Page 311 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 16 ■ Hemolytic Anemias 295

Alternative Pathway

BOX 16.2
T m

q . H , ,
m - Examples of Inherited Hemolytic Anemias

, m m m -

– - . STRUCTURAL MEMBRANE DEFECTS
A

H
Mannose-Binding Lectin Pathway H

M - m m m m- H m

, ( ), H x

m C q. M - , R

m mm m, ERYTHROCYTIC ENZYME DEFECTS

m m G PD

. G

H x k
Hem olytic Anem ia: Mem brane Disruption P k

H m -

m m . T m m- DEFECTS OF THE HEMOGLOBIN MOLECULE

H C
H S-C

■ I m ( m H S-S ( k m )

m ) T m

■ A q m

( x m m )

F

m m. T m x

x m m

, , - . T m

( . ). q

m

Inherited Hemolytic Anemia . F x m m ,

Etiology m m k
. C m m m
I m m m m- m

, z m , m - . C – - m

m . B x .

x m m m . S m m m (

F . . B) m m -
Structural Mem brane Defects m . S , m

P m m m k , , , . . R

m x m , . T m m m j m m

TABLE 16.1 Comparison of Intravascular and Extravascular Hemolysis

Intravascular Extravascular

Site of destruction of Within blood vessels Spleen or liver

erythrocytes

Mechanism Activation of complement IgM or IgG Cell-mediated phagocytosis of IgM- or IgG-coated cells

Laboratory ndings Hemoglobinuria direct antiglobulin test Positive direct antiglobulin test erythrocytes

Hemosiderinuria