-

. .
-
, Wharton Jones
1846, Paul Erlich 1879,
.
10-17,
,
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o
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(EDN) - ()
, ,
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,
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( ),

. .

,
,
(IL-5, IL-3, IL-2, IL-13) (GM-CSF). IL-5
,
- .
3-5% ,
3-6 .
, .
30 .
,
L- 1-,
-1 (intracellular adhesion molecule 1, ICAM-1)
-1 (vascular adhesion molecule 1, VCAM-1).

, .
,
.
. L-2,
(LCF), ,
C5a , (PAF), -1
(MIP-1), IL-5 IL-3.
, ,
, 10-12
, .
APO-1/CD95 (Fas)
(Fas-ligand),
CD45 , /
CD69 .
: -
0,4x109/l (0.1-0.6x109/l)
,

 .
, ,

. ,

.

0.6x109/l.
0.6 1.5x109/l,
1.5 5x109/l,
5x109/l.


:
- .
, , , , ,
, .
.
,
(), , ,
() 16
(M4Eos), 8p11 (),
() - ,
() .


.
.

,
.
. ,
,
, . ,
Hodgkin Hodgkin
.
().
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6 , ,
, .

, ,
.. .

, ,
.

,
.
( Well), ( Loeffler), Shulman
( ,
), , ,
.
. Shulman

.

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T-, ,
: () 1 -
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(2) IL-4 IL-5.
IL-3 GM-CSF
. ( )
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,
1 2 .
, IL-1, IL-5 ,
- (TGF-) PAF,
.

.
. ,
,
-, IL-3, IL-5, GM-CSF.
,
,
.



.

.
,
C- ,
.
(Isospora belli
Dientamoeba fragilis) ,
, Trichinella spiralis,
Strongyloides stercoralis Toxocara canis,
. ,
,
. ,
,

, .
,
. IgE
500 kU/L .

1%
. ,

.
, ()
, () FISH (fluorescent in situ hybridization)
, ()
,
() (PCR)

 (HUMARA) , () DNA
(PGK), ()
- () Kras .
,
.
: () (ALIP)
, / , ,
() ( ), ()
()
(IL-3, IL-5 GM-CSF).
:

()
,
. ,

80%
.
. 0,4
400x109/L .
t(5;11), t(2;5), t(5;12)
5. ,
5q31 IL-3, IL-5 GM-CSF,
.
.
2003 FIP1-like1platelet derived
growth factor receptor- (FIP1L1-PDGFRA) 9/16
. .
.
ETV6-PDGFR
.
, , L
, PDGFR KIT
.
...
.
bcr-abl, ,
.

.

B12
.
. ,
.

.

() .
, ,
,
. 4eos). t(10;14),
.
.


-2, FAB , t(8;21)
,
. 4eos,
16,
.
16, t(16;16), del(16),
t(5;16) t(16;21). ,
,
.

1973.
.
15
t(5;14), ,
.

. ,

.
, .
( )
.
()
, . 114
Matsushima . (1993) 8 (7%)
. (RA), 5
(RAEB) (RAEBt).
7/8 .
0,7-1,4x109/L, 6/7 (86%)
, 7 () 5 (5
).
. Oscier 1996
5 10
.
0.8 4.6x109/l ( 1.5x109/l).
8 2 7 .
. 7
,

5%. 1996 Doorduijn .

. 24 ,
.
8, .
8 FISH.
.
.
.


.
, ,
. Spiers . 1977
50 (
bcr-abl ), 46 .
50 5x109/l (: 0.3-30x109/l).

 Jonte . (1992) 134

78% .

- ,
- -.

.
,
8 (8p11) .


, , , .
60 ,
.
,
, . 2
...
2 .
25% , ,
~35% .

.

()
.
:
() 1.5 109/l
, 6 , () ,
, , , ()
.
(<1% )
,
.
() , 90% ,
15 .

.

.

B7/CD28 LFA-3/CD2.

. 2, IL-2/ IL-2,
. ECP
.
-
.
L-2 L-15 -
(natural killers cells-NK).
IL-2 . Simon
. 60 : 12 IL5 -, IL-4
- 4 . 16
-.
, 8

 TCR TCR , .

.

.

.
. , ,

.

, ,
. ,

10 .
.

, IL-5
, . ,
, -
, .
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. -

. ,
(MBP, ECP) ,
. ,
,
.
, , .
, .
, PAF ICAM-1.
ICAM-1
. , , ,
IL-3, IL-5 GM-CSF, .

,
PAF VCAM-1, .


. IgE

.
, , .
, MBP, ECP
, ,
. ,
, , 12,
.
, ,
,
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.


15 .

,
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.
,
. 5
,
.

, , , , ,
. 13 ,
1-2 . 7
, (3-4 .) 4 , 2
.
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, , ,
.
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. .
/ .
,
,
.


1.

.
, , ,
.


.

.

.
, , (
1 mg/kg/), ,
,
- , ,
.
,
.
- (IFN-),
.
.
NF-AT AP-1
IL-2, IL-5
- .

. 1-2 g/ . ,

.

 . 100
mg/ 4 , 75 mg/.
200 mg .

.

, 25%
. ,
,
.
, ,
12, ,
, ,
, ,
. ,
.
.

Apperley JF, Gardembas M, Melo JV et al. Response to imatinib mesylate in patients with
chronic myeloproliferative diseases with rearrangements of the platelet-derived growth
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Bain B. Eosinophilic leukemia and idiopathic hypereosinophilic syndrome are mutually
exclusive diagnoses. Blood 2004; 104: 3836.
Bigoni R, Cunes A, Roberti MG et al. Cytogenetic and molecular cytogenetic
characterisation of 6 new cases of idiopathic hypereosinophilic syndrome. Haematologica
2000; 85: 486 491.
Brito-Babapulle F. The eosinophilias, including the idiopathic hypereosinophilic syndrome.
Br J Haematol 2003; 121: 203-223.
Chang HW, Leong KH, Koh DR, Lee SH. Clonality of isolated eosinophils in the
hypereosinophilic syndrome. Blood 1999; 93: 1651 1657.
Cools J, DeAngelo DJ, Gotlib J et al. A tyrosine kinase created by fusion of the PDGFRA
and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic
syndrome. N Engl J Med 2003; 348: 1201-1214.
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their concentration in peripheral blood. Haematologica 1999; 84: 240.
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2779.
. . , 6 ,
, 2003.
. . "", 2000.
, . CDROM - .
"", 1998.
, . , , . CDROM
. "", 1996.
. . , . 39, 16-24, 1981.
. . . , 46, 323-331,
1984.

 1. , -, .
, ,
(,
)
(V,HTLV-, )
( )

(,
, )

(visceral larva migrans)
:
(Wuchereria bancrofti, Brugia malayi)
Ancylostoma duodenale,
Toxocara canis,
scariasis,
Trichinella spiralis,
Nippostrongylus brasiliensis,
Fasciola hepatica,
Schistosoma mansoni,
Echincoccus-
Trichuris trichura,
Capillaria,
Gnathostoma,
Enterobius vermicularis,
Isospora belli,
Dientamoeba fragilis,
Toxoplasma: -

,
(
,

): , IL-2,
GM-CSF, ,

i
( Loeffler)

(PIE )

( Churg-Strauss)
A

Shulman
Wegener
( )
Sjogren

( Wells)



Wiskott-Aldrich
IgA
-IgE-
( Job)
Nezelof

Hodgkin
-Hodgkin

(
Kimura-Weil)
Langerhans







-


ddison

10

 2. ...

(indolent)

**

* FIPL1-PDGFRA
** 10%

2. ...

(> 15
)
.
(>25%)
. D816V
816 c-kit
. CD2
CD25
. >20 ng/mL.

11

E 1.

?
( )

( 6 ):
,
,
,
(IL-5, IL-2, IL-3, IL13, GM-CSF),

+ (>1,5x109/L) > 6

:
,IFN-, HU,

:

+/-

12