PAROXYSMAL NOCTURNAL

HEMOGLOBINURIA
 Paroxysmal nocturnal hemoglobinuria (PNH) is a rare,

acquired clonal stem cell disorder in which red cells are

abnormally sensitive to the hemolytic action of the

complement.

 PNH red cells are deficient in several cell membrane

proteins including CD 55 (decay accelerating factor) and

CD 59 (membrane inhibitor of reactive lysis or MIRL)

 In a typical case of PNH, red cell destruction occurs at

night so that hemoglobinuria (passage of reddishbrown

urine) is noticed after getting up in the morning.

 Laboratory diagnosis of PNH is based on:

•1- Evidence of intravascular hemolysis:

Hemoglobinuria, hemosiderinuria,

methemalbuminemia, and increased indirect serum

bilirubin.
  2-Demonstration of increased sensitivity of red cells to

complement: Sucrose hemolysis test, Ham’s test. •

 3- Flow cytometric analysis of blood cells for lack of

CD55 or CD59.

Tests for Paroxysmal Nocturnal

Hemoglobinuria..
1-Sucrose hemolysis test :

 This is the screening test.

 It is highly sensitive but less specific than Ham’s test.
 Red cells from the patient are mixed with fresh
ABOcompatible normal serum and isotonic sucrose
solution (a low ionic strength medium which activates
complement). Hemolysis >10% indicates PNH.
2. Ham’s acidified serum lysis test:

 Acidified serum lysis test or Ham’s test is the definitive

test for the diagnosis of PNH.
 Patient’s red cells are mixed with acidified normal
serum (acidification activates complement).
 Hemolysis occurs if red cells are abnormally sensitive to
complement action .
MICROANGIOPATHIC HEMOLYTIC ANEMIA :
 This is a form of mechanical hemolytic anemia resulting
from intravascular fragmentation and lysis of red cells
due to vascular endothelial abnormalities.
 Common causes include :
 1-thrombotic thrombocytopenic purpura,
 2-hemolytic uremic syndrome,
 3-disseminated intravascular coagulation, eclampsia,
malignancy, and generalized vasculitis. Blood film shows
numerous fragmented red cells (schistocytes)
Blood smear in microangiopathic hemolytic anemia showing
many fragmented red cell

Thank you

DR..Lubna aloshibi