few days
Haemolytic anemia (complete)
Hematology -
f life-span . .
to
fe
Haemolysis: premature destruction of RBCs
-
normally 120 days , Here a
Develop when B.M cannot compensate for erythrocyte loss extravascul
-
hemolysis -
\ intravascula
Pathophysiology:
Hereditary or acquired.
causes
can be due to: - red cell membrane disorder
– Intrinsic membrane defect: hereditary spherocytosis, eliptocytosis.. Red cell disorder 1
-
enzyme
– Abnormal Hb (HbS)
Hemoglobin opathies
–
-
Erythrocyte enzymatic defects: G6PD deficiency, Hereditary nonspherocytic hemolytic anemia (pyruvate kinase def.)
Ip thala
– Immune destruction of erythrocytes Anem
– mechanical injury: DIC, RBCs hit Fibrin lead to RBC damage, replaced valve: lead to RBCs hitting and damage
– Hypersplenism
un
c
urobilirogen-nve-ick
breakdown exc
pec
Hemolysis can be Intravascular and extravascular:
AU
bilirubin ☐ ✗
-
1- 9 Retie c
– shape globin
.
Intravascular: different blood group transfusion (more severe) •
– Extravascular: autoimmune hemolytic anemia, hereditary spherocytosis (RBCs destructed in spleen and other tissues)
2- Un conj
3- A urin
Hereditary disorders: Erythrocyte membrane abnormality (hereditary spherocytosis), enzymatic defects (G6PD def.), Hb Abnormalities (Sickle cell a
4- abnor
Acquired:
"
Immune disorders: autoimmune hemolytic anemia mut chromic ( sickle
very h
Toxic chemicals and drugs: MCH
:
Antivirals: ribavirin
,G6PD:
X-linked recessive, polymorphic (300 variants)
Hereditary, structural defect in G6PD (house keeping) enzyme
Imp for: survival of RBCs and response to oxidative stress
Work up:
CBC and reticulocyte count
LDH level
Indirect and direct bilirubin
Serum haptoglobin level
off
Urinalysis for hematuria
Urinary hemosiderin: Hb breakdown —> iron release
*haptoglobin is a protein released by liver to bind and clear free Hb (hapto hepato globin globulin)
C.F:
– Neonatal jaundice
– Intravascular hemolysis and consequent anemia
– Chronic hemolytic anemia
Herne body appear
eaten by macrophage ( the heine cells)
Bite all
I
, Keratocytes ( bite cells looks bitten) and heinz body
*Target cell in thalassemia, echinoycyte: renal problem?
*Myelofibrosis: eliptocytes
Management:
most no tx only avoidance
Favabeans, drugs, chemicals that —> oxidative stress
- - -
G6PD: avoid paracetamol and Aspirin
if
Haemolytic anemia (complete)
Hematology -
f life-span . .
to
fe
Haemolysis: premature destruction of RBCs
-
normally 120 days , Here a
Develop when B.M cannot compensate for erythrocyte loss extravascul
-
hemolysis -
\ intravascula
Pathophysiology:
Hereditary or acquired.
causes
can be due to: - red cell membrane disorder
– Intrinsic membrane defect: hereditary spherocytosis, eliptocytosis.. Red cell disorder 1
-
enzyme
– Abnormal Hb (HbS)
Hemoglobin opathies
–
-
Erythrocyte enzymatic defects: G6PD deficiency, Hereditary nonspherocytic hemolytic anemia (pyruvate kinase def.)
Ip thala
– Immune destruction of erythrocytes Anem
– mechanical injury: DIC, RBCs hit Fibrin lead to RBC damage, replaced valve: lead to RBCs hitting and damage
– Hypersplenism
un
c
urobilirogen-nve-ick
breakdown exc
pec
Hemolysis can be Intravascular and extravascular:
AU
bilirubin ☐ ✗
-
1- 9 Retie c
– shape globin
.
Intravascular: different blood group transfusion (more severe) •
– Extravascular: autoimmune hemolytic anemia, hereditary spherocytosis (RBCs destructed in spleen and other tissues)
2- Un conj
3- A urin
Hereditary disorders: Erythrocyte membrane abnormality (hereditary spherocytosis), enzymatic defects (G6PD def.), Hb Abnormalities (Sickle cell a
4- abnor
Acquired:
"
Immune disorders: autoimmune hemolytic anemia mut chromic ( sickle
very h
Toxic chemicals and drugs: MCH
:
Antivirals: ribavirin
,G6PD:
X-linked recessive, polymorphic (300 variants)
Hereditary, structural defect in G6PD (house keeping) enzyme
Imp for: survival of RBCs and response to oxidative stress
Work up:
CBC and reticulocyte count
LDH level
Indirect and direct bilirubin
Serum haptoglobin level
off
Urinalysis for hematuria
Urinary hemosiderin: Hb breakdown —> iron release
*haptoglobin is a protein released by liver to bind and clear free Hb (hapto hepato globin globulin)
C.F:
– Neonatal jaundice
– Intravascular hemolysis and consequent anemia
– Chronic hemolytic anemia
Herne body appear
eaten by macrophage ( the heine cells)
Bite all
I
, Keratocytes ( bite cells looks bitten) and heinz body
*Target cell in thalassemia, echinoycyte: renal problem?
*Myelofibrosis: eliptocytes
Management:
most no tx only avoidance
Favabeans, drugs, chemicals that —> oxidative stress
- - -
G6PD: avoid paracetamol and Aspirin
if
