APPROACH TO A PATIENT
Dr. Ramlah Hasan
Hemolytic are a group of disorders characterised
by shortened survival of RBCs in circulation .
Consequences of hemolysis
Shortening of red cells survival does not always cause
anemia as there is a compensatory increase in red cell
production by the bone marrow.If the red cell loss can be
contained within the marrow’s capacity for increased
output,then a hemolytic state can exist without anemia
(compensated hemolytic disease). The bone marrow can
increase its output by 6 to 8 times by increasing the
proportion of cells committed to erythropoiesis (erythroid
hyperplasia) and by expanding the volume of active
marrow. In addition, immature cells (reticulocytes) are
Sites of hemolysis
Extra vascular hemolysis
In most hemolytic conditions red cell
destruction is extra vascular. The red
Cells are removed from the circulation by
macrophages in the reticuloendothelial
system, particularly the spleen.
Sites of Hemolysis (contd.)
Intra vascular Hemolysis
When red cells are rapidly destroyed within
the circulation Hb is liberated. This is initially
bound to plasma haptoglobin but these soon
become saturated. Excess free plasma Hb is
filtered by the glomerulus and enters the urine,
although small amounts are reabsorbed by the
renal tubules. In the renal tubular cells Hb is
broken down and becomes deposited in the
cells as haemosiderin.
Classification of Hemolytic
Pyruvate Kinase Deficiency
Sickle Cells Anemia
Paroxysmal Nocturnal Haemoglobinuria
Classification of Hemolytic
Autoimmune Hemolytic Anemias
Associated with warm antibodies
Associated with cold antibodies
Isoimmune Hemolytic Anemias
Drug induced Hemolytic Anemias
Microangiopathic Hemolytic Anemias
Hemolytic anemia can occur at any age.
Hereditary disorders usually are evident early in life.
AIHA is more likely to occur in middle-aged and older
Most cases of hemolytic anemia are not specific to any
Autoimmune hemolytic anemia (AIHA) is slightly more
likely to occur in females than in males.
G-6-PD deficiency is an X-linked recessive disorder.
Males usually are affected, and females are carriers
Pallor, Fatigue, Worsening of preexisting, angina,
Palpitation, Postural dizziness
Mottled or Numb fingers or toes
Pigment gall stone
Reddish Brown first morning urine
Symptoms due to splenomegaly like dragging
sensation in abdomen
Acute painful episode include the bones
(especially the back and long bones) and the chest.
Positive Medical History for Associated
Signs of congestive cardiac failure
Poorly healing ulcers over lower tibia
Blood Complete Picture
Hb or N
Red Cell Indices
Bone Marrow Examination
The level of unconjugated bilirubin never exceeds 4 to 5 mg/dl.
Positive Urinary Haemosiderin
Peripheral Blood Film
Spherocytes No spherocytes Fragmentation
DCT+ DCT- Hereditary
Autoimmune Hereditary Microangiopathic,
hemolysis spherocytosis Traumatic
Sickle Cell Anemia
Osmotic fragility test
Specific enzymatic assays
Evaluation of Hemolysis
Extra vascular Intra vascular
Plasma Or Serum
Bilirubin Unconjugated Unconjugated
Haptoglobin , Absent Absent
Plasma Hb N-
Lactate Dehydrogenase ) variable) (variable)
Bilirubin 0 0
Hemosiderin 0 +
Haemoglobin 0 + in severe cases
More than 200 types of hemolytic anemia exist, and each type
requires specific treatment. Therefore, only the aspects of medical
care relevant to most cases of hemolytic anemia are discussed.
Avoid transfusions unless absolutely necessary, but they may be essential for
patients with angina or severely compromised cardiopulmonary status.
Administer packed RBC slowly to avoid cardiac stress.
In AIHA, type matching and crossmatching may be difficult. Use the least
incompatible blood if transfusions are indicated. The risk of acute hemolysis of
transfused blood is high, but the degree is dependent on the rate of infusion.
Slowly transfuse by administering half units of packed red cells to prevent rapid
destruction of transfused blood.
Discontinue penicillin and other agents that can cause immune hemolysis and
oxidant medication such as sulfa drugs
Administer folic acid because active hemolysis may consume folate
and cause megaloblastosis.
Corticosteroids are indicated in AIHA.
IV immunoglobulin G (IgG) has been used for patients with AIHA,
but only a few patients have responded, and the response has been
This is indicated for patients with severe intravascular hemolysis in
which persistent hemoglobinuria has caused substantial iron loss.
Before iron is administered, document the iron deficiency by
serum iron studies and, possibly, by assessing iron stores in bone
Because iron stores increase in hemolysis, iron administration
generally is contraindicated in hemolytic disorders, particularly
those that require chronic transfusion support.
Splenectomy may be the first choice of treatment in
some types of hemolytic anemia such as hereditary
In other cases, it is recommended when other measures,
such as in AIHA, have failed.
Splenectomy usually is not recommended in hemolytic
disorders such as cold agglutinin hemolytic anemia.
Immunize against infections with encapsulated organisms,
such as Haemophilus influenzae and Streptococcus
pneumoniae, as far in advance of the procedure as possible.
Points to Remember
Peripheral smear is the most important
initial investigation and
Coombs test with anti-IgG and anti-
complement in diagnosing hemolytic