clinpath hematology disorder (1)
TRANSCRIPT
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Indriani SilviaPatologi Klinik
FK UNSWAGATI06092015
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Transports o!g"n# $all"do!%"&oglo'in# (%"n it giv"s )p itso!g"n it is d"o!%"&oglo'in*
Also 'inds and transports $ar'ondioid"# $ar'a&ino%"&oglo'in*
,ak"s )p - 9. / o 3 → - 250&illion 4' &ol"$)l"s p"r 3
,"n 1718 &gdl 'lood
Wo&"n 12716 &gdl 'lood
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T!p"s o 4"&oglo'in :4'; 4' A
< 96/ o ad)lt 4' :=2 >2;
4' A2< ?/ o ad)lt 4' :=2 @2;
4' F< 1 / o ad)lt 4' :=2 2;
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Blood
group
Antigen Antibody Donor to Recipient
from
A A Anti-B A A, O
B B Anti-A B B, O
AB AB Neither AB A, B, AB, O
O Neither Anti-
A/Anti-B
O, A, B, AB O
Universal donor "O"Universal recipient "AB"
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Acute Hemolytic Reaction S!&pto&s
< F"v"r# $%ills and "v"r# t%" ""ling o %"at alongt%" v"in in (%i$% t%" 'lood is '"ing trans)s"d
< Pain in t%" l)&'ar r"gion< 3onstri$ting pain in t%" $%"st# ta$%!$ardia#
%!po7t"nsion< 4"&oglo'in"&ia (it% s)'s")"nt %"&oglo7
'in)ria and %!p"r'ilir)'in7"&ia* H""ling o i&p"nding doo&H
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Acute Hemolytic Reaction 3a)s"s
< 4)&an "rror Trans)s"d r"d $"lls r"a$t (it% $ir$)lating anti'od! int%" r"$ipi"nt (it% r"s)ltant intravas$)lar %"&ol!sis
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Acute Hemolytic Reaction Fr")"n$!
< ar"
Pr"v"ntion< Prop"r id"ntiE$ation o pati"nts# pr"trans)sion
'lood sa&pl"s and 'lood $o&pon"nts at t%"ti&" o trans)sion
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Delayed Hemolytic ReactionFalling %"&ato$rit
d)" to "travas$)lar d"str)$tion o t%" trans)s"dr"d 'lood $"lls;
Positiv" dir"$t antiglo')lin :3oo&'s; t"st:JAT;
$$)rs a'o)t 78 da!s at"r 'lood trans)sion
Pati"nts &a! &ani"st "v"r and l")ko$!tosisApp"aring to %av" an o$$)lt in"$tion*
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Allergic ! urticaria Bar!ng"al "d"&a and 'ron$%ospas& 1/ o r"$ipi"nts
< I $o)pl"d (it% anot%"r sign# s)$% as "v"r#"val)ation or a %"&ol!ti$ r"a$tion &a! '"indi$at"d*
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Allergic ! Anaphyla"is Anap%!la$ti$ or anap%!la$toid
< "spirator! involv"&"nt (it% d!spn"a or stridor
3ardiovas$)lar insta'ilit!< %!pot"nsion# ta$%!$ardia# loss o
$ons$io)sn"ss# $ardia$ arr%!t%&ia# s%o$k and
$ardia$ arr"st
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#olume $%erload Trans)sion7r"lat"d vol)&" ov"rload In)s" s&all"r vol)&"s &or" slo(l!
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&acterial 'ontamination 4!pot"nsion# s%o$k# "v"r and $%ills#
na)s"a and vo&iting# and r"spirator!
distr"ss Gra& stain and 'lood $)lt)r"
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Follo( proto$ol or trans)sionr"a$tions i&pl"&"nt"d '! t%"
instit)tion
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(top t%" trans)sion i&&"diat"l!
Disconnect the intra%enous line ro&t%" n""dl"*
(eek medical attention i&&"diat"l!* It%" pati"nt is s)D"ring $ardiop)l&onar!$ollaps"# and &"di$al att"ntion is not
i&&"diat"l! availa'l"# pr"ss or L3od"H
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'heck to "ns)r" t%at t%" pati"nt na&"and r"gistration n)&'"r on t%" 'lood 'agla'"l "a$tl! (it% inor&ation on t%"
pati"ntMs id"ntiE$ation
Do not discard the unit o blood t%at%as '""n dis$ontin)"d '"$a)s" it &a! '"
n"$"ssar! or t%" inv"stigation o t%"trans)sion r"a$tion*
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Reaction Type Treatment - Adult Pediatric Follow-up
Acute
HemolyticReactions
Diuretic therapy: Initially, give 40-80mg Furosemide (Lasix)intravenously. This dose can ere!eated once. Lac" o# res!onse to#urosemide in $-% hours indicatesthe !resence o# acute renal #ailure.
&ediatric dose' -$mg"gdose.*ay re!eat onceat $-4 mg"g.
Treat shoc" and disseminatedintravascular coagulation +itha!!ro!riate measures i# and +henthey a!!ear.
Water loading: The !atient should ehydrated to maintain urinaryout!ut o# at least 00 mLhr untilurine is #ree o# hemogloin.
In#use a loading dose o# 0. sodiumchloride or dextrose in 0.4sodium chloride. /hart hourlyurine out!ut. *aintain the urine
out!ut y administeringintravenous #luid at 00 mLhouruntil the urine is #ree o#hemogloin. I# the !atientsurinary out!ut does not increase,+ith this hydration any additional#luids should e in#used +ithcaution.
&ediatric !atientsshould receive asmaller loadingvolume o# #luidin !ro!ortion totheir odysur#ace area.
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DelayedHemolytic
TransfusionReactions
1!eci#ic treatment generally is notnecessary
1u!!lemental trans#usion o# lood lac"ingthe antigen corres!onding to theo##ending antiody may e necessaryto com!ensate #or the trans#used cells
that have een removed #rom thecirculation.
Reaction Type Treatment - Adult Pediatric Follow-up
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Reaction Type Treatment - Adult Pediatric Follow-up
AllergicTransfusion
Reactions
Antihistamines(e.g., 2enadryl). 3ive0-00 mg orally or intravenously. I#urticaria develo!s slo+ly,antihistamines may e given orally.
&ediatric dose' -$mg"g intramuscularlyor intravenously #or$-0 mg !er averagedose.
outine use o# 2enadryl as !remedication#or all trans#usions, regardless o# a historyo# allergic reactions, is discouraged.
Aminophylline #or +hee5ing, at a doseo# $-$0 mg intravenously slo+lyover a !eriod o# aout #ive minutes
&ediatric dose' %mg"gdose inintravenous dri! overo# $0 minutes.
Epinephrine #or severe, acute reactionsincluding laryngeal edema or
ronchos!asm 3ive 0.-0. mg (0.-0.mL o# a '000 solution)sucutaneously. 1ucutaneous dosemay e re!eated at 0- minuteintervals. The total sucutaneous dosein a $4-hour !eriod, +ith rareexce!tion, should not exceed mg.
&ediatric dose' 0.0%mL*$ (0.0% mg*$
o# a '000 solution)given sucutaneously.6 single !ediatricdose should notexceed 0.% mg.
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FerileTransfusion
Reactions
Premedicate the !atient +ithacetamino!hen or otheranti!yretic agents +hen !reviousreactions have een extremely
othersome. &ediatric dose' 0mg"g to a maximum o# 700 mg.
6s!irin +ill adversely a##ect the !atients !latelet #unction, so non-as!irinanti!yretic agents are !re#erale.
!e"ere sha#ing$hills
(rigors) can e controlled y thesedative e##ect o# 2enadryl oremerol ($-0 mg givenintramuscularly or intravenously
9ote' emerol may cause acuteres!iratory arrest. 6n o!iateantagonist (9arcan) should eimmediately availale.
!epsis Due to%acterial
$ontaminationof Donor %lood
Treatment o# se!tic shoc" includes'terminating the sus!ected
trans#usion immediately, cardio-vascular and res!iratory su!!ort,
lood culture o# the !atient, andadministration o# road s!ectrumantiiotics including anti-
!seudomonas coverage i# the lood com!onent involved is ed2lood /ells&
Reaction Type Treatment - Adult Pediatric Follow-up
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A'nor&all! lo( n)&'"r o 3 or 4'l"v"ls
"d)$"d o!g"n $arr!ing $apa$it!
'auses lood loss In$r"as"d rat" o r"d $"ll d"str)$tion
< 4"&ol!ti$ an"&ia
J"E$i"nt or i&pair"d r"d $"ll prod)$tion
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Risk actors Poor di"t Int"stinal disord"rs ,"nstr)ation Pr"gnan$! 3%roni$ $onditions Fa&il! %istor!
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LNT A JISASO ')t a s!&pto&< J"p"nd"nt on s"v"rit!# sp""d o d"v"lop&"nt#
ag"# %"alt% stat)s and $o&p"nsator!&"$%anis&s
< Asso$iat"d (it% i&pair"d 2 transport#alt"ration in 3 str)$t)r" or (it% $%roni$illn"ss
< Not "pr"ss"d )ntil 50/ o 3 &ass is lost
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(igns and symptoms T%" &ain s!&pto& o &ost t!p"s o
an"&ia is atig)"< W"akn"ss
< Pal" skin
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Iron De)ciency Anemia ,ost $o&&on or& o an"&ia
< AD"$ts a'o)t on" in Ev" (o&"n< 4al o pr"gnant (o&"n and ? p"r$"nt o &"n
in t%" Unit"d Stat"s* T%" $a)s" is a s%ortag" o t%" "l"&"nt iron
< N)tritional i&'alan$"< Slo(# $%roni$ 'l""ding disord"rs< Ina'ilit! to r"$!$l" plas&a iron
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#itamin De)ciency Anemias Folat" and vita&in 712 d"E$i"n$! Int"stinal disord"r t%at aD"$ts t%"
a'sorption o n)tri"nts Fall into a gro)p o an"&ias $all"d
&"galo'lasti$ an"&ias# in (%i$% t%" 'on"&arro( prod)$"s larg"# a'nor&al r"d'lood $"lls*
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Anemia o 'hronic Disease Int"r"r" (it% t%" prod)$tion o r"d 'lood
$"lls# r"s)lting in $%roni$ an"&ia Kidn"! ail)r" also $an '" a $a)s" o
an"&ia
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Aplastic Anemia Bi"7t%r"at"ning an"&ia $a)s"d '! a
d"$r"as" in t%" 'on" &arro(Ms a'ilit! toprod)$" all t%r"" t!p"s o 'lood $"lls Q r"d'lood $"lls# (%it" 'lood $"lls and plat"l"ts
3a)s" o aplasti$ an"&ia is )nkno(n< a)toi&&)n" dis"as"< 3%"&ot%"rap!
< adiation t%"rap!< nviron&"ntal toins
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Anemias associated *ith bone marro*disease
B")k"&ia and &!"lod!splasia# $an $a)s"an"&ia '! aD"$ting 'lood prod)$tion int%" 'on" &arro(
D"$ts var! ro& a &ild alt"ration in 'loodprod)$tion to a $o&pl"t"# li"7t%r"at"nings%)tdo(n o t%" 'lood7&aking pro$"ss,!"lod!splasia is a pr"7l")k"&i$
$ondition t%at $an $a)s" an"&ia*t%"r $an$"rs o t%" 'lood or 'on" &arro(#s)$% as &)ltipl" &!"lo&a# &!"loproli"rativ"disord"rs or l!&p%o&a# $an $a)s" an"&ia*
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Hemolytic Anemias "d 'lood $"lls ar" d"stro!"d ast"r t%an
'on" &arro( $an r"pla$" t%"&* A)toi&&)n" disord"rs $an prod)$"
anti'odi"s to r"d 'lood $"lls# d"stro!ingt%"& pr"&at)r"l!< 4"&ol!ti$ an"&ias &a! $a)s" !"llo(ing o t%"
skin :Ra)ndi$"; and an "nlarg"d spl""n*
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Hereditary (pherocytosis ,)tations in t%" ank!rin &ol"$)l" (it% a
s"$ondar! d"E$i"n$! o sp"$trin along t%"
$"ll &"&'ran"< "d)$"d r"d $"ll sta'ilit! Jo"s not aD"$t o!g"n $arr!ing $apa$it!
Spl"ni$ s")"stration
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(ickle cell anemia J""$tiv" or& o %"&oglo'in t%at or$"s
r"d 'lood $"lls to ass)&" an a'nor&al$r"s$"nt :si$kl"; s%ap"*
< ,)tation or t%" g"n" $oding or t%" >7glo')lin$%ain alin" is s)'stit)t"d or gl)ta&i$ a$id 4'S
"d $"lls di" pr"&at)r"l!# r"s)lting in a
$%roni$ s%ortag" o r"d 'lood $"lls*< lo$k 'lood o( t%ro)g% s&all 'lood v"ss"ls int%" 'od!# prod)$ing ot%"r# ot"n pain)l#s!&pto&s*
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Singl" 'as" pair &)tation r"s)lts in asingl" a&ino a$id $%ang"*
Und"r lo( o!g"n# 4g' '"$o&"s
insol)'l" or&ing long pol!&"rs T%is l"ads to &"&'ran" $%ang"s
:Lsi$klingO; and vasoo$$l)sion
!i #l $ ll ' t ti
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!ic#le $ell 'utation
+O2
-O2
+O2
-O2
5'α1α2
3'Chromosome 16
5' 3'Chromosome 11 ε γ γ G A
α αCCT GAG GAG
-Pro-Glu-Glu-5 6 7
CCT GTG GAG
-Pro-Val-Glu-5 6 7β
A βS
Normal (HbA) Abnormal (HbS)
(
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Deo"ygenation o (( erythrocytes leads tointracellular hemoglobin polymeri+ation, loss odeormability and changes in cell morphology-
OXY-STATE DEOXY-STATE
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./ Thalassemia 3o&&on in Asians J"l"tion o glo')lin $%ain lo$i
possi'l" d"gr""s o = t%alass"&ia< Sil"nt $arri"r# loss o a singl" = glo')lin g"n"< = t%alass"&ia trait# loss o a pair o glo')lin
g"n"
< 4'4 dis"as"# onl! a singl" g"n" is pr"s"nt< 4!drops "talis# d"l"tion o all = glo')lin
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T%" onl! tr"at&"nts ar" st"& $"lltransplant and si&pl" trans)sion*
3%"lation t%"rap! to avoid iron
ov"rload %as to '" start"d "arl!*
TA0A((12IA
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An a$)ir"d disord"r o t%" 'on" &arro(t%at $a)s"s t%" ov"rprod)$tion o all t%r""'lood $"ll lin"s
< (%it" 'lood $"lls# r"d 'lood $"lls# and plat"l"ts It is a rar" dis"as" t%at o$$)rs &or"
r")"ntl! in &"n t%an (o&"n# and rar"l!in pati"nts )nd"r 0 !"ars old*
$a)s"s is )nkno(n
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Us)all! d"v"lops slo(l!# and &ost pati"ntsar" as!&to&ati$< a'nor&al 'on" &arro( $"lls proli"rat"
)n$ontrolla'l! l"ading to a$)t" &!"log"no)sl")k"&ia
Pati"nts %av" an in$r"as"d t"nd"n$! toor& 'lood $lots t%at $an r"s)lt in strok"sor %"art atta$ks< So&" pati"nts &a! "p"ri"n$" a'nor&al
'l""ding '"$a)s" t%"ir plat"l"ts ar" a'nor&al
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(ymptoms 4"ada$%" Jiin"ss Pr)rit)s F)lln"ss in t%" l"t )pp"r a'do&"n r!t%"&a :a$"; S%ortn"ss o 'r"at% rt%opn"a S!&pto&s o p%l"'itis
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3oll"$tiv"l! kno(n as W%it" lood 3"lls:W3;
For&"d "l"&"nts o t%" 'lood (it%
organ"ll"s and a n)$l")s ')t la$k%"&oglo'in
Prot"$t t%" 'od! against &i$roorganis&sand r"&ov" d"ad $"lls and d"'ris ro& t%"
'od!
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Per µl blood Per µl of blood
Total WBC count 5,000 – 10,000
Neutrophils 50 - 70% 2,000 – 7,000
Lymphocytes 20 - 40% 1,000 – 4,000Monocytes 1 – 6% 50 – 600
Eosinophils 1 – 5% 50 – 500
Basophils 0 – 2% 0 - 100
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B")kop"nia< J"$r"as"d p"rip%"ral (%it" $"ll $o)nt d)" to
d"$r"as" n)&'"rs o an! sp"$iE$ t!p"s ol")ko$!t"s
B")ko$!tosis< NonCn"oplasti$ "l"vation o W3 $o)nt
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3eutropenia "d)$tion in t%" n)&'"r o gran)lo$!t"s
:1500Vl;
In$r"as"d risk o in"$tion< "d)$"d p%ago$!tosis r"spons"
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3eutropenia J"$r"as"d or d""$tiv" gran)lopoi"sis
< Aplasti$ an"&ia
<Anti7n"oplasti$ ag"nts< t%"r dr)gs $%lora&p%"ni$ol# s)lona&id"s#$%lorpro&ain"
A$$"l"rat"d r"&oval or d"str)$tion<
Aggr"ssiv" and $%roni$ in"$tions
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,ani"station o N")trop"nia In"$tions
Signs and S!&pto&s ,alais"# $%ills# "v"r Ul$"rativ" n"$rotiing l"sions o t%" &o)t%#
skin vagina and GI tra$t
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Reacti%e 0eukocytosis In$r"as" n)&'"r o W3 3o&&on r"a$tion d)" to a vari"t! o
ina&&ator! stat"s $a)s"d '! &i$ro'ial ornon7&i$ro'ial sti&)li Us)all! non7sp"$iE$
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Causes of Leukocytosis
Polymorphonuclear leukocytosis Acute bacterial infections
Eosinophilic leukocytosis Allergic disorders
Monocytosis Chronic infections
Lymphocytosis Chronic immunologic disease
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B")k"&oid r"a$tion C t%is is an "tr"&" n")trop%ilia (it% aW3 $o)nt ?0 109B
,an! 'ands# &"ta&!"lo$!t"s# and &!"lo$!t"s ar" s""n
$$asional pro&!"lo$!t"s and &!"lo'lasts &a! '" s""n*
T%is $ondition r"s"&'l"s a $%roni$ &!"lo$!ti$ l")k"&ia:3,B;# ')t $an '" diD"r"ntiat"d ro& 3,B 'as"d on t%"a$t t%at in l")k"&oid r"a$tions
T%"r" is no P%ilad"lp%ia $%ro&oso&"
T%" $ondition is transi"nt
T%"r" is an in$r"as"d l")ko$!t" alkalin" p%osp%atas" s$or" :&or" on t%is lat"r;
B")k"&oid r"a$tions &a! '" s""n in t)'"r$)losis# $%roni$in"$tions# &alignant t)&ors# "t$*
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1* B")k"&ia C n"oiplas&s o t%"%"&atopoi"ti$ st"& $"lls
2* ,alignant l!&p%o&as C $o%"siv" t)&or
l"sionsX n"oplasti$ l!&p%o$!t"s?* Plas&a $"ll d!s$rasias C arising ro& t%"'on"sX lo$ali"d diss"&inat"dproli"ration o anti'od! or&ing $"lls
* 4isto$!tos"s C proli"rativ" l"sions o%istio$!t"s
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0eukemia ,alignant n"oplas& o t%" %"&atopi"ti$
st"& $"lls
, r"pla$"d '! )nr"g)lat"d# proli"rating#i&&at)r" n"oplasti$ $"lls 'lood
l")k"&ia "nt"r spl""n# l!&p% nod"s ,ost $o&&on $an$"r in t%" pa"diatri$ ag"
B"ading $a)s" o d"at% in $%ildr"n'"t(""n ? and 1 !"ars old
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'lassi)cation o 0eukemiaA* A$$ording to $"ll t!p" and stat" o $"ll
&at)rit!< B!&p%o$!ti$ C i&&at)r" l!&p%o$!t"s and
t%"ir prog"nators< ,!"lo$!ti$ C pl)ripot"nt &!"loid st"& $"lls
and int"r"r"s (it% &at)ration o allgran)lo$!t"s# 3 and plat"l"ts
* A$)t" or 3%roni$< A$)t" C i&&at)r" $"lls :'last;< 3%roni$ C ("ll diD"r"ntiat"d l")ko$!t"s
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67/103
3ong"nital< P"lg"r74)"t ano&al!
ilo'"d and o$$asional )ns"g&"nt"d n")trop%ils
A)toso&al r"$"ssiv" disord"r
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8/17/2019 ClinPath Hematology Disorder (1)
68/103
< N")trop%il %!p"r7s"g&"ntation ar" a)toso&al do&inant $ondition
N")trop%il )n$tion is "ss"ntiall! nor&al
< ,a!74"gglin ano&al!
N")trop%ils $ontain 'asop%ili$ in$l)sions o NA $$asionall! t%"r" is asso$iat"d l")$op"nia
T%ro&'o$!top"nia and giant plat"l"t ar" r")"nt
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8/17/2019 ClinPath Hematology Disorder (1)
69/103
< Ald"rYs ano&al! Gran)lo$!t"s# &ono$!t"s and l!&p%o$!t"s $ontain gran)l"s
(%i$% stain p)rpl" (it% o&ano(sk! stain
Gran)l"s $ontain &)$opol!sa$$%arid"s
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8/17/2019 ClinPath Hematology Disorder (1)
70/103
< 3%"diak74igas%i s!ndro&" A)toso&al r"$"ssiv" disord"r
Giant gran)l"s in gran)lo$!t"s# &ono$!t"s andl!&p%o$!t"s
Partial o$$)lo$)tan"o)s al'inis&
J"pr"ss"d &igration and d"gran)lation
"$)rr"nt p!og"ni$ in"$tions
B!&p%oproli"rativ" s!ndro&" &a! d"v"lop
Tr"at&"nt is ,T
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8/17/2019 ClinPath Hematology Disorder (1)
71/103
A$)ir"d Toi$ gran)lation
Jo%l" 'odi"s
P"lg"r $"lls
4!p"rs"g&"nt"d n")trop%ils
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8/17/2019 ClinPath Hematology Disorder (1)
72/103
B")$o$!t" ad%"sion d"E$i"n$! 3%roni$ gran)lo&ato)s dis"as" 3%"diak74igas%i s!ndro&" Pri&ar! i&&)nod"E$i"n$!
S"v"r" $o&'in"d i&&)nod"E$i"n$!
3o&&on varia'l" i&&)nod"E$i"n$!
Isolat"d IgA d"E$i"n$!
T7$"ll i&&)nod"E$i"n$!
T%!&i$ aplasia :Ji G"org" s!ndro&";
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8/17/2019 ClinPath Hematology Disorder (1)
73/103
lo$k in t%" diD"r"ntiation o l")k"&i$$"lls (it% prolong"d g"nration ti&"
$lonal "pansion o t%" transor&"d st"&
$"lls Z ail)r" o &at)ration
accumulation o leukemic blast
s)ppr"ss nor&al %"&atopoi"ti$ st"& $"lls
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8/17/2019 ClinPath Hematology Disorder (1)
74/103
F"at)r"s S)dd"n ons"t :? &ont%s; J"pr"ss"d &arro( )n$tion on" pain and t"nd"rn"ss G"n"rali"d l!&p%ad"nop%at! Spl"no&"gal!# %"pato&"gal! 3NS %"ada$%"# vo&iting
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8/17/2019 ClinPath Hematology Disorder (1)
75/103
,ost $o&&on l")k"&ia in $%ildr"n:80/;
Tr"ata'l" and pot"ntiall! $)ra'l" 3lassiE"d a$$ording to l!&p%o$!t"s and
stat" o &at)ration1* arl! $"ll
2* Pr"7 $"ll
?* ,at)r" $"ll* arl! T $"ll
5* ,at)r" T $"ll
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8/17/2019 ClinPath Hematology Disorder (1)
76/103
A$)t" Non7l!&p%o$!ti$ B")k"&ia :ANBB; ,ost $o&&on in ad)ltsX 50/ 60!"ars old .0/ o ad)lts (ill "nt"r r"&ission (it%
ind)$tion $%"&o< 257?5/ o t%os" in r"&ission (ill %av" a 5 !"ar
s)rvival rat" , transplant
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8/17/2019 ClinPath Hematology Disorder (1)
77/103
Tr"at&"nt S"l"$tiv" radiation 3%"&ot%"rap!
1* Ind)$tion
2* Int"nsiE$ation
?* ,aint"nan$" and $onsolidation on" &arro( transplant
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8/17/2019 ClinPath Hematology Disorder (1)
78/103
Insidio)s ons"t In$id"ntal Endings d)ring ro)tin" "a&
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8/17/2019 ClinPath Hematology Disorder (1)
79/103
Proli"ration and a$$)&)lation o &at)r"l!&p%o$!t"s (%i$% ar" i&&)nologi$all!in$o&p"t"nt< $"ll lin" :US;
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8/17/2019 ClinPath Hematology Disorder (1)
80/103
15/ o all l")k"&ias 3%ro&oso&al a'nor&alit! :P%1; ,ostl! $"ll dis"as"
< B")ko$!tosis
< Spl"no&"gal!
< 4"pato&"gal!
< B!&pad"nopat%!
on" &arro( transplant
5 !"ar s)rvivalor 507.5/ o pati"nts
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8/17/2019 ClinPath Hematology Disorder (1)
81/103
T(o distin$t p%as"s 3%roni$
< Bast a'o)t ?7 !"ars
< N"ar "nd a$$"l"rat"d p%as" "v"r# nig%t
s("ats# &alais" A$)t"
< 27 &ont%s
< Poor prognosis# palliativ" &anag"&"nt
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8/17/2019 ClinPath Hematology Disorder (1)
82/103
Pri&ar! solid t)&ors o t%" l!&p%oids!st"&
3an$"rs involving l!&p%o$!t"s d)ring&at)ration or storag" in t%" 'on" &arro(
T%ird &ost $o&&on &aligna$! in $%ildr"n
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8/17/2019 ClinPath Hematology Disorder (1)
83/103
4odgkinYs B!&p%o&a Jisord"rs pri&aril! involving t%" l!&p%oid
tiss)"s Anato&i$al spr"ad ,orp%ologi$al pr"s"n$" o Reed-Sternbergcells
60790/ $)r" rat"
-
8/17/2019 ClinPath Hematology Disorder (1)
84/103
,ani"stations o 4odgkinYs A s!&pto&s
< Painl"ss progr"ssiv" "nlarg"&"nt o a singl" orgro)p o nod"s :n"$k;
< ,a! spr"ad $ontin)o)sl! t%ro)g% o)t t%"l!&p%ati$ s!st"&
s!&pto&s< F"v"r# nig%t s("at# ("ig%t loss
< Fatig)"# an"&ia
-
8/17/2019 ClinPath Hematology Disorder (1)
85/103
Tr"at&"nt or 4odgkinYs adiation 3%"&ot%"rap!
-
8/17/2019 ClinPath Hematology Disorder (1)
86/103
Non74odgkinYs B!&p%o&a Involv"s l!&p%oid tiss)" and &a! spr"ad
to vario)s tiss)"s ,ostl! $"ll :80/; 3a)s" &a! '" viral or g"n"ti$
<
< I&&)nos)ppr"s"d pati"nts AIJS
At"r organ transplant
-
8/17/2019 ClinPath Hematology Disorder (1)
87/103
Tr"at&"nt arl! stag" radiation Bat" stag" $%"&o and radiation
, transplant
-
8/17/2019 ClinPath Hematology Disorder (1)
88/103
Trans)s" or an! s"v"r" an"&ia (it%p%!siologi$ $o&pro&is"*
J"$id" "arl! (%"t%"r trans)sion (ill
'" rar" or part o t%"rap!* Avoid long7t"r& $o&pli$ations '!
(orking (it% !o)r 'lood 'ank and
)sing $%"lation t%"rao!*
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8/17/2019 ClinPath Hematology Disorder (1)
89/103
-
8/17/2019 ClinPath Hematology Disorder (1)
90/103
-
8/17/2019 ClinPath Hematology Disorder (1)
91/103
-
8/17/2019 ClinPath Hematology Disorder (1)
92/103
-
8/17/2019 ClinPath Hematology Disorder (1)
93/103
-
8/17/2019 ClinPath Hematology Disorder (1)
94/103
-
8/17/2019 ClinPath Hematology Disorder (1)
95/103
-
8/17/2019 ClinPath Hematology Disorder (1)
96/103
-
8/17/2019 ClinPath Hematology Disorder (1)
97/103
-
8/17/2019 ClinPath Hematology Disorder (1)
98/103
-
8/17/2019 ClinPath Hematology Disorder (1)
99/103
-
8/17/2019 ClinPath Hematology Disorder (1)
100/103
-
8/17/2019 ClinPath Hematology Disorder (1)
101/103
-
8/17/2019 ClinPath Hematology Disorder (1)
102/103
-
8/17/2019 ClinPath Hematology Disorder (1)
103/103