Hemolytic Anemia: Classification and Management
الدكتور
ماهر جبار صالح
اختصاص دقيق (دكتوراه) اورام وامراض الدم
كليه طب البصره
classification of hemolytic anemia
عنوان المحاظره :
10-11-2019
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This results from increased red cell
destruction due to red cell autoantibodies.
•
The antibodies may be IgG or IgM, or
more rarely IgE or IgA.
•
If an antibody avidly fixes complement, it
will cause intravascular haemolysis, but if
complement activation is weak, the
haemolysis will be extravascular (in the
reticulo-endothelial system).
•
Warm antibodies
bind best at 37°C and
account for 80% of cases.
•
the majority are IgG and often react against
Rhesus antigens.
•
Cold antibodies
bind best at 4°C but can bind
up to 37°C in some cases. They are usually
IgM and bind complement. To be clinically
relevant, they must act within the range of
normal body temperatures. They account for
the other 20% of cases.
Causes
:
Investigations
:
•
General
: ……
•
Specific
: The standard Coombs reagent
will
miss
IgA or IgE antibodies.
•
Around 10% of all warm autoimmune
haemolytic anaemias are Coombs test-
negative.
Management
warm
type
•
If the haemolysis is
secondary
to an
underlying cause, this must be treated and
any implicated drugs stopped.
1
st
line treatment
•
prednisolone (1 mg/kg orally).
•
A response is seen in 70–80% of cases but
may take up to 3 weeks; a rise in
haemoglobin will be matched by a fall in
bilirubin, LDH and reticulocyte levels. Once
the haemoglobin has normalised and the
reticulocytosis resolved, the glucocorticoid
dose can be reduced slowly over several
weeks.
•
Q…Glucocorticoids probably work by..?
2
nd
line :
•
These include immunomodulation/suppression and
splenectomy.
•
Currently, there are fewer splenectomies than
previously and the second-line drug of choice in
current UK guidance is the anti-CD20 monoclonal
antibody rituximab.
•
Splenectomy is associated with a good response in
50–60% of cases.
•
The operation can be performed laparoscopically with
reduced morbidity.
•
If splenectomy is not appropriate, alternative
immunosuppressive therapy with azathioprine,
ciclosporin.
C
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d
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s
e
•
This is mediated by antibodies, usually
IgM, which bind to the
•
red cells at low temperatures and cause
them to agglutinate
•
This can be chronic when the antibody is
monoclonal…..
lymphoma
•
or acute or transient when the antibody is
polyclonal……
infection
.
Chronic cold agglutinin disease
•
This typically affects elderly patients and
may be associated with an underlying low-
grade B-cell lymphoma. It causes a low-
grade intravascular haemolysis with cold,
painful and often blue fingers,
•
toes, ears or nose (so-called
acrocyanosis).
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Cold agglutination can occur in association
with
Mycoplasma
•
pneumoniae
or with infectious
mononucleosis
Treatment
:
•
Treat the underlying cause :
infection , lymphoma .
•
Warm the peripheries in winter .
•
less likely steroid /rituximab .
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unmatched blood transfusion maternal
sensitisation to paternal antigens on fetal
cells.
•
(haemolytic disease of the newborn,
N
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1-Endothelial damage
:
•
Mechanical heart valves.
•
March haemoglobinuria.
•
Thermal injury.
•
Microangiopathic haemolytic anaemia.
2
-
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n
f
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t
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Plasmodium falciparum
malaria may be
associated with
•
intravascular haemolysis; when severe, this
is termed blackwater
•
fever because of the associated
haemoglobinuria
•
Clostridium perfringens
sepsis usually in the
context of ascending cholangitis or
necrotising fasciitis .
3
-
C
h
e
m
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c
a
l
s
o
r
d
r
u
g
s
•
Dapsone and sulfasalazine.
•
Benzine
•
Nitrate .
•
Arsenic.
P
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rare acquired,non immune non-malignant
clonal expansion of haematopoietic stem
cells
deficient
in
glycosylphosphatidylinositol (GPI) anchor
protein which is
important
to protect the
cell against the complement mediated
hemolysis.
•
…..complement activation
Clinical features
:
•
Hemolysis.
•
Thrombosis at unusual sites ..liver /abdomen .
•
Aplastic an./MDS
TREATMENT
:
•
Supportive …..
•
Anti complement C5 Ab…ECULIZUMAB.
Cases
:
•
THANKS
Hemolytic anemia encompasses various subtypes like autoimmune hemolytic anemia, with warm and cold antibodies, each requiring unique management strategies. Diagnosis involves specific investigations like the Coombs test. Treatment typically involves corticosteroids as first-line therapy, while second-line options include immunosuppression and splenectomy. Cold agglutinin disease is another notable subtype. Understanding the classification and management of hemolytic anemia is crucial for effective patient care.
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Presentation Transcript
Autoimmune haemolytic anaemia This results from increased red cell destruction due to red cell autoantibodies. The antibodies may be IgG or IgM, or more rarely IgE or IgA. If an antibody avidly fixes complement, it will cause intravascular haemolysis, but if complement activation is weak, the haemolysis will be extravascular (in the reticulo-endothelial system).
Warm antibodies bind best at 37C and account for 80% of cases. the majority are IgG and often react against Rhesus antigens. Cold antibodies bind best at 4 C but can bind up to 37 C in some cases. They are usually IgM and bind complement. To be clinically relevant, they must act within the range of normal body temperatures. They account for the other 20% of cases.
Investigations : General : Specific : The standard Coombs reagent will miss IgA or IgE antibodies. Around 10% of all warm autoimmune haemolytic anaemias are Coombs test- negative.
Management warm type If the haemolysis is secondary to an underlying cause, this must be treated and any implicated drugs stopped.
1stline treatment prednisolone (1 mg/kg orally). A response is seen in 70 80% of cases but may take up to 3 weeks; a rise in haemoglobin will be matched by a fall in bilirubin, LDH and reticulocyte levels. Once the haemoglobin has normalised and the reticulocytosis resolved, the glucocorticoid dose can be reduced slowly over several weeks. Q Glucocorticoids probably work by..?
2ndline : These include immunomodulation/suppression and splenectomy. Currently, there are fewer splenectomies than previously and the second-line drug of choice in current UK guidance is the anti-CD20 monoclonal antibody rituximab. Splenectomy is associated with a good response in 50 60% of cases. The operation can be performed laparoscopically with reduced morbidity. If splenectomy is not appropriate, alternative immunosuppressive therapy with azathioprine, ciclosporin.
Cold agglutinin disease This is mediated by antibodies, usually IgM, which bind to the red cells at low temperatures and cause them to agglutinate
This can be chronic when the antibody is monoclonal ..lymphoma or acute or transient when the antibody is polyclonal infection .
Chronic cold agglutinin disease This typically affects elderly patients and may be associated with an underlying low- grade B-cell lymphoma. It causes a low- grade intravascular haemolysis with cold, painful and often blue fingers, toes, ears or nose (so-called acrocyanosis).
Other causes of cold agglutination Cold agglutination can occur in association with Mycoplasma pneumoniae or with infectious mononucleosis
Treatment : Treat the underlying cause : infection , lymphoma . Warm the peripheries in winter . less likely steroid /rituximab .
Alloimmune haemolytic anaemia unmatched blood transfusion maternal sensitisation to paternal antigens on fetal cells. (haemolytic disease of the newborn,
Non-immune haemolytic anaemia 1-Endothelial damage: Mechanical heart valves. March haemoglobinuria. Thermal injury. Microangiopathic haemolytic anaemia.
2-Infection Plasmodium falciparum malaria may be associated with intravascular haemolysis; when severe, this is termed blackwater fever because of the associated haemoglobinuria Clostridium perfringens sepsis usually in the context of ascending cholangitis or necrotising fasciitis .
3-Chemicals or drugs Dapsone and sulfasalazine. Benzine Nitrate . Arsenic.
Paroxysmal nocturnal haemoglobinuria rare acquired,non immune non-malignant clonal expansion of haematopoietic stem cells deficient in glycosylphosphatidylinositol (GPI) anchor protein which is important to protect the cell against the complement mediated hemolysis. ..complement activation
Clinical features : Hemolysis. Thrombosis at unusual sites ..liver /abdomen . Aplastic an./MDS
TREATMENT : Supportive .. Anti complement C5 Ab ECULIZUMAB.
