Page 143 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 6 ■ Erythrocytes: Hemoglobin 127

DISORDERS RELA ED O HEMOGLOBIN

BIOSYN HESIS

Disorders of Heme (Porphyrin) Synthesis

D (F . . )

m m m q . I

m , -

.

A q ,

m . I ,

z m , m , m -

, ALA PBG

m . N m

m ( C ),

q ALA m x m FIGURE 6.10 I m . B m

m . m (N m green granules -

P m m m m). (R m M C KD. Clinical

m m Laboratory Medicine, , P , PA: L W m

x m x & W k , , m .)

/ .

P :

- -
■ C ( . ) . PBG m x m m

■ S z m ; , m

■ S z m m m , m

E ’ . I

C , - PBG, m m

m k m . S m m .

m m . D m W m , m

: m , m x

m . T -

m .

P m G k , porphyra, , P (F . . ). T

m . T - m ( ) sideroblasts.

Glycine
δ-ALA
Porphobilinogen (PBG)
Synthase
Succinyl CoA (4-PBG)
δ-ALA

Hydroxymethylbilane
Heme Heme proteins

Fe 2+

Protoporphyrin IX Uroporphyrinogen III Uroporphyrinogen I Uroporphyrin

(excretion)

Protoporphyrinogen IX Coproporphyrinogen III Coproporphyrinogen I Coproporphyrin
(excretion)

FIGURE 6.9 T m . I m z m x

δ- m k . (R

m M MW, . Green eld’s Surgery Scienti c Principles and Practice, , P , PA: L

W m & W k , , m .)