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第二十 六 章 免疫缺陷性疾病及其免疫检验 PowerPoint PPT Presentation


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第二十 六 章 免疫缺陷性疾病及其免疫检验. 第一节免疫缺陷病的分类和特点. 一、免疫缺陷病的分类 二、免疫缺陷病的特点 三、常见发病原因. Immunodeficiency Disease. 机体免疫系统由于先天性发育不全或后天获得性损伤 导致机体的免疫功能降低或缺乏 临床表现为反复迁移性或机会性感染,自身免疫现象,某些肿瘤的发生率增高. 一、免疫缺陷病分类. 两大类. 原发性免疫缺陷性疾病 ( primary immunodeficiency disease, PIDD ) 继发性免疫缺陷病

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第二十 六 章 免疫缺陷性疾病及其免疫检验

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Immunodeficiency Disease


primary immunodeficiency disease, PIDD

secondary immunodeficiency disease, SIDD





B

T


primary immunodeficiency disease, PIDD

congenital immunodeficiency disease, CIDD


B

primary B lymphocytes deficiency

BBT


Ig

B

T


XX-linked agamaglobulinemiaXLA

XBruton

BBrutons tyrosine kinaseBtkBBBB

6,IgB


XIgMX-linked high IgM syndromeXLHM

X-CD40LTCD40LBCD40BIgIgM

IgMIgGIgAIgEIgM


IgAselective IgA deficiency

IgABIgA

IgA<50mg/LsIgAIg


Tprimary T lymphocytes deficiency

TT

TTTCL-B


DiGeorgeT

TTB

24


TCR

TT

  • CD3----TCR-CD3

  • ZAP-70ZAP-70


severe combined immunodeficiency diseaseSCID

Ig


T

BIg

IL-2


20ADAADAdATPdGTPTBRNADNATB


primary phagocytes deficiency


granulocytopeniaagranulocytosis

G-CSF

1


leukocyte adhesion deficiencyLAD

LAD-1LAD-2

LAD-1CD-18TNK

LAD-2Sialyl-LewisxSlex

LAD


chronic granulomatous diseaseCGD

NADPH


primary complement system deficiency

,


C1C1 inhibitor,C1INH


glycosyl phosphatidylinositol,GPIpig-GPI




secondary immunodeficiency disease, AID

T


1

2HIV

3

4

5

6


acquired immunodeficiency syndromeAIDS

human immunodeficiency virusHIV

CD4+T



AIDS human immunodifiency virusHIV

HIVHIV-1HIV-2


HIV

HIV

HIV-1

HIV-2

Types

M

O

Groups

N

ROD NIH2

A, B, C, D,

E, F, G, H, I, J

ANT 70,

MVP5180,

VAU

Clades


HIV


HIV

1.CD4+T

HIVgp120CD4+ThCD4+/CD8+

HIVCD4+T,

gp120

HIVgp120CTLHIV


2.gp120CCRHIV

M

3. DC CD4, CCR

4.B HIVB


HIV

1CD4T

2Th1Th2

3

4BIg


HIV

HIVHIV


HIV

HIVHIV2-6


HIV

AIDS

AIDS

AIDSKaposiAIDS

60%AIDSAIDS





B

T


B

Ig

smIg

IgA


T

T

T

T


Boyden

NBT



HIV


CD4


EIA

WB


EIA

EIA


HIV


OraQuick test


HIV

1

2156030

3


4

5 HIV-1/230 12 2~8


WB


RNA

4-11RNA

NASBA,RT-PCR,b-DNA


22HIV, 2

1898.8%


CD8/PBMC


102~105106>50000

FDA


3

HIV

2EIA


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