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Paroxysmal cold hemoglobinuria - PCH
Autoimmune haemolytic anaemia
Developed by-Dr.Abdulrazzaq Othman Alagbare
M.D M.S.c C.P - Lecturer of Hematology and Immunohematology
1
Paroxysmal cold hemoglobinuria - PCH
Definition: It is cold agglutinin disease With An IgG biphasic Donath-
Landsteiner antibody (Donath-Landsteiner antibody.)
ANEMIA -Dr.Alagbare
What means the
IgG is biphasic?
In low
tempreture
binding with
RBC
causes intravascular
hemolysis after
warming.
In 37 C
Paroxysmal cold hemoglobinuria - PCH
MECHANISM
The antibodies cause intravascular hemolysis with hemoglobinuria and
hemosiderinuria
Hemolysis results from exposure to cold, which may even be localized (eg,
from drinking cold water, from washing hands in cold water
IgG binds to RBCs at low temperatures and causes intravascular
hemolysis after warming.
ANEMIA -Dr.Alagbare
Paroxysmal cold hemoglobinuria - PCH
Risk factor: viral illness in young children
or acquired syphilis
CBC: anemia, increase plasma Hb,
Urine : hemoglobinuria and hemosiderinuria
ANEMIA -Dr.Alagbare
Paroxysmal cold hemoglobinuria - PCH
PBS: Erythrophagocytosis by neutrophils is common and is distinctive
Peripheral blood film showing auto-agglutination, spherocytes and
reticulocytes
ANEMIA -Dr.Alagbare
Erythrophagocytosiauto-agglutination, spherocytes
Paroxysmal cold haemoglobinuria:
Donath Landsteiner antibody positive haemolytic anaemia.
Peripheral blood film showing auto-agglutination,
spherocytes and reticulocytes
‫رب‬ ‫هلل‬ ‫الحمد‬
‫العالمين‬
‫ابتغي‬ ‫العمل‬ ‫هذا‬
‫الكريم‬ ‫هللا‬ ‫وجه‬ ‫به‬
7

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Paroxysmal cold hemoglobinuria -PCH

  • 1. Paroxysmal cold hemoglobinuria - PCH Autoimmune haemolytic anaemia Developed by-Dr.Abdulrazzaq Othman Alagbare M.D M.S.c C.P - Lecturer of Hematology and Immunohematology 1
  • 2. Paroxysmal cold hemoglobinuria - PCH Definition: It is cold agglutinin disease With An IgG biphasic Donath- Landsteiner antibody (Donath-Landsteiner antibody.) ANEMIA -Dr.Alagbare What means the IgG is biphasic? In low tempreture binding with RBC causes intravascular hemolysis after warming. In 37 C
  • 3. Paroxysmal cold hemoglobinuria - PCH MECHANISM The antibodies cause intravascular hemolysis with hemoglobinuria and hemosiderinuria Hemolysis results from exposure to cold, which may even be localized (eg, from drinking cold water, from washing hands in cold water IgG binds to RBCs at low temperatures and causes intravascular hemolysis after warming. ANEMIA -Dr.Alagbare
  • 4. Paroxysmal cold hemoglobinuria - PCH Risk factor: viral illness in young children or acquired syphilis CBC: anemia, increase plasma Hb, Urine : hemoglobinuria and hemosiderinuria ANEMIA -Dr.Alagbare
  • 5. Paroxysmal cold hemoglobinuria - PCH PBS: Erythrophagocytosis by neutrophils is common and is distinctive Peripheral blood film showing auto-agglutination, spherocytes and reticulocytes ANEMIA -Dr.Alagbare Erythrophagocytosiauto-agglutination, spherocytes
  • 6. Paroxysmal cold haemoglobinuria: Donath Landsteiner antibody positive haemolytic anaemia. Peripheral blood film showing auto-agglutination, spherocytes and reticulocytes
  • 7. ‫رب‬ ‫هلل‬ ‫الحمد‬ ‫العالمين‬ ‫ابتغي‬ ‫العمل‬ ‫هذا‬ ‫الكريم‬ ‫هللا‬ ‫وجه‬ ‫به‬ 7