I-Histiocytosis

I-Histiocytosis yigama elijwayelekile leqembu lezifo noma "ama-syndromes" afaka ukwanda okungavamile kwinani lamaseli egazi amhlophe abizwa ngama-histiocyte.

Muva nje, ulwazi olusha ngalo mndeni wezifo kuholele ochwepheshe ukuthi bakhe isigaba esisha. Kuphakanyiswe imikhakha emihlanu:

• L iqembu - kufaka phakathi iLangerhans cell histiocytosis kanye nesifo i-Erdheim-Chester
• Iqembu C - lifaka i-non-Langerhans cell histiocytosis ebandakanya isikhumba
• M iqembu - kufaka phakathi i-histiocytosis ebulalayo
• R group - kufaka phakathi isifo seRosai-Dorfman
• H Iqembu - kufaka phakathi i-hemophagocytic lymphohistiocytosis

Lo mbhalo ugxile kuphela eqenjini le-L, elibandakanya i-Langerhans cell histiocytosis nesifo i-Erdheim-Chester.

Kube nenkulumompikiswano ngokuthi ngabe iLangerhans cell histiocytosis kanye ne-Erdheim-Chester isifo ukuvuvukala, ukuphazamiseka komzimba, noma izimo ezifana nomdlavuza. Muva nje, ngokusebenzisa ososayensi be-genomics bathole ukuthi lezi zinhlobo ze-histiocytosis zikhombisa izinguquko zofuzo kumaseli egazi okuqala amhlophe. Lokhu kuholela ekuziphatheni okungavamile kumaseli. Amaseli angajwayelekile abese enyuka ezingxenyeni ezahlukahlukene zomzimba kufaka phakathi amathambo, isikhumba, amaphaphu, nezinye izindawo.

ILangerhans cell histiocytosis yisifo esingajwayelekile esingathinta abantu bayo yonke iminyaka. Izinga eliphakeme kakhulu liphakathi kwezingane ezineminyaka engu-5 kuya kwengu-10. Ezinye izinhlobo zalesi sifo zofuzo, okusho ukuthi zizuzwe njengefa.

Isifo i-Erdheim-Chester siyindlela engajwayelekile ye-histiocytosis ethinta ikakhulukazi abantu abadala ebandakanya izingxenye eziningi zomzimba.

Kokubili isifo seLangerhans cell histiocytosis kanye nesifo i-Erdheim-Chester singathinta umzimba wonke (systemic disorder).

Izimpawu zingahluka phakathi kwezingane nabantu abadala, kepha zingaba nezimpawu ezifanayo.Izimila emathanjeni athwala isisindo, njengemilenze noma umgogodla, zingadala ukuthi amathambo aqhekeke ngaphandle kwesizathu esisobala.

Izimpawu ezinganeni zingabandakanya:

• Ubuhlungu besisu
• Ubuhlungu bamathambo
• Ukuthomba okulibazisekile
• Isiyezi
• Ukuhanjiswa kwendlebe okuqhubeka isikhathi eside
• Amehlo avele aphume kakhudlwana
• Ukuthukuthela
• Ukwehluleka ukuchuma
• Imfiva
• Ukuchama njalo
• Ubuhlungu bekhanda
• I-jaundice
• Ukuxhuga
• Ukwehla kwengqondo
• I-Rash
• I-Seborrheic dermatitis yesikhumba
• Ukuquleka
• Isiqu esifushane
• Izindlala ze-lymph ezivuvukile
• Omile
• Ukuhlanza
• Ukwehla kwesisindo

Qaphela: Izingane ezingaphezulu kweminyaka emihlanu ubudala zivame ukubandakanyeka emathanjeni kuphela.

Izimpawu kubantu abadala zingafaka:

• Ubuhlungu bamathambo
• Ubuhlungu besifuba
• Khwehlela
• Imfiva
• Ukungakhululeki okuvamile, ukungakhululeki, noma ukugula
• Inani elandayo lomchamo
• I-Rash
• Ukuphelelwa umoya
• Ukuphuza uketshezi ukoma nokwanda
• Ukwehla kwesisindo

Azikho izivivinyo ezithile zegazi zeLangerhans cell histiocytosis noma isifo se-Erdheim-Chester. Izicubu zikhiqiza ukubukeka "okushayiwe" ku-x-ray yethambo. Ukuhlolwa okukhethekile kuyehluka, kuya ngeminyaka yomuntu.

Ukuhlolwa kwezingane kungafaka:

• I-biopsy yesikhumba ukuhlola amaseli aseLangerhans
• I-bone marrow biopsy ukuhlola amaseli aseLangerhans
• Qedela ukubalwa kwegazi (CBC)
• Ama-X-ray awo wonke amathambo emzimbeni ukuthola ukuthi mangaki amathambo athintekile
• Hlola ukuguqulwa kwezakhi zofuzo ku-BRAF V600E

Ukuhlolwa kwabantu abadala kungafaka futhi:

• I-biopsy yanoma yisiphi isimila noma isisindo
• Ukufanekisa umzimba, kufaka phakathi i-x-ray, i-CT scan, i-MRI, noma ukuskena kwe-PET
• I-Bronchoscopy ene-biopsy
• Ukuhlolwa komsebenzi wePulmonary
• Ukuhlolwa kwegazi nezicubu zokuguqulwa kwezakhi zofuzo kufaka phakathi i-BRAF V600E. Lokhu kuhlolwa kungadinga ukwenziwa esikhungweni esikhethekile.

ILangerhans cell histiocytosis kwesinye isikhathi ixhunyaniswa nomdlavuza. Ukuhlolwa kwe-CT kanye ne-biopsy kufanele kwenziwe ukuze kukhishwe umdlavuza ongaba khona.

Abantu abane-Langerhans cell histiocytosis ebandakanya indawo eyodwa kuphela (enjengethambo noma isikhumba) bangelashwa ngokuhlinzwa kwendawo. Kodwa-ke, kuzodingeka zilandelwe eduze ukubheka izimpawu zokuthi lesi sifo sesisakazekile.

Abantu abanesifo seLangerhans cell histiocytosis esandile noma isifo i-Erdheim-Chester badinga imishanguzo yokwehlisa izimpawu nokulawula ukusabalala kwalesi sifo. Ucwaningo lwakamuva lukhombisa ukuthi cishe bonke abantu abadala abane-histiocytosis esabalele banezakhi zofuzo kwizicubu, ezibonakala zidala lesi sifo. Imithi evimbela lokhu kuguquka kwezakhi zofuzo, njenge-vemurafenib iyatholakala njengamanje. Ezinye izidakamizwa ezifanayo nazo ziyathuthukiswa.

ILangerhans cell histiocytosis nesifo i-Erdheim-Chester yizinkinga ezingavamile kakhulu. Ngakho-ke kunemininingwane elinganiselwe ngenkambo engcono yokwelashwa. Abantu abanezi zimo bangafuna ukubamba iqhaza ezivivinyweni eziqhubekayo zomtholampilo ezenzelwe ukukhomba ukwelashwa okusha.

Eminye imishanguzo noma ukwelashwa kungasetshenziswa, kuya ngombono (ukubikezela) nokuphendula emithini yokuqala. Ukwelashwa okunjalo kungafaka:

• I-Interferon alpha
• I-cyclophosphamide noma i-vinblastine
• I-Etoposide
• IMethotrexate
• Vemurafenib, uma ukuguqulwa kwe-BRAF V600E kutholakala
• Ukufakelwa kabusha kwe-stem cell

Ezinye izindlela zokwelashwa zingafaka:

• Ama-antibiotics ukulwa nezifo
• Ukusekela ukuphefumula (ngomshini wokuphefumula)
• Ukwelashwa kokufaka esikhundleni seHormone
• Ukwelashwa ngokomzimba
• Ama-shampoo akhethekile wezinkinga ze-scalp
• Ukunakekelwa okusekelayo (okubizwa nangokuthi ukunakekelwa kwenduduzo) ukuqeda izimpawu

Ngaphezu kwalokho, abantu abanalezizimo ababhemayo bayakhuthazwa ukuthi bayeke ngoba ukubhema kungadabukisa impendulo ekwelashweni.

Inhlangano ye-Histiocytosis www.histio.org

ILangerhans cell histiocytosis kanye nesifo i-Erdheim-Chester singathinta izitho eziningi futhi kungaholela ekufeni.

Cishe uhhafu walabo abane-histiocytosis yamaphaphu bayathuthuka, kanti abanye banokulahleka okungapheli komsebenzi wamaphaphu ngokuhamba kwesikhathi.

Kubantu abasha kakhulu, umbono ubheka i-histiocytosis ethile nokuthi inzima kangakanani. Ezinye izingane zingaphila impilo ejwayelekile ngokubandakanyeka okuncane kwezifo, kanti ezinye zenza kabi. Izingane ezincane, ikakhulukazi izinsana, zinamathuba amaningi okuba nezimpawu zomzimba wonke eziholela ekufeni.

Izinkinga zingafaka:

• Ukusabalalisa i-interstitial pulmonary fibrosis (izicubu ezijulile zamaphaphu ezivuthayo bese zilimala)
• Ngokuzenzakalelayo kwawa amaphaphu

Izingane nazo zingathuthukisa:

• I-anemia ebangelwa ukusabalala kwezimila emnkantsheni wethambo
• Isifo sikashukela insipidus
• Izinkinga zamaphaphu eziholela ekuhlulekeni kwamaphaphu
• Izinkinga ngendlala ye-pituitary eholela ekuhlulekeni kokukhula

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma wena noma ingane yakho unezimpawu zalesi sifo. Iya egumbini lezimo eziphuthumayo uma kuba nokuphefumula noma ubuhlungu besifuba.

Gwema ukubhema. Ukuyeka ukubhema kungathuthukisa imiphumela kubantu abane-Langerhans cell histiocytosis ethinta amaphaphu.

Akukho ukuvimbela okwaziwayo kwalesi sifo.

ILangerhans cell histiocytosis; Isifo i-Erdheim-Chester

• I-eosinophilic granuloma - i-x-ray yogebhezi
• Uhlelo lokuphefumula

UGoyal G, uJR osemncane, uKoster MJ, et al. Isitatimende sokuvumelana seMayo Clinic Histiocytosis Working Group sokuxilongwa nokuhlolwa kweziguli ezindala ezinama-histiocytic neoplasms: Isifo i-Erdheim-Chester, iLangerhans cell histiocytosis, nesifo sikaRosai-Dorfman. IMayo Clin Proc. 2019; 94 (10): 2054-2071. I-PMID: 31472931 pubmed.ncbi.nlm.nih.gov/31472931/.

URollins BJ, uBerliner N. Ama-Histiocytoses. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 160.