You are on page 1of 24

Acquired Hemolytic Anemia

Dr Peter Maturi Mwamba


Senior Lecturer
Hematology and Blood Transfusion
Hemolytic anemia is anemia due to
hemolysis which can be either:
intravascular
Extravascular
General classification of hemolytic anemia
is either:
Acquired or
Inherited.
Acquired Hemolytic Anaemia
Acquired hemolytic anemia can be further divided
into:
Immune and
Non-immune mediated.
This is according to the mechanism by which
hemolysis is produced.
In immune the antibodies are the main agents in the
immature destruction of red cell
Direct Coombs test is positive
Immune mediated Hemolytic
Anaemia
Main causes are:
Autoimmune hemolytic anemia
Alloimmune hemolytic anemia
Drug induced immune mediated hemolytic anemia
Autoimmune
1. Autoimmune hemolytic anemia
A. Warm antibody autoimmune hemolytic
anemia
B. Cold antibody autoimmune hemolytic
anemia
A: Warm antibody autoimmune hemolytic anemia
Idiopathic - cause not known

Associated with lymphoproliferative disorders and


other malignancies

Associated with autoimmune diseases e.g. SLE


B: Cold antibody autoimmune hemolytic anemia
Idiopathic

Associated with infections:


Infectious mononucleosis and
Mycoplasma ( atypical) pneumonia

Paroxysmal nocturnal hemoglobinuria (PNH)


2. Alloimmune hemolytic anemia
Haemolytic disease of the newborn (HDN)
Rh disease (Rh D)

Other blood group incompatibility (RhC, Rhe, Kidd, Duffy,


MN, P and others)

Alloimmune hemolytic blood transfusion reactions (ie


from a non-compatible blood type
3. Drug induced immune mediated
hemolytic anemia

a) Membrane modification mechanism


b) Drug adsorption mechanism: (Hapten type)
c) Immune complex mechanism: (Innocent
bystander mechanism)
a) Membrane modification mechanism
- Causes a positive DAT by modifying the red cell
membrane components

- Examples: Cephalosporin
a) Drug absorption mechanism: (Hapten type)
- Here drugs readily form hapten-carrier
complexes with plasma proteins which enhance
drug-specific antibody production.
- As a result a variety of plasma proteins
including immunoglobulins and complement
attach through non-immune mechanisms to the
membrane.
- Examples here include eg for penicillin,
cephalosporins and tetracyclines
a) Immune complex mechanism: (Innocent bystander
mechanism)
- Here drugs form hapten-carrier complexes with plasma
proteins which enhance drug-specific antibody production.
- Once drug antibodies are present, reintroduction of the
drug causes immune complexes to form which are
absorbed on to the red cell membrane and complement
activated.
- Classicallly hemolysis occures on the second or
subsequent exposure to the drug and may develop within
minutes or hours of drug injestion.
- Examples of drug:
Rifampicin, phenacetin, quinine, quinidine,
chlorpropramidePenicillin (high dose)
Non-immune mediated HA
Direct Coombs test is negative
1. Drugs
Some drugs and other ingested substances lead to haemolysis
by direct action on RBCs, e.g. ribavirin
Via oxidative mechanisms. This is particularly likely to occur
when there is an enzyme deficiency in the antioxidant defence
system of the redcell eg G6PD deficiency where antimalarial
oxidant drugs like primaquine damage red blood cells
Via cell volume control mechanisms; for example drugs can
directly or indirectly impair regulatory volume decrease
mechanisms, which become activated during hypotonic RBC
swelling to return the cell to a normal volume. e.g. ouabain at
very high doses
Some drugs cause RBC (red blood cell) lysis even in normal
individuals. These include dapsone and sulfasalazine.
2. Toxins
Snake venom;
Plant poisons such as aesculin
3. Trauma
Mechanical eg heart valves, extensive
vascular surgery, microvascular disease)
4. Microangiopathic hemolytic anemia
Thrombotic Thrombocytopenia Purpura (TTP)

Hemolytic Uremic Syndrome (HUS)

Disseminated Intravascular Coagulation(DIC)


5. Infections
Malaria Falciparum:
Babesiosis
Septicaemia
Here hemolysis involves extravascular destruction of
parasitized cells in the spleen and liver and intravascular
lysis when the spozoites break out of the cells.
Laboratory Workup

Initial Investigations:
FBC

Reticulocyte count
Raised indicating increased erythropoietc
activity
Blood film

Peripheral Blood Film


Important morphologic changes e.g.
Spherocytes (Immune haemolytic anaemias,
toxins, burns)
Red cell fragments (fragmentation anaemias)
Polychromasia
Nucleated red cells
Parasites etc
Biochemical tests for
haemolysis
Liver function tests
Bilirubinemia (increased indirect bilirubin)
LDH increased
Haptoglobulin, hemopexin decreased

Urinalysis
Haemosiderin
Haemoglobinuria
Urobilinogen
Specific studies:
Directed by history and PE and preliminary tests:
Direct antiglobulin test (Immune mediated
haemolysis)
Antibody identification
Tests for drug mediated haemolysis
Hams-Dacie/Hams test (serum acidified lysis
test) PNH (sugar or sucrose lysis test)
Flow cytometry
Red blood cell survival [51 Cr] survival) -rarely
used, can demonstrate shortened RBC
(hemolysis).
FRAGMENTS/SCHISTOCYTES
Polychromasia
Malaria
parasites