0I L0kh 6k0 Tk0 Mk0

Fhkh I
0I L0kh 6k0 Tk0 h0h 6k0
Z
80 8Ihh Mk0
Te bao goc tao mau (hemooietic stem cell)
La te bao goc van nang (luriotential stem cell)
So luJng te bao on dinh vao khoang 1,7x1O
7
Te bao goc duJc duy tri J muc do vua hai
hang ngay mot so luJng nho (khoang O,8%) ra mau ngoai vi
trong vai hut roi trJ ve tuy
8
80 8Ihh Mk0 (Z)
Te bao tien than dong tuy la te bao da nang (CFu0EMM:
colonyorming unit) biet hoa thanh cac te bao dJn nang
Tien than dong hong cau (CFuE)
Tien than dong hat (CFu0)
Tien than dong tieu cau (CFuMeg)
Tien than dong dJn nhan (CFuM)
Te bao tien than dong lymho (CFuL)
4
80 8Ihh Mk0 (8)
CFu0EMM CFuL
6
0h h0h 6k0
hong cau mang hemoglobin vJi nong do cao luu thong
trong toan cJ the de mang oxy duJc nhan tu hoi den mo
hong cau hai di chuyen 8OO dam, qua tim 5x1O
5
lan
hong cau hai ben vung va hb khong bi bien tinh
hinh dia lom, d= 78, qua duJc nJi he den 1
hC chuyen hoa theo 2 duJng:
6
h00h 06 h0h 6k0
Erythroblast
hong cau con nhan
hC luJi
hC luJi (reticulocyte) 1% so luJng hC con luJi RhA, mat luJi sau
24 48 giJ
hhuom bang blue cresyl brillant
ThJi gian truJng thanh 7 ngay
7
Mkh h0h 6k0
hai lJ hosholiid gom hoshatidyl(ethanolamine, inositol,
choline, serine ...)
Sialorotein cua nhom mau gan vJi hoshatidylinositol
glycan anchor (F0A)
Bien di (acquired somatic mutation) tu te bao goc dong hong
cau thieu F0A gay tieu hb ve dem
Sectrin, actin, rotein 4.1, ankyrin tao thanh bo xuJng cua
hong cau
Sectrin gom 2 chuoi va xoan vao nhau dia lom
8
Mkh h0h 6k0
9
6h0YEh h0k 60k h0h 6k0
Embdan MayarhoII. gIyroIyIIr paIhWay
Tao hA0F: methemoglobin redutase (giu Fe J dang Fe
++
)
Men yruvate kinase
FanIosa phosphaIa paIhWay
Tao hA0Fh: chong goc tu do va cac chat oxy hoa.
0GF0 (glucoseGhoshate dehydrogenase)
Thieu 0GF0: huyet tan khi co chat oxy hoa
10
11
hEM0L08Ih
Bon chuoi globin gom 2 chuoi (hST 1G) va 2 chuoi khong
(hST 11)
hbA (2, 2)
hbA2 (2, 2) 8%
hbF (2, 2 )
Moi chuoi olyetid gan vao mot dJn vi heme boc quanh mot
han tu Fe
++
1Z
hEM0L08Ih
hama
hhan proIoporphyrIna
Fa
++
18
kIEM 80kT 80 8Ihh h0h 6k0
So luJng tuJng doi on dinh
So luJng hong cau cua tre con it hJn J nguJi lJn
So luJng hong cau J nam cao hJn nu
Thieu oxy la kich thich chinh cho su tao mau
Erythrooietine:OO% san xuat J than, 51O% J gan va
cac mo khac
Tang su biet hoa, tang toc do truJng thanh va dua hC
luJi vao mau
Androgene cung co the kich thich su sinh hong cau mot
cach gian tie
14
oI song hong rau
hapIogIobIn
16
8aI, VIIamIn 81Z va IoIIr arId
LuJng sat trong cJ the la 84 g
75% chua trong hemoglobin
0u tru J dang erritin (hemosiderin) J te bao gan va dai
thuc bao
van chuyen trong mau nhJ transerin (siderohilin)
hhu cau sat hang ngay la 1mg, nhu cau cua hu nu 2
8mg/ ngay, bao thai 84mg/ngay.
hhu cau tha vi duJc tai su dung
16
6h0YEh h0k 8kT
17
oI Ioan Iong hop hb khI IhIau saI
18
5,1Omethylene
tetrahydroalate
Tetrahydroolate
VIIamIn 8
1Z
5,1O methylene
tetrahydroolate
olyglutamate
0esoxyuridine
monohoshate
0esoxythymidine
monohoshate
0esoxythymidine
dihoshate
0esoxythymidine
trihoshate
hk
19
khh Ik 0h h0h 6k0
Bem hC/mm
8
; hC luJi; 0ung tich hC; hb.
M6V (the tich hC): (hct/hC) hC to, nho, hoac binh thuJng.
M6h (hb trong hC): (hb/hC) hC nhuJc sac hay dang sac.
M6h6 (hb trong hC): (hb/hct) it co cong dung trong xe
loai thieu mau.
W (RBC distribution width): (1115%) cho he danh gia
tinh dong nhat cua MCv
Fhet mau cho he biet cJ kho, mau sac, hinh thai cua hong
cau
Tuy do khi can thiet.
Z0
hong cau binh thuJng
Z1
ThIE0 Mk0
0iam so luJng hC hoac hb hoac ca hai duJi muc binh thuJng
Co nhieu cach han loai:
Fhan loai tren lam sang theo MCv va MCh
Fhan loai thieu mau theo cJ che benh sinh
ZZ
ThIE0 Mk0
Fhan loai tren lam sang theo M6V va M6h
Thieu mau hong cau nho, nhuJc sac (MCv78l)
Thieu mau hong cau binh thuJng, dang sac
Thieu mau hong cau to (MCv>1OOl)
Thieu mau hong cau nho la loai thieu mau thuJng ga do roi loan
chuyen hoa sat
Thieu sat
Thieu mau trong cac benh man tinh (roi loan su dung)
hoi chung thalassemia (thieu hut chuoi globin)
Thieu mau nguyen bao sat (sideroblastic anemia)
Z8
ThIE0 Mk0
Thieu mau hong cau to (macrocytic anemia) vJi MCv, MCh tang
Thuc hien het mau va dem reticulocyte neu:
Reticulocyte tang thi co the do huyet tan hoac mat mau.
Retyculocyte binh thuJng hoac giam, hai lam tuy do.
heu khong co nguyen hong cau khong lo (megaloblaste)
thi co the la benh gan do ruJu hoac suy gia hoac do loan
sinh tuy (myelodyslasia)
heu co nguyen hong cau khong lo co the la thieu vitamine
B12 hoac acid olic
Z4
ThIE0 Mk0
Fhan loai thieu mau theo cJ che benh sinh
Thieu mau do mat mau.
Thieu mau do hong cau bi huy hoai.
Thieu mau do giam san xuat vi thieu nguyen lieu hoac
bJi roi loan J tuy xuJng
Z6
MkT Mk0
MkT Mk0
6ap
0uJi 1O%: bu tru
8O4O%: shock (mat hC+ the tich)
Bang sac, dang bao
Can mau toan han
Man
Thieu hC, the tich binh thuJng
hhuJc sac, hC nho
Sat / huyet thanh giam
Chi can hC
Z6
Thieu mau do mat mau man
Thieu sat
hb giam hC nhuJc sac
Z7
h0YET Tkh
hb tu do / mau hb nieu (tieu huyet sac to)
Fe
++
/ huyet thanh tang
Bilirubin tu do tang
Tang chuyen hoa bilirubin> Stercobilinogen, urobilinogen
hC luJi tang cao
Thieu mau ro hJn vang da
Z8
Mang HC
Hb bat thng
Thieu enzyme
Thuoc
VK, virus
KST, oc to
Khang the
Chan thng
Z9
h0YET Tkh 0 h6 (1)
Mang h6:
hC hinh cau (Minkowski Chauard)
Frotein mang (sectrin) bt thng
Fhosholiid mang giam
ATFase (ri lon v cu truc va chc
nng)
Mang hC tng tinh thm vi hatri lam
cho hC hng len dng hinh cau
Chi co J Chau Au
0i truyen tinh tri,hST thng
80
hong rau hnh rau (sherocytosis)
81
h0YET Tkh 0 h6 (Z)
8anh huyaI sar Io (hemoglobinoathy)
khi co sai lech ve cau truc cua hb
Sai trinh tu amino acid
Khong tong hJ hoac thieu mot chuoi
hb8 (Sickle cell anemia)
Chuoi , vi tri G, valin thay glutamin
Khi thieu oxy hb J dang olymer hC bien dang, tac
mach, vJ.
8Z
88
hong cau hinh liem (sikcle cell anemia)
84
h0YET Tkh 0 h6 (8)
8anh ThaIassamIa: tuy chuoi bi khiem khuyet.
Thalassemie
Thieu hoac giam chuoi ,
Thay the bang chuoi , hbA (2,2) giam co hbF (2, 2)
The dong hJ tu hbF cao>GO% hC bia >8O4O%
The di hJ tu chuoi thieu it hbF4O% hC bia 1O%
Thalassemie
Thieu chuoi
Bac diem: thieu mau nhuJc sac, co hC bia.
86
86
87
h0YET Tkh 0 h6 (4)
ThIau antyma 0GF0
ThuJng ga
Lien quan hST X
Bi ton thuJng bJi chat co tinh oxy hoa
0oc tu do tu hagocyte
hb nieu
Binh luJng 0GF0
88
Benh tieu huyet sac to ve dem
(Faroxysmal nocturnal hemoglobinuria)
Thieu F0A gene thieu 0F (glycosyl hoshatityl
inositol J mang hC)
Thieu mot so rotein bam len 0F J mang (0F
linked rotein)
C055: decayaccelerating actor, C05O, C8 binding
rotein
C05O bat hoat in vivo C8 convertase
hong cau nhay cam vJi bo the khi h giam
89
40
huyaI Ian do nguyan nhan ngoaI h6 (1)
kT rhong h6.
KT tu nhien (gM): AB0 ngung ket, ly giai
KT do da ung M0 (g0) thuc bao J gan, lach
Rh
KhhC khac do sinh nhieu lan, truyen mau.
Thuoc: Methyl doa, FhC.
Ac tinh: leukemia(lymho), lymhoma
Benh collagen: Luus ban do rai rac
vo can (GO%)
Coombs test (+)
41
4Z
Coomb test
48
huyaI Ian do nguyan nhan ngoaI h6 (Z)
Th006.
0o tinh oxy hoa cua
thuoc
Thuoc la haten bam
tren be mat hC
KhKh gan tren thu
the CR1
Fha vJ su tu dung
na
44
h0YET Tkh 0 h0YEh hhkh h0kI h6 (8)
VI khuan
Clostridium erringens
lecithinase
Folysaccharide tu vK
bam len hC
ky sInh Irung
6haI dor: con trung
kIm IoaI nang: chi dong,
arsenic
6o hor: valve tim nhan tao
46
ThIE0 Mk0 0 IkM 8kh X0kT
6o quan Iao mau
Khong bi xam lan
Khong bi ton thuJng
Khong thieu erythrooietine
hguyan IIau da san xuaI.
Frotid,
Fe++, vit B12, olic acid,
BG, B2, vit C...
46
ThIE0 Mk0 0 ThIE0 8kT (1)
ThuJng ga (OO%)
Thieu mau nhuJc sac hong cau nho
MCv, MCh, MChC giam
Fe / hT, Ferritin,
Siderohylline, bao hoa siderohyllin1G%
47
ThIE0 Mk0 0 ThIE0 8kT (Z)
6h0YEh h0k 8kT
van chuyen nhJ transerrin (siderohylline)
Transerrin glycoroteine (gan), bao hoa 8O% thu the cua
erythroblast.
Fe con du tru bJi erritin (hemosiderin) trong bao tuJng cua
macrohage.
Khi viem nhiem sat bi giu bJi macrohage Fe / hT giam
Thieu mau nhuJc sac do viem
hct khong duJi 82%
48
ThIE0 Mk0 0 ThIE0 8kT (8)
h0YEh hhkh
Mat mau (OO%)
Cung ca thieu (sJ sinh, co thai)
Roi loan han hoi: viem, ung thu (sat bi macrohage,
to chuc u thu giu)
Chlorose (green sickness)
Thieu mau hC nho khong acid chlorhydric/da day
49
ThIE0 Mk0 0 ThIE0 8kT (4)
ThIau mau nguyan bao saI (sideroblastic anemia)
0o roi loan su dung sat
hhiem sac the X, nhiem sac the thuJng
Thu hat do dung thuoc lao (hh, FAS, Cycloserine)
Chloramhenicol, thuoc chong ung thu (chong olic va nhom
alkyl), ruJu, chi, thieu BG..., vo can.
Thieu mau hong cau nho nhuJc sac, sat / hT, erritin tang
Sideroblast la cac nguyen hC vJi nhung hat sat quanh nhan
60
hguyen bao sat (sideroblaste)
61
ThIE0 VIT 81Z & F0LI6 k6I
vit B12 va olic can de tong hJ A0h
Thieu roi loan tang sinh va biet hoa
hhieu loai te bao bi anh huJng:
hC bi chet J cuoi giai doan truJng thanh
hC to (MCv>1O5l) de vJ, co nguyen hC khong lo (megaloblast)
BC gia co nhieu mui
0iam TC
0iam sinh san tb mo bi mo sinh duc
6Z
hong cau to
hguyen hC khong lo
Bach cau nhieu mui
68
ThIE0 VIT 81Z & F0LI6 k6I (Z)
Thieu olic thuJng thay hJn B12
Cung ca (an chay)
ha thu
Cat da day, hoi trang
San de 0ihyllobotrium latum
Thieu 0MF (gastromucorotein)
Tu KT chong 0MF
Teo niem mac bam sinh (gastric atrohy)
KT chong te bao thanh da day
64
6o rha IhIau mau do IhIau 81Z
66
IkM 8kh X0kT h6 0 T0Y X00h
Cot hoa, xJ tuy
Xam lan
hgo doc:thuoc, ben/en, chi, tia xa
Thieu erythrooietine
Khong ro nguyen nhan (OO%)
66
IkM 8kh X0kT h6 0 T0Y X00h (Z)
Ton thuJng te bao goc
Ton thuJng te bao chat dem (vi moi truJng)
Mien dich: do da ung M0, tu mien.
Tb lymho da hoat tac
Fh va ThF
uc che su tang truJng te bao goc (C084
+
)
Chet te bao goc
Te bao chat dem bi uc che bJi Fh
67
68
IkM 8kh X0kT h6 0 T0Y X00h (8)
h0uYEh LY BEu TR
Thieu hut te bao goc ghe tuy
uc che su ha huy te bao goc
AL0 (Anti Lymhocyte 0lobulin)
AT0 (Anti Thymocyte 0lobulin)
Cyclosorin
Corticoid
Kich hoat su sinh te bao
0CSF, 0MCSF
69
k h0h 6k0
Ba hC tuJng doi
Ba hC tuyet doi
Ba hC nguyen hat
Ba hC thu hat

hC>GT
hb>1Gg/dl
ham: hct>54%, hu: >47%
60
k h0h 6k0 (Z)
Ba hC nguyen hat (Benh vaque/: Folycythemia vera)
0o su tang sinh mot clone te bao goc
hC Erythrooietine
hC Tang do quanh cua mau
Co tang BC, TC kem theo
ham, trung nien, 5O tuoi la luc co xJ vua dong mach
Cao hA, cJn dau that nguc, TBMMh, dau khJ (uric acid)
61
k h0h 6k0 (8)
Ba hC thu hat: secondary olycythemia
Thieu oxy Erythrooietine hC
hJi cao
Benh tim , shunt
Benh hoi man
Thong khi giam do TK: viem nao, TBMMh
0o ton thuJng TK: u nao, benh Cushing
0o cac nguyen nhan khac: u than, K gan, u xJ TC, hc than
hu, loet da day ta trang, lao lach.