ηωσινοφιλία 14 δεκεμβρίου 2011-νέο

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  • 14 2011 1

  • 2

  • ~13

    450/l

    ( )

    3

  • 1-2 %

    >90 %

    4

  • ;

    eotaxin-1 CCR3

    ,

    5

  • ;

    ,

    ( tissue remodeling)

    MHC class I- ,

    : ,

    6

  • Illustration of a Model of Eosinophils as Antigen Presenting Cells. Eosinophils can be stimulated to express MHC Class II and co-stimulatory molecules by granulocyte-macrophage colony stimulating factor (GM-CSF) under experimental conditions. Eosinophils achieve the same expression of antigen presentation machinery in vivo in various disease states including asthma, chronic eosinophilic pneumonia, and parasitic infection. They then traffick to regional lymph nodes where they encounter CD4+ T cells, promoting proliferation and cytokine production (IL-4, IL-5, IL-13) by T cells.

    7

  • llustration of a Model of Eosinophil Immunoregulation on CD4+ T cells. Eosinophils are capable of producing IL-12 and IL-4, which polarize T cells to Th1 cells and Th2 cells, respectively. Eosinophils express both Th1- and Th2-associated cytokines and chemokines.IL-25 is secreted by eosinophils and promotes Th2 activity. IL-4 has an amplifying effect on the Th2 response and promotes further eosinophil recruitment. IL-5 is a potent autocrine and paracrine growth and survival cytokine for eosinophils.

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    Ig, , 2

    /

    ,

    ,

    ,

    , Ig

    ;

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    Major basic protein(MBP1) Eosinophil cationic protein(ECP) Eosinophil-derived neurotoxin(EDN) Eosinophil peroxidase

    2

    -

    2 2 Hydroxyl radicals

    Collagenase 92 kDa Gelatinase (MMP-9)

    Leukotriene C4/D4 Platelet activating factor 5-HETE 5,15-and 8,15-diHETE Prostaglandine E1 ,E2 Thromboxane B2

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    &

    IL-1 IL-2 IL-3 IL-4 IL-5 IL-6 IL-8 IL-10 IL-11

    IL-12 IL-13 IL-16 RANTES EOTAXIN MIP-1a

    GM-CSF TNF-a SCF

    TGF-a TGF-b1 PDGF NGF BDGF NT-3

  • ,

    : >450/..

    >1500/..

    15

    : 450-1500/ :1500-5000/ : >5000/

  • 16

    H:

    /

    (Churg- Strauss vasculitis, ,

    Loeffler HIV....

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  • 18

    E

    DRESS syndrome . -

    , , GM-CSF ,IL-2 , ,NSAIDs , , , , NSAIDs,.. L-tryptophan contaminant

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    : ,

    :,

    : HIV

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    Angiostrongyliasis Echinostomiasis Metagoniamiasis

    Anisakiasis Enterobiasis Opisthorciasis

    Ascariasis* Fascioliasis* Paragonomiasis*

    Capillariasis Fasciolopsiasis* Schistosomiasis*

    Clonorchiasis* Filariasis Sparganosis

    Coenurosis Gnathostomiasis Strongyloidiasis*

    Cysticercosis Heterophyiasis Trichinosis*

    Dicrocoeliasis Hookworm* Trichuriasis

    Echinococcosis Hymenolepsiasis VLM

    (>3000/l), *

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    Myiasis

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    Cysticercosis

    Echinococcosis

    Filariasis loiasis,

    onchocerciasis,

    mansonellosis,

    TPE

    Gnathostomiasis

    Flukes schisto, fascioliasis, clonochiasis, paragonimiasis

    Hookworm Strongyloidiasis

  • Ascaris Haiti Paraguay

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    Guinea Worm

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    (HES)

    K 1975:

    1. >1500/ > 6

    2.

    3. /

    1. >1500/ 2

    2. , , , ,

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    FIG 1. Revised classification of HESs. Changes from the previous classification3 are indicated in red.

    Dashed arrows identify HES forms for which at least some patients have T celldriven disease. Classification of myeloproliferative forms has been simplified, and patients with HES and eosinophil

    hematopoietinproducing T cells in the absence of a T-cell clone are included in the lymphocytic forms of HES. IBD:Inflammatory bowel disease CSS:Churge Strauss Syndrome, CEL:Chronic Eosinophilic Leukemia, FIP1L1:Fip1-Like-1, PDGFRA: Platelet derived growth factor receptor a

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    myeloproliferative hypereosinophilic syndrome

    FIP1L1-PDGFRA fusion (+) 10%20%

    vitamin B12

    tryptase

    /

    mast cells

    imatinib

    lymphocytic hypereosinophilic syndrome

    FIP1L1-PDGFRA fusion (-)

    immunoglobulin E

    interleukin (IL)-5 and/or IL-4

    TARC (thymus and activation-regulated chemokine)

    T-cell

    1/4 - 1/3 HES

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    FIP1L1/PDGFRA-(+)

    4

    , 20-40 50% 5

    tyrosine kinase inhibitor, imatinib mesylate

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    5q31-33

    ,

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    >1,500/mm3

    , , Churg-Strauss

    undefined

    Overlap HES

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    >1,500/mm3

    . , , ALPS(Autoimmune Lymphoproliferative

    Syndrome)

    Associated HES

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    >1500/mm3

    Undefined

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    > 50%

    ;

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    =105

    100%

    58%

    56%

    54%

    49%

    43%

    30%

    23%

    23%

    3 HES

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    HES

    TGF-b,IL-4, IL-6, TNF-a, ECP, MBP

  • 35

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    Cytoplasmic clearing Dysplastic nucleus

    Lipid body

    Piecemeal degranulation

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    Prednisone

    Hydroxyurea

    Interferon- Imatinib mesylate

    Mepolizumab ( anti-IL-5)

    cyclosporin A, 2-CDA, vincristine, chlorambucil, ,

    stem cells,

    HES

  • HES

    38

    >1500/

    ,

    >100.000/

    :

    , , 12

    FIP1L1/PDGFRA

    , CT /, CHO

    +invermectin

    Strongyloides

    HES

    +

    -

    +

    -

  • HES

    39

    >1500/

    ;

    HES :

    , , 12

    FIP1L1/PDGFRA

    , CT //, CHO

    ,

    ,

    - +

    ;

    - +

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    ;

    . !

    , , .

    , EPO, ECP...

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    Strongyloides . . ivermectin 3 ( .. Toxocara)

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    . !!!

    -

    Chung-Strauss

    ,

    ( L-tryptophan)

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    1. -;

    2.

    /;

    (; ;)

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