-

MD, Ph.D ( c)
/


(MDS)

, )

(,

(>60 ),
(melphalan, cyclophosphamide,
busulfan, chlorambucil)

(
-)

F.A.B.
1976 ,
(F.A.B.) France.American.British group 1982


( , Auer, ,
)


W.H.O.
1999 100
W.H.O. ( )
2001.

20%
30%( RAEB-T )
RA, RARS

RCMD (
)
RAEB RAEB-1 ( 5%-9%)
RAEB-2 ( 10%-19%)
5q-
CMML MDS/MPD
1 2
MDS (MDS-U)

 W.H.O.

 2008
:

,
,
.

- : ,
< 1%.
- : : > 10%
, < 5% , < 15%
.


(- )
-

, ,

(
)

( )

(, ,
,,
)

()




(CV 100-110)
RAS

(-5q)



( )

(RAS)



( )

( PelgerHuet)

Peripheral blood smear from

a patient with refractory
anemia with excess blasts
(RAEB) shows a neutrophil
with a bilobed pseudoPelger-Huet (Pelgeroid)
nucleus. The two lobes are
connected by a thin strand
(arrow) giving a "pince-nez"
appearance. These nuclei
look identical to the those
seen in the inherited PelgerHuet anomaly. This
neutrophil also has markedly
reduced granulation, a
finding commonly seen in
the myelodysplastic
syndromes



( )


.
- , .

- : ,
,
,
Howell-Jolly,
,
.
- : ,
,
, .
- :

,
, .

Bone marrow
aspirate from a
patient with
myelodysplastic
syndrome. The
megakaryocytes are
abnormal, with
multiple small lobes
seemingly
disconnected from
each other ("Pawn
ball" changes,
arrows). (WrightGiemsa)

Prussian blue stain of

the bone marrow in a
patient with
refractory anemia
and ringed
sideroblasts (RARS).
Blue-stained
hemosiderin deposits
in the mitochondria
of erythroid
precursors form an
apparent ring around
the nucleus (see
arrows).

-
-
- ALIP (bnormal Localization of Immature
Progenitors)



RA,RARS
>60%
5q-, 7,
8

/ MDS
(, 19,
)
(12- , }
( )

( Felty)

( Pearson)
( Fanconi ,
, Shwachman-Diamond,
)
PNH




.
FAB 1982
1985



,

( )

 (IPSS)

1997
,


ISPP (International Prognostic

Scoring System)

WPSS ( WHO Classification-based prognostic scoring

system for MDS )

RA,RARS,5q-

RCMD,RCMD-RS

RAEB-1

3
RAEB-2

( 1
8 4 )

( : ,-, del5q, del20q , : , 7 , :

)

()

Very low

103

Low

72

Intermediate

40

High

3-4

21

Very high

5-6

12


( Low ,
Int-1 Risk )

( Int-2 ,
High Risk )



(Low,INT-1 risk)

Epo > 500 mU/ml

:
/

: .

Epo > 500 mU/ml
: ,



(Low,INT-1 risk)

-

Epo < 500 mU/ml : Epo / G-CSF

: /
,

Del 5q :



(Low,INT-1 risk)
,

,
:

-A

-
G-CSF &
-A




: - >20-30 >1000
g/L
- >2
-

- ( Desferal, DFO)
- (Exjade)

)
)

- ( -
- (



(Int-2,High Risk)
<60 - :
(
)
- :
5-
>60 - :
5-
- : 5 , ,
.

-

,

.



po 40.000-60.000 IU 1-3x/
- 150-300 g/ 6-8

Hb 1.5 g/dl

-
Hb <12 g/dl

- ( Fe)
G-CSF 2 g/kgr x 1-3/
Hb
<12 g/dl <20.000/l
6-8 .




~10% . .
/ ,