Medicine / haemolytic anemia

1. HEMOLYTIC ANEMIAS
Prof.Dr. Hayder S.Qasim
consultant physician

2. Factors which influence the size of red cells in
anaemia. ↓ MCV is < 76 fL; ↑ MCV is > 100 fL.
Printed from: Davidson's Principles and Practice of Medicine
21E (on 25 March 2013)
© 2013 Elsevier

4. HEMOLYTIC ANEMIA
• Anemia of increased destruction
– Normochromic, normochromic anemia
– Shortened RBC survival
– Reticulocytosis - Response to increased
RBC destruction
– Increased indirect bilirubin
– Increased LDH

7. HEREDITARY SPHEROCYTOSIS
• Defective or absent spectrin molecule
• Leads to loss of RBC membrane, leading to
spherocytosis
• Decreased deformability of cell
• Increased osmotic fragility
• Extravascular hemolysis in spleen
• Autosomal domenent

8. 0
20
40
60
80
100
0.3 0.4 0.5 0.6
NaCl(%ofnormalsaline)
%
Hemolysis
Normal HS
Osmotic
Fragility

10. Diagnosis
flow cytometric tests, detecting binding of eosin-5-
maleimide to red cells, are recommended in borderline
cases.
An osmotic fragility test may show increased sensitivity
to lysis in hypotonic saline solutions but is limited by
lack of sensitivity and specificity.
Family screening
Blood film show spherocytes

11. Treatment
Folic acid prophylaxis, 5 mg once weekly, should be
given for life.
Consideration may be given to splenectomy, which
improves but does not normalise red cell survival.
Potential indications include moderate to severe
haemolysis with complications (anaemia and
gallstones), although splenectomy should be delayed
until after 6 years of age in view of the risk of sepsis

13. Paroxysmal Nocturnal
Hemoglobinuria

18. • When to suspect G6PD deficiency? —
Glucose-6-phosphate dehydrogenase
(G6PD) deficiency should be suspected in
any subject with an episode of non-
immune hemolytic anemia, especially if
occurring after drug ingestion, exposure
to fava beans, or associated with an
infection.

19. Care must be taken close to an acute haemolytic
episode because reticulocytes may have higher
enzyme levels and give rise to a false normal
result
Management
aims to stop any precipitant drugs and treat any
underlying infection. Acute transfusion support
may be life-saving.
Administration of folic acid in a dose of 1 mg/day
may prevent worsening of anemia due to folate
depletion in this group of patients.
G6PD level

20. HEMOLYTIC ANEMIA

24. Suspecting hemolysis —
• Rapid onset of pallor and anemia
• Jaundice with increased indirect bilirubin concentration
• History of pigmented (bilirubin) gallstones
• Splenomegaly
• Presence of circulating spherocytic red cells or red cells
with other shape changes
• Increased serum lactate dehydrogenase (LDH)
• Reduced (or absent) level of serum haptoglobin
• A positive direct antiglobulin test (Coombs test)
• Increased reticulocyte percentage or absolute reticulocyte
number, indicating the bone marrow's response to the
anemia

25. AUTOIMMUNE HEMOLYSIS
Warm Type
• Usually IgG antibodies
• Fix complement only to level of C3,if at all
• Immunoglobulin binding occurs at all temps
• Fc receptors/C3b recognized by
macrophages;
• Hemolysis primarily extravascular
• 70% associated with other illnesses

26. Management
• prednisolone 1 mg/kg orally. A response is seen in 70-
80% of cases but may take up to 3 weeks;
• Transfusion support may be required for life-threatening
problems,
• splenectomy should be considered. with a good response
in 50-60% of cases.
• immunosuppressive therapy with azathioprine or
cyclophosphamide may be considered.
• The anti-CD20 (B cell) monoclonal antibody, rituximab,
has shown some success in difficult cases.

27. AUTOIMMUNE HEMOLYSIS
Cold Type
• Most commonly IgM mediated
• Antibodies bind best at 30º or lower
• Fix entire complement cascade
• Leads to formation of membrane attack complex,
which leads to RBC lysis in vasculature
• Typically only complement found on cells
• 90% associated with other illnesses
• Poorly responsive to steroids, splenectomy;
responsive to plasmapheresis

28. • Abnormalities on the peripheral blood smear
suggesting extravascular hemolysis include:
spherocytes, microspherocytes, elliptocytes,
"bite" or blister cells, acanthocytes, and
teardrop red cells.
• Abnormalities that suggest that the
hemolysis is intravascular include the
presence of free hemoglobin in plasma or
urine, a urine sediment positive for iron
(hemosiderinuria), and, in rare cases, the
presence of circulating red cell "ghosts."

29. Cold agglutinin disease
• 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).
• MCV may be spuriously raised
• Treatment is directed at any underlying lymphoma but if
the disease is idiopathic, then patients must keep
extremities warm, especially in winter.
• Some patients respond to corticosteroid therapy and
blood transfusion may be considered, but the cross-
match sample must be placed in a transport flask at a
temperature of 37 °C and blood administered via a
blood-warmer.

30. • Other causes of cold agglutination Cold
agglutination can occur in association with
Mycoplasma pneumoniae or with infectious
mononucleosis.
• Paroxysmal cold haemoglobinuria is a very rare
cause seen in children in association with
congenital syphilis.
• An IgG antibody is termed the Donath-
Landsteiner antibody and has specificity against
the P antigen on the red cells.

31. Other causes of hemolytic anemia