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ANGELES UNIVERSITY FOUNDATION
GRADUATE SCHOOL
Master of Arts in Nursing
A Case Stud On
ACUTE MYELOGENOUS LEUKEMIA
!RESENTED TO
ANNA LYN M"!AANO# RN# MAN
!rofessor# Con$e%ts I
!resented &
E!IFANIA !" GOGOLIN# RN
MAN Student
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I. INTRODUCTION
The terms malignant and cancer!s descri"e cells that are gr#ing and
$rli%erating in a disrderl&' chatic %ashin. In ad!lts' cancer !s!all& cc!rs in the %rm
% a slid t!mr. In children' the mst %re(!ent t&$e % cancer is that % an immat!re cell
)ergr#th' r le!*emia +McCance , -e!ther'//01.
Ac!te m&elgen!s le!*emia +AML1 is res!lts %rm a de%ect %rm a
hemat$eitic stem cells that di%%erentiates int all m&elid cells2 mnc&tes'
gran!lc&tes +ne!tr$hils' "as$hils' esin$hils1' er&thrc&tes and $latelets +Smelt3er'
et al.' //01. AML cm$rises a$$r4imatel& 556 % the cases r le!*emia in childhd
in the United States' #ith a$$r4imatel& 0// children diagnsed #ith AML ann!all&.
Unli*e ALL' the incidence % AML is cnstant %rm "irth thr!gh!t the %irst 5/
&ears % li%e #ith a slight $ea* in late adlescence. Internatinall&' AML is mre
cmmnl& diagnsed in de)el$ed c!ntries. AML is als mre cmmn in men than in
#men. There is n *n#n relatinshi$ "et#een race and the incidence % AML. It #as
"ser)ed th!gh' that AML is mre cmmn in #hites than in ther $$!latins. The
di%%erence is e)en mre a$$arent in lder $atients. 7re)alence increases #ith age.
-#e)er' this disease a%%ects all age gr!$s +Seiter' //81.
The $rgnsis is highl& )aria"le. 7atient age ma& "e a %actr9 $atients #h are
&!nger ma& s!r)i)e %r : &ears r mre a%ter diagnsis % AML. -#e)er.' $atients #h
are lder r ha)e a mre !ndi%%erentiated %rm % AML tend t ha)e #rse $rgnsis.
7atients #ith s!$$rti)e care !s!all& s!r)i)e less than 5 &ear' #ith death !s!all& a res!lt
% in%ectin and hemrrhage +American Cancer Sciet&' //81.
AML can $resent #ith relati)e s!"tle' nns$eci%ic s&m$tms s!ch anre4ia'
malaise' irrita"ilit&' and #eight lss. As the disease $rgresses' the& $resent #ith signs
and s&m$tms assciated #ith the %ail!re % nrmal hemat$iesis' #hich res!lts %rm
the re$lacement % nrmal marr# cells "& malignant cells. The child ma& e4hi"it %atig!e
$allr' and decreased acti)it& d!e t anemia9 eas& "r!ising and "leeding assciated #ith
thrm"c&t$enia9 r %e)er and in%ectin secndar& t ne!tr$enia. ;ne $ain and
arthralgias are less
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ALL. Massi)e he$ats$lenmegal& is !ncmmn e4ce$t in in%ants #ith AML +Seiter'
//81.
;ne marr# as$iratin and "i$s& are the de%initi)e diagnstic tests.
A$$r4imatel& 5/6 % $atients #ith ac!te le!*emia d nt ha)e circ!lating "lasts atdiagnsis. One r mre % the %ll#ing la" tests ma& "e !sed t diagnse AML and=r
t determine the s$eci%ic s!"t&$e % AML. Sam$les % "ld' "ne marr#' r CS> are
l*ed at !nder a micrsc$e "& a $athlgist and ma& "e re)ie#ed "& the $atient?s
hematlgist=nclgist. ;ased n the cells? si3e' sha$e' and ther traits' dctrs can
classi%& them int s$eci%ic cell t&$es. A *e& element % this classi%icatin is #hether the
cells l* li*e nrmal cells % circ!lating "ld +mat!re1 r lac* %eat!res % nrmal "ld
cells +immat!re1.
The $ercentage % cells in the "ne marr# r "ld that are "lasts +immat!re1 is
$artic!larl& im$rtant. -a)ing at least /6 "lasts in the marr# r "ld is generall&
re(!ired %r a diagnsis % ac!te m&elid le!*emia. AML can als "e diagnsed i% the
"lasts ha)e a chrmsme change that cc!rs nl& in a s$eci%ic t&$e % AML' e)en
th!gh the "last $ercentage desn?t reach /6. In rder %r a $atient t "e cnsidered
t "e in remissin a%ter treatment' the "last $ercentage m!st "e n higher than :6.
Additinal tests that are !sed t cn%irm the diagnsis % AML are as %ll#s.
C&tchemistr& tests in)l)e e4$sing cells t chemical stains +d&es1 that react #ith nl&
sme t&$es % le!*emia cells. >l# c&tmetr& n the ther hand is a techni(!e #hich is
%ten !sed t l* at the cells %rm "ne marr# and "ld sam$les. It is )er& acc!rate
in determining the e4act t&$e % le!*emia. Related tests' called imm!nhistchemistr&
tests' can "e !sed t detect these s!"stances #hen )ie#ing cells !nder a micrsc$e.
These tests are !sed %r i''uno%(enot%ingIS-1 is a $rced!re similar t c&tgenetic testing. It can %ind mst
chrmsme changes +s!ch as translcatins1 that are )isi"le !nder a micrsc$e in
standard c&tgenetic tests' as #ell as sme changes t small t "e seen #ith !s!al
c&tgenetic testing. @er& sensiti)e DNA tests s!ch as $l&merase chain reactin +7CR1
tests can als %ind translcatins t small t "e seen !nder a micrsc$e' e)en i% )er&
%e# le!*emia cells are $resent in a sam$le. These tests ma& als "e !sed a%ter
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treatment t %ind small n!m"ers % le!*emia cells that ma& nt "e )isi"le !nder a
micrsc$e.
There are als se)eral imaging st!dies that might "e dne in $e$le #ith AML'
"!t the& are dne mre %ten t l* %r in%ectins r ther $r"lems' rather than t l*%r the le!*emia itsel%. ;eca!se le!*emia des nt !s!all& %rm )isi"le t!mrs' imaging
tests are % limited )al!e. In sme cases imaging st!dies ma& "e dne t hel$
determine the e4tent % the disease' i% it is th!ght it ma& ha)e s$read "e&nd the "ne
marr# and "ld. R!tine chest 4
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MDS=M7D "!t #ith d&s$lasia in at least :/6 % cells in r mre lineages12 1 AML and
MDS' thera$& related + Al*&lating agent r radiatinancni anemia' and ne!r%i"rmatsis. Us!all&' these $atients
de)el$ AML d!ring childhd9 rarel&' sme ma& $resent in &!ng ad!lthd. Mres!"tle genetic disrders' incl!ding $l&mr$hisms % en3&mes that meta"li3e
carcingens' als $redis$se $atients t AML. Se)eral st!dies demnstrate a
relatinshi$ "et#een radiatin e4$s!re and le!*emia. Earl& radilgists +$rir t
a$$r$riate shielding1 #ere %!nd t ha)e an increased li*elihd % de)el$ing
le!*emia. 7ersns #h sm*e ha)e a small "!t statisticall& signi%icant +dds rati' 5.:1
increased ris* % de)el$ing AML. In se)eral st!dies' the ris* % AML #as slightl&
increased in $e$le #h sm*ed cm$ared #ith thse #h did nt sm*e. E4$s!re t
"en3ene is assciated #ith a$lastic anemia and $anc&t$enia. These $atients %tende)el$ AML.
Treatment % AML is di)ided int t# stages2 ind!ctin % remissin and $st
remissin cnslidatin r intensi%icatin. Lng
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>r c!rrent trends and iss!es' a n!m"er % ne# dr!gs are c!rrentl& in clinical
trials. The t# mst $rmising areas are %arnes&l trans%erase inhi"itrs and dr!gs that
target FLT-. >arnes&l trans%erase inhi"itrs target ras' a mlec!le that is in)l)ed in the
$ath$h&silg& % )ari!s hematlgic malignancies. T# dr!gs % this t&$e' ti$i%arni"
and lna%arni"' are c!rrentl& !nderging clinical trials. Earl& res!lts ha)e demnstrated
res$nses in 5:
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S(ort.ter' o/0e$ti1es2
5. Recgni3ed the cnditin that are threatening t his health
. A$$reciate all the health teaching and c$erate #ith the n!rsing
inter)entins dne %r his cm%rt
. A$$l& meas!res ta!ght t $re)ent the #rsening % his cnditin
Long.ter' o/0e$ti1es2
5. Maintain a health& li%est&le that is cntri"!tr& t health maintenance and
$ersnal de)el$ment
. In time' s!stain li%est&le that "est ens!re $tim!m le)el % %!nctining
.;raden *n#ledge regarding sel%
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5. Im$r)e= dee$en the n!rses !nderstanding' *n#ledge and s*ills #ith the
disease.
. 7rmte e%%ecti)e n!rsing care t all the clients that ma& "e handled in the
%!t!re #ith the same disease cnditin.
. A$$l&=share the learning %rm the e4$erience t clleag!es' st!dents' clients ,
demnstrate a caring rle as a n!rse has.
0. Identi%& areas related t illness in children that c!ld "ene%it %rm additinal
n!rsing research r a$$licatin % e)idenceili$in citi3en' "a$ti3ed as a Rman Cathlic'
and a resident % 0 ; Ri3al St.' La$a3 Tarlac. -e #as admitted in a tertiar&
g)ernment hs$ital in Tarlac' last Oct"er ' // #ith chie% cm$laints %
e$ista4is' g!m "leeding and l# "ld and $latelet c!nt.
The ;i" %amil& is a n!clear t&$e % %amil& cm$sing % three mem"ers9
his mther' %ather and Tt& ;i" himsel%. -is %ather and mther are "th 5
&ears % age. -is %ather %rmerl& #r*s as a sec!rit& g!ard at a %irm in Manila and
is c!rrentl& sel%
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"!ilding that is in "ad cnditin "eca!se % a %ire incident lng ag. The residents
are als cm$laining % %!l smelling air "eca!se % a $!ltr& lcated in%rnt %
their )icinit&. Their tilet is #ater sealed and the& ha)e #ater $!m$ as the s!rce
% #ater s!$$l&. Accrding t the mther their $lace is small "!t clean and #ell
maintained' #ith a small li)ing rm' ne "edrm #here the %amil& slee$s
tgether' a *itchen and a cm%rt rm. Their #ater is "iled "e%re it is !sed "&
the %amil&. Mrs. ;i" als mentined that the %ather % Tt& ;i" ccasinall&
sm*es and drin*s #ith his $eers. The mther n the ther hand is %nd %
s#eets and %ds rich in car"h&drates' es$eciall& d!ring her $regnanc&. She
als admits that at an earl& age' the& alread& %%ered Tt& ;i" s%tdrin*s r
car"nated drin*s %r re%reshment.
As %r the %amilies health "elie% and $ractices' the %amil& "elie)e in se)eral
s!$erstitins #hich a%%ect their F!dgment and decisins smetimes. The ;i"
%amil& "elie)es in %aith healers and Jmanghihilt es$eciall& #hen smene get
%ract!res r s$rains. The& als ma*e !se % her"al medicines t heal se)eral
cnditins #hich the& cntem$late t "e e%%ecti)e and at the same time
ine4$ensi)e. The& als $ractice sel%
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his mther. -e #as %irst %ed "& %rm!lated mil* *n#n as &ona' then a%ter t#
mnths #as changed t rice mil* r #hat is cmmnl& called as 3a'4 deri)ed
%rm "iled rice. A%ter that Tt& ;i" #as %ed in cm"inatin % &ear &rand# a'
and Mi5o. -e #as than intrd!ced t eating 5uga6' mashed carrt' $tat and
s(!ash. It #as als mentined that e)en "e%re reaching his %irst "irthda&' the
$arents alread& gi)e s%tdrin*s %r Tt& ;i" t drin*. -e is cm$letel&
imm!ni3ed at the lcal health center in La$a3' and #as cmmnl& nted t "e
)er#eight d!ring his mnthl& chec* !$s.
Tt& ;i" "eing three &ears % age is a tddler !nder the A!tnm& @S
shame and D!"t in Eri* Eric*sns 7s&chscial De)el$ment ther&' #here
the child %c!ses n sel%AMILY
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Legend:
Red < -&$ertensin=-eart Disease Yell#T!"erc!lsis
Green Asthma
Figure 1. Genogram
The $aternal grand$arents % Tt& ;i" are *n#n t ha)e h&$ertensins and
all % his !ncle and a!nts incl!ding his %ather ha)e asthma. In his maternal side' Tt&
;i"s grand%ather ha)e alread& $assed a#a& d!e t str*e. -is grandmther n the
ther hand is still li)ing "!t has a histr& % t!"erc!lsis. There are n *n#n disease
r illness that ha)e "e%allen her a!nts and !ncles in his mther side. All are in gd
health as % the $resent.
D. -ISTORY O> 7AST ILLNESS
Tt& ;i" has a histr& % "eing "r!ght at the emergenc& de$artment % a
district hs$ital in La$a3 Tarlac se)eral times at an earl& age % 5
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March ' //' Tt& ;i" #as r!shed t a tertiar& g)ernment hs$ital in
Tarlac Cit& a%ter "eing s!ggested "& the dctrs in the district hs$ital in La$a3 #hen
res!lts % his $latelet and "ld cm$nents #ere %!nd t "e "el# nrmal and in need
% immediate "ld trans%!sin. Se)ere $aleness and a mar*ed decreased %
hemgl"in c!nt % /. 7-YSICAL EAMINATION
Oct"er ' //' Emergenc& Rm 7ediatrics resident
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Genera! "ur#e$
Seen $atient n "ed' #ith I@> % D: /. NaCl at :/ cc le)el in%!sing n his le%t ce$halic
)ein. 7atient is cr&ing and restless9 #ith "d& malaise9 increased res$iratr& e%%rt #ithminimal m)ement9 needs %!ll assistance t d ADL. -e #as #earing a shirt and a $air
% $aFamas. N a$$etite "!t #ith increased #ater inta*e +0
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a. General2 n di%%ic!lt& #ith nec* m"ilit&". ;ld )essels2 slight distensin % F!g!lar )ein is nted.c. Trachea2 n masses nted' and n th&rid gland enlargement.
Integumentar$:a. S*in2 $ale in clr' #ith #arm s*in' $etechial rashes n "th $alm % the
hands' #ith min!te hematma nted n Fint areas n "th !$$er and
l#er e4tremit&'". Nails2 shrt and clean9 #ith ca$illar& re%ill % secnds' a"sence %
cl!""ing and disclratinc. -air2 #ith e(!al distri"!tin % hair
L$m+% NodesBith slightl& enlarged cer)ical l&m$h ndes
%estac*:a. Res$iratin2 #ith nta"le increase in res$iratr& rate' #ith slight
di%%ic!lt& in "reathing". L!ngs2 Rales a!sc!ltated %rm "th l!ng %ields.c. Chest2 #ith e(!al chest e4$ansin' #ith e(!al anteri$sterir
diameter' n a"nrmal retractin' circ!m%erence meas!ring 0 cm in
#idth. @isi"le heart "eat ntedd. ;ac*2 #ith min!te hematma at the "ac* , $rminent at sh!lder
"ladesardio#ascu!ar:
a. -eart2 $siti)e S5 and S s!nds' n m!rm!r". 7eri$heral $!lses2 negati)e $al$itatins #ith nrmal rate
Gastrointestina!:a. A"dmen2 #ith rigid a"dmen9 a"dminal girth % 8 cm9 n striae r
rashes nted' n $rtr!ding mass r discharge n the !m"ilic!s' nascites' nrmacti)e "#el s!nds' n re"!nd tenderness' n
tenderness n *idne&s and li)er !$n $al$atin.uscu!os*e!eta!:
a. U$$er E4tremities2 e(!al in si3e' s&mmetrical in sha$e #ith n e)identdistensin % )eins' and e(!al $!lses' n edema nted. 7ale #ith$etechial rashes n "th $alms % the hands and min!te hematman Fint areas.
". L#er E4tremities2 e(!al in si3e' s&mmetrical in sha$e #ith n e)identdistensin % )eins' and e(!al $!lses. Bith enlarged "leedinghematma at the le%t %t meas!ring a"!t 0
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s#all#ing the %d1. Bith increased #ater inta*e +0
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". Nails2 shrt and clean9 #ith ca$illar& re%ill % secnds' a"sence %cl!""ing and disclratin
c. -air2 #ith e(!al distri"!tin % hairL$m+% Nodes
Bith slightl& enlarged cer)ical l&m$h ndes%estac*:
a. Res$iratin2 #ith nta"le increase in res$iratr& rate' #ith slightdi%%ic!lt& in "reathing
". L!ngs2 Rales a!sc!ltated %rm "th l!ng %ields.c. Chest2 #ith e(!al chest e4$ansin' #ith e(!al anteri$sterir
diameter' n a"nrmal retractin' circ!m%erence meas!ring 0 cm in#idth. @isi"le heart "eat nted
d. ;ac*2 #ith min!te hematma at the "ac* , $rminent at sh!lder"lades
ardio#ascu!ar:a. -eart2 $siti)e S5 and S s!nds' n m!rm!r". 7eri$heral $!lses2 negati)e $al$itatins #ith nrmal rate
Gastrointestina!:
a. A"dmen2 #ith rigid a"dmen9 a"dminal girth % 8 cm9 n striae rrashes nted' n $rtr!ding mass r discharge n the !m"ilic!s' nascites' nrmacti)e "#el s!nds' n re"!nd tenderness' ntenderness n *idne&s and li)er !$n $al$atin.
uscu!os*e!eta!:a. U$$er E4tremities2 e(!al in si3e' s&mmetrical in sha$e #ith n e)ident
distensin % )eins' and e(!al $!lses' n edema nted. 7ale #ith$etechial rashes n "th $alms % the hands and min!te hematman Fint areas.
". L#er E4tremities2 e(!al in si3e' s&mmetrical in sha$e #ith n e)identdistensin % )eins' and e(!al $!lses. Bith enlarged "leedinghematma at the le%t %t meas!ring a"!t :
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P%$sica! assessment:&ead and Face:
a. -air2 #ith %ine shrt "lac* hair". Scal$2 n e)idence % dandr!%% r %la*ing9 n tenderness % the scal$
nted.c. S*!ll2 nrmal s*!ll cn%ig!ratin' n a"nrmal ele)atin r de$ressin.
d. >ace2 s&mmetrical in sha$e' n tenderness !$n $al$atin and ncre$it!s heard #hile m)ing the Fa#
'$es:a. General2 e&es are s&mmetrical' #ith nted hematma $erir"ital
edema +racn
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a. Res$iratin2 #ith nta"le increase in res$iratr& rate' #ith slightdi%%ic!lt& in "reathing
". L!ngs2 Rales a!sc!ltated %rm "th l!ng %ieldsc. Chest2 #ith e(!al chest e4$ansin' #ith e(!al anteri$sterir
diameter' n a"nrmal retractin' circ!m%erence meas!ring 0 cm in#idth. @isi"le heart "eat nted
d. ;ac*2 #ith min!te hematma at the "ac* , $rminent at sh!lder"lades
ardio#ascu!ar:a. -eart2 $siti)e S5 and S s!nds' n m!rm!r". 7eri$heral $!lses2 negati)e $al$itatins #ith nrmal rate
Gastrointestina!:a. A"dmen2 #ith a"dminal distentin' a"dminal girth % 80 cm9 n
striae r rashes nted' n $rtr!ding mass r discharge n the!m"ilic!s' n ascites' nrmacti)e "#el s!nds' n re"!ndtenderness' n tenderness n *idne&s and li)er !$n $al$atin.
uscu!os*e!eta!:a. U$$er E4tremities2 e(!al in si3e' s&mmetrical in sha$e #ith n e)ident
distensin % )eins' and e(!al $!lses' n edema nted. 7ale #ith$etechial rashes n "th $alms % the hands and min!te hematman Fint areas.
". L#er E4tremities2 e(!al in si3e' s&mmetrical in sha$e #ith n e)identdistensin % )eins' and e(!al $!lses. Bith enlarged "leedinghematma at the le%t %t meas!ring a"!t 8 % 7lain NSS 5 liter at // cc
le)el in%!sing n his le%t ce$halic )ein' #ith nging 5 !nit % 7R;C SQ:50 as side dri$'
+1 $allr' restless and irrita"le' "arel& *n# the $e$le handling him incl!ding his
$arents' generali3ed "d& #ea*ness9 #ith e4ertinal "reathing.
P%$sica! assessment:&ead and Face:
a. -air2 #ith %ine shrt "lac* hair". Scal$2 n e)idence % dandr!%% r %la*ing9 n tenderness % the scal$
nted.
c. S*!ll2 nrmal s*!ll cn%ig!ratin' n a"nrmal ele)atin r de$ressin.d. >ace2 s&mmetrical in sha$e' n tenderness !$n $al$atin and n
cre$it!s heard #hile m)ing the Fa#'$es:
a. General2 e&es are s&mmetrical' #ith nted hematma $erir"italedema +racn
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d. E&elids and E&elashes2 n e)idence % in%ectin' n nd!les andtenderness nted' #ith minimal s#elling % l#er e&elid.
e. Lacrimal $!ncta2 n nd!les and tenderness nted.%. Sclerae2 #hitish sclerae #ith e)ident ca$illaries' n Fa!ndice nted.g. CnF!ncti)a2 $ale cnF!ncti)a.h. @isin2 #ith gd )is!al ac!it&
'ars:a. E4ternal2 s&mmetrical a$$earance % $inna' n lesins r a"nrmal
discharges r s#elling.". Internal2 n discharges ntedc. Cranial Ner)e @III2 n hearing im$airment % "th ears
Nose:S&mmetrical nares' n discharge' e4!dates' n "leeding nr s#elling
nted.out% and )%roat:
a. M!th=Li$s2 dr& , crac*ed' #ith dried #!nd n "th li$s' #ith mildsres n ral ca)it&' #ith n "leeding nted.
". Tng!e2 nt assessed
c. S%t $alate and !)!la2 nt assessedd. Thrat2 nt assessed
Nec*:a. General2 n di%%ic!lt& #ith nec* m"ilit&". ;ld )essels2 distended F!g!lar )ein.c. Trachea2 n masses nted' and n th&rid gland enlargement.
Integumentar$:a. S*in2 $ale in clr' #ith #arm s*in' $etechial rashes n "th $alm %
the hands' #ith min!te hematma nted n Fint areas n "th !$$er
and l#er e4tremit&". Nails2 shrt and clean9 #ith ca$illar& re%ill % :
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a. U$$er E4tremities2 e(!al in si3e' s&mmetrical in sha$e #ith n e)identdistensin % )eins' and e(!al $!lses' n edema nted. 7ale #ith$etechial rashes n "th $alms % the hands and min!te hematman Fint areas.
". L#er E4tremities2 e(!al in si3e' s&mmetrical in sha$e #ith n e)identdistensin % )eins' and e(!al $!lses. Bith enlarged "leeding
hematma at the le%t %t meas!ring a"!t 8
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Medical $rced!res ha)e "een de)el$ed t e4amine the "ne marr# +"ne marr#
as$iratin and "i$s&1 % $atients and als t trans%er nrmal stem cells %rm a dnr
int a reci$ient +"ne marr# trans$lantatin1.
STRUCTURE
Red marr# cnsists $rimaril& % a lse' s%t net#r* % "ld )essels and $rtein
%i"ers inters$ersed #ith de)el$ing "ld cells. The "ld )essels are termed the
)asc!lar cm$nent' and the $rtein %i"ers and de)el$ing "ld cells cllecti)el& are
re%erred t as the strma' r the e4tra)asc!lar cm$nent. The $rtein %i"ers crisscrss
the marr#' %rming a mesh#r* that s!$$rts the de)el$ing "ld cells cl!stered in
the s$aces "et#een the %i"ers.
Red marr# cntains a rich "ld s!$$l&. Arteries trans$rt "ld cntaining 4&gen
and n!trients int the marr#' and )eins rem)e "ld cntaining car"n di4ide and
ther #astes. The arteries and )eins are cnnected "& ca$illaries' "ld )essels that
"ranch thr!gh!t the marr#. In )ari!s $laces' the ca$illaries "alln !t' %rming
n!mer!s thin' "ld
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Gra$s natom$ i!!ustration o3 ce!!s in bone marro. (From Ne 4or!d'nc$c!o+edia
>UNCTIONS
Red marr# $rd!ces all % the "d&s "ld cellsred "ld cells' #hite "ld cells'
and $latelets. Red "ld cells in the circ!latr& s&stemtrans$rt 4&gen t "d& tiss!es
and car"n di4ide a#a& %rm tiss!es. Bhite "ld cells are critical %r %ighting "acteria
and ther %reign in)aders % the "d&. 7latelets are essential %r the %rmatin % "ld
clts t heal #!nds.
Bithin red "ne marr#' all "ld cells riginate %rm a single t&$e % cell' called a
hemat$ietic stem cell. Stim!lated "& hrmnes and gr#th %actrs' these stem cells
di)ide t $rd!ce immat!re' r $rgenitr "ld cells. Mst % these $rgenitr cells
remain in the strma and ra$idl& !nderg a series % cell di)isins' $rd!cing either red
"ld cells r #hite "ld cells. At an& ne time' the strma cnsists largel& %
$rgenitr cells in )ari!s stages % de)el$ment. At the a$$r$riate de)el$mental
stage' the %resh' ne# cells s(!ee3e thr!gh the #alls % the ca$illaries. >rm there' the
cells lea)e the "ne and enter the "d&s circ!latr& s&stem. Sme $rgenitr cells
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migrate t the sin!sids' #here the& $rd!ce $latelets' #hich als tra)el t the
circ!latr& s&stem )ia the ca$illaries.
Alth!gh stem cells are relati)el& rarea"!t 5 in e)er& 5/'/// marr# cells is a stem
cellthe& t&$icall& $rd!ce the %rer!nners % an estimated millin red cells $ersecnd and "illin $latelets $er da&. -#e)er' i% signi%icant am!nts % "ld are lst
r ther cnditins red!ce the s!$$l& % 4&gen t tiss!es' the *idne&s secrete the
hrmne er&thr$ietin. This hrmne stim!lates stem cells t $rd!ce mre red "ld
cells. T %ight %% in%ectin' hrmnes cllecti)el& termed $o5on sti'u5ating gro6t(
fa$tors are released "& the imm!ne s&stem. These hrmnes stim!late the stem cells t
$rd!ce mre in%ectin
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Note t(at so'e $o'%5e7it is o'itted fro' t(e diagra'" L'%(o$tes $o'e fro'
8L'%(oid8 5ine# 6(ereas granu5o$tes# 'ono$tes# 'ega9aro$tes# and ert(ro$tes
$o'e fro' 8Me5oid8 5ine" A'ong 'e5oid $e55s# granu5o$tes and 'ono$tes(a1e a
$o''on %re$ursor# 8CFU.GM8"
-emat$iesis cc!rs in the "ne marr#. The degree and lcatin % "ne
marr# acti)it& )aries de$ending n the age and health stat!s % &!r $atient. Bithin the
"ne marr# there is a $l!ri$tent stem cell. This stem cell is the JMther Cell r the
riginatr % all "ld cells. It has the a"ilit& t sel%
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;ld is a li(!id tiss!e. S!s$ended in the #ater& $lasma are se)en t&$es % cells
and cell %ragments. The Red ;ld Cells +R;Cs1 r er&thrc&tes ' 7latelets r
thrm"c&tes' and %i)e *inds % #hite "ld cells +B;Cs1 r le!*c&tes. Three *inds %
gran!lc&tes are as %ll#s2 Ne!tr$hils' Esin$hils' ;as$hils. T# *inds %
le!*c&tes #ith!t gran!les in their c&t$lasm are2 L&m$hc&tes and Mnc&tes'
Functions o3 t%e !ood
;ld $er%rms t# maFr %!nctins2 trans$rt thr!gh the "d& % 2 4&gen and
car"n di4ide' %d mlec!les +gl!cse' li$ids' amin acids1' ins +e.g.' Na' Ca'
-CO1' #astes +e.g.' !rea1' hrmnes' heat and de%ense % the "d& against in%ectins
and ther %reign materials. All the B;Cs $artici$ate in these de%enses.
Red Blood Cells (erythrocytes)
The mst n!mer!s t&$e in the "ld. Bmen a)erage a"!t 0. millin % these
cells $er c!"ic millimeter +mm9 #hich is the same as a micrliter VlW1 % "ld. Men
a)erage a"!t :.0 4 5/8$er Vl. These )al!es can )ar& )er (!ite a range de$ending n
s!ch %actrs as health and altit!de. +7er!)ians li)ing at 5'/// %eet ma& ha)e as man&
as . 4 5/8R;Cs $er Vl.1 R;C $rec!rsrs mat!re in the "ne marr# clsel& attached
t a macr$hage. The& man!%act!re hemgl"in !ntil it acc!nts %r sme /6 % the
dr& #eight % the cell. The n!cle!s is s(!ee3ed !t % the cell and is ingested "& the
macr$hage. N
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Mst % the irn in their hemgl"in is reclaimed %r re!se. The remainder % the heme
$rtin % the mlec!le is degraded int "ile $igments and e4creted "& the li)er. Sme
millin R;Cs die and are sca)enged "& the li)er each secnd. Red "ld cells are
res$nsi"le %r the trans$rt % 4&gen and car"n di4ide.
Hemoglobin
-emgl"in is a $rtein that is carried "& red cells. It $ic*s !$ 4&gen in the l!ngs
and deli)ers it t the $eri$heral tiss!es t maintain the )ia"ilit& % cells. -emgl"in is
made %rm t# similar $rteins that stic* tgether. ;th $rteins m!st "e $resent %r
the hemgl"in t $ic* !$ and release 4&gen nrmall&. One % the cm$nent $rteins
is called al$ha' the ther is "eta. ;e%re "irth' the "eta $rtein is nt e4$ressed. A
hemgl"in $rtein %!nd nl& d!ring %etal de)el$ment' called gamma' s!"stit!tes !$
!ntil "irth.
In ad!lt h!mans the hemgl"in +-"1 mlec!le cnsists % %!r $l&$e$tides2 t#
al$ha +X1 chains % 505 amin acids and t# "eta +1 chains % 508 amin acid. Each %
these is attached the $rsthetic gr!$ heme. There is ne atm % irn at the center %
each heme. One mlec!le % 4&gen can "ind t each heme.
Li*e all $rteins' the "l!e$rint %r hemgl"in e4ists in DNA +the material that
ma*es !$ genes1. Nrmall&' an indi)id!al has %!r genes that cde %r the al$ha $rtein'
r al$ha chain. T# ther genes cde %r the "eta chain. +T# additinal genes cde %r
the gamma chain in the %et!s1. The al$ha chain and the "eta chain are made in $recisel&
e(!al am!nts' des$ite the di%%ering n!m"er % genes. The $rtein chains Fin in
de)el$ing red "ld cells' and remain tgether %r the li%e % the red cell.
-emgl"in s&nthesis re(!ires the crdinated $rd!ctin % heme and gl"in.
-eme is the $rsthetic gr!$ that mediates re)ersi"le "inding % 4&gen "& hemgl"in.
Gl"in is the $rtein that s!rr!nds and $rtects the heme mlec!le.
'r$t%roc$tes and 6e!ated 7a!ues
6ed !ood e!! (6
Norma! 6ange: .9-9. ;109mm (ma!es
.2 -/. ;109mm (3ema!es
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Er&thrc&tes' r red "ld cells' riginate %rm a stem cell. @itamin ;5' %lic acid' irn'
and c$$er are essential in the %rmatin % er&thrc&tes. Er&thr$ietin is released "&
*idne&s in res$nse t h&$4emia #hich stim!lates the "ne marr# t $rd!ce red
"ld cells. T&$icall&' red "ld cells li)e a$$r4imatel& 5/ da&s. Bhen the red "ld
cells "ecme ld and damaged' the li)er' s$leen' and "ne marr# cleanse them %rm
the "ld.
6eticu!oc$te ount
Norma! 6ange: 0./-2./< o3 6s
Bhen released %rm the "ne marr# red "ld cells are slightl& immat!re and are
*n#n as retic!lc&tes. Retic!lc&tes mat!re int red "ld cells #ithin a %e# da&s.
&emog!obin
Norma! 6ange: 1-18 gdL (ma!es
12-19 gd! (3ema!es
-emgl"in is a $rteinr e4am$le2
I% &!r $atients hemgl"in is ./ g=dl' and &! gi)e him ne !nit % $ac*ed red "ld
cells' &!r $atients hemgl"in sh!ld cme !$ t ./ g=dl.
&ematocrit
Norma! 6ange: 2-/2< (ma!es
=-/=< (3ema!es
-ematcrit is an e4$ressin % the ttal $ercentage % "ld )l!me that is cm$sed %
red "ld cells. It is als *n#n as the $ac*ed cell )l!me % &!r "ld +Sher#d'
51.
Iron
Norma! 6ange: /0-1/0 mcgdL
As mentined earlier' irn is necessar& %r the %rmatin % hemgl"in' an essential $art
% the red "ld cell. Irn is a"sr"ed %rm the small intestine int the "ld and "inds
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#ith trans%errin. Trans%errin trans$rts irn tthe "ne marr# #here it is !sed t ma*e
hemgl"in.
)ota! Iron inding a+acit$
Norma! 6ange: 2/0-10 mcgd!
The am!nt % irn that can still "ind #ith trans%errin +t "e trans$rted t "ne marr#
t ma*e hemgl"in1 is *n#n as the ttal irn "inding ca$acit& r TI;C. Thin* % &!r
TI;C as the ttal am!nt % $e$le that can get n a "!s. The irn is the $e$le and the
"!s is trans%errin. Bhen &!r ser!m irn le)els increase' &!r TI;C decreases. Bhen
&! ser!m irn le)els decrease' then &!r TI;C increases.
Ferritin
Norma! 6ange: 20 - 00 ngmL (ma!es
20 - 120 ngmL (3ema!es
>erritin is a $rtein that "inds t irn. Mst % the irn stred in the "d& is attached t
%erritin. >erritin is %!nd in the li)er' s$leen' and "ne marr#. Onl& a small am!nt is
%!nd in the "ld. Li*e the TI;C' the am!nt % %erritin in the "ld ma& hel$ indicate
the am!nt % irn stred in &!r "d&.
4%ite !ood e!! ount (4 and >i33erentia!
Bhite "ld cells' r le!*c&tes' are classi%ied int t# main gr!$s2
gran!lc&tes and nngran!lc&tes +als *n#n as agran!lc&tes1. The gran!lc&tes'
#hich incl!de ne!tr$hils' esin$hils' and "as$hils' ha)e gran!les in their cell
c&t$lasm. Ne!tr$hils' esin$hils' and "as$hils als ha)e a m!ltil"ed n!cle!s. As a
res!lt the& are als called $l&mr$hn!clear le!*c&tes r $l&s. The n!clei %ne!tr$hils als a$$ear t "e segmented' s the& ma& als "e called segmented
ne!tr$hils r segs. The nngran!lct&e #hite "ld cells' l&m$hc&tes and
mnc&tes' d nt ha)e gran!les and ha)e nnl"!lar n!clei. The& are smetimes
re%erred t as mnn!clear le!*c&tes.
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The li%es$an % #hite "ld cells ranges %rm 5 t / da&s' a%ter #hich time the&
are destr&ed in the l&m$hatic s&stem. Bhen immat!re B;Cs are %irst released %rm the
"ne marr# int the $eri$heral "ld' the& are called "ands r sta"s. Le!*c&tes
%ight in%ectin thr!gh a $rcess *n#n as $hagc&tsis. D!ring $hagc&tsis' the
le!*c&tes s!rr!nd and destr& %reign rganisms. Bhite "ld cells als $rd!ce'
trans$rt' and distri"!te anti"dies as $art % the "d&?s imm!ne res$nse.
Leu*oc$tes
)ota! 4
Norma! 6ange: /,000 -10,000micro!iter
Le!*c&tes' r #hite "ld cells' $rtect the "d& %rm "acteria and in%ectin.
The #hite "ld cell c!nt is e4$ressedas the n!m"er % le!*c&tes $er micrliter %
"ld. The ttal B;C c!nt increases in res$nse t in%ectin r tra!ma.
L$m+%oc$tes
Norma! 6ange: 19-9inall&' the
clt m!st "e dissl)ed in rder %r nrmal "ld %l# t res!me %ll#ing tiss!e re$air.
The dissl!tin % the clt cc!rs thr!gh the actin % $lasmin. 7lasmin is a $rtein that
is res$nsi"le %r digesting %i"rin. E)ent!all&' scar tiss!e %rms cm$leting the healing %
the inF!red )essel +Sher#d' 51.
P!asma
7lasma is a stra#
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I@. T-E 7ATIENT AND -IS ILLNESS
A.7AT-O7-YSIOLOGY +;OOK CENTERED1
7redis$sing >actrs2Race + Bhite race 1Gender +Male1
Age +increases #ith age1-eredit&=>amilial Tendenc&
7reci$itating >actrs2Antecedent -ematlgicDisrdersCngenital DisrderEn)irnmental E4$s!res +high dses% radiatin' chemicals li*e "en3ene't"acc Sm*e17rir E4$s!re T Chemthera$e!tic
Agents >r Anther Malignanc&
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A%%ectatins in di%%erent cmmitted cells
M!tant le!*emia cells $rli%erate and %ill the"ne marr#
Cm$ete and in%iltrate hemat$eisis ,s$reads "e&nd the marr# and tiss!e
rgans
Er&thr"lasts
7rli%eratin %
immat!re$hagc&tes
Decreased
$rd!ctin %nrmal R;C
Decreased
4&gencarr&ing
ca$acit& % the"ld
Mega*ar&idCmmitted Cells
Mega*ar&"last
7rli%eratin %immat!re
mega*ar&c&tes
Immat!re$latelet
$rd!ctin
M&elid Cmmitted CellsM&el"lasts
7rli%eratin %
immat!rem&elc&tes
Increased
immat!re B;C$rd!ctin
L&m$h"las
7rli%eratin immat!re
l&m$hc&te
A%%ectatins ; l&m$hc&t
, T T-E DISEASE
G'N'6L >'"6IP)ION
The !nderl&ing $ath$h&silg& cnsists % a mat!ratinal arrest % "ne marr#
cells in the earliest stages % de)el$ment. The mechanism % this arrest is !nder st!d&'
"!t in man& cases' it in)l)es the acti)atin % a"nrmal genes thr!gh chrmsmal
translcatins and ther genetic a"nrmalities. This de)el$mental arrest res!lts in
disease $rcesses. >irst' the $rd!ctin % nrmal "ld cells mar*edl& decreases'
#hich res!lts in )ar&ing degrees % anemia' thrm"c&t$enia' and ne!tr$enia.
7etechiaeEcch&msis
Gingi)al"leedinge$ista4is
;leedingTendencies
$allrAnemia
Bt. lss Malaise
Eas& %atiga"ilit&
Ris* %r in%ectin
A%%ectatinsin B;C cellscm$nents
Ne!tr$hils;as$hils
Esin$hils
Ina"ilit& t $rtect"d& against
in)asin
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Secnd' the ra$id $rli%eratin % these cells' alng #ith a red!ctin in their a"ilit& t
!nderg $rgrammed cell death +a$$tsis1' res!lts in their acc!m!latin in the "ne
marr#' "ld' and' %re(!entl&' the s$leen and li)er.
". RISK >ACTORS
P6'->I"PO"ING F)O6"2
6' < AML is mre cmmn in #hites than in ther $$!latins.
"'; < AML is mre cmmn in men than in #men. The di%%erence is e)en mre
a$$arent in lder $atients. Sme ha)e $r$sed that the increased $re)alence %
AML in men ma& "e related t cc!$atinal e4$s!res.
G' < 7re)alence increases #ith age. The median age % nset is 8: &ears. -#e)er'this disease a%%ects all age gr!$s.
>AMILIAL TENDENCY < Germ
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thera$e!tic irradiatin %r an*&lsing s$nd&litis #ere at increased ris* % le!*emia.
S!r)i)rs % the atmic "m" e4$lsins in Ha$an #ere at a mar*edl& increased ris* %r
the de)el$ment % le!*emia. 7ersns #h sm*e ha)e a small "!t statisticall&
signi%icant +dds rati' 5.:1 increased ris* % de)el$ing AML. In se)eral st!dies' the ris*
% AML #as slightl& increased in $e$le #h sm*ed cm$ared #ith thse #h did nt
sm*e. E4$s!re t "en3ene is assciated #ith a$lastic anemia and $anc&t$enia.
These $atients %ten de)el$ AML. Man& % these $atients demnstrate M8 mr$hlg&.
7RIOR E7OSURE TO C-EMOT-ERA7EUTIC AGENTS >OR ANOT-ER
MALIGNANCY < As mre $atients #ith cancer s!r)i)e their $rimar& malignanc& and
mre $atients recei)e intensi)e chemthera$& +incl!ding "ne marr# trans$lantatin
;MTW1' the n!m"er % $atients #ith AML increases "eca!se % e4$s!re t
chemthera$e!tic agents. 7atients #ith a $rir e4$s!re t al*&lating agents' #ith r#ith!t radiatin' %ten ha)e a m&eld&s$lastic $hase $rir t the de)el$ment % AML.
The t&$ical latenc& $erid "et#een dr!g e4$s!re and ac!te le!*emia is a$$r4imatel&
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.Fe#er and ot%er signs o3 in3ectioncan cc!r' incl!ding l!ng %indings % $ne!mnia
These are d!e t the lac* % nrmal #hite "ld cell $rd!ctin that ma*es the $atient
s!sce$ti"le t in%ectins' #hile the le!*emic cells are deri)ed %rm #hite "ld cell
$rec!rsrs' the& ha)e n in%ectin
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. P)&OP&5"IOLOG5 (LI'N)-'N)'6'>
7redis$sing >actrs2
Gender +Male1Age + &ears ld1
7reci$itating >actrs2
En)irnmental E4$s!res< Cigarette Sm*e< E4$s!re t certain chemicals+car"nated drin*s e)en "e%rereaching 5
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A%%ectatins in di%%erent cmmitted cells
M!tant le!*emia cells $rli%erate and %ill the"ne marr#
Cm$ete and in%iltrate hemat$eisis ,s$reads "e&nd the marr# and tiss!e
rgans
Er&thr"lasts
7rli%eratin %
immat!re$hagc&tes
Decreased
$rd!ctin %nrmal R;C
Decreased
4&gencarr&ing
ca$acit& % the"ld+-g"1
Mega*ar&idCmmitted Cells
Mega*ar&"last
7rli%eratin %immat!re
mega*ar&c&tes
Immat!re$latelet
$rd!ctin
M&elid Cmmitted CellsM&el"lasts
7rli%eratin %
immat!rem&elc&tes
Increased
immat!re B;C$rd!ctin
Er&thrid CmmittedCells
Disr!$tin % $l!ri$tent stem cells
Disr!$tin % s$eci%ic genes
A%%ectatins in di%%erent cmmitted cells
Mn"lasts
7rli%eratin %immat!re
mnc&tes
Decreased$hagc&tic acti)it&
% mnc&tes
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a. SYNT-ESIS O> T-E DISEASE +CLIENT ;ASED1
GENERAL DESCRI7TION
The !nderl&ing $ath$h&silg& cnsists % a mat!ratinal arrest % "ne marr#
cells in the earliest stages % de)el$ment. This de)el$mental arrest res!lts in
disease $rcesses. >irst' the $rd!ctin % nrmal "ld cells mar*edl& decreases'
#hich res!lts in )ar&ing degrees % anemia' thrm"c&t$enia' and ne!tr$enia.
Secnd' the ra$id $rli%eratin % these cells' alng #ith a red!ctin in their a"ilit& t
] 7etechiae^+"th $alms % the hand1
] Ecch&msis^
] Gingi)al "leeding^
] E$ista4is +!$nadmissin1
] -ematma^
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!nderg $rgrammed cell death +a$$tsis1' res!lts in their acc!m!latin in the "ne
marr#' "ld' and' %re(!entl&' the s$leen and li)er. In AML' the "ne
". RISK >ACTORS
P6'->I"PO"ING F)O6"2
"'; < AML is mre cmmn in men than in #men. The di%%erence is e)en mre
a$$arent in lder $atients.
G'< 7re)alence increases #ith age. The median age % nset is 8: &ears. -#e)er'
this disease a%%ects all age gr!$s.
P6'IPI))ING F)O6"2
EN@IRONMENTAL E7OSURES < In se)eral st!dies' the ris* % AML #as slightl&
increased in $e$le #h sm*ed cm$ared #ith thse #h did nt sm*e.
C. SIGNS AND SYM7TOMS BIT- RATIONALE
5. nemia, Neutro+enia, and )%romboc$to+enia These are d!e t "ne marr#
%ail!re. It res!lts %rm the %act that as le!*emic clne % cells gr#s' it tends t dis$lace
de)el$ment % nrmal "ld cells in the "ne marr#. There is als decreased
ne!tr$hil le)els des$ite an increased ttal B;C c!nt.
.P%$sica! signs o3 anemia- incl!ding $allr and d&s$nea !$n e4ertin.These are
d!e t the increased n!m"er % #hite "ld cells dis$lacing r ther#ise inter%ering #ith
the $rd!ctin % nrmal "ld cells in the "ne marr#. The mst cmmn s&m$tm %
anemia is %atig!e.
. Fe#er and ot%er signs o3 in3ection7atients $resent #ith %e)er' #hich ma& cc!r
#ith r #ith!t s$eci%ic dc!mentatin % an in%ectin. These are d!e t the lac* %
nrmal #hite "ld cell $rd!ctin that ma*es the $atient s!sce$ti"le t in%ectins' #hile
the le!*emic cells are deri)ed %rm #hite "ld cell $rec!rsrs' the& ha)e n in%ectin