More Related Content Similar to 4a..hemolytic anemia (20) More from Afrina Qureshi (20) 4a..hemolytic anemia2. "Pleasure in the job"Pleasure in the job
putsputs
perfection in the work."perfection in the work."
-- Aristotle-- Aristotle
4. C.B.C / FBC / Hemogram
Haemoglobin - 15±2.5, 14 ±2.5 - g/dl
PCV - 0.47 ±0.07, 0.42 ±0.05 - l/l (%)
Haematocrit, Total RBC volume - better
RBC count - 5.5 ±1, 4.8 ± 1 x1012/l
MCHC - Hb/PCV - 30-36 - g/dl
Hb synthesis within RBC
MCH - Hb/RBC - 29.5 ± 2.5 pg/l
Average Hb in RBC
MCV - PCV/RBC 85 ± 8 – fl
RBC Maturation
5. RBC disorders (Anemias) :
““Anemia is decreased red cell massAnemia is decreased red cell mass
affecting tissue oxygenation”affecting tissue oxygenation”
* Low Hb <13.5 (males), <11.5 (females)* Low Hb <13.5 (males), <11.5 (females)
Acquired / Congenital disorders:
Decreased production / Increased loss
6. Haemolytic An. Introduction
Anemia due to Increased RBC destruction
Decreased life span (<120d)
Breakdown ↑Bilirubin (Unconj) Jaundice
Increased RBC production - ↑ reticulocytes
Low Haptoglobins – Hb carrier proteins.
10. Clinical Features:
Pallor mild – mucosal
Jaundice - Mild fluctuating
Splenomegaly
No bile in urine (dark on standing-UBG)
Pigment gall stones – in chronic forms
Crisis – aplastic, hemolytic, vascular
Ankle ulcers
11. Hemolytic Anemia - Types:
Immune lysis
• Warm & Cold Ab, Auto & Allo immune
Mechanical Damage
• Valve, Microangiopathy (DIC), prosthesis, march
Hereditary Defects
• Membrane, Hb & Enzyme defect
Infection induced
• Clostridia, malaria, septicemia
13. Congenital RBC Disorders:
Membrane Disorders:
Spherocytosis, Elliptocytosis
Hemoglobin Disorders:
Hemoglobinopathies - Sickle cell, HbC etc.
Thalassemia Syndromes - α, β, δ
Enzyme disorders:
G6PD, PK deficiency
14. Laboratory Evaluation:
Features of RBC breakdown:
Hyperbilirubinemia
Increased Urine UBG & Faecal stercobilinogen.
Low or absent Haptoglobins
Features of increased RBC Production:
Reticulocytosis
Marrow erythroid hyperplasia – bone changes
Damaged RBC
Morphology, Osmotic Fragility
Decreased RBC survival – 51
Cr labelling.
Hemoglobin electrophoresis, enzyme abnormality
17. Blood Smear Interpretation:
A B C D
E F G H
I J
A. Normal
B. Micro/hypo
C. Macro
D. Target
E. Sphero
F. Heinz body
G. Schistocyte
H. nRBC
I. Polychrom
J. Teardrop
19. ““Seeing much, suffering muchSeeing much, suffering much
and studying much are theand studying much are the
three pillars of learning.”three pillars of learning.”
–Benjamin Disraeli
20. MOLECULAR PATHOLOGY :
Normal adult blood contain 3 types of Hb.
The major component is HbA - α2
ß2
.
The minor component fetal Hb (α2
γ2
) and Hb
A2 (α2
δ2
)
Structure & Synthesis of Haemoglobin:
Hb in adult Hb A Hb F Hb A2
Structure α2
ß 2
α2
γ2
α2
δ2
Normal % 96-98 0.5-0.8 1.5-3.2
24. Thalassemia Syndromes:
Group of disorders with decreased production
of α or β chains.
Features:
Low Hb – depending on type.
Microcytic Hypochromic RBC
Unlike IDA, uniform - low RDW
Target forms typical. No pencil forms.
Heinz bodies – globin deposits
HBH inclusions – Golf ball cells
25. Thalassemia Syndromes:
Etiologically α, β, δβ thalassemia, HbH dis.
Clinically classified into
Hydrops fetalis(α) – IU death
Thalassemia major (β) – transfusion dep
Thalassemia intermedia (αβ) –
spleenomegaly, Fe
Thalassemia minor (αβ) - symptomless.
26. α-Thalassemia:
Decreased production of α chains.
Classification
α0
– (four gene deletion) - Hydrops fetalis
α+
2/3 gene deletion – Thal. Intermedia, HbH dis.
α0
trait, α+
trait – Thal Minor, HPFH
No α thalassemia major.
HbH inclusions can be demonstrated in some
cases. (less in trait, more in intermedia)
39. If you don'tIf you don't
stand for something,stand for something,
you will fall for anything…!you will fall for anything…!
40. Hb Barts levels in Cord blood inHb Barts levels in Cord blood in αα thalassemiathalassemia
Phenotype Equivalent No
of Functional
Genes
% Barts
Normal 4 0
α thal trait
(mild)
3 0-1
α thal trait
(severe)
2 2-8*
Hb H disease 1 10-40
Hb Barts Hydrops 0 ~80
Higgs DR et al Blood 73, 1081, 1989
* Some references mention upto 15%