BT AIDS

Immunodeficiency Disease ?

primary immunodeficiency disease,PIDD

secondary immunodeficiency disease,SIDD

TBB T BmX-IgMIgWiskott-Aldrich Chediak-HigashiX-DiGeorge

27-1

B T

primary immunodeficiency disease,PIDDcongential immunodeficiency disease, CIDD

B

primary B lymphocytes deficiencyBBT

IgBT

X X-linked agamaglobulinemiaXLA

XBrutonBBrutons tyrosine kinaseBtkBBBB6IgB

XIgMX-linked high IgM syndromeXLHM X-CD40LTCD40LBCD40BIgIgM IgMIgGIgAIgEIgM

IgA selective IgA deficiencyIgABIgA IgA

T

primary T lymphocytes deficiencyTTTTTCL-B

DiGeorgeTTTB24

TCR TT

CD3----TCR-CD3 ZAP-70ZAP-70

severe combined immunodeficiency diseaseSCIDIg

T BIg IL-2

20ADAADAdATPdGTPTBRNADNATB

primary phagocytes deficiency

granulocytopeniaagranulocytosisG-CSF1

leukocyte adhesion deficiencyLAD LAD-1LAD-2LAD-1CD-18TNKLAD-2Sialyl-LewisxSlexLAD

chronic granulomatous diseaseCGD NADPH

primary complement system deficiency,

C1C1 inhibitor,C1INH

C1-INH

secondary immunodeficiency diseaseT

1 2HIV34 56

acquired immunodeficiency syndromeAIDShuman immunodeficiency virusHIVCD4+T

AIDS human immunodeficiency virusHIV

HIVHIV-1HIV-2

HIV

HIV

1.CD4+T HIVgp120CD4+ThCD4+/CD8+HIVCD4+T,gp120HIVgp120CTLHIV

2.gp120CCRHIV M 3.DC CD4, CCR 4.B HIVB

HIV1CD4T2Th1Th234BIg

HIV

HIVHIV

HIVHIV26HIV

AIDSAIDSAIDSKaposioAIDS60%AIDSAIDSHIV

B T

B 1. 2.IgA 3. 4. 5.IgA 6. 7.B 8.

T1.2.T3.E4.T12T

1.2.3.4.NBT5.

CH50 C3C4C1qB C1

HIV

CD4

EIAWB

OraQuick test

WB

4-11RNA

RNA

102-105106>50000 FDA

CD8/PBMC

HIV2EIA

CD4+ CD4+HIV