Paroxysmal nocturnal hemoglobinuria (PNH) For Lab.students

1. Paroxysmal Nocturnal Hemoglobinuria (PNH)
Autoimmune haemolytic anaemia
Developed by-Dr.Abdulrazzaq Othman Alagbare
M.D M.S.c C.P - Lecturer of Hematology and Immunohematology
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2. Paroxysmal Nocturnal Hemoglobinuria (PNH)
 It is non-immune but acquired hemolytic anemia, unknown causes, It is
intravascular hemolysis
Why its nocturnal?
 sleep causes relative acidosis and activate the complements
An acquired membrane defect of the MSC.
There is a loss of anchor protein which hold different antigens e.g. CD59,
CD55.
These are regulatory proteins for the complement pathway.
ANEMIA -Dr.Alagbare

3. Paroxysmal Nocturnal Hemoglobinuria (PNH)
All cells are abnormally sensitive to lysis by complement
Which cells affected?
 RBC , leukocytes and platelets,
ANEMIA -Dr.Alagbare

4. Paroxysmal Nocturnal Hemoglobinuria (PNH):
• It is  rare, non immune hemolytic anemia,
acquired
ANEMIA -Dr.Alagbare
Mechanism: loss of anchor protein (GPI glycosylphosphatidyl inositol) which
hold different antigens e.g. CD59, CD55.

5. Paroxysmal Nocturnal Hemoglobinuria (PNH
Lab finding - CBC
1- Pancytopenia -Patients with PNH may develop pancytopenia with aplastic
anaemia
Urine:
Hemoglobinuria and Hemosiderinuria is a constant feature and can give
rise to iron deficiency which may make worse the anemia
When the patient wakes up in the morning and passes urine, the urine is
dark due to hemoglobinuria (thus the name).
The urine will clear during the day.
ANEMIA -Dr.Alagbare

6. Paroxysmal Nocturnal Hemoglobinuria (PNH
Modern diagnosis by flow cytometry,
 The standard diagnostic test for PNH , CD59 and CD55 are absent or
decrease amount
Old diagnostic methods:
acidified serum (Ham’s) test (more specific) no longer used for diagnosis
sucrose hemolysis test (more sensitive) no longer used for diagnosis
ANEMIA -Dr.Alagbare

7. 1- tube Patient cells with patient serum (unacidified).
2-Patient cells with patient serum (acidified).
3- tube Patient cells with patient serum (acidified
and heated).
Ham test
Heat inactivates complement,
whereas low pH activates complement
Ham’s test is no longer used for diagnosis but is included
here as it illustrates the mechanism of haemolysis.
ANEMIA -Dr.Alagbare
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8. ‫رب‬ ‫هلل‬ ‫الحمد‬
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