Transcript 파일명 : 14-10-02_혈액학_특강

Erythrocyte
전적모구(풋정상적모구)
(Rubriblast, Pronormoblast, Proerythroblast)
호염성적모구
(Prorubricyte, Basophilic normoblast, Basophilic erythroblast)
다염성적모구
(Rubricyte, Polychromatic normoblast, Polychromatic erythroblast)
정염성적모구
(Metarubricyte, Orthochromatic normoblast, Orthochromatic erythroblast)
다염적혈구(뭇색적혈구)
(Polychromatic erythrocyte)
Wright stain
그물적혈구(망상적혈구)
(Reticulocyte)
New methylene blue Stain
Brilliant cresyl blue Stain
Erythropoietic activity
Hemolytic anemia
Pluripotential
stem cell
대소부동증 (Anisocytosis )
RDW: 11.5 – 14.5%
Price-Jones Curve
l
20
건강인 평균
철결핍성빈혈
악성빈혈
15
l
10
l
5
l
%
Um
ㅣ
5.5
ㅣ
6.0
ㅣ
6.5
ㅣ
7.0
ㅣ
7.5
ㅣ
8.0
ㅣ ㅣ
8.5 9.0
ㅣ
ㅣ
ㅣ
ㅣ ㅣ
9.5 10.0 10.5 11.0 11.5
Target cell(codocyte, leptocyte)
Thalassemia Major
Spherocyte ; Spectrin 감소
Osmotic fragility 증
가
Hemolytic anemia
Osmotic fragility test
Hereditary
Spherocytosis
Acantocyte
liver chirosis
Echinocyte(Burr cell)
Uremia, gastric ulcer
Sickle cell(Drepanocyte)
Hemolytic anemia
HbS
Tear drop cell(Dacrocyte)
fibrosis
Stomatocyte
Howell-Jolly body
; DNA
splenectomy, hemolytic anemia
Super vital stain
Heinz body;
abnormal globin
G6PD deficiency anemia
Basophillic stippling
lead poisoning
; ribosome
Cabot ring ; 방추사 잔존물
Pappenheimer body
; Hemosiderin
Ringed sideroblastic anemia
Prussian blue stain
Ringed sideroblastic anemia
Schuffner’s granules
Malaria infection
Rouleaux formation(연전형성)
Plasma protein(fibrinogen과globulin)의 변성
Multiple myeloma
Blood smear시 너무 오랜 시간방치 후 밀었을 때 Artifact
ESR ?
Heme
Synthesis
Succinyl CoA
+ activated
Glycine
7)
Heme
(Heme synthetase or Ferrochelatase)
1) -aminolevulinic acid
feedback
inhibition
(ALA synthetase)
Fe++
6) Protoporphyrin IX
(Protogen oxidase)
3H2
5) Protoporphyrinogen IX
Mitochondria
(Coprogen oxidase)
O2
2H2O
4CO2
4NH3
2) Porphobilinogen
(2ALA : ALA dehydrase)
3) Uroporphyrinogen III
(4PBG : Urogen III synthetase)
Uroporphyrinogen I
Cytoplasm
4) Coproporphyrinogen III
(Urogen decarboxylase)
Coproporphyrinogen I
Globin
Globin Heme 합성
7) Heme
(Heme synthetase or
Ferrochelatase)
Succinyl CoA
+ activated
Glycine
1) -aminolevulinic
acid (ALA synthetase)
feedback
inhibition
6)
IX
Fe+
+
Protoporphyrin
(Protogen oxidase)
3H2
5) Protoporphyrinogen IX
(Coprogen oxidase)
Mitochondrion
O2
2H2O
4CO2
4NH3
2)
Porphobilinogen
(2ALA : ALA dehydrase)
Cytoplasm
3) Uroporphyrinogen
III
(4PBG :
4) Coproporphyrinogen III
(Urogen decarboxylase)
Urogen III synthetase)
Uroporphyrinogen
I
Coproporphyrinogen I
이상혈색소증 (Hemoglobinopathy)
1. Hb S = 226Val
2. Hb C = 226Lys
3. Hb D = 22121Gln
4. Hb E = 2226Lys
5. Hb H = 4
6. Hb Bart = γ4
HbO2 친화도(%)
100
O2 친화성 
온도
80
pH 
23DPG 
60
myoglobin
hemoglobin
O2 친화성 
온도
pH 
23DPG 
40
정맥혈
동맥혈
20
“p50선”
0
20
40
60
80
100
PO2(mmHg)
정상인의 혈색소-산소해리곡선
Glucose metabolism
Emden-Meyerhof pathway
(Anaerobic glycolysis: 90%)
Hexose monophosphate
pathway(Aerobic: 10%)
Glucose
Anti-oxidant activity
2ATP
2H2O
2GSH
GSSG + H+
2ADP
Methemoglobin
reductase pathway
Met.Hb(Fe+++)
H2O2
NAD
NADPH
NADP
G6PD
NADH
4ADP
Hb(Fe++)
Luebering-Rapoport
pathway
4ATP
2,3-DPG production
Lactate
1. Embden-Meyerhof Pathway
2. Hexose-monophoshate Pathway
.....
G6PD
3. Methemoglobin Reductase Pathway
.....
4.
Fe
Leubering-Rapaport Pathway
.....
2,3-DPG
IDA
RBC index(MCV, MCH, MCHC ???)
Microcytic
hypochromic
Megaloblastic anemia
Schilling
Macrocytic
normochromic
test
RBC
Hemolysis
Extravascular
hemolysis
Intravascular
hemolysis
plasma Hb
+ Haptoglobin(Hp)
Hb-Hp
Free Hb
Macrophage
Fe
Hb
Globin
Bilirubin
Unconjugated
bilirubin
(Plasma)
Met-Hb
Liver
Conjugated
bilirubin
Globin
Ferriheme
+ Hemopexin(Hpx)
Heme-Hpx
+Albumin
Hemosiderinuria
Hemoglobinuria
Urobilinogen
Methemalbumin
Urine
Stool
Hemolytic anemia..
적혈구 수명 감소:
51Cr
혈장: unconjugated bil↑
*
*
*
*
혈액 :
reticulocytosis
macrocytosis
erythroblastosis
LDH↑
haptoglobin↓
hemoglobinemia↑
골수 :
hemopexin↓
erythroid hyperplasia
methemalbuminemia
요: * hemosiderin(+)
* hemoglobin(+)
urobilinogen↑
Hemolytic anemia..
1) Congenital spherocytosis ; Osmotic fragility test
2) Hemoglobinopathy
; Hb Electrophoresis
Sickle cell anemia, Thalassemia..
3) G6DP deficiency anemia ; Heinz body test
4) Hemolytic disease of newborn
Erythroblastosis fetalis ; Coombs' test
(antiglobulin test)
5) Immunologic hemolysis
Transfusion reaction
; Donath-Landsteiner test
PCH
6) PNH ; Sucrose hemolysis test
Ham's test
Leukocyte
골수모구 (Myeloblast)
전골수구 (Promyelocyte)
간상헥구 (band
cell)
White cell maturation
골수구
(myelocyte)
후골수구
(metamyelocyte)
WBC maturation
림프구 (lymphocyte)
T cell
•
•
•
•
•
•
Thymus
Cellular immunity
Lymphokines producing
Long life span
CD4, CD8
Sheep cell rosette
B cell
•
•
•
•
•
Bursa of Fabricius
Humaral immunity
Antibody producing
Short life span
CD19, CD20
단구 (monocyte)
Toxic granule
Döhle body
pelger-Huet anomaly
Faggot cell
Auer body
;
;M3
Drum stick
LE factor
LE cell
Le body (hematoxylin body)
cf. Tart cell
Hairy cell
Plasma cell (plasmocyte)
Plasma cell
Russell body
Reactive lymphocyte(atypical lymphocyte)
M0
M4
M1
M5
AML
M6
M2
M3
M7
M0 ; Acute myeloblastic leukemia
No maturation
M1 ; Acute myeloblastic leukemia
Without maturation
M2 ; Acute myeloblastic leukemia
With maturation
M3 ; Acute promyelocytic leukemia
Faggot cell
DIC
t(15;17)
PML-RaRa
rearrangement
Peroxidase(POD)
- primary granule에서 발견되는 효소
- AML과 ALL 감별
Basic Principle
SH2 + H2O2
Peroxidase
S + 2H2O
Sudan Black B stain
- 지질을 함유하고 있는 과립구계에 흑갈색의 양성과립
- AML과 ALL 감별
- Peroxidase와 유사