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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

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Text of 免疫不全・免疫増殖性症候群 · PDF file7 重症免疫不全症候群 •...

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    595102

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    1TB

    2

    3

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    BpreBBBB

    XLAXq23.1-Xq22agammaglobulinemia tyrosine kinaseatkXLAsrc Btk

    X Bruton

    Bruton Brutons tyrosine kinase (Btk)

    Btk BBBBB

    CD19/CD20B

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    3-23

    IgM

    IgMXLHM, XHIMCD40

    CD40BBT

    CD40gp39q26T

    XLHMgp39CD40

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    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

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    SCIDSCIDADA adenosin deaminaseHLAclassTIL2

    ADAATP

    ADATSCID

    ADA121990

    SCID

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    ADA

    T,B ADA ADADNADNA

    SCID

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    X

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

    TIL-7 NKIL-15 BB

    T-B+SCID

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

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    CGD

    NADPHCGD

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    LADLADLFA-1Mac-1P150.95CD18

    CD18

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    LAD

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    AIDS

    HTLV AIDS

    T

    EB

    B

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    ICAM-1

    AA

    N

    BCR2EB

    TCD4HIV

    IFNTNF

    IFN

    IFNRTNFRIL-10

    EB

    MHC

    MHC

    E3

    MHC

    Fc

    gE/gl

    EBERSRNA)eIF-2

    EB

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    HPV1618

    DNA BEB

    HBV

    HCV

    ATLTHTLV-1HTLV-2

    RNA

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

    HIVlentivirusHIVCD4Tgp120TCD4HIVT

    TCD4CD4CD8TNKLAK

    KaposiHIVHIVHIV.

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    AIDS

    AZT3azido3deoxythymidineHIVHIVT

    HTLV-1,2 TTATL)C

    TC

    HTLV-1HTLV I associated myelopathy(HAM)

    HTLV-1ATLHAM2

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    ATL,HAM

    70100.

    EB

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    55

    .

    opportunistic infectionazathioprineCyclosporin

    CFS5061988

    NKNKCFIDS

    NKIL2IL2IL2

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    TB

    CD512

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    TCRIg51356

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    APL90t1517q22q122117q2115q22PMLMYL2DNA

    BALL30t119q23P13319p133E2A1q23PRLE2ADNA

    T7q3514qll7q35TCR14qllTCRTCR

    CMLt922q34qllPhl

    TATLHTLV-1ATLATLIL2p40taxNFKBIL2R

    1EBEBVCD21BEBVMYC

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