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anemia
Young man of 19
Complains of giddiness
weakness, pallor
Laboratory investigations:
Severe normochromic, normocytic
anemia (hemoglobin level of 6.4 g/dL
Reticulocyte count of 12.2%.
Introduction
Mean
Hemolytic Anemia
Definition:
Those
Classification:
Congenital
Acquired
/ Hereditary
HEMATOLOGIC
Polychromatophilia
Polychromatophilia
Erythroid
hyperplasia
Erythroid
hyperplasia
PLASMA OR SERUM
Bilirubin
Haptoglobin
Plasma hemoglobin
Lactate dehydrogenase
Unconjugated
, Absent
N/
(Variable)
Unconjugated
Absent
(Variable)
URINE
Bilirubin
Hemosiderin
Hemoglobin
0
0
0
0
+
+ severe cases
Hemoglobinuria
Hereditary
Acquired
Ref : Harrisons
Features of HEMOLYSIS
Bilirubin
LDH
Reticulocytes, n-RBC
Haptoglobulins
+ve Urinary hemosiderin, Urobilinogen
Blood Film
Spherocytes
DCT +ve
AI Hemolysis
No spherocytes
Fragmentation
DCT ve
H. Sherocytosis
Malaria,
Clostidium
Hereditery enzymopathies Microangiopathic,
inherited as AD disorder
Defect: Deficiency of Beta Spectrin or Ankyrin
Loss of membrane in Spleen & RES
becomes more spherical Destruction in
Spleen
RBC Membrane
C/F:
Asymptomatic
Fluctuating
hemolysis
Splenomegaly
Pigmented gall stones- 50%
Complications
Clinical
Crisis:
Hemolytic
Inv:
Test
Osmotic Fragility
Management:
Folic Acid
2.Hereditary Elliptocytosis
Equatorial Africa,
SE Asia
AD
/ AR
Functional abnormality in one or more anchor
proteins in RBC membrane- Alpha spectrin ,
Protein 4.1
Usually asymptomatic
Mx: Similar to H. spherocytosis
Variant:
3.SE-Asian ovalocytosis:
Common in Malaysia , Indonesia
Asymptomatic-usually
Cells oval , rigid ,resist invasion by malarial
parasites
Elliptocytosis
1. Glucose-6-Phosphate Dehydrogenase
( G6PD ) Deficiency
Pivotal
Most
(Reduced form)
(Oxidised form)
Clinical
Acute
Features:
drug induced hemolysis:
Aspirin,
Chronic
compensated hemolysis
Infection/acute illness
Neonatal jaundice
Favism
Inv:
e/o
non-spherocytic intravascular
hemolyis
P. Smear: Bite cells, blister cells,
irregular small cells, Heinz bodies,
polychromasia
G-6-PD level
Treatment:
Stop
Chronic
hemolytic anemia
Inv;
P. Smear: Prickle cells
Decreased enzyme activity
Treatment:
Transfusion may be required
Hemolobinopathies
Celsius
Cold AI Hemolysis: Ab binds at 4 degree
Celsius
1.Warm AI Hemolysis:
Can
MACROCYTE
SPHEROCYTE
IMMUNOHEMOLYTIC ANEMIA
complement
Inv:
e/o
hemolysis, MCV
P Smear: Microspherocytosis, n-RBC
Confirmation: Coombs Test / Antiglobulin test
Treatment
Correct
2. Cold AI Hemolysis
Usually Ig M
Acute or Chronic form
Chronic:
C/F:
Elderly
patients
Cold , painful & often blue fingers, toes,
ears, or nose ( Acrocyanosis)
Inv:
e/o
hemolysis
P Smear: Microspherocytosis
Ig M with specificity to I or I Ag
Other
Infection:
Treatment:
Treatment
1. Mechanical Trauma
TRAUMATIC HEMOLYSIS
Acquired hemolysis
2.Infection
F. malaria: intravascular hemolysis: severe
called Blackwater fever
Cl. perfringens septicemia
3.Chemical/Drugs: oxidant denaturation of
hemoglobin
Eg: Dapsone, sulphasalazine, Arsenic
gas, Cu, Nitrates & Nitrobenzene
Immunophenotyping of bone
marrow cells showed that 10% of
all the cells were CD20 and CD19
positive.
Rituximab is a monoclonal
antibody that binds to CD 20
Rituximab was started at the dose
of 375 mg/mq once weekly, for a
total of 4 doses
Summary of lecture
Learning points