clinical presentation, laboratory manifestations, and diagnosis of immunoglobulin light chain...

8/11/2019 Clinical presentation, laboratory manifestations, and diagnosis of immunoglobulin light chain (AL).pdf

1/12

() ( )

. 2013. 7, 2013.

. ( ),

,

( )

( ).

20 .

:

() ( )

.

( ,

).

,

(, , , )

.

. () .

( " () ".)

,

( ), , ,

, .

, .

( " " " " "

".)

, , .

, ,

. ( " " "

() "

" ()

".)

.

, 6 10

,

, , ,

,


2/12

1.

. ,

40 1.

64 5 40 24.

65 70 .

; , .

(). ,

,

, (), 1

5. ( "

", ' '.)

,

, , .

,

. ,

( ):

1596 1960

1994, (0.4 ) ( 10 81 )

6.

.

4319 1990 2008

, 47 (1.1 ) 7.

, ,

(95% 414 ).

30 , , ,

8.

. ,

. , , "" .

. ( "

", ' '.)

, ,

3.

:

70

(50

). ( " ".)

60


3/12

( 1).

.

,

. () A

.

( " ".)

(20 ) /

(15 ) A .

, , ,

. ,

, .

.

( " ", 'A

'.)

70 . A

25 .

,

8 1 , , 769

9.

( ), ,

, , ,

. ( " ".)

A

(, ). A (, ),

, A

( 1). A .

" "

/

. ( " ", '

A '.)

,

( ) ,

, A ( 2) 10.

, () ( 3),

.

.

A 1113.

337 , 28

51 , 12.

;

; . ,

11.

, .

( " ", 'A

'.)

A 10 >30

/ .


4/12

, , , ,

. ( "C , , ",

'C '.)

A I

, () 1417. I

F 64 I A ,

(78 ), I G (14 ), H (8 ) 16.

59 F 46 103

K (K) 17. ( "E, ,

", 'A'.)

I F , (55 ),

(53 ), (36 ) 16. 99 I

A 18, (31 3 ) (17 2

) I A .

,


5/12

C ( ) ( 6

7) ( ) 24. ,

(, 6 ) C

.

(

C ),

A :

I (, , ,

A ) 25.

A ;

(A) ; A

(AA) . H, A

23.

I ( 8)

( ) A ,

(65 ) 26. I

A ( 9) 27,28.

A

29.

. I

50 (

) 41 ,

100 98 3033.

G ( ) ,

. H,

, .

A

, .

. I

FIH 21 A , 18 , 72 ,

34. 19

(G). ( "D

".)

C A

(11;14)(13;32), G; (1314),

; 121 3538.

( " ", 'C ' "C ,

, ", 'C'.)

A

() , ,


6/12

.

A ()

. A 35 , A

10 , 5 , 1 , ( )

39. A ,

.

70 , 25 , 5 40.

:

(), ,

50 A .

( " ", ' ()'.)

90 A . ,

(, ) 19. ( " ", '

'.)

,

40. ( "

", ' '.)

.

23. , .

A ( )

( 8) 41. , A

.

(A)

A

42,43.

A 44,45.

46 42.

A , , , . A A

, .

A :

,

, , ,

, , , .

A

, , . A

, (, , ) . ( '


7/12

'.)

, .

( " ", ' '.)

47,48:

(, , , ,

). ,

,

.( ' '.)

(, ,

) . ( ' '

.)

(, ;

). ( ' '.)

(,

, , ).

( " ", ' '.)

2 3

;

.

() , ,

.

,

, . ,

.

(" ".)

, ,

.

,

, . (

),

( ).

().

( )

,

, , ' , ,

.

,


8/12

. A AA A

A , .

( " (AA) " "C (AA)

".)

,

.

(),

A, A A, , .

A ,

.

350 A

; 34 (9.7 )

"" , A 49.

( 0.2 /)

34 . ( "

".)

A ,

A (, , )

, . A ,

. ( "A

", 'D'.)

A (),

A , ( ) (, ). ,

,

(),

(). A () (A)

() ()

50,51. .

A ,

. A ,

, .

( "A ", 'A () '.)

A A

().

(DD)

.

DD

. DD:

A

A . , A

C .


9/12

(CDD)

A ,

2,24. CDD

/ 52.

53. ,

, . ,

CDD .

; .

A , CDD C . ( "

(A) ",

' CDD CDD'.)

(CDD)

A

. D CDD ()

, , C . ( " "

" (A)

", ' '.)

, , ;

, .

.

(), ,

, , 5457. ,

, .

(, ,

, ), 56,58,59.

, A

(, , 24 ,

) .

A

. A

( )

. A

(

, ). ( 'E'.)

A

. , ,

. ,

, , .

, ,

. ( ' '.)

A . A

A . E

90 A . ( ' '.)


10/12

D A ( 'D

'):

(, , , ,

). ( ' '.)

C (, ,

). ( ' '.)

E

(, ;

). ( 'D

'.)

E (,

, ,

). ( " ", ' '.)

A ,

, (DD) .

C ( ),

( A ).

DD

. , A DD

C . ( 'D '.)

D A.

1. A, A, B C, . C, , 1950 1989. B 1992; 79:1817.

2. A, . A (A). C 229 . C 1983; 58:665.

3. A, A. : 474 . 1995; 32:45.

4. A, , 'F . : 168 . B 1986; 68:220.

5. A, , , . A . E 2002; 346:564.

6. , A, A. . C 1998; 82:1501.

7. , D A, , . C (A) . C 2010; 85:232.

8. A CE, C . . 1986; 30:465.

9. D, A, F C, . . C 1993; 68:763.

10. E , B . . A . E 2007; 356:2406.

11. A, , A, . B 100 .AA 1983; 249:1322.

12. AD, 'D , D, . B


11/12

337 A. B 2000; 110:454.

13. C, , B, . A : , , . A 2006; 47:947.

14. A, A, . : ' . C 1993; 11:914.

15. A, A. A . 2003; 30:325.

16. B, A, , . : 72 . (B) 2008; 87:99.

17. A, , , . A : . B 2008; 112:4009.

18. A, , , . A . 2007; 357:1083.

19. A, B , , . : . B2012; 119:44.

20. A, , , C A. . A ' . A 1987; 82:412.

21. A, , , C A. , ,

. A 1989; 32:82.22. C, A, , .

. 1993; 44:834.

23. , C , , . C , AC , 2008.

24. . A . ( ). 1980; 302:1283.

25. , , B , . : 117 . B 2012; 119:488.

26. , C , A, . A . 2004; 19:3050.

27. A , , , . . A 2001; 111:243.

28. A, , B, . : . 1994; 18:401.

29. , B , , . A . C 1989; 42:817.

30. A, , B, . C . B 2009;114:4957.

31. , , , . . C A 2010; 5:2180.

32. C, A, , . . A 2011; 68:195.

33. B , , , . . B 2012; 119:1844.

34. , A , , . C . B 1998; 103:704.

35. , B , , . . B 2001;98:2266.

36. C, , , . 1432 1314 . B 2002; 117:427.

37. B , , C , . . B 2008; 111:4700.


12/12

38. B AH, K , G MA, . (11;14) (AL) . H 2009; 94:380.

39. K A, G MA, G , . A : , , , , . E J M1997; 336:1202.

40. G, , B , . I . C C

2009; 55:499.

41. B, MB, JJ, . . B 2011; 117:3613.

42. H , L J, MB. E 123I . E J M 1990; 323:508.

43. G G, , CM H, . E .A J 1994; 145:526.

44. H , MB. I A. E J M 1995;22:595.

45. J L, H B, F EJ, . K 123 AA AL

. J M 1998; 39:699.46. , MB, H . . A J K D 1995; 26:267.

47. K A, . C , , . L 2009; 23:3.

48. . M : 2011 , , .A J H 2011; 86:57.

49. L HJ, B D, B E, . M AL (). E J M 2002; 346:1786.

50. G MA, K A, E D. . B 1989; 43:101.

51. B, C LH, D , . :

. A I M 2005; 165:1425.

52. H, AM, C LD, . : 64 . C J A 2012; 7:231.

53. C, D'A M, F GB, . L : . A J K D 2003; 42:1154.

54. G G K M F, B C H, B H. G

AL . B J H 2004; 125:681.

55. F , A , M KC, . . J A A D 2006; 54:712.

56. K A, G MA, L M, D A. L AL :

. A 2003; 10:36.

57. , DL, M , . : 31 . M C 2000; 75:1264.

58. D , , C , . . A . I J 1996; 36:61.

59. , M K, F , . E . B J H 2004; 125:679.

6668 12.0

2013 D, I. A . L A : 21.8 C21.121

L : : 110585.52.244.57FF9A9B30A9I3867.14

clinical presentation, laboratory manifestations, and diagnosis of immunoglobulin light chain...

Documents