Langerhans Cell Histiocytosis – Children

previously known as Histiocytosis X

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) – Child­ren
Gui­de­lines for dia­gno­sis, cli­ni­cal work-up and tre­at­ment du­ring child­hood; Ver­si­on 1.1, March 2011 http://www.eurohistio.net/e1623/e1554/e1856/index_eng.html

The Guidelines include:
· Me­thods and Pro­cess of de­ve­lop­ment
· Pur­po­se and Re­stric­tions
· Ge­ne­ral Con­s­ide­ra­ti­ons

· Dia­gno­sis

De­fi­ni­ti­ve dia­gno­sis of LCH re­qui­res clas­si­cal his­topa­tho­lo­gy con­fir­med by de­mons­tra­ti­on of CD1a or Lan­ge­rin (CD207) or by the ul­tra­struc­tu­ral pre­sence of Bir­beck gra­nu­les.

o Bio­p­sy and His­to­lo­gi­cal Ex­ami­na­ti­on
o Dia­gnostic Cri­te­ria

· Pre­tre­at­ment Cli­ni­cal Eva­lua­ti­on

The fol­lo­wing pa­ti­ent eva­lua­ti­on is re­com­men­ded on­ly for pa­ti­ents with de­fi­ni­te dia­gno­sis of LCH and for tho­se with pres­ump­ti­ve LCH in whom a bio­p­sy is de­fer­red for the re­a­sons men­tio­ned in the chap­ter Bio­p­sy / His­to­lo­gy. In all other ca­ses we suggest eit­her con­sul­ting the re­fer­ral cen­ter of your coun­try or sha­ring the his­to­lo­gi­cal sli­des with other re­fe­rence or spe­cia­li­zed cen­ters:
o Re­com­men­ded Eva­lua­ti­on in Every Ca­se
o Spe­ci­fic Cli­ni­cal Sce­na­ri­os and Re­com­men­ded Ad­di­tio­nal Tes­ting
o De­fi­ni­ti­on of Or­gan In­vol­ve­ment
o Head MRI in LCH Pa­ti­ents

· Cli­ni­cal Clas­si­fi­ca­ti­on
Tre­at­ment re­com­men­da­ti­ons are ba­sed on site and ex­ten­si­on of the di­sea­se which is why it is im­portant to dis­tin­gu­ish bet­ween sin­gle sys­tem LCH and mul­ti­sys­tem LCH.

· Dif­fe­ren­ti­al Dia­gno­sis
· Tre­at­ment

Whe­ne­ver pos­si­ble pa­ti­ents should be en­rol­led in on­go­ing cli­ni­cal tri­als and trea­ted ac­cor­ding to the pro­to­col. CHILD­REN who are not en­rol­led in a cli­ni­cal tri­al should be trea­ted ac­cor­ding to the gui­de­lines pre­sen­ted he­re. Due to the di­ver­si­ty of cli­ni­cal cour­se of LCH, even re­com­men­da­ti­ons which are es­ta­blis­hed as stan­dard of ca­re may need to be cri­ti­cal­ly ap­prai­sed in an in­di­vi­du­al ca­se. We sug­gest that you ne­ver he­si­ta­te to con­ta­ct LCH ex­perts in ca­se that the cli­ni­cal cour­se rai­ses ques­ti­ons or doubts.

o Ma­nage­ment Al­go­rithms
o Lo­cal The­ra­py or Care­ful Ob­ser­va­ti­on
o Sys­te­mic The­ra­py
o Tre­at­ment Op­ti­ons in Ca­se of Re­ac­tiva­ti­on

· Fol­low-up of LCH pa­ti­ents

Ge­ne­ral Con­s­ide­ra­ti­ons; Dia­be­tes in­si­pi­dus; Hor­mo­ne De­fi­ci­en­cies; Or­tho­pa­edic Se­que­lae; Pro­blems with Ears, Oral Tis­sue and Jaw; Neu­ro­lo­gi­cal Se­que­lae; Da­ma­ge of Lung and Li­ver
o Mo­ni­to­ring for Per­ma­nent Con­se­quen­ces
o Fol­low-up – Re­qui­red Tests and Fre­quen­cy
o Ma­li­gnan­cies As­so­cia­ted with Lan­ger­hans Cell His­tio­cy­to­sis

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