免疫不全・免疫増殖性症候群 · PDF file7 重症免疫不全症候群 • 重症免疫不全症候群（scid）は細胞性免疫と体液性 免疫の両方を欠く重症疾患である．scidには多くの

1

595102

2

3

1TB

2

3

4

BpreBBBB

XLAXq23.1-Xq22agammaglobulinemia tyrosine kinaseatkXLAsrc Btk

X Bruton

Bruton Brutons tyrosine kinase (Btk)

Btk BBBBB

CD19/CD20B

5

3-23

IgM

IgMXLHM, XHIMCD40

CD40BBT

CD40gp39q26T

XLHMgp39CD40

6

IgM IgG,IgAIgM B B CD40LXHIM)activation-induced cytidine deaminase (AID)non-XHIM)

TypeType CD40LT

B IL-12T

AIDRNA editing XHIM Non-XHIM XHIMT

T B

CD28 CD80

CD40L CD40

FAS-L FAS

TCR MHC-II

peptide

BCR

7

SCIDSCIDADA adenosin deaminaseHLAclassTIL2

ADAATP

ADATSCID

ADA121990

SCID

8

ADA

T,B ADA ADADNADNA

SCID

9

X

IL-2 IL-2IL-4IL-7IL-9IL-15

TIL-7 NKIL-15 BB

T-B+SCID

p84IL-2IL-4IL-7IL-9IL-15

10

CGD

NADPHCGD

11

12

LADLADLFA-1Mac-1P150.95CD18

CD18

13

LAD

14

15

AIDS

HTLV AIDS

T

EB

B

16

ICAM-1

AA

N

BCR2EB

TCD4HIV

IFNTNF

IFN

IFNRTNFRIL-10

EB

MHC

MHC

E3

MHC

Fc

gE/gl

EBERSRNA)eIF-2

EB

17

HPV1618

DNA BEB

HBV

HCV

ATLTHTLV-1HTLV-2

RNA

AIDS AIDS1981 LAV 1ymphadenopathyassociatedvimsARVAIDSrelatedvimsHTLV-humanTcel1 1eukemia vimsHIVhuman immuno deficiency virus).

HIVlentivirusHIVCD4Tgp120TCD4HIVT

TCD4CD4CD8TNKLAK

KaposiHIVHIVHIV.

18

AIDS

AZT3azido3deoxythymidineHIVHIVT

HTLV-1,2 TTATL)C

TC

HTLV-1HTLV I associated myelopathy(HAM)

HTLV-1ATLHAM2

19

ATL,HAM

70100.

EB

20

55

.

opportunistic infectionazathioprineCyclosporin

CFS5061988

NKNKCFIDS

NKIL2IL2IL2

21

22

TB

CD512

23

24

TCRIg51356

25

APL90t1517q22q122117q2115q22PMLMYL2DNA

BALL30t119q23P13319p133E2A1q23PRLE2ADNA

T7q3514qll7q35TCR14qllTCRTCR

CMLt922q34qllPhl

TATLHTLV-1ATLATLIL2p40taxNFKBIL2R

1EBEBVCD21BEBVMYC

26