My hand is bluer than blue

A case of a 39 year old male with cyanosis and numbness of the distal phalanges of the left hand
Angelo P. Ampong, MD Emergency Medicine Resident East A enue Medical !enter

HISTORY OF PRESENT ILLNESS Patient is a 39 year old single unemployed male from Payatas !ue"on #ity $%o &ame in $it% a &%ief &omplaint of &yanosis of t%e left finger tips' (t appro)imately *am patient e)perien&ed sudden &yanosis num+ness and tingling sensation of t%e distal p%alanges of t%e left %and' Patient soug%t &onsult at a lo&al &lini& +e&ause of persisten&e of pre,ious symptoms' He $as found to %a,e a -P of .*/0.// on t%e rig%t %and +ut a+sent +lood pressure and radial pulse on t%e left %and' Patient $as ad,ised to see1 furt%er e,aluation from a tertiary %ospital' RE2IE3 OF SYSTE4S 567 anore)ia 567 $eig%t loss 567 malaise 567 fe,er

567 &%est pain diffi&ulty of +reat%ing 567 dysuria PERSON(L SO#I(L HISTORY Non smo1er O&&asional (l&o%oli& +e,erage drin1er No inta1e of illi&it su+stan&es F(4ILY HISTORY 587 Hypertension 6 4ot%er 567 (st%ma

567 ,omiting a+dominal pain diarr%ea

567 9ia+etes

567 PT-

567 #ardia& disease 567 T%yroid disorder

PHYSI#(L E:(4IN(TION #ons&ious &o%erent am+ulatory not in distress -P; "#$%"$$ 5R(7 no appre&ia+le -P 5L(7 HR; *< RR; .9 Temp; 3<'< HEENT; an&teri& s&lerae pin1 palpe+ral &on=un&ti,ae 567 nasal flaring 567 #L(9 #%est; S#E 567 $%ee"e 567 &ra&1les 567 retra&tions Heart; (dynami& pre&ordium normal rate regular r%yt%m 567 murmur (+domen; flat normo a&ti,e +o$el sounds soft non6tender E)tremity; 587 &yanosis on t%e distal p%alanges of t%e left %and 567 &lu++ing 567 +ipedal edema 4otors; >0> on all e)tremities Sensory; .// ?

INITI(L I4PRESSION (&ute (rterial O&&lusion t0& Ta1ayasu@s (rteritis t0& Poly&yt%emia ,era

#OARSE (T THE E4ERBEN#Y 9EP(RT4ENT

9;C* pm R(; .*/0.// L(; / pulses R(; 88 L(; /

9(T I2F; PNSS .L ) DH Diagnostics& #:R P6( #-# PT aPTT Na E -AN &rea .CL E#B T%erapeuti&s; gi,e (spirin D/ mg0ta+ F ta+s &%e$ed P'O' gi,e #lopidogrel *> mg0ta+ F ta+s PO

"$&3' pm R(; .</0.// L(; / pulses R(; 88 L(; /

gi,e Heparin F <// units I2 gi,e Prednisone </ mg P'O'

""&($ pm R(; .</0.// L(; /

Patient still for &%est )ray follo$ up la+ results

pulsesR(; 88 Hemoglo+in Hemato&rit 3-# Neut Lym 4ono Platelet 4#2 4#H 4#H# R93

L(; / .9C g0L /'>*> 5H7 5H7 .F/6.*/ /'F/6/'>/

*'* )./90L /'*< ? /'.D ? /'/< ? .*> )./90L .//'D fL 5H7 33'* pg 5H7 33> g0L .>'> ? 5H7 D/6.// C*63. 3C/63</ ..'<6.F'<

PT ? (&ti,ity INR #ontrol apTT #ontrol -AN #rea Na E #%est :ray; Fi+ro %a"y infiltrates RALF Normal &ardia& si"e

C.'9 *> .'9* .3'< 3.'F CD'/ C'D mg0dL /'F mg0dL .3>'3 mmol0L C'<C mmol0L

.C'96 .>'*

C<63.

Anremar1a+le (orta

#OARSE (T THE 3(R9S ./090.C F;F> am 9(T (ssessment; t0& (&ute arterial o&&lusion C to Hyper&oagula+le state t0& Poly&yt%emia ,era For )A*+ assay &0o NETI If $it%out funds may do EP, le el For Arterial Duple- study of t%e Apper E)tremity Start Heparin drip; PNSS.L 8 >/// units of Heparin to run at .<&&0%r Sim,astatin F/ mg O9

Refer to Hematology Se&tion for &o6management and phlebotom

FIRST HOSPIT(L 9(Y

10/9/12

9:50 am For arterial duplex study of UE & LE Start Cilostazol 100 m !o" t#e! $%& Start 'S' (0 m )&

*+, radial- .ra/#ial 12:10 pm pulse L

'ssessme!t: t/c Acute Limb Ischemia t/c Takayasus Arteritis

THIR9 HOSPIT(L 9(Y

FOARTH HOSPIT(L 9(Y

FIFTH HOSPIT(L 9(Y

On t%e nint% %ospital day patient under$ent p%le+otomy' F>/ mL of +lood $as o+tained' On t%e t%irteent% %ospital day patient under$ent repeat p%le+otomy'

FOARTEENTH HOSPIT(L 9(Y

9IFFERENTI(L 9I(BNOSES

9IS#ASSION Poly&yt%emia ,era 5P#27 +eing a primary poly&yt%emia is &aused +y neoplasti& proliferation and maturation of eryt%roid mega1aryo&yti& and granulo&yti& elements to produ&e $%at is referred to as panmyelosis In &ontrast to se&ondary poly&yt%emias P#2 is asso&iated $it% a lo$ serum le,el of t%e %ormone eryt%ropoietin 5EPO7' Instead P#2 &ells %a,e a mutation in t%e tyrosine 1inase 5G(EC7 $%i&% a&ts in signaling pat%$ays of t%e EPO6re&eptor rendering t%ose &ells %ypersensiti,e to EPO' Patients $it% poly&yt%emia ,era &an +e asymptomati&' ( &lassi& symptom of poly&yt%emia ,era is pruritus or it&%ing parti&ularly after e)posure to $arm $ater Patients $it% poly&yt%emia ,era are prone to t%e de,elopment of +lood &lots' ( ma=or t%rom+oti& &ompli&ation may sometimes +e t%e first symptom or indi&ation t%at a person %as poly&yt%emia ,era

P%ysi&al e)am findings are non6spe&ifi& +ut may in&lude enlarged li,er or spleen plet%ora or gouty nodules' T%e diagnosis is often suspe&ted on t%e +asis of la+oratory tests' #ommon findings in&lude an ele,ated %emoglo+in le,el or %emato&rit refle&ting t%e in&reased num+er of red +lood &ellsH t%e platelet &ount or $%ite +lood &ell &ount may also +e in&reased' T%e eryt%ro&yte sedimentation rate 5ESR7 is de&reased due to an in&rease in "eta potential' -e&ause poly&yt%emia ,era results from an essential in&rease in eryt%ro&yte produ&tion patients %a,e a lo$ eryt%ropoietin 5EPO7 le,el' Re&ently in C//> a mutation in t%e )A*+ .inase 52<.*F7 $as found +y multiple resear&% groups to +e strongly asso&iated $it% poly&yt%emia ,era' JAK2 is a mem+er of t%e Ganus 1inase family and ma1es t%e eryt%roid pre&ursors %ypersensiti,e to eryt%ropoietin 5EPO7'

Efficacy and /afety of 0ow1Dose Aspirin in Polycythemia 2era

Raffaele Landolfi 4'9' Ro+erto 4ar&%ioli 4'9' Ga&1 Eutti 4'9' Hein" Bisslinger 4'9' Bianni Tognoni 4'9' #arlo Patrono 4'9' and Ti"iano -ar+ui 4'9' for t%e European #olla+oration on Lo$69ose (spirin in Poly&yt%emia 2era In,estigatorsI

-(#EBROAN9 T%e use of aspirin for t%e pre,ention of t%rom+oti& &ompli&ations in poly&yt%emia ,era is &ontro,ersial' 4ETHO9S >.D patients $it% poly&yt%emia ,era $ere enrolled no &lear indi&ation for aspirin treatment and no &ontraindi&ation to su&% treatment in a dou+le6+lind pla&e+o6&ontrolled randomi"ed trial to assess t%e safety and effi&a&y of prop%yla)is $it% lo$6dose aspirin 5.// mg daily7' T%e t$o primary end points $ere t%e &umulati,e rate of nonfatal myo&ardial infar&tion nonfatal stro1e or deat% from &ardio,as&ular &auses and t%e &umulati,e rate of nonfatal myo&ardial infar&tion nonfatal stro1e pulmonary em+olism ma=or ,enous t%rom+osis or deat% from &ardio,as&ular &auses' T%e mean duration of follo$6up $as a+out t%ree years ' RESALTS Treatment $it% aspirin as &ompared $it% pla&e+o reduced the ris. of t%e &om+ined end point of nonfatal myo&ardial infar&tion nonfatal stro1e or deat% from &ardio,as&ular &auses 5relati,e ris1 /'F.H 9> per&ent &onfiden&e inter,al /'.> to .'.>H PJ/'/97 and t%e ris1 of t%e &om+ined end point of nonfatal myo&ardial infar&tion nonfatal stro1e pulmonary em+olism ma=or ,enous t%rom+osis or deat% from &ardio,as&ular &auses 5relati,e ris1 /'F/H 9> per&ent &onfiden&e inter,al /'.D to /'9.H PJ/'/37' O,erall mortality and &ardio,as&ular mortality $ere not redu&ed signifi&antly' T%e in&iden&e of ma=or +leeding episodes $as not signifi&antly in&reased in t%e aspirin group 5relati,e ris1 .'<CH 9> per&ent &onfiden&e inter,al /'C* to 9'*.7' #ON#LASION

Lo$6dose aspirin &an safely pre,ent t%rom+oti& &ompli&ations in patients $it% poly&yt%emia ,era $%o %a,e no &ontraindi&ations to su&% treatment