Hematopoietic Disorder
-
Upload
en-oh-o-end -
Category
Documents
-
view
220 -
download
0
Transcript of Hematopoietic Disorder
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 1/30
Hematopoietic Disorder
Iron‐defciency anemia
Vit B12 De & Folate De Anemia
anemia
G-6-PD Defciency
Thalaemia
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 2/30
• Defnition- A red!ction in n!m"er o #B$
% World Health Organization defnedanemia as
'ale ( )" * 1+ ,d. /03 mmol.4
Female ( )" * 12 ,d. /35 mmol.4
Anemia
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 3/30
Morphology
Macrocyti
c
Hypochromic
, microcytic
Normocytic
anemias Vit B12 de Folic acidde Dr!,‐
ind!ced
Iron de Genetic a"n 7ic8le cellanemia
Thalaemia
#ecent "lood lo% )emolyi% B' ail!re% A$D
% #enal ail!re% 9ndrocrine di% 'yelo:laticanemia
Etiology
Defciency Central Peripheral
IronVitamin B12 Folic acid Pyrido;ine
im:aired "onemarro< !nction -A$D -Anemia oelderly
-'ali,nant "onemarro< di
Bleedin,/hemorrha,e4)emolyi/hemolyticanemia4
Pathophysiology
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 4/30
Normal Hematologic Vales
!est "e#erence "ange $%&'() yr*
Hgb (g/dL) M 13.5 17.5; F 12.0 16.0‐ ‐
Hct (%) M 41.0 53.0; F 35.0 46.0‐ ‐
MCV (µm3) 80-100
MCHC (%) 31-37
MCH (g) 28-34
!"C (106/mm3) 4.5-5.#
!$ (%) 11-16
!&t'c*c+t& ',d& (%) 1-2.5
&m '*, (µg/dL) M 50 160; F 40 150‐ ‐
"C(µg/dL) 250-400
F&'t', (,g/mL) M 15 200; F 12 150‐ ‐
F*t& (,g/mL) 1.8-16
V't "12 (g/mL) 100-#00
+t**'&t', (/mL) 0.01-0.03
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 5/30
Clinical Presentations
Acte onset Chronic anemia
$ardiore:iratory
ym:tom
-Tachycardia
-.i,ht‐headedne
-Breathlene
Fati,!e= headache=
>erti,o= aintne
7eniti>e to cold= :allor.o o 8in tone
+oals% Alle>iate i,n and ym:tom% $orrect the !nderlyin, etiolo,y
% Pre>ent rec!rrent anemia
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 6/30
Iron‐defciency anemia
8oilonychia An,!lar tomatiti and,loiti
Treatment
1 $ontrol o the !nderlyin, ca!e2 Dietary !::lement+ Thera:e!tic iron :re:aration
% 2 m, Feday
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 7/30
Salt Elemental Iron (%)
F&* 9t& 20
F&* 9t&: &'cct& 30
F&* gc*,t& 12
F&* 9mt& 33
F&* +**t& 12
F&* cb*,t& 48
ron prodcts
Common AD"- ide e?ect GI
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 8/30
Parenteral ronprodctsIndication
Iron mala"or:tion Intolerance o oral iron thera:y @oncom:liance
Iron de;tran /I' or IV4 Tet doe o 2m, /I' or IV4 o>er ‐1 min in
dil!ted IV ol!tion 'onitor or 1 ho!r or ad>ere reaction
% Aller,ic reaction incl!din, ana:hyla;i
+oals ),B riin, 1‐2 ,d. :er <ee8 and contin!e
treatment at leat +‐6 month
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 9/30
Vit B12 De & Folate De anemia
Inacti
>e
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 10/30
$ardiore:iratory ym:tom
@e!ro:ychiatric a"normalitie /Delay & nlyB124
Paretheia Ata;ia- Vi!al dit!r"ance
Pernicio! anemia+oals
7i,n and ym:tom !!ally !"ide aterre:lace
Vit B12 or olate
Clinical manifestations
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 11/30
De#. Etiology !reatment
Vit B12 - หล ง intrinic actor
ลดลง
- IM
$yanoco"alamin orhydro;yco"alamin
- ral Vit B12 i
rarely indicated
Folate -ร บจากอาหารไม เพ ยงพอ- การรบกวนenterohe:aticcirc!lation เช นPhenytoin= oralcontrace:ti>e=
ioniaCid
-Oral olic acid m,D
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 13/30
emolytic Anemia
G6PD /Gl!coe‐6‐Pho:hate Dehydro,enae4
Defciency Genetic a"n X-link recessive(sex??) lac8 o ,l!coe‐6‐:ho:hate
dehydro,enae
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 14/30
/ymptoms o# hemolytic anemia
!reatment• หล กเล ยง o;idati>e treor
% Folic acid and iron• ให เล อด /#arely4
อาการมกจะเกดข นทนทหลงเปนโรคต ดเช ขอ หรชอ
หลงไดรบา - ไข!"ง หนา#!$น % &ดเหลชอง%อ'อนเ(ลมาก % ป!!า#ะ!คลาน) าปลา หรชอโคล'า-*า#ะโ(+ท!เ&ม,นเลชอด!"ง
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 15/30
Antimalarials: Prima!ine, $hloro!ine,
)ydro;ychloro!in
Antibiotics: Dapsone, Sulasalasine,!uinolone
Anal"esics: ASA
Miscellaneous: ThiaCide Di!retic= Vitamin
E= Penicillamine= Acor"ic /lar,e doe4=
Fa>a "ean
Drg indced Hemolytic anemia
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 16/30
Thalaemia
• Defnition- โรคโลห ตจางพ นธกรรมจากความผ ดกต !องการ"ร าง#
โมโกลบ น- "ร างน อยลง="ร าง# โมโกลบ นผ ดกต
- เม $ดเล อด%ดงม ล ก&'(ผ ดกต %ล(ม อาย" น/hemolytic anemia4
- .'าทอดทาง(น/กรรม+บบ
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 17/30
Pathophysiology
Fetal )"F /2H24
Ad!lt )"A /224 J0K
'inor )"A2 /2L24 2K
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 18/30
01!halassemia
!ype +enotype Phenotype'inor /4 or /M4 )" N 1
Aym:tomaticcarrier
'aOor (0/0): (0/<) * (</<) )" * 3
Intermediate (0/<) * (</<) )" 3-1
= < = = 0
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 19/30
21!halassemia
!ype +enotype Phenotype
ilent carrier /- 4 Aym:tomatic
minor /- -4 = /- -4 Aym:tomatic
)") dieae /- - - 4 'ild to mod 'icrocytichy:ochromic
)" Barthydro:
/- - - -4 ม กเ" ยช วตต ง%ต ในครร) *หร อ ตายคลอด
#(- -) t$al %, (- & ) t$al '
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 20/30
Classifcation o# !halassemia
/e3ere!halassemia
Moderatelyse3ere
!halassemia
Mild!halassemia
Homo. 4thal4 thal5H4
E4 thal5H4 E
H4 Hdisease4 thal5H4
E
tem cell tran:lantation
lo< tran!ion M ironchelationQ :lenectomy
hi,h tran!ion Q ironchelation
lo< tran!ion Q:lenectomy
*> t,9'*, (&m*+')
* ให เล อดร มา'มากเพ อร(ง บการ"ร างเม $ดเล อด Gold: keep Hb > 10 g /dl
hi,h tran!ion M ironchelation
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 21/30
Clinical manifestations
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 22/30
crease "6C destrction
Iron chelation
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 24/30
Pititary gland Im:aired,ro<th
Heart cardiac ail!re7i3er he:atic cirrhoiPancreas dia"ete mellit!+onadal inertility
!halassemia 8 ron o3erload
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 26/30
ว ดรมา'ธาตเหล $กในต บ%ล(ในห วใจ/T24
T2 * 2 m .V9F ลดลง
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 27/30
ndication
89 ไดรบเลชอดมา : 8;%<; ครง<9 =>?26 @>??A3AB : 8C;;; BDE67
+oal- ลดร มา'
@TBIให ได มาก+
"ด
-Deero;amine /DF4
-Deeri:rone /DFP4
-Deerairo; /DF74
!reatment-
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 28/30
Agent Ad Half life Clearance S/E
FGH %I4B39JB@25A4B K;%L;
6DEMDEN1O PNN9QA3 I
<; 6AB 2?AB1?OC@>R17%S>3AB4T13UO%H3434VARA3O%W?4X3U
?>31?N13A4B%Y4R17 5MAB?>1R3A4B
%=MAB 27R>?13A4B%Z>?5ABA1AB@>R3A4B
%H?17 3AN ^%_ %\>23?4 >BA1
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 29/30
Monitor DFO DFP DFS
P2NA46>3?O 1BN >O> 1BB2177O E
IdI X>>M7O E
PYe 64B3U7O `@A?53 ^%L 64BaC 3U>B>]>?O L64B3U5
E
PYe 64B3U7O E
=>?26 R?>13ABAB> 64B3U7O E
=>?26 @>??A3AB 7>]>7 f21?3>?7O E E E
7A]>? A?4B 1BB2177O E E E
7/26/2019 Hematopoietic Disorder
http://slidepdf.com/reader/full/hematopoietic-disorder 30/30
• !otal iron 4inding capacity $!6C*-ร มา'!องเหล $ก+ ต องการเพ อน ามาจ บก บ tranerrin
•!rans#errin ( โรต นในเล อด, - ง+ าหน า+ !น" งเหล $ก
• Reticulocyte index (%): อ ตราการ"ร างเม $ดเล อด%ดง
•!rans#errin satration $!/A!*- จ .านวน ต .า%หน งบน tranerrin + มเหล $กจ บอย/
•/erm #erritin- ร มา'เหล $ก"("ม!อง
ร างกาย