Acquired Hemolitic Anemia

Cokorda A Wahyu P

Hematology and Medical Oncology Division

Internal Medicine Department Udayana
University/Sanglah General Hospital
 SKDI (Standar Kompetensi Dokter
Indonesia)
• Hemolytic Anemia (3A)
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rujukan.
 Hemolytic Anemia
Definition
• A group of disorders leading to anemia caused
by a reduction in red cell life span.
– RBC’s normally survive 60 - 120 days .
– Bone marrow has the capacity to increase erythropoiesis 6 - 8 times
than normal.

• Anemia is the result of premature destruction

of red cells exceeding the erythropoietic
capacity of the bone marrow.
 Classification
• Hemolytic anemia may be classified as

I- Hereditary and acquired

or
II- Intracorpuscular and Extracorpuscular
Classification of Hemolytic Anemia

1- Abnormalities of RBC interior

a. Enzyme defects Hereditary
b. Hemoglobinopathies & Thalassemia
2-RBC membrane abnormalities
a. Hereditary spherocytosis, elliptocytosis etc
b. Paroxysmal nocturnal hemoglobinuria
c. Spur cell anemia
3- Extrinsic factors
Acquired

a. Hypersplenism
b. immune hemolysis
c. Traumatic & Microangiopathic hemolysis
d. Infections , toxins , etc
Diagnosis of hemolytic syndrome:
1. Anemia
2. Reticulocytosis
3. Indirect hyperbilirubinemia
 Diagnosis of Hemolysis
• Symptoms depend on degree of anemia (ie, rate of
destruction)
• Clinical features: anemia, jaundice, reticulocytosis, high MCV
& RDW, elevated indirect bili, elevated LDH, low
haptoglobin, positive DAT (AIHA)
• Acute intravascular hemolysis: fever, chills, low back pain,
hemoglobinuria
• Smear: polychromatophilia, spherocytosis &
autoagglutination
 Features of HEMOLYSIS
Bilirubin
LDH
Reticulocytes, n-RBC
Haptoglobulins
+ve Urinary hemosiderin, Urobilinogen

Blood Film

Spherocytes No spherocytes Fragmentation

DAT +ve DAT –ve

AI Hemolysis H. Sherocytosis Malaria,

Clostidium
Hereditery enzymopathies
Microangiopathic,
Traumatic
 AIHA
• Result from RBC destruction due to RBC
autoantibodies: Ig G, M, E, A
• Most commonly-idiopathic
• TRIAD :
– Abrupt onset
– Jaundice
– Splenomegaly
• Classification
– Warm AIHA: reacting ab will show IgG +/- C3
– Cold AIHA: reacting ab will have C3 only
• Cold Aglutinin Disease (CAD)
• Paroxysmal Cold Hemoglobinuria (PCH)
– Mixed type AIHA
1.Warm AI Hemolysis:
Can occurs at all age groups
F > M
Causes:
50% Idiopathic
Rest - secondary causes:
1.Lymphoid neoplasm: CLL, Lymphoma,
Myeloma
2.Solid Tumors: Lung, Colon, Kidney, Ovary,
Thymoma
3.CTD: SLE,RA
4.Drugs: Alpha methyl DOPA, Penicillin , Quinine,
Chloroquine
5.Misc: HIV
• Inv:
– hemolysis, MCV N/
– Blood Smear: Microspherocytosis, n-RBC
– Confirmation: Coomb’s Test / Antiglobulin test

• Treatment
– Correct the underlying cause
– Prednisolone 1mg/kg po until Hb reaches
10mg/dl then taper slowly and stop
– Transfusion: for life threatening problems
– If no response to steroids  Spleenectomy or,
– Immunosuppressive: Azathioprine,
Cyclophosphamide
 Management of Warm-Ab
Autoimmune Hemolysis
• Steroids block clearance of RBCs with IgG or C3 on surface
and decrease production of IgG antibody
• Prednisone 1 to 1.5 mg/kg/day is usual dose
• Most respond within 2 weeks
• Very slow taper required
• Chemotherapy or splenectomy may help if steroids fail
• Transfusions given if needed, may require “least
incompatible” blood; likely will be destroyed at the same
rate as the patient’s own blood
2. Cold AI Hemolysis
Usually Ig M
Acute or Chronic form
Chronic:
Elderly patients
Cold , painful & often blue fingers, toes, ears,
or nose ( Acrocyanosis)
Inv:
 hemolysis
Blood Smear: Microspherocytosis
Other causes of Cold Agglutination:
Infection: Mycoplasma pneumonia, Infec
Mononucleosis
• Treatment:
– Treatment of the underlying cause
– Keep extremities warm
– Steroids treatment
– Blood transfusion
Management of Cold-Ab Autoimmune
Hemolytic Anemia
• Usually no treatment required in setting of mycoplasma or
EBV infection.
• Occasionally transfusion is needed. Washed RBCs have less
complement and are less likely to trigger further hemolysis.
• Steroids usually do not help
• Chemotherapy (eg, cyclophosphamide or chlorambucil) may
help
• In severe cases, plasmapheresis can reduce intravascular
antibody titer
• May have dramatic cold sensitivity; warm infusions, avoid
cold exposure
 Non-Immune Acquired Hemolytic
Anemia
1. Mechanical Trauma
A). Mechanical heart valves, Arterial grafts: cause
shear stress damage
B).March hemoglobinuria: Red cell damage in
capillaries of feet
C). Thermal injury: burns
D). Microangiopathic hemolytic anemia (MAHA): by
passage of RBC through fibrin strands deposited in
small vessels  disruption of RBC eg: DIC,PIH,
Malignant HTN,TTP,HUS
 Non-Immune Acquired Hemolytic
Anemia
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
 SUMMARY
• Hemolytic anemias result from a shortened
red blood cells (RBC) survival rate as a result
of an increased rate of RBC destruction
• Hemolytic anemia may be classified as
Hereditary and acquired
• AIHA is one of acquired type of HA which the
most happen in clinical setting
• AIHA  Warm and Cold  different
patofisiologi and different treatment
 puribatuaya@yahoo.co.id

Thank You Cokorda Agung Wahyu

cokordawahyu