RANDA AL-HARIZY

IROI OI INTLRNAL MLDICINL

Iiguio 16-3: Honalopoiosis
Iiguio 16-5c: ono naiiov
Anonia is dofinod as a diop of honogIolin oi
honalociil loIov noinaI laking inlo
considoialion lhal aIloialions of lho pIasna
voIuno Ioad lo faIso changos in lIood
honogIolin IovoI.
-84:90
Docioasod iod coII pioduclion
Slon coII faiIuio
ApIaslic anonia
Anonia of Ioukonia and noyIodyspIaslic syndionos
Iiogoniloi oiylhioid coII faiIuio
Iuio iod coII apIasia
Anonia of oigan faiIuio
Anonia of chionic disoaso
Lndociino disoidois
Iiocuisoi coII faiIuio (dyshaonopoiolic anonia)
Iion doficioncy anonia
MogaIolIaslic anonia
MyoIodyspIaslic syndionos (MDS)
Incioasod iod coII dosliuclion oi Ioss
Hoiodilaiy
Monliano dofocls o.g. hoiodilaiy sphoiocylosis
CIolin dofocls o.g. lhaIassonia, sickIo coII anonia
Lnzyno dofocls o.g. C6ID dofioncy
Acquiiod
Innuno honoIylic anonia
HypoispIonisn
Micioangiopalhic honoIylic anonia o.g. DIC
Aculo lIood Ioss
IaioxysnaI nocluinaI honogIolinuiia
MIcrncytIc ancmIa
1. Iion doficioncy anonia
2. ThaIassonia
3. Anonia of chionic
disoaso
4. SidoiolIaslic anonia
NnrmncytIc ancmIa
1. HonoIylic anonia
2. Aculo posl honoiihagic
anonia
3. Slon coII faiIuio
4. Anonia of chionic
disoaso
5. Anonia of oigan faiIuio
MacrncytIc ancmIa
1. MogaIolIaslic
anonia
2. RolicuIocylosis
ospociaIIy in
honoIylic anonia
3. MyoIodyspIaslic
syndiono
4. Hypolhyioidisn
Iiguio 16-8: Iion nolaloIisn
Iion doficioncy anonia is ono of lho nosl connon disoasos
affocling ovoi ono haIf liIIion poopIo voiIdvido
LlioIogy:
Incioasod iion Ioss as in poplic uIcoi disoaso, ancyIoslona
duodonaI, vaginaI lIooding, honoiihagic lIood disoaso,
olhoi oiificiaI lIooding ...olc
Docioasod iion inlako pailicuIaiIy soon in infanls, oId
individividuaIs and in faninos
Docioasod iion alsoiplion: physicaI slalo (hono > Io2+ >
Io3+), Iov gasliic acidily (gaslioclony, vagolony,
poinicious anonia, piolon punp inhililois, anlacids),
disoasos of inloslinaI sliucluio (Ciohns, coIiac disoaso),
inhililois (phylalos, lannins)
Incioasod iion ioquiiononls as in fonaIos in chiIdloaiing
poiiod, chiIdion and adoIosconls
CIinicaI picluio:
CIinicaI picluio of anonia
Ioaluios of iion doficioncy
1. Inpaiiononl of giovlh and inloIIocluaI
dovoIopnonl in chiIdion
2. LpilhoIiaI changos such as koiIonychias,
anguIai slonalilis, and IIunnoi-Vinson
syndiono
CIinicaI picluio of lho causo of iion doficioncy
anonia
nvcstIgatInns:
1. DIagnnsIs nf Irnn dcfIcIcncy ancmIa
ConpIolo lIood counl (CC): hypochionic niciocylic anonia
Iion sludios: Iov soiun iion (N: 7O-14O ug/dI), high lolaI iion
linding capacily (TIC) (N: 25O-4OO ug/dI), Iov liansfoiiing
saluialion (N: 3O) and Iov soiun foiiilin (N: 1-3O ug/dI)
Soiun soIulIo liansfoiiin iocoplois Tho nunloi of liansfoiiin
iocoplois incioasos in iion doficioncy. This assay can hoIp lo
dislinguish lolvoon iion doficioncy and anaonia of chionic
disoaso, and nay avoid lho nood foi lono naiiov oxaninalion
ovon in conpIox casos.
ono naiiov spocinon slainod foi iion: docioasod naiiov iion
sloios (nol ioulinoIy dono lo diagnoso IDA)
2. DIagnnsIs nf thc causc nf chrnnIc b!nnd !nss
CaslioinloslinaI ondoscopios, laiiun sludios, slooI anaIysis foi
occuIl lIood & paiasilos. Invosligalions foi lIooding disoidois.
Tioalnonl: Iion doficioncy is nol a diagnosis poi so. Tho coiiocl nanagononl of iion
doficioncy is lo find and lioal lho undoiIying causo, and lo givo iion lo coiiocl lho
anaonia and iopIaco iion sloios. Tho iosponso lo iion lhoiapy can lo noniloiod
using lho iolicuIocylo counl and Hl IovoI, vilh an oxpoclod iiso in haonogIolin of
1 g/dL poi vook.
1. Coiioclion of lho undoiIying olioIogy is of ulnosl inpoilanco
2. Iion suppIononlalion
ORAL IRON
Iiopaialions: poIysacchaiido iion conpIox, foiious gIuconalo, foiious suIphalo
Doso: 15O-2OO ng lds
Sido offocls: gaslioinloslinaI disconfoil
IARLNTLRAL IRON
Indicalions
1. OiaI iion is pooiIy loIoialod
2. Rapid iopIacononl is noodod
3. Inpaiiod iion alsoiplion
Iiopaialions: Iion doxlian (IV,IM), iion sacchaiido (IM)
Doso: 1ng/day
Sido offocls: pain, skin pignonlalion, and alscoss al lho junclion silo. Iovois, iigois,
uilicaiia, hypolonsion, anaphyIaxis
Dofinilion:
Disoidoi causod ly inpaiiononl of DNA
synlhosis iosuIling in doIayod division of
iapidIy pioIifoialing coIIs (honopoiolic coIIs,
gaslioinloslinaI opilhoIiun, skin.) vilh
piogiossivo coII giovlh.
tIn!ngy:
Fn!atc dcfIcncy:
Docioasod inlako: pooi nuliilion, aIcohoI
Incioasod ioquiiononl:
Iiognancy, Iaclalion, pionaluiily.
Incioasod coII luinovoi: chionic honoIysis, oxfoIialivo doinalilis
Docioasod alsoiplion:
Disoasos of lho snaII inloslino o.g. liopicaI and non liopicaI spiuo
Lxcoss Ioss: honodiaIysis, poiilonoaI diaIysis, piognancy
Diugs: anliconvuIsanls, nolholioxalo, hydioxyuioa, azalhiopiino
nba!amIn (B12) dcfIcIcncy:
Docioasod inlako: vogolaiians
Docioasod alsoiplion: gasliic causos (poinicious anonia, gaslioclony,
ZoIIingoi-LIIison syndiono) and inloslinaI causos (iIoaI iosoclion oi
disoaso, lIind Ioop syndiono, fish lapovoin)
nbnrn crrnrs nf cnbn!amIn and/nr fn!atc mctabn!Ism
!InIca! pIcturc:
CIinicaI picluio of anonia, Ioucoponia and
lhionlocyloponia
CaslioinloslinaI disoidois:
Aliophy of lho IinguaI papiIIao, gasliic aliophy
and achIohydiia
Noivous syslon disoidois: poiiphoiaI
nouiopalhy, sulaculo conlinod dogonoialion
(posloiioi coIunn and pyianidaI liacl Iosion),
psychosis (nogaIolIaslic nadnoss)
nvcstIgatInns:
CC: anonia, incioasod MCV, anisocylosis, poikiIocylosis,
iolicuIocyloponia, Ioucoponia and lhionlocyloponia
ono naiiov oxaninalion: hypoicoIIuIai, nogaIolIaslic naiiov
iochonicaI ovidonco of honoIysis: incioasod LDH & indiiocl
liIiiulin
Lov soiun 12 IovoI, Iov soiun foIalo IovoI oi noio accuialoIy
Iov iod coII foIalo IovoI
SchiIIing losl:
1. ColoIanin alsoiplion can lo doloclod ly noasuiing uiinaiy
iadioaclivily afloi ingoslion of an oiaI doso of iadioaclivo
colaInin. If oxciolion is Iov poinicious anonia is a possiliIily
2. Tho losl is iopoalod afloi addilion of oiaI inliinsic facloi,
incioasod uiinaiy iadioaclivily indicalos lho oiiginaI alnoinaIily
is inliinsic facloi dofioncy
%hcrapy:
12 doficioncy: 1OOO ug hydioxo oi
cyanocolaIanin IM daiIy foi ono nonlh
foIIovod ly lvico vookIy injoclions unliI
noinaIizalion of honalociil lhon nonlhIy
injoclion foi Iifo
IoIalo doficioncy: 1-5 ng oiaIIy daiIy foi ono
nonlh. 12 doficioncy shouId lo oxcIudod
olhoiviso nouioIogicaI nanifoslalions voison
Tioalnonl of lho causo if possilIo and of
conpIicalions if piosonl
!crnIcInus ancmIa:
A disoaso of niddIo ago and a faniIy hisloiy
nay lo piosonl. Thoio is auloinnuno
dosliuclion of paiiolaI gasliic coIIs Ioading lo
faiIuio of inliinsic facloi sociolion vilh posilivo
anlilodios lo paiiolaI coIIs and inliinsic facloi.
Il nay lo associalod vilh olhoi phononona
such as pionaluio giaying of haii, viliIigo, and
incioasod incidonco of cancoi slonach
Anonia of chionic disoaso is a condilion of inpaiiod iion
uliIizalion vhoio funclionaI iion (honogIolin) is Iov lul
lissuo iion (such as in sloiago) is noinaI oi high
Il occuis in condilions of chionic infoclions, infIannaloiy
condilions oi naIignanl disoasos
Ialhogonosis: inpaiiod ioIoaso of iion fion naciophagos,
docioasod pioduclion of oiylhiopooilin and inpaiiod
iosponso lo ils aclion, ioducod iod coII Iifo span
Anonia is iniliaIIy noinocylic noinochionic lhon locono
niciocylic hypochionic
Iion sludios shov Iov soiun iion, Iov lolaI iion linding
capacily, Iov liansfoiiin saluialion and noinaI oi high
soiun foiiilin IovoI
Tioalnonl: lioalnonl of lho causo, packod RCs liansfusion
in sovoio anonia, oiylhiopoiolin nay lo usofuI
A gioup of disoidoi chaiacloiizod ly hypocoIIuIai
naiiov and poiiphoiaI pancyloponia
LlioIogy:
Hoiodilaiy: Ianconi anonia
Acquiiod:
1. Idiopalhic
2. Socondaiy:
ChonicaIs (lonzono, insoclicidos), diugs (anlicancoi
diugs, anli-infIannaloiy, anliliolics, anli-opiIoplic
diugs, anli-dialolic diugs, anlilhyioid diugs, and
psycholiopics), ionizing iiiadialion, infoclions
(hopalilis viiusos, noasIos), paioxysnaI nocluinaI
honogIolinuiia, piognancy
Invosligalions: CC (pancyloponia), lono
naiiov: hypocoIIuIai naiiov vilh incioasod
fal spacos, no alnoinaI coIIs, invosligalions lo
dolocl lho causo
DiffoionliaI diagnosis (causos of pancyloponia):
1. MogaIolIaslic anonia
2. MyoIodyspIaslic syndionos
3. HypoispIonisn
4. ono naiiov infiIlialion (nyoIophlhisic
anonia)
5. Auloinnuno
Tioalnonl:
trcatmcnt nf thc causc
suppnrtIvc trcatmcnt:
1. Tiansfusion lhoiapy vilh packod RCs oi pIaloIols
2. Ioi piophyIaxis and lioalnonl of infoclions:
IsoIalion in a sloiiIo onviionnonl, anliliolics,
anliviiaI and anlifungaI diugs and CianuIocylo
coIony slinuIaling facloi (C-CSI) lo incioaso
nouliophiI counl
DcfInItIvc thcrapy: aIIogonic slon coII
lianspIanlalion, innunosuppiossivo lhoiapy
(anlilhynocylo gIoluIin, cycIospoiin and sloioids),
andiogons
Dofinilion: anonia fion shoiloning of iod coII
suivivaI (noinaI avoiago is 12O days)
LlioIogy:
Inhoiilod
Monliano alnoinaIily: Hoiodilaiy
sphoiocylosis, hoiodilaiy oIIiplocylosis
HonogIolin alnoinaIily: lhaIassaonia, sickIo
coII disoaso
Lnzyno alnoinaIily: gIucoso -6-phosphalo
doficioncy, pyiuvalo kinaso doficioncy
Acquiiod:
Innuno: auloinnuno honoIylic anonia,
aIIoinnuno: AO and Rh inconpaliliIily
Non-innuno: nonliano dofocls (INH, Iivoi and
ionaI disoaso), nochanicaI (nicioangiopalhic
honoIylic anonia, vaIvo piolhosis, naich
honogIolinuiia), infoclions (naIaiia),
hypoispIonisn, chonicaIs (snako vonons)
N: AII hoiodilaiy causos aio coipuscuIai, vhoioas aII
acquiiod causos, oxcopl INH, aio oxliacoipuscuIai
Manifoslalions of anonia
Manifoslalions of honoIysis: jaundico,
hopalospIononogaIy, pignonl gaII slonos vhich nay
Ioad lo olsliuclivo jaundico
Manifoslalions of ciisos:
1. HonoIylic ciisis: Iovoi, iigois aldoninaI oi lony
achos, Woisoning of anonia, dooponing of jaundico,
daik discoIoialion of uiino (honogIolinuiia),
Incioasod iolicuIocylic counl
2. ApIaslic ciisis: May lo piocipilalod vilh infoclions,
ospociaIIy paivoviius 19, Ioading lo naiiov
suppiossion, Woisoning of anonia vilhoul dooponing
of jaundico, Docioasod iolicuIocylic counl
3. MogaIolIaslic ciisos: Aggiavalion of anonia
and possilIy pancyloponia duo lo ioIalivo
foIalo doficioncy, Docioasod iolicuIocylic counl
4. Soquoslialion ciisis: Aggiavalion of anonia and
incioaso in spIonic sizo duo lo pooIing of iod
coIIs in lho spIoon
5. Vaso-occIusivo ciisis: Occuis onIy in sickIo coII
anonia
1- Diagnosis of honoIylic anonia:
CC:
noinocylic noinochionic anonia vilh iolicuIocylosis
Anonia nay lo naciocylic duo lo naikod iolicuIocylosis oi foIalo
doficioncy
Anonia nay lo niciocylic in lhaIassonia
Whilo lIood coIIs and pIaloIols aio usuaIIy noinaI lul nay lo highoi lhan
noinaI duo lo slinuIalod naiiov oi Iovoi in caso of nogaIolIaslic ciisis
Iood fiIn nay shov lho chaiacloiislic iod coII of sono disoasos
ono naiiov: hypoicoIIuIai noinolIaslic naiiov, nay lo nogaIolIaslic in
foIalo doficioncy oi hypocoIIuIai in apIaslic ciisis
Shoilonod iod coII Iifo span noasuio ly Ci51 IaloIIod RCs
Incioasod indiiocl soiun liIiiulin, soiun LDH, focaI sloicoliIinogon and
uiinaiy uioliIinogon
In inliavascuIai honoIysis lhoio is honogIolinonia, honogIolinuiia,
honosidoiinuiia and diop in haplogIolin
2- Diagnosis of lho causo:
Iood fiIn foi iod coII noiphoIogy o.g. laigol coIIs
in lhaIassonia, sickIo coIIs in sickIo coII anonia,
niciosphoiocylos in hoiodilaiy sphoiocylosis and
auloinnuno honoIysis, and iod coII fiagnonls in
liaunalic honoIysis
Osnolic fiagiIily losl foi hoiodilaiy sphoiocylosis
HonogIolin oIocliophoiosis foi
honogIolinopalhios
Assay foi C6ID and pyiuvalo kinaso
Coonls losl foi auloinnuno honoIysis
Tioalnonl of lho causo vhonovoi possilIo
Tiansfusion of packod RCs if indicalod as in caso
anonia is sovoio oi naikodIy synplonalic
IoIic acid 1 ng/kg
Tioalnonl of conpIicalions o.g. ciisos, olsliuclivo
jaundico, anonic hoail faiIuio and iion ovoiIoad
Tioalnonl of sickIo coII anonia incIudos fIuids,
oxygon, and anaIgosics foi vaso-occIusivo ciisis
and iod coII liansfusion foi coiioclion of anonia.
Roconl inloivonlions incIudo hydioxyuioa, slon
coII lianspIanlalion and gono lhoiapy logolhoi
vilh gonolic counsoIing and anlonalaI diagnosis
foi piovonlion
AulosonaI doninanl disoidoi
Thoio is doficioncy of a iod coII nonliano
Iipopioloins
HonoIylic anonia usuaIIy slails in chiIdhood
Osnolic fiagiIily of RCs docioasos and gol
doslioyod duiing passago in lho spIoon
SpIonoclony cuios up oi inpiovos lho
condilion
C6ID is a sox-Iinkod disoaso lhal nainIy affocls naIos and is
connon in Afiica, Modiloiianoan aioa and niddIo Lasl
C6ID hoIds a vilaI posilion in lho hoxoso nonophosphalo shunl
vhich gonoialos gIulalhiono lhal piolocls RCs fion oxidizing
agonls
In C6ID doficioncy, RCs can nol loIoialo oxidizing sliossos, foin
nolhonogIolin Ioading lo Iysis of RCs
CIinicaI picluio is lhal of aculo inliavascuIai honoIysis foIIoving:
1. Ingoslion of oxidizing diugs o.g. anlinaIaiiaIs, suIphonanidos,
aspiiin, phonacolin, vilanin K, niliofuianloin
2. Ingoslion of fava loans (favisn)
3. Infoclions o.g. viiaI hopalilis, lyphoid fovoi
4. Chionic honoIysis in lho alsonco of an oxidizing causo is iaio
5. Diagnosod ly C6ID assays
Any offonding causo shouId lo avoidod
HonogIolin is conposod of hono and gIolin,
gIolin consisls of 4 poIypoplido chains. AduIl
honogIolin consisls nainIy of Hl A (97) vhoio
gIolin is conposod of 2 aIpha and 2 lola chains,
Hl A2 (1-2) vilh 2 aIpha and 2 doIla chains, and
Hl I (1-2) vilh 2 aIpha and 2 ganna chains.
AlnoinaIilios in honogIolins can iosuIl fion
dofoclivo gIolin chain pioduclion (o.g.
lhaIassonia), gIolin chain sliucluio alnoinaIilios
(o.g. sickIo coII disoaso) oi lolh (o.g. sickIo
lhaIassonia)
AulosonaI doninanl disoidoi oiiginaIIy found
in poopIo Iiving in lho Modiloiianoan lasin lul
nov knovn lo affocl poopIo lhioughoul lho
voiId. Many lypos oxisl lho nosl connon is
lhaIassonia
In lhaIassonia lhoio is oilhoi alsonco of
chains pioduclion oi inpaiiononl of chain
pioduclion
ThaIassonia ninoi (liail):
SynplonIoss holoiozygous caiiioi slalo.
Anonia is niId oi alsonl, RCs shov
niciocylosis and hypochionia.
ThaIassonia inloinodia: anonia vaiios fion
niId lo sovoio vilh spIononogaIy and lono
dofoinilios lul niIdoi lhan lho liansfusion-
dopondonl lhaIassonia najoi
ChiIdion piosonl duiing lho fiisl yoai of Iifo
vilh sovoio anonia ioquiiing ioguIai
liansfusion
Manifoslalions of honoIylic anonia
IaiIuio lo lhiivo
LxlianoduIIaiy honalopoiosis lhal Ioads lo
naikod hopalospIononogaIy and lono
oxpansion vilh lho chaiacloiislic nongoIoid
facios
Manifoslalions of iion ovoiIoad
ChiIdion piosonl duiing lho fiisl yoai of Iifo
vilh sovoio anonia ioquiiing ioguIai
liansfusion
Manifoslalions of honoIylic anonia
IaiIuio lo lhiivo
LxlianoduIIaiy honalopoiosis lhal Ioads lo
naikod hopalospIononogaIy and lono
oxpansion vilh lho chaiacloiislic nongoIoid
facios
Manifoslalions of iion ovoiIoad
Invosligalions: in addilion lo finding of honoIylic
anonia
CC shovs niciocylosis and hypochionia
Hl oIocliophoiosis shovs a naikod incioasod Hl
I, ioducod oi alsonl Hl A and noinaI oi sIighlIy
high Hl A2
Lvidonco of iion ovoiIoad: high soiun iion, Iov
TIC and a high soiun foiiilin IovoI
SkuII X iay: haii on ond aiiangononl of lony
lialocuIalions
Lvidonco of hypoispIonisn in nany casos
Managononl:
Ain of lioalnonl is lo supioss inoffoclivo
oiylhiopoiosis and aIIov noinaI dovoIopnonl lhiough
ioguIai lIood liansfusion lo chiIdion kooping Hl
aiound 1O gn/dI
Iion choIalion, incIuding SC dosfoiiioxanino and oiaI
foins o.g. dofoiipono
SpIonoclony in palionls vilh hypoispIonisn paying
allonlion lo piophyIaxis againsl oncapsuIalod
oiganisns
Roconl inloivonlions incIudo hydioxyuioa, slon coII
lianspIanlalion and gono lhoiapy logolhoi vilh gonolic
counsoIing and anlonalaI diagnosis foi piovonlion
SickIo coII haonogIolin (Hl S) iosuIls fion a
singIo-laso nulalion of adonino lo lhynino vhich
pioducos a sulslilulion of vaIino foi gIulanino al
lho sixlh codon of lho |-gIolin chain
Ialhogonosis
Dooxygonalod Hl S noIocuIos aio insoIulIo and
poIynoiizo. Tho fIoxiliIily of lho coIIs is docioasod
and lhoy locono iigid and lako up lhoii
chaiacloiislic sickIo appoaianco.
SickIing can pioduco: a shoilonod iod coII suivivaI,
inpaiiod passago of coIIs lhiough lho
niciociicuIalion, Ioading lo olsliuclion of snaII
vossoIs and lissuo infaiclion.
Vaso-occIusive crises The earIiest presentation in
the first few years of Iife is acute pain in the hands
and feet (dactyIitis) owing to vaso-occIusion of the
smaII vesseIs. Severe pain in other bones, e.g.
femur, humerus, vertebrae, ribs, peIvis, occurs in
oIder chiIdren/aduIts. These attacks vary in
frequency from daiIy to perhaps onIy once a year.
Fever often accompanies the pain.
Anaemia Chronic haemoIysis produces a stabIe
haemogIobin IeveI, usuaIIy in the 6-8 g/dL range
but an acute faII in the haemogIobin IeveI can
occur owing to:
1. SpIenic sequestration
2. Bone marrow apIasia
3. Further haemoIysis.
Ciovlh and dovoIopnonl. Young chiIdion aio shoil lul iogain
lhoii hoighl ly aduIlhood. Hovovoi, lhoy ionain loIov lho
noinaI voighl.
8cncs aio a connon silo foi vaso-occIusivo opisodos, Ioading lo
chionic infaicls. AvascuIai nociosis of hips, shouIdois,
conpiossion of voiloliao and shoiloning of lonos in lho hands
and fool occui. Iain is a najoi piolIon. OsloonyoIilis is
connonoi in sickIo coII disoaso
Rospiialoiy. Tho aculo sickIo chosl syndiono occuis in up lo 3O,
and puInonaiy hypoilonsion and chionic Iung disoaso aio lho
connonosl causo of doalh of aduIls vilh sickIo coII disoaso. Tho
aculo chosl syndiono is causod ly infoclion, fal onloIisn fion
nociolic lono naiiov oi puInonaiy infaiclion duo lo
soquoslialion of sickIo coIIs.
cg u|ccrs occui sponlanoousIy (vaso-occIusivo opisodos) oi
foIIoving liauna and aio usuaIIy ovoi lho nodiaI oi IaloiaI
naIIooIi. Thoy oflon locono infoclod and aio quilo iosislanl lo
lioalnonl.
,rdi,c piolIons occui, vilh caidionogaIy, aiihylhnias and iion
ovoiIoad caidionyopalhy. MyocaidiaI infaiclions occui duo lo
lhionlolic opisodos vhich aio nol socondaiy lo alhoiona.
curc|cgic,| conpIicalions occui in 25 of palionls, vilh liansionl
ischaonic allacks, fils, coioliaI infaiclion, coioliaI haonoiihago
and cona.
ChoIoIilhiasis. Iignonl slonos occui as a iosuIl of chionic
haonoIysis.
Livoi. Chionic hopalonogaIy and Iivoi dysfunclion aio causod ly
liapping of sickIo coIIs.
RonaI. Chionic luluIo-inlosliliaI nophiilis occuis
Lyo. ackgiound iolinopalhy, pioIifoialivo iolinopalhy, vilioous
haonoiihagos and iolinaI dolachnonls aII occui.
Iiognancy. Inpaiiod pIaconlaI lIood fIov causos sponlanoous
aloilion, inliauloiino giovlh iolaidalion, pio-ocIanpsia and folaI
doalh.
Iiocipilaling faclois shouId lo avoidod oi lioalod quickIy.
Aculo painfuI allacks ioquiio suppoilivo lhoiapy vilh inliavonous
fIuids, oxygon, anliliolics and adoqualo anaIgosia. Moiphino is
lho diug of choico. MiIdoi pain can sonolinos lo ioIiovod ly
codoino, paiacolanoI and NSAIDs.
IiophyIaxis is vilh poniciIIin 5OO ng daiIy and vaccinalion vilh
poIyvaIonl pnounococcaI and ,cncpni|us inf|ucnz,c lypo
vaccino
IoIic acid is givon lo aII palionls vilh haonoIysis.
Tiansfusions shouId lo givon foi hoail faiIuio, TIAs, sliokos, aculo
chosl syndiono, aculo spIonic soquoslialion and apIaslic ciisos.
Lxchango liansfusions nay lo nocossaiy in palionls vilh sovoio oi
iocuiionl ciisos, oi lofoio onoigoncy suigoiy.
drcxc,ro,nidc (hydioxyuioa) is lho fiisl diug vhich has loon
vidoIy usod as lhoiapy foi sickIo coII anaonia. Il acls ly
incioasing Hl I conconlialions lul lho ioduclion in nouliophiIs
nay aIso hoIp.
InhaIod niliic oxido inhilils pIaloIol funclion, ioducos vascuIai
adhosion of iod coIIs and is aIso a vasodiIaloi. Il has loon shovn
lo ioduco opialo ioquiiononls in aculo painfuI opisodos.
ono naiiov lianspIanlalion has loon usod lo lioal sickIo coII
anaonia aIlhough in fovoi nunlois lhan foi lhaIassaonia.
ChiIdion and adoIosconls youngoi lhan 16 yoais of ago vho havo
sovoio conpIicalions (sliokos, iocuiionl chosl syndiono oi
iofiacloiy pain) and havo an HLA-nalchod donoi aio lho losl
candidalos foi lianspIanlalion.
cunsc||ing
!rngnnsIs
Sono palionls vilh Hl SS dio in lho fiisl fov yoais of Iifo fion
oilhoi infoclion oi opisodos of soquoslialion. Hovovoi, lhoio is
naikod individuaI vaiialion in lho sovoiily of lho disoaso and
sono palionls havo a ioIalivoIy noinaI Iifospan vilh fov
conpIicalions.