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.. , ..

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. ..

[[


 ,
116 (). 
() 82,8%, 
() 14,6%, 2,6%. 
. 
,
25 .
 (88,4%) 
(11,6%). 
.   
.   (54,1%) 
(44,3%) .   
, . 
58,3% ( ), 
30% ( t (12; 21) (13; q22). 
( , ) 75%
; 40% , 
t (15; 17) (q22; q21).
: , ,  , 
.
: , ..., .
.: +7 (342) 2218615.
, 2011
N.B. MERZLOVA, D.V. MERKURIEV, V.I. BATURIN, O.E. NIKONOVA

Structure and genetic and immunological features of acute leukemia in children


of the Perm region
A study was made into agegender and morphological structure, inmmunological and genetic peculiarities of 166 children
diagnosed with having acute leukemias (AL). In the structure of AL acute lymphoblastic leukemia (ALL) 82.8% was found
to be predominant, as well as acute myeloblastic leukemia (AML) 14.6% and acute biphenotypical leukemia 2.6%.
Overall AL and ALL occurred significantly more frequently among boys. A significantly early manifestation of ALL was revealed
in comparison to AML, and also an increased frequency of progression of ALL in children aged 2 to 5 years. Typical for ALL
immunological structure was predominance of Bcell leukemia (88.4%) over Tcell leukemia (11.6%). Early manifestation
is more common for BALL than for TALL. A significant prevalence of TALL over BALL was detected among schoolage
patients. Dominant in the structure of BALL were prepreB (54.1%) and preB (44.3%) subvariants. TALL was represented
by preT and cortical subvariants which showed similar frequency rates. During ALL chromosome rearrangements were detect
ed in 58.3% of patients (more often hyperdiploidy), transgenation in 30% (more commonly translocation t (12; 21) (13; q22).
In patients with AML an aberrant karyotype (various digital, structural anomalies) was recorded in 75% of cases; at the molec
ular level rearrangements were detected in 40% of patients, half of them were found to have the t (15; 17) (q22; q21)
translocation.
Key words: acute leukemias, children, agegender structure, immunophenotypic and genetic features.

22

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(n=96)

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,
(L1)
(L2) .
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; 1
; 2 ;
3 ; 4 ;
5 . 
0 5 ( 29,4%);
3 (17,6%).
(6)
(7) .
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;  
11,6% ( 7,62:1). 
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(<0,05).

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25

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, 
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3 3,5
46 . , 3/4
(717 )
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 12,5%.
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. 
(common),
CD10+, CD19+, cIg, sIg,  (CD10+, CD19+,
cIg+, sIg). 
 (CD10, CD19+, cIg, sIg).
, 25,4% 

 .
CD33+ (9,1%),
CD13+ CD33+ ( 9,1%).

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( ) CD2+ (
).
, 8
, ,
, .


:  CD7+, TDT+,CD3+,
CD2, CD1a (50% )
CD7+, TDT+,CD3, CD2+, CD1a+,
CD4+ CD8+ (50% ). 62,5%
 
, CD33
(50%),  CD10 (12,5%).
,
, (
90% ) 
, 

. 

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36 :
41,7%, 

1 .

2 0 1 1 .

58,3%. 
(23,8%), 


1 dup(1) (q32; q21)
6 del (6) (q15; q22). 

t (11; 14) (1315; q11), 
 .
t (; 7)
(q22; q32), t (8; 11) (22;
q13). 
t (6; 14) (q27; q12), t (1; 4) (q21; 15), t (12; 21) (13;
q22) t (9; 22) (q34; q22); 11 del
(11) (q23),
12. 
. 
t (1; 12) (q21; 13) t (9; 12) (12; 13) 
del (9) (q22) 
. ,
48 ,
, 21,
20 .
 ,
30 , 
30% .
t (12; 21) (13; q22) 
L/AML1 (20%).
t (9; 22) R/ABL;
t (4; 11) MLL/AF4; t (1; 19) E2A/PBX,
del (1) (p32) SILTAL.

,
 

t (12; 21) (13;
q22), 33,3 20% 
.  
t (11; 14) (1315; q11), 
25% .

8 . 
75% (6 ); 
3 .
/
/ 
t (6;22) (p24; q11), t (7; 14) (p13; q31),
del (1) (p35) del (11) (q23), Y;
17 del (17) (p11) 
9 
9 (9ph);
21 
t (12; 17) (p13; p13)
del (11) (q23). 
( 8,

26

21, ).
 ,
10 , 
40% 
: t (15; 17) (q22; q21) L/RAR
(20%), t (9; 11) (22; q23) MLL/AF9 (10%)
inv (16) (p13; q22) CBFB/MYH11 (10%).
, 

58,3% 75% .
 

t (12; 19).

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.
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,
[10, 11]. 
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1 ,
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t (1; 4) (q21; 15)
 .

1 .

2 0 1 1 .

2. ..
. : , 2004, 191 .
3. / ( .
.. , . ). .: ,
2004, 792 .
4. : ( .
.. ). .: , 2002, 351 .
5. Voute P.A., Kalifa C., Barret A. Cancer in children: clinical
management Oxford; 1998. . 359.
6. ..
/ . .
... ., 1996, 25 .
7. ..
//
, 2003, . 4, . 471478.
8. .., .., .. .

// . .,
2005, . 50, 3, . 814.
9. .., .. /
: .  .
. 2, . . .: , 2007, . 409501.
10. .. // .
., 1997, 5, . 6570.
11. Parkin D.M., Muir C.S., Whelan S.L., et al. Cancer in fife
continents. IARC Sci Publicat 1992; 120: 82670.
12. Parkin D.M., Stiller S.A., Draper G.J., et al. International
incidence of childhood cancer. IARC Sci Publicat 1988; 84:
2571.
13. .., .., .. 
. .:
, 2005, 176 .
14. Hamouda F., ElSissy A.H., Radwan A.K., et al. Correlation
of karyotype and immunophenotype in childhood acute
lymphoblastic leukemia; experience at the National Cancer
Institute, Cairo University Egypt. J Egypt Natl Canc Inst 2007;
19 (2): 8795.
15. .., .., ..

// . 
., 2000, . 45, 1, . 2834.
16. .., .., ..

// /
, 2004, . 3, 2,
. 510.
17. .., .. 
. . .:
, 2006, 112 .
18. Gmidene A., Sennana H., Elghezal H., et al. Cytogenetic
analysis of 298 newly diagnosed cases of acute lymphoblastic
leukaemia in Tunisia. Hematol Oncol 2008; 26 (2): 917.
19. Paulsson K., Johansson B. High hyperdiploid childhood
acute lymphoblastic leukemia. Genes Chromosomes Cancer
2009; 48 (8): 63760.

1. 

(82,8%),
.
14,6% , 

.
.
, 

.
2. 
, 

25 .
,

.
3. 
 (88,4%)
 (11,6%); /
 7,62:1.  
.

 
 
3 46 .
4. 
  .
  
, 
.
5.

58,3%;
.

30% ,

t (12; 21) (13; q22).
75% , 

.
40% , 
t (15; 17)
(q22; q21).

1. . . .:
, 2005, 766 .

27

1 .

2 0 1 1 .