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150 109 /L ACOG Practice Bulletin No. 95: Anemia in pregnancy.
1% 100 109/L, 2/3 Obstet Gynecol 2008;112:201-7.
130 109/L 150 7. Horowitz KM, Ingardia CJ, Borgida AF. Anemia in preg-
nancy. Clin Lab Med 2013;33:281-91.
109/L.
8. Stephansson O, Dickman PW, Johansson A, Cnattingius
: 1. , S. Maternal hemoglobin concentration during pregnancy
2. ( - and risk of stillbirth. JAMA 2000;284:2611-7.
), 3. 9. Tsatalas C, Chalkia P, Pantelidou D, Margaritis D,
, 4. , 5. Bourikas G, Spanoudakis E. Pregnancy in beta-thalas-
. semia trait carriers: an uneventful journey. Hematology
. - 2009;14:301-3.
10. Whittaker PG, Macphail S, Lind T. Serial hematologic
3-4 .18-20 changes and pregnancy outcome. Obstet Gynecol
1996;88:33-9.
-
11. Lurie S, Mamet Y. Red blood cell survival and kinetics
,
during pregnancy. Eur J Obstet Gynecol Reprod Biol
(HELLP, ), - 2000;93:185-92.
, 12. Milman N, Graudal N, Nielsen OJ, Agger AO. Serum
. , erythropoietin during normal pregnancy: relationship to
- hemoglobin and iron status markers and impact of iron
supplementation in a longitudinal, placebo-controlled
.18-20 study on 118 women. Int J Hematol 1997;66:159-68.
13. Bille-Brahe NE, Rrth M. Red cell 2.3-diphospho-
- glycerate in pregnancy. Acta Obstet Gynecol Scand
1979;58:19-21.

14. Kuvin SF, Brecher G. Differential neutrophil counts in
, . pregnancy. N Engl J Med 1962;266:877-8.
15. Molberg P, Johnson C, Brown TS. Leukocytosis in
labor: what are its implications? Fam Pract Res J
1994;14:229-36.
1. Rizack T, Rosene-Montella K. Special hematologic is- 16. Siegel I, Gleicher N. Peripheral white blood cell altera-
sues in the pregnant patient. Hematol Oncol Clin North tions in early labor. Diagn Gynecol Obstet 1981;3:123-6.
Am 2012;26:409-32. 17. Khnert M, Strohmeier R, Stegmller M, Halberstadt E.
2. Townsley DM. Hematologic complications of pregnancy. Changes in lymphocyte subsets during normal pregnan-
Semin Hematol 2013;50:222-31. cy. Eur J Obstet Gynecol Reprod Biol 1998;76:147-51.
3. Bernstein IM, Ziegler W, Badger GJ. Plasma vol- 18. Bussel JB, Druzin ML, Cines DB, Samuels P. Thrombo-
ume expansion in early pregnancy. Obstet Gynecol cytopenia in pregnancy. Lancet 1991;337:251.
2001;97:669-72. 19. McCrae KR. Thrombocytopenia in pregnancy. Hematol-
4. Nadel AS, Ballermann BJ, Anderson S, Brenner BM. ogy Am Soc Hematol Educ Program 2010;2010:397-402.
Interrelationships among atrial peptides, renin, and blood 20. ACOG practice bulletin: Thrombocytopenia in pregnancy.
volume in pregnant rats. Am J Physiol 1988;254:R793- Number 6, 1999. Clinical management guidelines for
800. obstetrician-gynecologists. American College of Ob-
5. http://www.who.int/nutrition/publications/en/ida_assess- stetricians and Gynecologists. Int J Gynaecol Obstet
ment_prevention_control.pdf 1999;67:117-28.

14
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11. Dreden P, Woodhams B, Rousseau A et al. Comparative
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24

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25
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29
.

14,9-23,7 g/dl.
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100-125 fl 9 1. Golub R, Cumano A. Embryonic hematopoiesis. Blood
. , cells, molecules & diseases. 2013;51(4):226-31.
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journal of developmental biology. 2010;54(6-7):1061-5.
3. Chen D, Lewis RL, Kaufman DS. Mouse and human
-
embryonic stem cell models of hematopoiesis: past,
3-5 . present, and future. BioTechniques. 2003;35(6):1253-
61.
. 4. Wilkinson RN, Pouget C, Gering M, Russell AJ, Davies
SG, Kimelman D, et al. Hedgehog and Bmp polarize
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. dorsal aorta. Developmental cell. 2009;16(6):909-16.
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31
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39
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44


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45
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31,8%, IUGR 24,2%
72,7%, 60-80% - -
( 87,5%). 2 ,
26 36 30%
7,2%.14 .15
-

(4-30%). .
IUGR,
-
.
100 g /L.
H -
.
-
60 34 -
18,3%, . ,
bF.15 -

/ .

-
, , - 5-30%,

Hb>9,5-10,5 g/L ,
. - .16 ,
-
.9
-
, .
-
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. -
,
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48


. , Schuman . -

.
-,
-
, - -
. -,

. ,


- -
.21-23
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).17-19

- -
. ,
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.7-9,24,25
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.20
- -. ,

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b

.
H -,
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-
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, rh-Epo (
. ).
rh-Epo
2,3-DPG (2,3- ) -,
, . ,

. , - - . -
,
. , rh-Epo
- 2,3-DPG SPC (summary of product
- characteristics) epoetin-
.
.
- ,
, ,

49
. .

7. Aessopos A, Karabatsos F, Farmakis D, et al. Pregnancy

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51
 :

, ,

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52


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53
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55
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56


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57
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59
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60


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 :

- HELLP

, ,
, .. ,

Hypertension in Pregnancy (ISSHP), Society of

Obstetricians and Gynaecologists of Canada (SOGC)).

140/90mmHg (
. 10% -
. - ) [300 mg
50% . 24 /
- ( mg/dL) 0,3
7% (dipstick) 1+ (1+ -
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( ) dL)- ],
. , 20
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. - -,
. -
. (
O > ), -
, ( <100000/L),
, ( >1,1 mg/dL, -
/
), ,
. A - (,
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pregnancy (HDP))
.

160/110mmHg, ( <100000/
(National Institute for Health and Clinical L), >
Excellence (NICE), World Health Organization (WHO), ,
American College of Obstetricians and Gynecologists
(ACOG), International Society for the Study of , ( >1,1 mg/dL,

63
. .

.
), -
, . -
(65%) .
. .
,
, ,
-
. . 50%
40,
.
. : ,
, -
, , , (BMI >30 .3
) ,
, , -
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..). - -
.
, .
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)
. , (.. ).
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.
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- ,
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. , ,
.
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. .
20 . .
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.
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20 ,
23 . .
. -
, -
. ,
- ,
.
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. - . -
. -
.

64
 - HELLP

- - ,
, 100.000- 150.000/ VEGF (vascular endothelial growth factor)
mm3 . PlGF (placental growth factor). VEGF -

- . PlGF VEGF
.
-
, .
- sFlt-1 (Soluble Fms-like tyrosine
, kinase 1),
,
, VEGF,
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. VEGF
PlGF .
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VEGF PlGF
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, sFlt-1
- ,
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.
,
. - -
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sFlt-1
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. ,
. ,
- (soluble Endoglin, sEng) sFlt-1
.
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TGF-) TGF-
.

. sEng
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-

, .
human interferon-inducible protein (CXC 10).
, stress, , -
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65
. .

adhesion molecule-1 [VCAM-1], intercellular adhesion .

molecule-1 [ICAM-1]) -
- 6 (IL-6), TNF- (Tumor (TNF, IL-1, IL-12).
necrosis factor-) stress. IL-12
- -1

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,
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DNA
. ,
. ,
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-
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-
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.

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,
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66
 - HELLP

C
. .
-
C .
. . HELLP
C :

( : apoptotic syncytial .13
knots and syncytial sprouts).
C, C3a Bb (Platelet-associated regu-
C latory system: PARS).
20 , .
C
. ( ,
- ,
C3a
Bb ,
). C .14
-
: MCP CD46: membrane cofactor protein, (TMA)
non GPI -linked C .
C3b C4b ,
C ,
C3 convertase, CD55: (decay accelerating , -
factor: - DAF) .
.
C3 convertase (C4b2a) C5 convertase -
CD59: (membrane inhibitor of ,
reactive lysis: - ( ).
-MIRL)
MAC (embrane Attack Complex:
C5-C9 complex) .
C9. -
C: MCP, Factor I, Factor H . -

. .15
To HELLP
C
, ,
,
. .
C
,
;11,12 ,
() , 48-72
, ( ) .
70%
. ( 27 - 37 , 10%
27 20% 37
, ) 30%
. To .

67
. .

I: : 50000-100000/mm3 II:
/ : 100000-150000/mm3
/ .
34 , ,
,
7 . , -
0.5-0.9% .
10-20%
. 10-20% .
HELLP , - .17

,
.
, 25 . LLP, :

19-27%. ( 90% ).
HELLP.16 (29-84%).
Prichard 1954 - ( 40-90%,
, ,
1982 -
Louis 5%).
Weinstein, -
HELLP
: ) (Hemolysis), )
(Elevated Liver .
enzymes) ) (Low 30-60%
Platelet count). 20% . ,
(complete or true HELLP (cHELLP))
HELLP (partial HELLP). - ,
cHELLP .
pHELLP 50-60% (
87%). ,

cHELLP . .
A ,
Tennessee Classification System
Sibai 30% . -
: A <100000/ .18-20
mm3, SGOT 70 IU/L LDH600 IU/L. -
Tennessee - .21
1) -
,
LDH, .
2) SGOT 70 IU/L ( .
)
1,2mg/dl 3) .
<100000/mm3. -
ELLP ,
.
ississippi classification .
-
: <50000/mm3, .

68
 - HELLP

, -
. -

. - ,
, 1,2mg/dl (
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.17,28 1. von Dadelszen P, Magee LA. Pre-eclampsia: an update.
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,
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71
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2/3


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 :

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.7 117 Haemorrhage (PPH)
2.3%, 5.9%
0% 87 , 17 ,
5 .18
- 600 ml >
1000 ml 24h.
PPH 4-6% .
PPH
( , , () 1 24
). 6 1-3%
, .
, -
, ,
.
.
: ,
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, ,
.
-

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,
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.

/

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.
/- -
.
- vWD 34%,
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.
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, vWD, ITP 10-37% 50-54% 1
-

.
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.
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.

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, , 1.
. .

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,
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,
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,

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-




(DDAVP) ia Glanzmann (GT) -
. BernardSoulier (BSS)
DDAVP: H DDAVP -
vWD - GP IIb/IIIa GPIb/IX/V .
-
.12 BSS GT,
.
, -
.
,
. - BSS PPH
, 34%
, 41% 30 .1,28
V2
. GT,28 -
:
.
PPH -

90g/Kg rFVIIa 2h 4-5 -
. : (), .
- ( EACA) (APR). .25
APR PPH
10 , rFVIIa
2008
Creutzfeldt-Jakob (- 2 .
). GT/BSS
TA

, .

() .
, .
(World Maternal Antifibrinolytic [WOMAN])11

PPH (-
) 1. Kadir DJ, Winikoff R, Pollard D, et al. Pregnancy com-
. plications and obstetric care in women with inherited
bleeding disorders Haemophilia 2013;19(Suppl 4):1-1.

86

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women: a systematic review of literature Haemophilia
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7. Huq FY, Kadir R A Management of pregnancy, labour
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8. Kadir R, Chi C, Bolton-Maggs P Pregnancy and rare
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87
 :

1, 2
..., , .. ,
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2

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,

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.
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o

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, .9
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, .

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.
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89
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,
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.
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- HMWK), 2 37C.15
,
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. ,
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.
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-
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VIII -
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) - -
. .
,
. ,
.
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, - , -
, , ,
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:
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,
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(DDAVP), O VII , -
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91
. .

, : 1 mg/
, kg 46
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, 176 ,
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. 1 ,
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, VIII -
, rFVIIa, 3 6 ,
95% .22
bypassing ,
, (2 mg/kg
6 ), ,
, (5
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(IVIgG) -,
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, ROTEM Baudo .,
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Bonfanti ., .30
, 1
2 Rituximab,

,
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 :

1, 2, 1
1
- , , 2Klinik fr Gynkologie und Geburtshilfe, Klinikum Links
der Weser, Bremen, Germany

0.5% 0.03 0.08%
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,

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.

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. .
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.
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. 1. ( High Risk Pregnancy-Management Options 4th Ed.-Saunders 2011).

95
. .

iv) . ,
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.
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104


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 :

, , .. ,

, -

.
, American College of Obstetrics and Gynecology
, , - (ACOG)
. 10%
,
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. -
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in Significant Haemorrhage) .
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istry off label rFVIIa
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VIIa (rFVIIa),
- ,

110
 -

, .30 during delivery and the postpartum. J Thromb Haemost

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 :

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114
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. Royal College of
Obstetricians and Gynaecologists (RCOG)
. LMWH OHSS 2
ART 1

115
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-
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,
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. ( n=386
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116
 iVF

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117
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119
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120


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121
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.35

122


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,
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, 1 . 2005; 341-346.
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139
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142


(2+) .18
RhD .
Rhesus D DVI.
D
( Du) , -

(, , )
D - .
RhDIG : ) (12
: )
.16,17

Coombs RhD RhDIG
27 . ) -
- (12 : )
(, , )
Coombs (indirect antiglobulin 18 , 4 28
test- IAT), 2
. .
Rhesus D -
, Rhesus D
.
-D
(12 ). ,
D-. 40%
D
D .

Coombs ( D )
RhDIG .
D

.18 D
27 .

-

, RhDIG (
) D
D
. , 38% RhDIG.
- DNA -
-
. C, E, c
.19,20

, .
.
-
Doppler
(middle cerebral arteria-peak systolic velocity, MCA-
PSV)

143
. .

-
. 21,22 : )
, ) -
)
Coombs
. 1 .18 DAT

100%
10%.23 ,
18-35 .
- HDFN.2,25
. DAT (+) -D
DAT
, 3 .2
, - DAT
. -

-
. -
.24 -

.
.25

HDFN
HDFN
(, , , :
- .

- ).
(IUT).
, - IUT
. -
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,
.
-
. 24 ,

Coombs DAT -,
FcRn -
Coombs (Direct Antigloboulin Test DAT) IgG
.26
.
-
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.
Coombs
, .27
DAT .2 HDFN
DAT

144


- .
-D - (RhDIG)
. -
- (antibody-mediated immune
( ..). suppression- AMIS), -
-). ,
, RhDIG -
, . RhDIG
- ,

.28
D . RhDIG
(160 ml/kg) -
, ,
. T -
- -
,
.37
, BSCH
74% D D
2%.29 -
-
.
- D D
HDFN 250 IU
(IVIg) . - 6 .38
, , Rh D
IVIg , RhDIG
D
-D D : )
.30-32 , 72 , )
- (28 -34 )
.33 )
, -, : -
(RCT) IVIg , , ,
, ,
. , ,
, , .38

IVIg,34-36 IVIg D
HDFN .33 D ,
RhDIG D -
D .
Rh D , ,
-
- -
.20
Rh D
. () -
Rhesus D - (RhIG) RhDIG -
-D - ,39-41

145
. .

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147
 :
-

1, 2, 3
, , .. , ... , , 2, ,
1

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, .1
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.
, .
. HbF HbA,
- 2 .
-
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.
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148


.
, .
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,
-
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dl ( 14-20gr/dl). Hb
<14 <13 gr/dl.
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.
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).7
Rh.
-
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,
. (HDFN) (HDN). -
- , -
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/Rh .
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( guidelines
-
) (7).
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( 25
1

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2 Hb 11 gr/dl 35
, Hb 7 gr/dl ,
42 . 4C.
,
2. .
( - guidelines
- ) ,
Ht >40-45% , 2,3
DPG ,
Ht >30-35%
(,
Ht >30- 35% ).
( Ht >20-25%
, :
) -

149
. .

(10-15 ml/Kgr) - -
0.3-0.4 mEq HDN. -
2-3 mEq/Kgr,
.10 D
- - Rhesus,
2,3 DPG , Rhesus
.
2,3 DPG - , -
.11 ,

.
.12 -
( 25 mg/dl).
.
(BCSH) - 8-12 mg/dl.16
.
(AABB) 5%
- , -
.13,14 , ,
- .
, . - 3-4/1000 .16
,
(CMV). <5106 , ,
-
, BCSH <1106 .
. , <1,5 Kgr,
, Rh Kell .
(stem cells), CMV
.14 .
- Ht 40%- 50 %.
.
.17
. 5%,

, .
, CMV /
. 24 .
- -
. G6PD .17
-
(<1,5 <1 Kgr), (5-10ml/Kgr
<4 .15 ) -
BCSH - .
14 -
14 , (160-200 ml).

14
28 .

30-35%
.

() , , -

150


, , .
.
. - , -
, ,
. .
15 ml/
Kgr . -
.20
.
HPA -1A.


.18

75% -
50
-100109, 5% .
<50109.

. .20,21
10 ml/Kgr
50-100109 (HDFN). H HFDN
30-60 min.
- . anti- Rh (RhIg)
3.19
, - 6,8 1000
- .22 -
.
RhD .23
,
19,
. -
.24


HDFN :
- .
. , -
,
- -
, .. - D 8
32.25
3. . - Doppler (MCA).
( -
)

<30.000 .26
- D50. H
30.000-50.000 - 1500 gr

-

(
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,
.
-
MCA .27
- - .
50.000 100.000

151
. .

. (Fy, Jk, MNS

, ).29
Coombs,
. - . -
-
6-7 (SD) .
.28
,
: .
Ht Ht / Ht Ht
,
(100-200ml) -
1960 -
, . -/
- .29
.
, - - -
<18 ,
.
13 .29
, -

, ,
- .
. -
,
.29
- Ht
( 40-50%.
). 15 ,
-
3-4 40 60 . ( 3 )
3 4 35 .
, CMV , ( <1106/ -
), Rh(-) - 1-2%.
Kell(-) (3-5 -
) .
75-85%. - . ,
,
RhD , .29
6 6
.28
.
- 10 ,
. , 2
- 16 , .30
.
19,
. ' , -
.29

152

15. Transfusion guidelines for neonates and older children
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16. American Academy of pediatrics, Subcommittee on
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153
 :
-

()

, nkeleida Trajce
, .. ,

NAIT
, -
,
(NAIT) (, ,
,
..). ,

-
1:800-1000 .
.
-
-

,
IgG
,


-
(PAs - Human Platelets antigens) .
. -
, .
(RhD ), , ,
,
20% NAIT
.1-3 10% 20%.
75%
20 .6
,

. NAIT.3,7
1953 Harrington.4 T, NAIT
. ,
, HPAs. -
24-48 . -
(<20109/L).
.
(<150109/L) , ,

.1,5 NAIT -

154
 ()

cost-effective.8,9 , -

, .15
- HPA
, ,
. anti-HPA .

NAIT -
,
-
. ,
, 24 - . HPA
. ,
-
(GPs) IIb/IIIa, Ib/IX, Ia/IIa. 12, 24, 32 .
1990 - (
HPA)
(HPA) ,
, 30 .
, a
b .10,11 NAIT
, HPA-1a .16
NAIT. HPA -
HPA- 5b, ( ) -
- NAIT,
. HPA- 3a HPA-1b. ,
, 12, 24, 32
HPA-4b.12,13 20
. -
HPAs NAIT - HLA DBR3*0101, NAIT
, PCR. , .2
HPA -1a/1a 1a/1b (98% PA

1a), HPA-1b/1b. , (: HPA 1a/1b)
10% anti . -
HPA -1a , NAIT. DNA
15
HLA DRB3*0101, -16 .
.
( DNA
99.6%), ,
(35%).14 .17



MAIPA (monoclonal antibody specific im-
mobilization of platelet antigens) MACE (modified NAIT
antigen capture enzyme-linked immunosorbent as-
say).
. <30x109/L 24

155
. . Trajce

.
,
< 50109/L. ,
.
, HPA - -
. ,
.
(IVIG 1g/kg/day
2 ). .
50109/L. ,
>30109/L
.20,21
-
,
, . .
8 10 32
.2 ( 48 ,
)
<50109/L.
>100109/L,
.16

.18
, .
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.
-
,
(active decision).
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