Page 287 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )
P. 287
CHAPTER 14 ■ Disorders of Iron Metabolism and Heme Synthesis 271
k HLA-A m m T HH m
. E m SF /mL.
m z SF
k m . ,
T HLA-A m . A SF
HFE. mm m HFE , C282Y , x ,
H63D, HH. I m , x k . SF -
. ( - ) -
T m m HH.
. G
M m q . . T m S SF
A HFE m - .
m m A m m
k m m ,
m - , m m z k m ,
IDA. ,
. Hemochromatosis ype 2: Juvenile hemochromatosis. . F m , z
x . , m
m HJV , . T , m
m m q . m - m m
z m m - S . I -
, m m q . %
H m j x , k ,
m , . M m m j HH %. A
m . m .
HAMP . M
m j j m - Treatm ent
m z m m
. N . R m ( mL)
m k j m m . k
. Hemochromatosis ype 3: Mutation o rans errin receptor m - m m HH -
2 ( R2) gene. T m m m m .
m . T m E x m m . I
m FR , - m ,
, m m m q . I m .
.
. Hemochromatosis ype 4: Mutations o Ferroportin-1 DISORDERS RELA ED O HEMOGLOBIN
gene. I , BIOSYN HESIS: HEME (PORPHYRIN)
SLC40A1 . SYN HESIS
A m m
m . T m D (F . . )
m m m m m q . I
m m , -
m MPS. T m .
k k HLA . A q ,
m . I ,
Pathophysiology and Laboratory Characteristics z m , m , m -
HH m x , ALA PBG
. T m - m . N m
m . A m , q ALA
m m - m x m m
x m .
, , x P m m m
m m m. m m
A - x m x
m HH m / L. / .

