Hemolytic anemias are characterized by premature destruction of red blood cells (RBCs) and can be classified into congenital/acquired and acute/chronic types, with various intrinsic and extrinsic causes. Clinical features may include jaundice, splenomegaly, and black urine, while lab tests reveal abnormalities in RBCs, reticulocyte counts, and cellularity in bone marrow. Treatment options vary based on the underlying condition, including supportive care, immunosuppressive drugs, and potential genetic therapies.

1. Hemolytic Anemias
Hemolytic Anemias

2. Hemolytic
Hemolytic Anemia
Anemia
Definition
Definition:
:
Group of anemias due premature destruction
Group of anemias due premature destruction
of RBCs
of RBCs
OR
OR
Shortening of RBCs life span
Shortening of RBCs life span

3. Classification
Classification
Criteria of classification
Criteria of classification
 Congenital / Acquired.
Congenital / Acquired.
 Acute /Chronic.
Acute /Chronic.
 Site of hemolysis : intravascular/ Extra..
Site of hemolysis : intravascular/ Extra..
 Basic defect ( cause).
Basic defect ( cause).

4. Basic Causes
Basic Causes
Intrinsic defects
•Membrane
•Enzymes
•HB%
Extrinsic factors
•Infections
•hyperspleenism
•Immune
•Biochemical
•Mechanical

5. General C/F
General C/F
 ...of anemia…
...of anemia…
 Marked Jaundice , stones.
Marked Jaundice , stones.
 Black urine.
Black urine.
 Splenomegally.
Splenomegally.
 Skeletal deformities.
Skeletal deformities.
 Leg ulcrs.
Leg ulcrs.

6. Lab Features of Hemolysis
Lab Features of Hemolysis
 CBC:
CBC: normo-normo, Retic count, nucleated
normo-normo, Retic count, nucleated
RBCs, polychromasia, Poikelocytosis,
RBCs, polychromasia, Poikelocytosis,
fragmented cells...
fragmented cells...
 BM
BM:
: marked
marked cellular
cellular
 Biochemical
Biochemical : bilirubin,free HB, HB urea, LDH,
: bilirubin,free HB, HB urea, LDH,
ALP.
ALP.
 RBCs studies
RBCs studies: fragility, life span
: fragility, life span

8. Membrane Defects
Membrane Defects
 Hereditary spherocytosis.
Hereditary spherocytosis.
 Hereditary eleptocytosis
Hereditary eleptocytosis
 Pyropoikelocytosis
Pyropoikelocytosis
 PNH.
PNH.

9. Hereditary Spherocytosis
Hereditary Spherocytosis
 Common ( AD).
Common ( AD).
 Due to defect in cell membrane proteins;
Due to defect in cell membrane proteins;
spectren, anchyren, B and 4 band.
spectren, anchyren, B and 4 band.
 Deformidibility exra-vascular H.
Deformidibility exra-vascular H.
 Clinically
Clinically: early jaundice, large spleen
: early jaundice, large spleen
 Complications
Complications : leg ulcer, bile stones, Aplastic
: leg ulcer, bile stones, Aplastic
anemia
anemia
 Management?
Management?

10. Hereditary Spherocytosis
Hereditary Spherocytosis
Diagnosis
Diagnosis
 .. Of H. anemia
.. Of H. anemia
 Specific tests:
Specific tests:
 Osmotic fragility test
Osmotic fragility test
 Auto- heamolysis test
Auto- heamolysis test
 Membrane protein studies
Membrane protein studies
 Genetic studied
Genetic studied

11. OFT
OFT
Normal
High fragility
eg: HS
Leg ulcer

12. Paroxysmal nocturnal Hurea
Paroxysmal nocturnal Hurea
 Acquired stem cell disorder resulting in abnormal
Acquired stem cell disorder resulting in abnormal
cell membrane defect.
cell membrane defect.
 DAF complements activity heamolysis
DAF complements activity heamolysis
 Clinically
Clinically: of IVH, morning black urine, thrombosis,
: of IVH, morning black urine, thrombosis,
iron deficiency. AA
iron deficiency. AA
 Specific tests
Specific tests: Ham test, stem cell study
: Ham test, stem cell study

13. Enzymes Deficiency
Enzymes Deficiency
1.
1. G6PD
G6PD
Function: Antioxdation
Function: Antioxdation
1.
1. Pyrophate kinas
Pyrophate kinas

14. G6PD deficiency
G6PD deficiency
 Decreased level or activity
Decreased level or activity
 Tow types:-
Tow types:-
 A- in Africa ( mild)
A- in Africa ( mild)
 B- in middle East ( sever)
B- in middle East ( sever)
 X linked recessive.
X linked recessive.
 Mechanism: G6PD NADH Membrane
Mechanism: G6PD NADH Membrane
oxidation IVH
oxidation IVH

15. G6PD deficiency
G6PD deficiency
 Precipitating factors
Precipitating factors
1.
1. Drugs : antimalarials
Drugs : antimalarials
2.
2. Diet : vava been
Diet : vava been
3.
3. Acidosis
Acidosis
4.
4. Infections, surgery, ...
Infections, surgery, ...
 Clinically:
Clinically: of IVH , after induction
of IVH , after induction

16. Diagnosis and TTT
Diagnosis and TTT
 Diagnosis
Diagnosis:
:
 General.....
General.....
 Specific : Enzymes assay, Heinz bodies
Specific : Enzymes assay, Heinz bodies
 Treatment :
Treatment : try to give option!!!!
try to give option!!!!

17. Immune hemolytic Anemia
Immune hemolytic Anemia
 Shortening of RBCs life span due to immune
Shortening of RBCs life span due to immune
mechanisms (Abs)
mechanisms (Abs)
 Types
Types :-
:-
 1- Clod : 15-20 Cº: IgM
1- Clod : 15-20 Cº: IgM
 2- Worm: 37 Cº IgG
2- Worm: 37 Cº IgG
Allo-immune
Auto-immune
Drug induced

18. Worm IHA
Worm IHA
 37 Cº, IgG
37 Cº, IgG
 Causes
Causes
 Idiopathic
Idiopathic
 Drugs ( methyledopa)
Drugs ( methyledopa)
 Lymphoma / leukemia
Lymphoma / leukemia
 CT. diseases : Rh artheritis, SLE,..
CT. diseases : Rh artheritis, SLE,..

19. Mechanism of worm IHA
Mechanism of worm IHA
B Abs
IgG
Complement
activation
Removal by
spleen
IVH
EVH
Opsonization

20. Diagnosis and treatment
Diagnosis and treatment
 ..General
..General!
! Autoagglutination and spherocytosis in
Autoagglutination and spherocytosis in
Blood film.
Blood film.
 Specific (serology)
Specific (serology)
 DCT (direct Coomb test)(100%)
DCT (direct Coomb test)(100%)
 IDCT (75%)
IDCT (75%)
 TTT:
TTT:
 Of the cause
Of the cause
 Supportive
Supportive
 Immunosuppressive drugs
Immunosuppressive drugs

21. Autoaglutination Spherocytosis

22. Cold IHA
Cold IHA
 15 C
15 Cº
º. IgM
. IgM
 Caused mainly by infection( Mycoplasma).
Caused mainly by infection( Mycoplasma).
 Agg > hemolysis
Agg > hemolysis
 Clinically : peripheral cyanosis( Rhenould)
Clinically : peripheral cyanosis( Rhenould)
 Diagnosed by DCT only ( at 4 C
Diagnosed by DCT only ( at 4 Cº
º)
)

23. Drug induced hemolytic aneamia
Drug induced hemolytic aneamia
How drug induce IHA?
How drug induce IHA?
1.
1. As a hatpin
As a hatpin
2.
2. Innocent by-stander
Innocent by-stander
3.
3. Auto IHA.
Auto IHA.
Diagnosis and TTT ?
Diagnosis and TTT ?

24. Hemoglobinopathy
Hemoglobinopathy
Hemolytic Anemias
Sickle cell
Anemia Thalassemia Others

25. Normal HB.
Normal HB.
 Hem + 4 chains of globing
Hem + 4 chains of globing
 Deferent types of chains:-
Deferent types of chains:-

26. HB Abnormalites
HB Abnormalites
1.
1. Reduced chains production (thalassemia)
Reduced chains production (thalassemia)
2.
2. Abn HB. eg (HBS, C,D, E,...)
Abn HB. eg (HBS, C,D, E,...)
3.
3. Altered O
Altered O2
2 affinity
affinity
4.
4. Failure of reduction (Met HB)
Failure of reduction (Met HB)

27. Thalassemia
Thalassemia
Definition
Definition
Group of genetic disorders due to reduced rate
Group of genetic disorders due to reduced rate
of chain production.
of chain production.
Classification
Classification
_
_ alfa thal.
alfa thal.
- B thal
- B thal
Clinical classification
1. Major
2. Intermedia
3. Minor

28. Genetic Base
Genetic Base
B thalassemia
B thalassemia
 Controlled by tow genes
Controlled by tow genes
 (Point) mutation either complete loss B º
(Point) mutation either complete loss B º
Or partial loss B
Or partial loss B
 So there are many combinations of B, Bº and B .
So there are many combinations of B, Bº and B .
 Clinical severity depends on this combinations
Clinical severity depends on this combinations
+
+

29. Clinical features
Clinical features
 Clinical severity depends on the genetic
Clinical severity depends on the genetic
defect : Major, intermedius, minor.
defect : Major, intermedius, minor.
Generally:
Generally:
 .. Transfusion dependent H. anemia
.. Transfusion dependent H. anemia
 Hepatosplenomegaly
Hepatosplenomegaly
 Skeletal deformities (Hair on the end, short, ...)
Skeletal deformities (Hair on the end, short, ...)
 Infections
Infections
 Iron over load ( heart, liver , glands,...)
Iron over load ( heart, liver , glands,...)

30. Hair on the end
Thal. Faces

31. Lab Findings
Lab Findings
 CBC
CBC : micro- hypo + features of heamolysis +
: micro- hypo + features of heamolysis +
target cells.
target cells.
 BM
BM : hype cellular
: hype cellular
 Biochemical:
Biochemical: of H. anemia?
of H. anemia?
 HB electrophoresis
HB electrophoresis: No HB A, HB F.
: No HB A, HB F.
 Iron profile
Iron profile ( store)
( store)
 Genetic
Genetic studies.
studies.

32. Electrophoresis
Normal
B thal.Major
B thal trait
S/ B thal
HB H
SS

33. Treatment
Treatment
 Recurrent transfusion ( every 4-6 weeks)
Recurrent transfusion ( every 4-6 weeks)
 Folic acid.
Folic acid.
 Iron chelating (Dysferexamine+ Vit C).
Iron chelating (Dysferexamine+ Vit C).
 Treat complications:
Treat complications:
 Infections
Infections
 Endocrinal disorders
Endocrinal disorders
 skeletal deformities
skeletal deformities
 Spleenectomy , gene therapy.
Spleenectomy , gene therapy.

34. Sickle Cell Disorders
Sickle Cell Disorders
 Group of genetic disorders in which HB S is
Group of genetic disorders in which HB S is
inherited.
inherited.
Types :
Types :
 Sickle cell aneamia: HB SS
Sickle cell aneamia: HB SS
 Sickle cell trait :HB AS
Sickle cell trait :HB AS
 HB SC disease
HB SC disease
 Sickle cell thalassemia : HB SB thl.
Sickle cell thalassemia : HB SB thl.
 ......
......

35. Pathogenesis
Pathogenesis
O2
Heamolysis
Vaso-occlusion
sequestration
..............
Genetic
defect

36. Sickle cell anemia C/F
Sickle cell anemia C/F
 H. anemia, mild in relation to low HB% ( 6 g/dl),
H. anemia, mild in relation to low HB% ( 6 g/dl),
start at 6
start at 6th
th
month.
month.
 Acute complications (crisis)
Acute complications (crisis)
Hemolytic crisis
Hemolytic crisis
Vasso- occlusive
Vasso- occlusive
Visceral sequestration
Visceral sequestration
A plastic crisis
A plastic crisis

37. Sickle cell anemia C/F
Sickle cell anemia C/F
 Spleen first , then autosplenectomy (6
Spleen first , then autosplenectomy (6th
th
year)
year)
 Infections ( pneumonia, osteomyelitis,..)
Infections ( pneumonia, osteomyelitis,..)
 Leg ulceration
Leg ulceration
 Bile stones
Bile stones
 Skeletal deformities
Skeletal deformities