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2017年检验技师考试临床血液学第八章复习笔记(2)


2017 2


(hereditary spherocytosis) (HA) 8 Ca2+ Mg2+-ATP

2/3 (50%) ()
(aplastic crisis)(parvovirus) 1014

HA () (10%)Coombs / / O.51%O.72% O.45%O.36% 37 24
AAAAAA

HA

10
-6-
-6-(()6PI)) G6PI) G6PD HA 2 (58.2%)(15.7%)(13.7%)

X (Xq28) G6PD
G6PD G6PD (NADPH)(GSH) (Heinz body) 12 m

()
AAAAAA

(75%) 30%
()(Heinz body)
37G6PD 5%
() 6 (G6PD)
23 G6PD

HA ( ) G6PD G6PD
()(favism)
3 70%
1. 35 40%
2.( 2 )( 12 15 ) () 7
3.G6PD 10%
AAAAAA

() HA
( K ) 13 () 7 20 G6PD G6PD HA K

HA

a 141 a ( ) 146 a 16 11 2 (4 ) A(HbA) 95% a (a2 2) A2(HbA2) a (a2 2) 2%3%(HbF) a (a2 2) 6 1%
(hemoglobinopathy) ()
AAAAAA



()
6 S(HbS) HbS () HA 34 HbS
()
120 a G6PI)
() M(HbM)
HbM 5 4 a 30% NADH( 1)
AAAAAA

()
HbA 46
()
HbE HA 25%75%
DNA PCR
()
(thalassemia) HA
()
a a a 14.9% 4.1l%
1. a 4 a 1 2 Hb Bart() 5%15% H
2. H(HbH) 4 a 3 H( 4) Hb Bart 25% Hb Bart HbH
AAAAAA

HbH HbH pH 8.6 8.8 HbA
3. Bart a 4 a a HbBart( 4) Bart Hb Bart 80%100%Hb Bart 3040
()
1.5
1/4 ()2/4 () 1/4 a a HbA2(a2 2) HbF(a 2 2)HbF
1. HbA2 3.5%(4% 8%)HbF ( 5%)
2. HbF 10%
3.(Cooley ) 60g/L 10%35% HbF 30%90%HbA 40% O%
AAAAAA

X
DNA (mRNA)()

110g/L130g/L (deferoxamine)


AAAAAA

""

AAAAAA



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