Page 310 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

294 PART 4 ■ Erythrocyte Disorders

■ C mm G (I G)
mm M (I M) BOX 16.1

mm .

■ M - -
- Complementary Regulatory Proteins

. CD ( ) x

■ A (GPI) m m-
m m m , m m C -

C . T . m m m m k

■ T m C C m x (MAC).
. CD [m m (MCP)]

■ P m k k C m m
m . C .

A m C - CD GPI- m m
. F m , m m m k C C m

m m MAC.
m m k m x (MAC) q - F I m m . I

m . , C .
T m m m m F H

C m . A m m - . I C -
m m . I m I.

. S m - m m I–m C
m m - .

m m; m
f x , C C .

Activation of Complement m C C . T

q m —C , C , C , C , C ,
N m , m m m - C , C , C , C — x m

m. C m m .

m m - m - C m q ; x -

m . C m m -m m C m j q m -

m paroxysmal nocturnal hemoglobinuria m .

PNH m m m ( HUS) T m j :

m /

m m m . R

m m . Am m m

m m . S m m . M m k m x (MAC)

( B x . ) k m-

m . T m m k m x (MAC) q m

A m m m x m m m

m m . W m

C , x m m m . , C , C , C , C m MAC.

B C m m T C C C C m x m z C m

MAC m m m ( ), m m

. k ,

A m m , z m - x . B m x C , osmotic

m m m cytolytic reaction . T -

m x. T C

z m . B z m j m m m . A

m z m , m C m m f

; , m m m . x m
m m . I , m

, . T q
Classic Pathway
x m (N )
+
T m m m j H O m ,

m m -m mm . T .