AIIMS- NICU protocols 2010

Management of Polycythemia in Neonates

M Jeeva Sankar, Ramesh Agarwal, Ashok Deorari, Vinod Paul
Division of Neonatology, Department of Pediatris
All !ndia !nstitute of Medial Sienes
Ansari Nagar, New Delhi "##$$%&
Address for correspondence:
Prof Ashok Deorari
Department of Pediatrics
All India Institute of Medical Sciences
Ansari Nagar, New Delhi 110029
mail! ashokdeorari_56@hotmail.com
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AIIMS- NICU protocols 2010
Abstract
Pol"c"themia is defined as a #enous hematocrit a$o#e %&'( )he hematocrit in a new$orn
pea*s at 2 hours of age and decreases graduall" after that( )he relationship $etween
hematocrit and #iscosit" is almost linear till %&' and e+ponential thereafter( Increased
#iscosit" of $lood is associated with s"mptoms of h"po,perfusion( -linical features
related to h"per#iscosit" ma" affect all organ s"stems( Neonates $orn small for
gestational age . S/A 0 , $orn to dia$etic mothers. IDM 0 ,multiple $irths are at ris* for
pol"c"themia ( )he" should therefore undergo screening at 2, 12 , 21 hour of age(
Pol"c"themia ma" $e s"mptomatic or as"mptomatic and guidelines for management of
$oth t"pes $ased on the current e#idence are pro#ided in the protocol(
'eywords( polyythemia, )lood visosity, new)orn, therapy
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AIIMS- NICU protocols 2010
ntrod!ction
Pol"c"themia or an increased hematocrit is associated with h"per#iscosit" of $lood( As
the #iscosit" increases, there is an impairment of tissue o+"genation and perfusion and a
tendenc" to form microthrom$i( Significant damage ma" occur if these e#ents occur in
the cere$ral corte+, *idne"s and adrenal glands( 2ence this condition re3uires urgent
diagnosis and prompt management(
)he #iscosit" of $lood is directl" proportional to the hematocrit and plasma #iscosit" and
in#ersel" proportional to the deforma$ilit" of red $lood cells( S"mptoms of
h"poperfusion correlate $etter with #iscosit" as compared to hematocrit( 4iscosit" is,
howe#er, difficult to measure at the $edside( 2"per#iscocit" is therefore suspected in the
presence of an a$normall" high hematocrit with or without suggesti#e s"mptoms(
5elationship $etween #iscosit" and hematocrit is almost linear upto a hematocrit of %&'
and e+ponential thereafter
1,2
( )he pol"c"themia,h"per#iscocit" s"ndrome is thus usuall"
confined to infants with hematocrits of more than %&'6 it is #er" rare with hematocrits of
7%0'( Definition
A diagnosis of pol"c"themia is made in the presence of a #enous hematocrit more than
%&' or a #enous hemoglo$in concentration in e+cess of 22(0 gm8dl( 2"per#iscosit" is
defined as a #iscosit" greater than 11(% centipoise at a shear rate of 11( & per second
9
(
ncidence
)he incidence of pol"c"themia is 1(&,1' of all li#e $irths
1,&
( )he incidence is higher
among $oth small for gestational age .S/A0 and large for gestational age .:/A0 infants(
)he incidence of pol"c"themia is 1&' among term S/A infants as compared to 2' in
term appropriate for gestational age .A/A0 infants
%
( Neonates $orn at high altitudes also
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AIIMS- NICU protocols 2010
ha#e a higher incidence of pol"c"themia
1
( Pol"c"themia is unli*el" to occur in neonates
$orn at less than 91 wee*s gestation(
Physiological changes in postnatal life
Significant changes ta*e place in the hematocrit from $irth through the first 21 hours of
life( )he hematocrit pea*s at 2 hours of age and #alues upto ;1' ma" $e normal at this
age
;,<
It graduall" declines to %<' $" % hrs and usuall" sta$ili=es $" 12 to 21 hours( )he
initial rise in hematocrit is related to a transudation of fluid out of the intra#ascular space(
"linical feat!res
Pol"c"themia can result in a wide range of s"mptoms in#ol#ing se#eral organ s"stems
.)a$le 10( A$out &0' of neonates with pol"c"themia de#elop one or more s"mptoms(
2owe#er, most of these s"mptoms are non,specific and ma" $e related to the underl"ing
conditions rather than due to pol"c"themia per se(
#creening for polycythemia
Screening should $e done for pol"c"themia in certain high,ris* groups .)a$le 20( >e
recommend screening in high,ris* neonates at 2 hours of age( A normal #alue at 2 hours
of age .hematocrit 7%&'0 does not merit an" further screening unless the infant is
s"mptomatic( 2ematocrit #alues ?%&' at 2 hours of age merit repeat screening at 12 and
21 hours( An" infant with clinical features suggesti#e of pol"c"themia should $e
in#estigated for the same(
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AIIMS- NICU protocols 2010
*apillary vs+ venous hematorit
-apillar" hematocrit measurements are unrelia$le and highl" su$@ect to #ariations in
$lood flow( -apillar" hematocrits are significantl" higher than #enous hematocrits( )his
difference is e#en more apparent in infants recei#ing large placental transfusion
9
(
-apillar" samples ma" $e used for screening, $ut all high #alues should $e confirmed $"
a #enous sample for the diagnosis of pol"c"themia(
Methods of hematorit analysis
)he two a#aila$le methods are
Atomated hematolog! anal!"er! )his calculates the hematocrit from a direct
measurement of mean cell #olume and the hemoglo$in(
M#cro-centr#fge! Alood is collected in heparini=ed micro,capillaries .110mm length and
1,2mm internal diameter0 and centrifuged at 10,000,1&,000 rounds per minute .rpm0 for
9,& minutes( Plasma separates and the pac*ed cell #olume is measured to gi#e the
hematocrit( An automated anal"=er gi#es lower #alues as compared to hematocrits
measured $" the centrifugation method
10
( Most of the reported data on pol"c"themia is on
centrifuged hematocrits(
Management
Aefore a diagnosis of pol"c"themia is considered, it is mandator" to e+clude deh"dration(
If the $irth weight is *nown, re,weighing the $a$" and loo*ing for e+cessi#e weight loss
would help in the diagnosis of deh"dration( If this is present, it should $e corrected $"
increasing fluid inta*e( )he hematocrit should $e measured again after correction of
deh"dration( Bnce a diagnosis of pol"c"themia is made, associated meta$olic pro$lems
including h"pogl"cemia should $e e+cluded(
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AIIMS- NICU protocols 2010
Management of pol"c"themia is dependent upon two factors .Cigure0!
1( Presence of s"mptoms suggesti#e of pol"c"themia and8or
2( A$solute #alue of hematocrit
.a0 #ymptomatic polycythemia
)he definiti#e treatment for pol"c"themia is to perform a partial e+change transfusion
.P)0( P) in#ol#es remo#ing some of the $lood #olume and replacing it with fluids so
as to decrease the hematocrit to a target pac*ed cell #olume of &&'( Collowing partial
e+change transfusion, s"mptoms li*e @itteriness ma" persist for 1,2 da"s despite the
hematocrit $eing lowered to ph"siological ranges(
)he #olume of $lood to $e e+changed is gi#en $" the formula shown in the $o+(
4olume to $e D Alood #olumeE + .B$ser#ed hematocrit F Desired hematocrit0
e+changed
B$ser#ed hematocrit
$%lood &olme #s est#mated to be '0-(0 ml)*g #n term bab#es and (0-100 ml)*g
#n preterm bab#es
As a rough guide, the #olume of $lood to $e e+changed is usuall" 20 ml8*g(
+,-. per#pheral &s. mb#l#cal rote
Partial e+change transfusion ma" $e carried out #ia the peripheral route or the central
route( In the former, $lood is withdrawn from the peripheral arterial line and replaced
simultaneousl" with saline #ia the #enous line( In the central route, the um$ilical #enous
catheter is used for withdrawing $lood while the same amount of saline is replaced
through a peripheral #ein( Alternati#el", the um$ilical #enous catheter ma" $e used $oth
for withdrawal of $lood and replacement with saline( )wo s"stematic re#iews .including
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AIIMS- NICU protocols 2010
a -ochrane re#iew0 ha#e shown that the partial e+change transfusion through um$ilical
route ma" $e associated with increased ris* of necroti=ing enterocolitis
11,12
(
+,-. fl#ds to be sed
-r"stalloids such as normal saline .NS0 or ringerGs lactate .5:0 are preferred o#er
colloids $ecause the" are less e+pensi#e and easil" a#aila$le, produce a similar reduction
in hematocrit as colloids
19,11
, and do not ha#e the ris* of transfusion associated infections(
Moreo#er, adult plasma has $een shown to increase the $lood #iscosit" when mi+ed with
fetal er"throc"tes( /e se onl! normal sal#ne for part#al e0change transfs#on.
$b% Asymptomatic polycythemia:
)he line of management in infants with as"mptomatic pol"c"themia depends upon their
hematocrit #alues(
i( 1ematocr#t 2345. )hese infants are usuall" managed a partial e+change
transfusion(
ii( 1ematocr#t between 305 and 345. -onser#ati#e management with h"dration
ma" $e tried in infants with hematocrit of ;0 to ;&'( An e+tra fluid ali3uot of
20 ml8*g ma" $e added to the dail" fluid re3uirements( )he additional fluid ma"
$e ensured $" either enteral .super#ised feeding0 or parenteral route .I4 fluids0(
)he rationale for this therap" is hemodilution and the resultant decrease in
#iscosit"( 2owe#er, li$eral fluid therap" ma" $e associated with pro$lems
especiall" in preterm neonates(
iii( 1ematocr#t between 645 and 305. )he" onl" need monitoring for an" s"mptoms
of pol"c"themia and re,estimation of hematocrit( Curther management depends
upon the repeat hematocrit #alues(
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AIIMS- NICU protocols 2010
&'idence for management of polycythemia
Partial e+change transfusion re#erses the ph"siological a$normalities associated with the
pol"c"themiaFh"per#iscocit" s"ndrome( It impro#es capillar" perfusion, cere$ral $lood
flow and cardiac function( 2owe#er, there is #er" little data to suggest that P) impro#es
long term outcome in patients with pol"c"themia( )he -ochrane re#iew , pu$lished this
"ear .20100 , concludes that Hthere are no pro#en clinicall" significant short or long,term
$enefits of P) in pol"c"themic new$orn infants who are clinicall" well or who ha#e
minor s"mptoms related to h"per#iscosit"6 P) ma" lead to an increase in the ris* of
N-
12
(I 2owe#er, as the re#iew authors pointed out, the data regarding de#elopmental
outcomes are e+tremel" imprecise due to the large num$er of sur#i#ing infants who were
not assessed and, therefore, the true ris*s and $enefits of P) are unclear( It is possi$le
that the underl"ing etiolog" of pol"c"themia is a more important determinant of ultimate
outcome( /i#en the uncertaint" regarding the long term outcomes, it is prefera$le to do
partial e+change transfusion in s"mptomatic infants with hematocrit of ?%&' and in
as"mptomatic neonates with hematocrit of ?;&'(
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AIIMS- NICU protocols 2010
(eferences
1( Mac*intosh )C, >al*ar -2( Alood #iscosit" in the new$orn( Arch Dis -hild 19;96
1<! &1;,&9(
2( Phi$$s 52! Neonatal Pol"c"themia( In 5udolph AA.ed0! Pediatrics, 1%
th
ed( New
Jor*! Appleton -entur" -rofts, 199;, pp 1;9(
9( 5amamurth" 5S, Arans >J Neonatal Pol"c"themia I( -riteria for diagnosis and
treatment( Pediatrics 19<16 %<!1%<,;1(
1( >irth C2, /old$erg K, :u$chenco :B! Neonatal h"per#iscocit" I( Incidence(
Pediatrics 19;96 %9! <99,%(
&( Ste#ens K, >irth C2( Incidence of neonatal h"per#iscosit" at sea le#el( Pediatrics
19<069;!11<
%( Aada 2S, Korones SA, Pourc"rous M, >ong SP, >ilson >M9rd, Kolni 2>, Cord
D:( As"mptomatic s"ndrome of pol"c"themic h"per#iscocit"! effect of partial
e+change transfusion( L( Pediatr 19926 120! &;9,<&
;( Shohat M, Merlo$ P, 5eisner S2! Neonatal Pol"c"themia( I( arl" diagnosis and
incidence relating to time of sampling( Pediatrics 19<16 ;9!;,10(
<( Shohat M, 5eisner S2, Mimouni C, Merlo$ P( Neonatal pol"c"themia II( Definition
related to time of sampling( Pediatrics 19<16 ;9!11,9(
9( Bh >( Neonatal pol"c"themia and h"per#iscosit"( Pediatr -lin North Am
19<%699!&29,92
10( /old$erg K, >irth C2, 2athawa" >, /uggenheim MA, Murph" L5, Araithwaite
>5, :u$chenco :B( Neonatal h"per#iscocit" II( ffect of partial e+change
transfusion( Pediatrics 19<26 %9! 119,2&(
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11( Dempse" M, Aarrington K( Short and long term outcomes following partial
e+change transfusion in the pol"c"thaemic new$orn! a s"stematic re#iew(
Arch Dis -hild Cetal Neonatal d( 200%691!C2,%(
12( B=e* , Soll 5, Schimmel MS( Partial e+change transfusion to pre#ent
neurode#elopmental disa$ilit" in infants with pol"c"themia( -ochrane Data$ase S"st
5e#( 2010 Lan 206.10!-D00&0<9(
19( Deorari AK, Paul 4K, Shreshta :, Singh M( S"mptomatic neonatal pol"c"themia!
-omparison of partial e+change transfusion with saline #ersus plasma( Indian Pediatr
199&692!11%;,;1
11( de >aal KA, Aaerts >, Bffringa M( S"stematic re#iew of the optimal fluid for
dilutional e+change transfusion in neonatal pol"c"thaemia( Arch Dis -hild Cetal
Neonatal d( 200%691!C;,10(
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)able *. "linical feat!res ascribed to polycythemia and hyper'iscosity
"entral ner'o!s system
arl"! 2"potonia and sleepiness, irrita$ilit", @itteriness , sei=ures and infarcts
:ate! motor deficits, lower achie#ement and IM scores
Metabolism
2"pogl"cemia
Laundice
2"pocalcemia
+eart and l!ngs
)ach"cardia, tach"pnea, respirator" distress
-"anosis, plethora
-hest radiograph"! cardiomegal", pulmonar" plethora
chocardiograph"! increased pulmonar" resistance, decreased cardiac output
,astrointestinal tract
Poor suc*, #omiting
Ceed intolerance F a$dominal distenstion
Necroti=ing enterocolitis
-idneys
Bliguria .depending on $lood #olume0
)ransient h"pertension
5enal #ein throm$osis
+ematology
Mild throm$oc"topenia
)hrom$osis .rare0
Miscellaneo!s
Peripheral gangrene,
Priapism,
)esticular infarction
)able .. #creening for polycythemia
Screening is recommended for the following!
.a0 Small for gestational age .S/A0 infants
.$0 Infants of dia$etic mothers .IDM0
.c0 :arge for gestational age .:/A0 infants
.d0 Monochorionic twins especiall" the larger twin
.e0 Morphological features of growth retardation(
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/ig!re: Algorithm for management of polycythemia
-apillar" hematocrit ?%&'
-onfirm with #enous hematocrit
+clude deh"dration
-hec* weight loss
S"mptomatic As"mptomatic
P&) P-4 %&,;0 P-4 ?;&'
-onsider
h"dration P&)
+,-. part#al e0change transfs#on
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P-4 ;0,;&
Monitoring
Acti#el" loo* for
s"mptoms