Senior Case Presentation 2

8/19/2019 Senior Case Presentation 2

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SENIOR CASE PRESENTATION

DEEPTHY DAMIEN01/21/2010


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Chief Complaint

4 y/o with spontaneous bruising 1 !onth


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H/O Present Illness

4 y/o pre"ious#y hea#thy $e!a#e brought in by !otherbe%ause !u#tip#e spontaneous bruising 1 !onth&Most#y upper an' #ower e tre!ities in"o#"e'& (een atanother hospita# an' got re$erre' to )*)M*No $e"er /%hi##s/weight #oss/bone pain /+oint pain/noseb#ee'/hea' a%he / b#urry "isionPositi"e h/o ,)I a#!ost 1 !onth agoNo si!i#ar prob#e!s in the past


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Past Medical History

No prior hospita#i-ations

(een in the .rtho %#ini% $or intoeing


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ROS

HEENT No hea'a%he /%ongestion/epista is

)esp No %ough /whee-e*ar'ia% No %hest pain /%yanosis/pa#pitation

Ab'o!en No ab' pain/'iarrhea/%onstipation

, neg(5in pos $or bruisingNeuro No wea5ness/sei-ure


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Physical Exam

6ita#s T 78&8 P) 70/!in )) 90/!in P 100/:0 wt 2:&; 5gen a%ti"e ta#5ing

(5in e%%hy!osis ) e#bow ) 5nee shin 3 5neeten'er no organo!ega#y (?E tre!ities no swe##ing/e$$usion nor!a# ).MNeuro no 'e$i%its


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Labs

* * :&@/12&7/94&:/@

P/( 'e%rease' p#ate#ets& Nor!a# !orpho#ogyo$ ) * *


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Dia nosisITP/ IMM,NEBIDI.PATHI*C TH).M .*YT.PENI* P,)P,)A


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Orders at !dmission

* * with 'i$$

*MP ,ri% a%i' 3DH E() P(PT/IN)/PTTN. N(AID(

N. (TE).ID(Monitor $or b#ee'ing)estri%t a%ti"ities


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D!" #Patient stab#eNo new #esions/ b#ee'ing

3A (* * :&@/12&7/9:&8/;E() @*)P 0&9PT 11&8/IN) 1&1/PTT 2:&:

*MP 142/9&;/104/2@/11/0&; A3P 1@0/A(T 92/A3T 20/ i#i 0&13DH :18,ri% a%i' 4&9


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Orders

I6 I 1 g / g I6 1


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D!" $

Patient stab#e

No new #esions* * 4&1/1&:/9:&;/4:Dis%harge' ho!e with a'"ise' to $/u withP*P in 1 w5 * * prior to %#ini% "isit


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%a&e Home Messa e

N. N(AID( A( THEY IN*)EA(E THE )I( .3EEDIN

D. N.T I6E (TE).ID( ,NTI33 .THE)DIA N.(I( A)E E *3,DED A( THE (TE).ID(*AN MA( EA)3Y 3E, EMIA&I$ the ITP is not reso#"e' in 4>: w5s they !ay nee' ahe!ato#ogy re$erra#/ M I$ M %o!es neg it is o5 to treat with steroi's


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Imm'ne(idiopathic) %hrombocytopenic P'rp'raIn Children

Thro!bo%ytopenia Busua##y F20 000/!i%ro3C that is a%Guire' an'genera##y benign,n5nown %ause

A%ute ITP is %o!!on in %hi#'ren A#so 5nown as autoi!!une thro!bo%ytopeni% purpura/

isoi!!une thro!bo%ytopeni% purpura


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Patho enesis

Auto antibo'ies Busua##y Ig C against p#ate#et!e!brane antigens su%h as g#y%oprotein%o!p#e II b/III a& The antibo'y %oate' p#ate#etsha"e a shortene' ha#$>#i$e be%ause o$ thea%%e#erate' %#earan%e by tissue !a%rophagesin the sp#een an' other portions o$ )E(&Thenet e$$e%t is a 'e%rease in the p#ate#et %ount&In %hroni% ITP T %e## !e'iate' %ytoto i%ity !ay%ause p#ate#et 'estru%tion&


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Epidemiolo y

ITP is one o$ the !ost %o!!on %auses o$

sy!pto!ati% thro!bo%ytopenia in %hi#'ren&In%i'en%e 9>8 /100 000 %hi#'ren/ year ,sua# age 2>10 yrs pea5 2>;yr (#ight#y boys gir#s


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Clinical Presentation(u''en appearan%e o$ bruising an'/or b#ee'ing in an otherwisehea#thy %hi#'&

Historyin :0 there is a history o$ prior in$e%tion& An in%rease' ris5 o$ITP is asso%iate' with MM) i!!uni-ation&No syste!i% sy!pto!s

Presen%e o$ syste!i% sy!pto!s #i5e $e"er anore ia +ointpain bone pain or weight #oss usua##y points to other 'iagnosisDrug in'u%e'Bheparin Guini'ine su#$ona!i'esC thro!bo%ytopeniais un%o!!on in 5i's


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Disease %ourse @0 o$ %hi#'ren ha"e the a%ute $or! o$ ITP whi%h is'e$ine' by re%o"ery Bp#ate#et %ount 1;0 000/ !i%ro 3C in : !onths o$presentation with or without treat!ent& Treat!ent 'o not a$$e%t the #ongter! out%o!e but !ini!i-e the ris5 o$ signi$i%ant b#ee'ing

Diagnosis 2 %riteria1&Iso#ate' thro!bo%ytopenia with otherwise nor!a# b#oo' %ounts an'

periphera# s!ear&2&No %#ini%a##y apparent asso%iate' %on'itions that !ay %ause

thro!bo%ytopeniaE %#u'e %on%urrent in$/autoi!!une 'isor'ers/!a#ignan%y/'rugs/geneti%

b#ee'ing 'isor'ers/ !arrow $ai#ureMost %h#'ren 2>10 yrs with abrupt bruising in otherwise hea#thy %hi#'


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3absC,C thro!bo%ytopenia usua##y the on#y abnor!a#ity 'ete%te' usua##y F20 000/!i%ro3Peripheral smear+no !orpho#ogi% abnor!a#ities in */) *&P#ate#et 'e%rease' in nu!ber o$ten #arge in

si-e&Other st'dies *oags %oo!bsJ test reti% %ount HI6 stu'ies $or %o##agen "as%u#ar/rheu!atoi' 'isor'ers&

Anti p#ate#et ab testing>not routine#y in'i%ate' in 5i's

,one marro- examin%rease nu!ber o$ !ega5aryo%ytes !ay appear #arge an' i!!ature)outine#y per$or!e' in the past to r/o !arrow $ai#ure or !a#ignan%yBa%ute

#y!phob#asti% #eu5e!iaC&New gui'e#ines K M e a! is unne%essary in the Ltypi%a#%ase o$ %hi#'hoo' ITP


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In'i%ation $or M e a!

>Atypi%a# presentation>Persistent thro!bo%ytopenia beyon' : !onths>subseGuent %#ini%a# %ourse that is not %onsistent

with ITP> e$ore (teroi' treat!ent in typi%a# ITP is

re%o!!en'e' by so!e &Bnot by ,pToDateC


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Differential Dia nosis

6ira# in$e%tionBIMN Hepatitis HI6> 1CDrug e posureBHeparin uini'ine (u#$ona!i'eC

Autoi!!une Disor'ersB(3EC3eu5e!ia BA33C

A%Guire' !arrow $ai#ure syn'ro!e Bap#asti% ane!iaC

Inherite' thro!bo%ytopeni%'isor'ersBthro!bo%ytopenia>absent ra'ius syn'ro!e

is5ott>A#'ri%h syn'ro!e !utation o$ MYH 7 geneC


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%reatment

Initia# !e'i%a# !anage!ent

>I'ea# !anage!ent sti## un%#ear >obser"ationa#one "s obser"ation with phar!a%o#ogi%inter"ention

>restri%t %onta%t sports/physi%a# a%ti"ity>a"oi' !e'i%ations with antip#ate#et or

anti%oagu#ant a%ti"ities


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Pharmacolo ic Inter.entionPresen%e o$ se"ere #i$e threatening b#ee'ing ris5 o$ signi$i%ant b#ee'ingBgoing $or pro%e'ures/%ount F10 000/!i%ro3 an' %utaneous b#ee'ingC%on%o!itant / pree isting %on'itionsBhe!ophi#iaC nee' inter"ention

Corticosteroids)e'u%e Ab pro'u%tion )E( phago%ytosis o$ antibo'y %oate'

p#ate#ets i!pro"e "as%u#ar integrity i!pro"e p#ate#et pro'u%tionPre'nisone 1>2 !g/5g/'ayB!a :0 /'ayC in 9 'i"i'e' 'oses 2>4 w5s

$o##owe' by a taper o$ 4!g / 5g/'ay 'i"i'e' into 9 'oses $or 4 'ays.)

Methy#pre'niso#oneB 90>;0 !g /5g/'ayC $or 9>@ 'ays(o!e %ases !ay nee' repeat %ourses


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I I0Me%hanis! o$ a%tion is !u#ti>$a%toria# inhibition o$

antibo'y a'sorption to p#ate#ets pre"ention o$ )E(upta5e o$ auto ab %oate' p#ate#ets intera%tion o$ autoabs with i'iotype abs in I6 I &

or5s better than steroi's but higher %ostDose400!g /5g/'ay ; 'ays.)(ing#e 'ose o$ 1g/5g


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!nti1Rho(D) imm'ne lob'lin has been shown to be e$$e%ti"ePlatelet transf'sion use' in %ase o$ #i$e threatening

b#ee'ingBI*HCThe therapy is targete' to in%rease %ount to 20 000&In ris5 o$ #i$ethreatening b#ee'ing I6 I %ou#' be repeate' or %o!bine' withsteroi'sMonitoring the patients getting phar!a%o#ogi% inter"ention theusua# hospita# stay is 2 'ays&In the a!bu#atory setting p#ate#et%ount !onitore' 1>2 ti!es /w5 inter"a# %an in%rease as thep#ate#et in%rease &!onitoring is ne%essary unti# the %ount return to

1;0 000/!i%ro3B;0 in 1 !ont @0 in 9 !onthsC


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Chronic I%P persistentthro!bo%ytopeniaBF1;0 000/!i%ro3C $or : !onths

20>90 wi## ha"e %hroni% ITP1/9 r' o$ the %ases wi## ha"e spontaneous re!ission in

!onths to years In %hroni% ITP the p#ate#et %ount ranges between

20 000>@; 000/!i%ro3&usua##y 'o not reGuire anytreat!ent

Manage!ent 'e%rease the ris5 $or b#ee'ing&


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Phar!a%o#ogi% therapy is use' when they ha"esigni$i%ant b#ee'ing/or nee' to go $or pro%e'ures

*.)TI*.(TE).ID(>short %ourse/pu#se %ourseI6 I K ;00 !g /5g/'ay 2 'ays an' !ay be repeate'i$ the sy!pto!s re%ur (P3ENE*T.MY> !ay be nee'e' in patients nee'ing

repeate' /%ontinuous phar!a%o#ogi% inter"ention e"en12 !onths a$ter 'iagnosis)itu i!ab Dana-o# Inter$eron *y%#osporin *y%#ophospha!i'e )o!ip#osti! E#to!bopag A-athioprine


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,iblio raphy

,p To Date Treat!ent an' prognosis o$ ITP in%hi#'ren %#ini%a# !ani$estation an' 'iagnosis o$ITP in %hi#'ren E"a#uating purpura in %hi#'ren

AA P August 1 2001 E"a#uating the *hi#'with Purpura&

Documents

Senior Case Presentation 2