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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

Immunodeficiency Disease


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primary immunodeficiency disease, PIDD

secondary immunodeficiency disease, SIDD


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B

T


Primary immunodeficiency disease pidd

primary immunodeficiency disease, PIDD

congenital immunodeficiency disease, CIDD


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B

primary B lymphocytes deficiency

BBT


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Ig

B

T


X x linked agamaglobulinemia xla

XX-linked agamaglobulinemiaXLA

XBruton

BBrutons tyrosine kinaseBtkBBBB

6,IgB


X igm x linked high igm syndrome xlhm

XIgMX-linked high IgM syndromeXLHM

X-CD40LTCD40LBCD40BIgIgM

IgMIgGIgAIgEIgM


Iga selective iga deficiency

IgAselective IgA deficiency

IgABIgA

IgA<50mg/LsIgAIg


T primary t lymphocytes deficiency

Tprimary T lymphocytes deficiency

TT

TTTCL-B


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DiGeorgeT

TTB

24


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TCR

TT

  • CD3----TCR-CD3

  • ZAP-70ZAP-70


Severe combined immunodeficiency disease scid

severe combined immunodeficiency diseaseSCID

Ig


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T

BIg

IL-2


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20ADAADAdATPdGTPTBRNADNATB


Primary phagocytes deficiency

primary phagocytes deficiency


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granulocytopeniaagranulocytosis

G-CSF

1


Leukocyte adhesion deficiency lad

leukocyte adhesion deficiencyLAD

LAD-1LAD-2

LAD-1CD-18TNK

LAD-2Sialyl-LewisxSlex

LAD


Chronic granulomatous disease cgd

chronic granulomatous diseaseCGD

NADPH


Primary complement system deficiency

primary complement system deficiency

,


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C1C1 inhibitor,C1INH


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glycosyl phosphatidylinositol,GPIpig-GPI


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Secondary immunodeficiency disease aid

secondary immunodeficiency disease, AID

T


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1

2HIV

3

4

5

6


Acquired immunodeficiency syndrome aids

acquired immunodeficiency syndromeAIDS

human immunodeficiency virusHIV

CD4+T


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AIDS human immunodifiency virusHIV

HIVHIV-1HIV-2


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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


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HIV


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HIV

1.CD4+T

HIVgp120CD4+ThCD4+/CD8+

HIVCD4+T,

gp120

HIVgp120CTLHIV


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2.gp120CCRHIV

M

3. DC CD4, CCR

4.B HIVB


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HIV

1CD4T

2Th1Th2

3

4BIg


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HIV

HIVHIV


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HIV

HIVHIV2-6


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HIV

AIDS

AIDS

AIDSKaposiAIDS

60%AIDSAIDS


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B

T


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B

Ig

smIg

IgA


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T

T

T

T


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Boyden

NBT


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HIV


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CD4


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EIA

WB


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EIA

EIA


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HIV


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OraQuick test


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HIV

1

2156030

3


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4

5 HIV-1/230 12 2~8


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WB


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RNA

4-11RNA

NASBA,RT-PCR,b-DNA


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22HIV, 2

1898.8%


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CD8/PBMC


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102~105106>50000

FDA


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3

HIV

2EIA


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