• Etiology
• Pathophysiology
• Clinical Features
• Investigation
• Treatment

3. Haemolysis: Introduction
• Often Acute, not always can be chronic too – Sickle cell and hereditary
spherocytosis
• Acute anaemia – loss (almost always GI loss) = Haemolysis

4. Hemolysis: Intravascular Vs Extravasucular

5. Hemolysis: Hemolytic Anemia
Common Characteristics
• Acute
• MCV: normal or slightly raised
• Increased reticulocyte count
• Raised LDH

6. • Raised indirect bilirubin
• Decreased haptoglobin (Haptoglobin : transports red cells Hb
from vascular space to RES, liver, spleen where it is recycled)
• Dark Urine: free Hb (Hemoglobinuria)
• Renal Failure
• Acute Tubular Necrosis
Indirect Bilirubin donot get into urine because it is bound to
albumin

8. Sickle Cell Anemia
• Why do shape Change??
• Normal RBC shape: Biconcave

9. Pathophysiology
• Normal Hb: 2 alpha and 2 beta chain
• In sickle cell anemia, single nucleotide is changed
• GAG is replaced by GTG
• GAG: Glutamic acid
• GTG: Valine
• Whole protein structure is changed: miss sense mutation

10. • Sickle Cell Disease
• Sickle Cell Trait
• Sickle cell trait: less severe form of malaria

11. Clinical Features
• Predisposing Factors
• Infection
• Fever
• Hypoxia
• Acidosis
• dehydration

12. • Predisposing factors lead to sickle
cell crisis
• Clinical manifestation:
• Chest/Back pain
• Lower extremity ulcers
• Osteomyelitis (bad bone circulation)
• Organism: staph aureus (mc)

13. • Visual Symptoms
• CNS/CVA
• Signs of stroke
• Priapism
• Aseptic necrosis of femoral head

14. Evaluation
• Lab values: as per hemolytic anemia
• Best Initial test: smear: sickled cells
• Most accurate test: Hb electrophoresis

15. Treatment
• Fluids
• O2
• Pain medication

16. Can smear tell you the difference between
sickle cell and sickle cell trait?
• Yes, sickle cell: sickled cells
• Trait: Normal smear, Normal CBC

17. Who should get Antibiotics?
• Infections, fever>100 degree fareinheit
• They don’t have spleen (functional), don’t wait for the result of culture
• They are immunocompromised

18. 3rd Hospital Day
• Hematocrit : 32 to 22 ???
• Parvovirus B19
• Diagnosis: PCR for DNA
• Treatment: IgM against organism, IV immunoglobulin

19. • Sickle cell crisis: (visual/CNS/CVA/priapism): exchange transfusion
• Hydroxyurea: prevent sickle cell crisis, increases HbF, dilutes sickle cell Hb
• Folate supplementation
• Pneumococcal vaccination

20. Hereditary Spherocytosis

21. Diagnosis and Management of HS

22. Revision
• 1. Hemolytic disruption of the erythrocyte involves
A. an alteration in the erythrocyte membrane
B. a defect of the hemoglobin molecule
C. an antibody coating the erythrocyte
D. physical trauma

23. • 2. _____ Intravascular hemolysis
• A. Destruction of RBCs outside the circulatory blood
B. Destruction of RBCs within the circulatory blood

24. • 3. Which of the following tests is not useful in determining increased
erythrocyte destruction?
A. Reticulocyte count
B. Total leukocyte count
C. Serum haptoglobin
D. Unconjugated bilirubin

25. • _____ G6PD deficiency
• A. Structural membrane defect
B. Erythrocytic enzyme defect
C. Defect of the hemoglobin molecule

26. • 6. _____ Hereditary spherocytosis
• A. Structural membrane defect
B. Erythrocytic enzyme defect
C. Defect of the hemoglobin molecule

27. • _____ Hereditary spherocytosis
• A. An overabundance of oval-shaped red cells
B. A permeability disorder
C. The most common prevalent hereditary hemolytic anemia among people
of Northern European descent
D. Can be seen in the genetic hemoglobin defect, thalassemia
E. A subgroup of common hereditary elliptocytosis

28. • A blood transfusion can cure sickle cell anemia
• True
• False

29. • One complication of sickle cell disease is a damaged spleen
• True
• False

30. • A "sickle cell crisis" means a sudden onset of pain throughout the body
• True
• False

31. • Normal red blood cells last about 120 days, but red blood cells affected by
sickle cell disease last only 60 days.
• True
• False
• Last only 20 – 30 days

32. • Sickle-shaped red blood cells don't move easily through blood vessels.
• True
• False

33. • People with sickle cell anemia are born with it.
True
False

34. Thank You