Imbali

I-Histiocytosis ligama eliqhelekileyo leqela leengxaki okanye "ii-syndromes" ezibandakanya ukwanda okungaqhelekanga kwinani leeseli ezimhlophe zegazi ezibizwa ngokuba zii-histiocytes.

Kutshanje, ulwazi olutsha malunga nosapho lwezifo lukhokelele kwiingcali ukuba zenze uluhlu olutsha. Iindidi ezintlanu ziphakanyisiwe:

• L iqela - kubandakanya i-Langerhans cell histiocytosis kunye nesifo i-Erdheim-Chester
• Iqela C-kubandakanya i-non-Langerhans cell histiocytosis ebandakanya ulusu
• Iqela M - kubandakanya i-histiocytosis enobungozi
• R - kubandakanya isifo sikaRosai-Dorfman
• H Iqela - libandakanya i-hemophagocytic lymphohistiocytosis

Eli nqaku ligxile kuphela kwiqela L, elibandakanya i-Langerhans cell histiocytosis kunye nesifo i-Erdheim-Chester.

Kubekho impikiswano malunga nokuba isifo seLangerhans cell histiocytosis kunye nesifo i-Erdheim-Chester sisifo sokudumba, ukuphazamiseka komzimba okanye imeko ezinje ngomhlaza. Kutshanje, ngokusebenzisa oososayensi be-genomics bafumanise ukuba ezi ndlela ze-histiocytosis zibonisa utshintsho kwimfuza (utshintsho) kwiiseli ezimhlophe zegazi zokuqala. Oku kukhokelela ekuziphatheni okungaqhelekanga kwiiseli. Iiseli ezingaqhelekanga emva koko ziyonyuka kwiindawo ezahlukeneyo zomzimba kubandakanya amathambo, ulusu, imiphunga kunye nezinye iindawo.

ILangerhans cell histiocytosis sisifo esinqabileyo esinokuchaphazela abantu bayo yonke iminyaka. Elona zinga liphezulu liphakathi kwabantwana abaneminyaka emi-5 ukuya kweli-10. Ezinye iindlela zokuphazamiseka zizinto zemfuza, oko kuthetha ukuba bafumana ilifa.

Isifo i-Erdheim-Chester yindlela enqabileyo ye-histiocytosis echaphazela ikakhulu abantu abadala ebandakanya amalungu amaninzi omzimba.

Zombini iiseli zeLangerhans ze-histiocytosis kunye nesifo i-Erdheim-Chester sinokuchaphazela umzimba uphela (inkqubo yesifo)

Iimpawu zinokwahluka phakathi kwabantwana nabantu abadala, kodwa banokuba neempawu ezifanayo.Izidumbu ezinamathambo athwala ubunzima, njengemilenze okanye umqolo, zinokubangela ukuba amathambo aqhekeke ngaphandle kwesizathu.

Iimpawu kubantwana zingabandakanya:

• Intlungu zesisu
• Intlungu yethambo
• Ukulibaziseka kokufikisa
• Ukuba nesiyezi
• Umsele wendlebe oqhubeka ixesha elide
• Amehlo abonakala ngathi aya kunamathela ngakumbi nangakumbi
• Ukucaphuka
• Ukungaphumeleli
• Ifiva
• Ukuchama rhoqo
• Intloko ebuhlungu
• I-jaundice
• Ukuqhwalela
• Ukwehla kwengqondo
• Irhashalala
• I-Seborrheic dermatitis yesikhumba
• Ukuxhuzula
• Isiqu esifutshane
• Ukudumba kwamadlala
• Unxaniwe
• Ukuhlanza
• Ukuhla ukusinda

Qaphela: Abantwana abangaphezulu kweminyaka emi-5 ubudala bahlala benothambo kuphela.

Iimpawu kubantu abadala zingabandakanya:

• Intlungu yethambo
• Iintlungu zesifuba
• Khohlela
• Ifiva
• Ukuphazamiseka ngokubanzi, ukungonwabi, okanye ukugula
• Ukwanda kwenani lomchamo
• Irhashalala
• Ukuqhawukelwa ngumphefumlo
• Ukunxanelwa nokwanda kotywala kulwelo
• Ukuhla ukusinda

Akukho luvavanyo lwegazi luthile kwiLangerhans cell histiocytosis okanye isifo se-Erdheim-Chester. Amathumba avelisa "ukubethelelwa" ngaphandle kwithambo le-x-reyi. Iimvavanyo ezithile ziyahluka, kuxhomekeke kubudala bomntu.

Uvavanyo lwabantwana lunokubandakanya:

• I-Biopsy yesikhumba ukujonga iiseli zeLangerhans
• Umongo we-Bone biopsy ukujonga iiseli zeLangerhans
• Gcwalisa ubalo lwegazi (CBC)
• X-reyi yawo onke amathambo emzimbeni ukufumanisa ukuba mangaphi amathambo achaphazelekayo
• Uvavanyo lokutshintsha kwemfuza kwi-BRAF V600E

Uvavanyo lwabantu abadala lunokubandakanya:

• I-Biopsy yalo naliphi na ithumba okanye ubunzima
• Ukufanekisa umzimba, kubandakanya i-x-ray, i-CT scan, iMRI, okanye ukuskena kwePET
• Bronchoscopy kunye biopsy
• Uvavanyo lomsebenzi wePulmonary
• Uvavanyo lwegazi kunye nezicubu zotshintsho kuhlobo olubandakanya i-BRAF V600E. Olu vavanyo lunokufuna ukwenziwa kwiziko elikhethekileyo.

I-Langerhans cell histiocytosis ngamanye amaxesha inxulunyaniswa nomhlaza. I-CT scans kunye ne-biopsy kufuneka zenziwe ukukhusela umhlaza onokwenzeka.

Abantu abane-Langerhans cell histiocytosis ebandakanya indawo enye kuphela (enjengethambo okanye ulusu) banokuphathwa ngotyando lwasekhaya. Nangona kunjalo, kuya kufuneka zilandelwe ngokusondeleyo ukujonga iimpawu zokuba isifo sisasazekile.

Abantu abane-Langerhans cell histiocytosis okanye isifo se-Erdheim-Chester bafuna amayeza ukunciphisa iimpawu kunye nokulawula ukusasazeka kwesifo. Izifundo zamva nje zibonisa ukuba phantse bonke abantu abadala abane-histiocytosis ebanzi banokutshintsha kwemfuza kumathumba, abonakala ngathi abangela ukuphazamiseka. Amachiza athintela oku kuguqulwa kwemfuza, okunje ngeemururafenib ziyafumaneka ngoku. Amanye amachiza afanayo nawo ayaphuhliswa.

I-Langerhans cell histiocytosis kunye nesifo i-Erdheim-Chester zizifo ezinqabileyo kakhulu. Ke ngoko kukho ulwazi olulinganiselweyo malunga nelona khondo lilungileyo lonyango. Abantu abanezi meko banokufuna ukuthatha inxaxheba kuvavanyo lweklinikhi oluqhubekayo olwenzelwe ukufumanisa unyango olutsha.

Amanye amayeza okanye unyango lunokusetyenziswa, kuxhomekeke kwimbonakalo (prognosis) kunye nempendulo kumayeza aqala ngawo. Olu nyango lunokubandakanya:

• I-alpha ye-interferon
• Cyclophosphamide okanye vinblastine
• Etoposide
• Imethotrexate
• Vemurafenib, ukuba utshintsho lwe-BRAF V600E lufunyenwe
• Ukutshintshwa kweseli

Olunye unyango lunokubandakanya:

• Iintsholongwane zokulwa nosulelo
• Inkxaso yokuphefumla (ngomatshini wokuphefumla)
• Unyango olubuyisela iHormone
• Ulungiso lwenyama
• I-shampoos ekhethekileyo yeengxaki ze-scalp
• Inkxaso yenkxaso (ekwabizwa ngokuba kukhathalelo lwentuthuzelo) ukunciphisa iimpawu

Ukongeza, abantu abanezi meko batshayayo bayakhuthazwa ukuba bayeke kuba ukutshaya kunokuba mandundu impendulo kunyango.

Umbutho we-Histiocytosis www.histio.org

I-Langerhans cell histiocytosis kunye nesifo i-Erdheim-Chester sinokuchaphazela amalungu amaninzi kwaye kunokukhokelela ekufeni.

Phantse isiqingatha sabo banesifo se-pulmary histiocytosis, ngelixa abanye benokuphulukana ngokusisigxina nomsebenzi wemiphunga ngokuhamba kwexesha.

Kubantu abancinci kakhulu, imbonakalo ixhomekeke kwi-histiocytosis ethile kunye nokuba kunzima kangakanani. Abanye abantwana banokuphila ubomi obuqhelekileyo ngaphandle kokubandakanyeka kwezifo, ngelixa abanye besenza kakubi. Abantwana abancinci, ngakumbi iintsana, kunokwenzeka ukuba babe neempawu zomzimba wonke ezikhokelela ekufeni.

Iingxaki zinokubandakanya:

• Ukusasaza i-pulstary pulmonary fibrosis (izicubu ezinzulu zemiphunga eziye zitshise kwaye zonakalise)
• Ngokukhawuleza kwawa imiphunga

Abantwana banako ukuphuhlisa:

• I-anemia ibangelwa kukusasazeka kwamathumba kumongo wethambo
• Isifo seswekile
• Iingxaki zemiphunga ezikhokelela ekusileleni kwemiphunga
• Iingxaki nge-pituitary gland ekhokelela ekusileleni kokukhula

Fowunela umboneleli wakho wezempilo ukuba wena okanye umntwana wakho uneempawu zesi sifo. Yiya kwigumbi likaxakeka ukuba ukuphefumla kancinci okanye iintlungu zesifuba ziyakhula.

Kuphephe ukutshaya. Ukuyeka ukutshaya kunokuphucula isiphumo kubantu abane-Langerhans cell histiocytosis echaphazela imiphunga.

Akukho kuthintelwa kwesi sifo.

I-Langerhans cell histiocytosis; Isifo i-Erdheim-Chester

• I-granuloma ye-Eosinophilic - i-x-ray yekhayi
• Inkqubo yokuphefumla

Ukunyaniseka G, uJR omncinci, uKoster MJ, et al. Isiteyitimenti seMayo Clinic Histiocytosis seQela eliSebenzayo sokuchonga kunye nokuvavanya abaguli abadala abane-neoplasms ye-histiocytic: Isifo i-Erdheim-Chester, iLangerhans cell histiocytosis, kunye nesifo sikaRosai-Dorfman. Ikliniki yaseMayo. Ngo-2019; 94 (10): 2054-2071. IINKCUKACHA: 31472931 pubmed.ncbi.nlm.nih.gov/31472931/.

I-Rollins BJ, iBerliner N. Imbali. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 160.