Mbiriyakale

Histiocytosis ndi dzina la gulu la zovuta kapena "syndromes" zomwe zimakhudza kuchuluka kwachilendo kwamaselo oyera amwazi omwe amatchedwa histiocytes.

Posachedwapa, chidziwitso chatsopano chokhudza matendawa chapangitsa akatswiri kuti apange gulu latsopano. Magulu asanu aperekedwa:

• L gulu - limaphatikizapo Langerhans cell histiocytosis ndi matenda a Erdheim-Chester
• Gulu la C - limaphatikizapo anti-Langerhans cell histiocytosis yomwe imakhudza khungu
• Gulu la M - limaphatikizanso ndi histiocytosis yoyipa
• R gulu - limaphatikizapo matenda a Rosai-Dorfman
• H Gulu - limaphatikizapo hemophagocytic lymphohistiocytosis

Nkhaniyi imangoyang'ana pagulu L, lomwe limaphatikizapo Langerhans cell histiocytosis ndi matenda a Erdheim-Chester.

Pakhala pali mtsutso wokhudza ngati matenda a Langerhans cell histiocytosis ndi Erdheim-Chester ndi otupa, matenda amthupi, kapena mikhalidwe yofanana ndi khansa. Posachedwa, pogwiritsa ntchito ma genomics asayansi apeza kuti mitundu iyi ya histiocytosis ikuwonetsa kusintha kwa majini (masinthidwe) m'maselo oyera amwazi woyambirira. Izi zimabweretsa machitidwe osadziwika m'maselo. Maselo achilendo amawonjezeka m'magulu osiyanasiyana amthupi kuphatikiza mafupa, khungu, mapapo, ndi madera ena.

Langerhans cell histiocytosis ndi matenda osowa omwe amatha kukhudza anthu azaka zonse. Mulingo wokwera kwambiri uli pakati pa ana azaka zapakati pa 5 ndi 10. Mitundu ina yamatendawa ndi majini, zomwe zikutanthauza kuti adalandira.

Matenda a Erdheim-Chester ndi mtundu wosowa wa histiocytosis womwe umakhudza makamaka achikulire omwe amakhudza ziwalo zingapo za thupi.

Matenda onse a Langerhans a histiocytosis ndi Erdheim-Chester amatha kukhudza thupi lonse (systemic disorder).

Zizindikiro zimatha kusiyanasiyana pakati pa ana ndi akulu, koma atha kukhala ndi zizindikilo zomwezo.Zotupa m'mafupa onyamula zolemera, monga miyendo kapena msana, zimatha kupangitsa mafupa kusweka popanda chifukwa chomveka.

Zizindikiro mwa ana zimatha kuphatikiza:

• Kupweteka m'mimba
• Kupweteka kwa mafupa
• Kuchedwa kutha msinkhu
• Chizungulire
• Kutulutsa kwamakutu komwe kumapitilira nthawi yayitali
• Maso omwe amawoneka kuti amatuluka kwambiri
• Kukwiya
• Kulephera kukula bwino
• Malungo
• Kukodza pafupipafupi
• Mutu
• Jaundice
• Kutsimphina
• Maganizo atsika
• Kutupa
• Seborrheic dermatitis ya pamutu
• Kugwidwa
• Msinkhu waufupi
• Kutupa kwamatenda am'mimba
• Ludzu
• Kusanza
• Kuchepetsa thupi

Chidziwitso: Ana opitilira zaka 5 nthawi zambiri amakhala ndi mafupa okhaokha.

Zizindikiro mwa akuluakulu zimatha kuphatikiza:

• Kupweteka kwa mafupa
• Kupweteka pachifuwa
• Tsokomola
• Malungo
• Kusapeza bwino, kusakhazikika, kapena kudwala
• Kuchuluka kwamkodzo
• Kutupa
• Kupuma pang'ono
• Ludzu ndi kuchuluka kwa zakumwa
• Kuchepetsa thupi

Palibe mayeso apadera a magazi a Langerhans cell histiocytosis kapena matenda a Erdheim-Chester. Zotupazo zimatulutsa mawonekedwe "omenyedwa" pa x-ray ya mafupa. Mayeso apadera amasiyanasiyana, kutengera msinkhu wa munthu.

Mayeso a ana atha kukhala:

• Chikopa cha khungu kuti muwone ngati maselo a Langerhans
• Kutsegula m'mafupa kuti muwone ngati maselo a Langerhans
• Kuwerengera kwathunthu kwa magazi (CBC)
• X-ray ya mafupa onse m'thupi kuti mudziwe kuti ndi mafupa angati omwe akukhudzidwa
• Yesani kusintha kwa majini mu BRAF V600E

Mayeso a akulu atha kuphatikizaponso:

• Chiwindi cha chotupa chilichonse kapena misa
• Kujambula kwa thupi, kuphatikiza x-ray, CT scan, MRI, kapena PET scan
• Bronchoscopy yokhala ndi biopsy
• Mayeso a ntchito yamapapo
• Kuyesa magazi ndi minofu pakusintha kwa majini kuphatikiza BRAF V600E. Kuyesaku kungafunikire kuchitidwa kumalo apadera.

Langerhans cell histiocytosis nthawi zina imalumikizidwa ndi khansa. Kufufuza kwa CT ndi biopsy ziyenera kuchitidwa kuti muchepetse khansa.

Anthu omwe ali ndi Langerhans cell histiocytosis yomwe imangokhala ndi gawo limodzi (monga fupa kapena khungu) atha kuchiritsidwa ndi opaleshoni yakomweko. Komabe, amafunika kuwatsata kuti aone ngati matendawo afalikira.

Anthu omwe ali ndi matenda a Langerhans cell histiocytosis kapena matenda a Erdheim-Chester amafuna mankhwala kuti achepetse zizindikilo ndikuletsa kufalikira kwa matendawa. Kafukufuku waposachedwa akuwonetsa kuti pafupifupi achikulire onse omwe ali ndi histiocytosis ambiri amasintha majini m'matumbo, omwe amawoneka kuti amayambitsa matendawa. Mankhwala omwe amaletsa kusintha kwa majini, monga vemurafenib alipo. Mankhwala ena ofanana nawo akupanga.

Langerhans cell histiocytosis ndi Erdheim-Chester matenda ndizovuta kwambiri. Chifukwa chake pali chidziwitso chochepa chokhudza njira yabwino kwambiri yothandizira. Anthu omwe ali ndi mikhalidwe imeneyi angafune kutenga nawo mbali pazoyeserera zamankhwala zomwe zimapangidwa kuti zidziwike za mankhwala atsopano.

Mankhwala ena kapena mankhwala atha kugwiritsidwa ntchito, kutengera mawonekedwe (malingaliro) ndi kuyankha kwa mankhwala oyambira. Mankhwalawa atha kuphatikiza:

• Zolemba za alpha
• Cyclophosphamide kapena vinblastine
• Etoposide
• Methotrexate
• Vemurafenib, ngati kusintha kwa BRAF V600E kungapezeke
• Kuphatikizira kwa cell

Mankhwala ena atha kukhala:

• Maantibayotiki olimbana ndi matenda
• Kupumira kothandizirana (ndimakina opumira)
• Thandizo m'malo mwa mahomoni
• Thandizo lakuthupi
• Ma shamposi apadera a mavuto amutu
• Chithandizo chothandizira (chomwe chimatchedwanso chisamaliro cha chitonthozo) kuti muchepetse zizindikiro

Kuphatikiza apo, anthu omwe ali ndi mikhalidwe imeneyi omwe amasuta amalimbikitsidwa kuti asiye chifukwa kusuta kumatha kukulitsa kuyankha kwamankhwala.

Mgwirizano wa Histiocytosis www.histio.org

Langerhans cell histiocytosis ndi matenda a Erdheim-Chester zimatha kukhudza ziwalo zambiri ndipo zimatha kupha.

Pafupifupi theka la iwo omwe ali ndi m'mapapo a histiocytosis amakula bwino, pomwe ena amakhala ndi kutayika kwamapapo kwa nthawi yayitali.

Kwa achichepere kwambiri, malingaliro amatengera histiocytosis yeniyeni komanso momwe imakhalira yolimba. Ana ena amatha kukhala moyo wabwinobwino osadwala matenda ena, pomwe ena samachita bwino. Ana aang'ono, makamaka makanda, amatha kukhala ndi zizindikilo zathupi lonse zomwe zimabweretsa imfa.

Zovuta zingaphatikizepo:

• Kutulutsa kwamkati mwa pulmonary fibrosis (matumbo am'mapapo omwe amatupa kenako nkuwonongeka)
• Mowopsa anakomoka m'mapapo

Ana amathanso kukula:

• Kuchepa kwa magazi komwe kumayambitsidwa ndikufalikira kwa zotupa m'mafupa
• Matenda a shuga
• Mavuto am'mapapo omwe amatsogolera m'mapapo kulephera
• Mavuto ndi chifuwa cha pituitary chomwe chimayambitsa kukula

Itanani omwe akukuthandizani ngati inu kapena mwana wanu muli ndi zizindikiro za matendawa. Pitani kuchipinda chadzidzidzi ngati kupuma pang'ono kapena kupweteka pachifuwa kumayamba.

Pewani kusuta. Kusiya kusuta kumatha kusintha zotsatira za anthu omwe ali ndi Langerhans cell histiocytosis yomwe imakhudza mapapu.

Palibe njira yodziwira matendawa.

Langerhans cell histiocytosis; Matenda a Erdheim-Chester

• Granuloma ya eosinophilic - x-ray ya chigaza
• Dongosolo kupuma

Goyal G, Wachichepere JR, Koster MJ, et al. Mgwirizano wa Mayo Clinic Histiocytosis Working Group wodziwitsa ndikuwunika odwala achikulire omwe ali ndi zotupa za histiocytic: Matenda a Erdheim-Chester, Langerhans cell histiocytosis, ndi matenda a Rosai-Dorfman. Chipatala cha Mayo. 2019; 94 (10): 2054-2071 (Adasankhidwa) PMID: 31472931 pubed.ncbi.nlm.nih.gov/31472931/.

Rollins BJ, Berliner N. Mbiriyakale. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 160.