previously known as Histiocytosis X

Management of Adult Patients with LCH.pdf

Dif­fe­rent working groups ha­ve re­view­ed avail­able in­ter­na­tio­nal and na­tio­nal gui­de­lines and re­com­men­da­ti­ons for dia­gno­sis, tre­at­ment and fol­low-up of LCH. A syn­the­sis of this in­for­ma­ti­on was ge­ne­ra­ted and publis­hed af­ter ap­pro­val of ma­ny in­vol­ved spe­cia­lists and is available from the EURO-Histio-Net website linked in the following LCH pages.

· Lan­ger­hans Cell His­tio­cy­to­sis (LCH) – Adults
Re­com­men­da­ti­ons for dia­gno­sis, cli­ni­cal work-up and treatment of adults; Ver­si­on 1, Au­gust 2013
http://www.eurohistio.net/e1623/e1554/e3219/index_eng.html

The Guidelines include:
· Back­ground, Pro­cess of De­ve­lop­ment and Re­stric­tions
The­re are no uni­ver­sal­ly ac­cep­ted in­ter­na­tio­nal gui­de­lines avail­able for the dia­gno­sis and tre­at­ment of adult LCH pa­ti­ents.
· Ge­ne­ral con­s­ide­ra­ti­on
· Dia­gno­sis
De­fi­ni­ti­ve dia­gno­sis of LCH re­qui­res clas­si­cal his­topa­tho­lo­gy con­fir­med by de­mons­tra­ti­on of CD1a or Lan­ge­rin (CD207) or by the ul­tra­struc­tu­ral pre­sence of Bir­beck gra­nu­les.

· Pre­tre­at­ment cli­ni­cal eva­lua­ti­on
o Histo­ry and phy­si­cal ex­ami­na­ti­on
o La­bo­rato­ry and ra­dio­gra­phic eva­lua­ti­on
o Fur­ther in­ves­ti­ga­ti­ons for spe­ci­fic cli­ni­cal sce­na­ri­os

· Or­gan in­vol­ve­ment
Af­ter the dia­gno­sis of LCH has be­en ma­de, in­vol­ve­ment of other or­gans should be eva­lua­ted and de­fi­ned ac­cor­ding to the cli­ni­cal, bio­lo­gi­cal or ra­dio­lo­gi­cal cri­te­ria.

o Stra­ti­fi­ca­ti­on / Cli­ni­cal clas­si­fi­ca­ti­on
o Risk or­gans and spe­cial sites
o En­do­cri­no­lo­gic dys­func­tion
o Der­ma­to­lo­gi­cal in­vol­ve­ment
o Gas­tro­in­tes­ti­nal in­vol­ve­ment

· Tre­at­ment

Tre­at­ment re­com­men­da­ti­ons are ba­sed on site and ex­ten­si­on of the di­sea­se.
o Ma­nage­ment al­go­rithm
o Care­ful ob­ser­va­ti­on, lo­cal or “mild sys­te­mic” the­ra­py
o Sys­te­mic the­ra­py
o Tre­at­ment op­ti­ons in ca­se of re­ac­tiva­ti­on
o Ra­dio­the­ra­py
o Tre­at­ment and hor­mone re­pla­ce­ment of en­do­cri­no­pa­thies
o Cen­tral ner­vous sys­tem in­vol­ve­ment

· Pri­ma­ry pul­mon­a­ry LCH

Alt­hough the lungs may be af­fec­ted si­mul­ta­neo­us­ly with other or­gans an iso­la­ted pul­mon­a­ry LCH is ob­ser­ved mo­re fre­quent­ly and re­p­res­ents a spe­cial form of adult LCH.
o Epi­de­mio­lo­gy and cli­ni­cal fea­tures
o Dia­gno­sis
o Tre­at­ment and pro­gno­sis

· Pre­g­nan­cy

· Fol­low-up

LCH may re­ac­tiva­te and lead to chro­nic lo­cal sym­ptoms or in­du­ce or­gan dys­func­tion. Ra­re­ly LCH is as­so­cia­ted with ma­li­gnant tu­mors. The­re­fo­re, fol­low-up in­ves­ti­ga­ti­ons of di­sea­se and mo­ni­to­ring of func­tio­nal im­pair­ments are ne­cessa­ry.

Our thanks to all of our Histio Partners for their input to these pages.