Kodi mukudziwa za matenda a Erdheim-Chester? Tiyeni tikambirane za matenda osowa awa!

Kodi mudamvapo za matenda a Erdheim-Chester (ECD)? Mwina mudadabwa pang'ono mutamva dzinali. Chifukwa chakuti uwu ndi matenda osowa kwambiri omwe amapezeka kawirikawiri padziko lonse lapansi. Komabe, popeza amatha kukhudza ziwalo zosiyanasiyana m'thupi lathu, ndikofunikira kwambiri kudziwa izi. Lero, tiyeni tikambirane za matenda a Erdheim-Chester (ECD).

Kodi matenda a Erdheim-Chester (ECD) ndi chiyani?

Mwachidule, matenda a Erdheim-Chester (ECD) ndi matenda osowa kwambiri m'magazi. Ndi a m'gulu la matenda otchedwa histiocytosis . Tsopano mwina mukudabwa kuti ma histiocytes awa ndi otani. Ma histiocytes ndi mtundu wapadera wa selo mu chitetezo chathu chamthupi . Ali ngati asilikali ang'onoang'ono omwe amateteza matupi athu. Maselo amenewa nthawi zambiri amapezeka m'malo monga mafupa athu, magazi, khungu, mapapo, ndulu, ndi chiwindi.

Komabe, mwa munthu amene ali ndi matenda a Erdheim-Chester (ECD), ma histiocyte amenewa amakula mosalamulirika . Zili ngati kukhala ndi asilikali ambiri. Ma histiocyte owonjezerawa amayamba kukula m'malo osiyanasiyana a thupi komwe sayenera kukula. Kenako amatha kupanga zotupa ndikuwononga minofu yathanzi . Izi ndi zomwe zimachitika mu ECD.

Kodi matendawa ndi ofala bwanji?

Monga tanenera kale, uwu ndi matenda osowa kwambiri . Tangoganizani, kuyambira pomwe matendawa adapezeka koyamba mu 1930, milandu pafupifupi 800 yalembedwa padziko lonse lapansi. Komabe, madokotala ena amakhulupirira kuti chiwerengerochi chikhoza kukhala chokwera kwambiri chifukwa anthu ena omwe ali ndi matendawa sapezeka mokwanira. Izi zitha kukhala chifukwa chakuti mayiko padziko lonse lapansi pakadali pano alibe njira yofanana yosungira zolemba za odwala awa.

Matenda a Erdheim-Chester (ECD) amapezeka kwambiri mwa akuluakulu azaka zapakati . Ku United States, zaka zapakati zomwe munthu amadwala matendawa ndi zaka 46. Komabe, zanenedwa kuti ana aang'ono kwambiri ndi awa. Chinanso n'chakuti amuna ndi omwe ali ndi mwayi waukulu wopeza matendawa. Akuti pakati pa 70% ndi 75% ya odwala ndi amuna.

Kodi zizindikiro zake ndi ziti?

Mmene matenda a Erdheim-Chester (ECD) amakhudzira munthu aliyense ndi osiyana kwambiri . Izi zikutanthauza kuti si aliyense amene ali ndi zizindikiro zofanana. Zizindikiro zimadalira ziwalo za thupi zomwe zawonongeka ndi ma histiocytes ochulukirapo, monga tafotokozera kale, komanso ziwalo zomwe zakhudzidwa. Nthawi zina, matendawa amatha kupezeka popanda kusonyeza zizindikiro zilizonse (zosawoneka). Pankhaniyi, dokotala amapeza chidziwitso cha izi kuchokera ku scan (`imaging`) kapena mayeso a magazi (`lab tests`) omwe amachitika pazifukwa zina.

Tiyeni tiwone ziwalo zomwe zimakhudzidwa kwambiri ndi zizindikiro zake.

Momwe zimakhudzira mafupa

ECD ingayambitse kukhuthala kwa mafupa kosazolowereka (osteosclerosis). Izi nthawi zina zingayambitse kupweteka kwa mafupa . Chizindikiro chodziwika bwino cha matendawa ndi kukhuthala kwa mafupa ndi kupweteka, makamaka m'miyendo yonse iwiri . Kukhuthala kwa mafupa kumeneku kumawoneka bwino pa scans.

Zotsatira pa impso

Impso zathu ndi minofu yozungulira ('retroperitoneum') zithanso kuwonongeka ndi ma histiocyte awa. Izi zingayambitse matenda monga:

• Kutupa kwa impso.
• Kufooka kwa impso.
• Kulephera kwa impso.

Zotsatira pa dongosolo la endocrine (dongosolo la mahomoni)

Ngati ma histiocyte amenewa awononga tiziwalo timene timatulutsa mahomoni omwe amalamulira machitidwe osiyanasiyana m'thupi lathu, mavuto osiyanasiyana a mahomoni angabuke. Zizindikiro zimasiyana malinga ndi chiwalo chomwe chawonongeka.

• Ngati pituitary gland yawonongeka: Kuchepa kwa kupanga kwa mahomoni amodzi kapena angapo a pituitary (`hypopituitarism`).
• Ngati chithokomiro chawonongeka: Kuchepa kwa mahomoni a chithokomiro (hypothyroidism).
• Ngati ma gonads awonongeka: Kuchepa kwa mahomoni ogonana (monga testosterone ndi estrogen) (`hypogonadism`).
• Ngati adrenal gland yawonongeka: Kuchepa kwa mahomoni a adrenal (kulephera kwa adrenal).

Kuwonongeka kwa pituitary gland kungayambitse matenda otchedwa diabetes insipidus , omwe amayambitsa zizindikiro monga kukodza pafupipafupi komanso ludzu lochuluka. Zapezeka kuti pafupifupi theka la odwala ECD alinso ndi vutoli.

Zotsatira pa dongosolo lamanjenje

Ubongo wathu ndi dongosolo lathu la mitsempha zimathanso kuwonongeka ndi ma histiocyte awa. Kenako mutha kuwona zizindikiro monga:

• Kutaya kulinganiza bwino poyenda, mavuto ndi mgwirizano (ataxia).
• Kusalankhula bwino (dysarthria) chifukwa cha kulephera kulamulira minofu panthawi yolankhula.
• Kuvuta kuganiza, kuganizira, kapena kukumbukira.
• Mutu.

Zotsatira pa maso

ECD ingakhudze diso limodzi kapena onse awiri.

• Matumbo achikasu, ofewa pa zikope (xanthelasma).
• Kutuluka kwa zikope (proptosis).
• Kupweteka kwa maso.
• Kuchepa kapena kutayika kwa masomphenya.

Zotsatira pa dongosolo la kupuma

Ngakhale kuti ma scan akusonyeza kuti mapapu amakhudzidwa ndi ma histiocyte amenewa, nthawi zambiri zizindikiro sizimachitika . Komabe, ngati zizindikirozo zichitika, zingaphatikizepo:

• Chifuwa.
• Kuvuta kupuma (kupuma movutikira).

Ngati sichinalandire chithandizo choyenera , chingayambitse zipsera zosatha m'mapapo (pulmonary fibrosis), matenda oopsa.

Zotsatira pa dongosolo la mtima

Dokotala wanu angaonenso pa scan kuti ma histiocyte akhudza mtima wanu ndi mitsempha yamagazi. Izi zitha kukhala zoopsa ngati sizikuthandizidwa. ECD ingayambitse zotsatirazi:

• Kutupa kwa thumba lozungulira mtima (pericardial effusion).
• Kuthamanga kwa magazi (kuthamanga kwa magazi m'thupi) kumachitika chifukwa cha kuchepa kwa magazi kupita ku impso.
• Kulephera kwa mtima.

Zotsatira pakhungu

Chizindikiro chachikulu chomwe chimawoneka pakhungu ndi ziphuphu zachikasu (`xanthelasma`) zomwe zimakula pa zikope. Kuphatikiza apo, ziphuphu zachikasu-bulauni zimatha kuwonekanso m'malo awa:

• Pankhope.
• Pakhosi.
• Chifuwa ndi mimba (`Mzimba`).
• M'dera la groin.

Kuphatikiza apo, ma histiocyte ochulukirapowa amatha kudziunjikira ndikuwononga minofu m'malo monga ndulu, chiwindi, ndi mafuta am'mafupa.

Kodi zimayambitsa matendawa ndi ziti?

Tsopano tikudziwa kuti ECD ndi matenda omwe ma histiocyte amachulukana mosalamulirika, amafalikira, ndikuwononga minofu ndi ziwalo zathanzi. Komabe, asayansi sakudziwabe chifukwa chake maselowa amakula mosalamulirika. Komabe, kafukufuku waposachedwapa wapeza kuti kusintha kwa majini ena kumachita nawo gawo.

Anthu opitilira theka la omwe ali ndi matenda a Erdheim-Chester (ECD) apezeka kuti ali ndi kusintha kwa majini a BRAF, komwe kumathandizira kukula kosalamulirika kwa ma histiocyte.

Ngakhale kuti jini ya BRAF ndiyo yofala kwambiri, asayansi apeza kusintha kwina kwa majini komwe kumakhudzana ndi ECD. Kupeza kumeneku kwathandiza asayansi kupanga njira zochizira majini omwe asinthidwa ndikuletsa kukula kwachilendo kwa ma histiocyte.

Kodi matendawa amapezeka bwanji?

Popeza ECD ndi matenda osowa kwambiri ndipo zizindikiro zimasiyana malinga ndi munthu, madokotala sangaganize nthawi yomweyo za vutoli. Chifukwa chake, zingatenge nthawi kuti mudziwe matendawa . Mungafunike kuonana ndi madokotala angapo.

Dokotala wanu adzaganizira zizindikiro zanu pamodzi ndi zotsatira za mayeso ena angapo kuti adziwe ngati muli ndi ECD. Nazi zina mwa zinthu zomwe zingakuthandizeni kupeza matenda:

• Njira zojambulira: Kujambula zithunzi zosiyanasiyana (X-ray, bone scans, PET scans, CT scans, MRIs) kungagwiritsidwe ntchito kuona komwe ma histiocyte awa alowa m'thupi. Kungasonyezenso kuwonongeka kwa minofu yofewa monga ubongo, chifuwa, ndi ziwalo zam'mimba.
• Mayeso a Laboratory:Mayesowa amatha kuzindikira mavuto ena okhudzana ndi kugwira ntchito kwa ziwalo (zomwe zingakhudze ECD), komanso kuwona zinthu monga kutupa, kusokonekera kwa kuchuluka kwa maselo m'magazi, komanso kusintha kwa kuchuluka kwa mahomoni.
• Kufufuza za maselo: Kufufuza za maselo kumaphatikizapo kutenga chitsanzo chaching'ono cha minofu yomwe yakhudzidwa ndikuyang'ana pansi pa maikulosikopu. Kenako maselowo amayesedwa ngati ali ndi zizindikiro za ECD komanso kusintha kwa majini monga BRAF. Kudziwa makhalidwe a maselo amenewa kungathandize dokotala wanu kusankha chithandizo chomwe chili chabwino kwa inu.

Kodi mankhwala ake ndi otani?

Ngati mulibe zizindikiro ndipo ECD yanu sikukukhudzani thanzi lanu, dokotala wanu angasankhe kuyang'anira vuto lanu. Komabe, anthu ambiri omwe ali ndi ECD adzafunika chithandizo . Ngakhale kuti palibe mankhwala, pali njira zingapo zothandizira vutoli.

Nazi njira zazikulu zochizira:

• Chithandizo Cholunjika: Izi zimaphatikizapo kugwiritsa ntchito mankhwala omwe amayang'ana kusintha kwa majini komwe kumapangitsa kuti ma histiocyte azichulukana mosalamulirika. FDA yavomereza mankhwala otchedwa Vemurafenib kwa odwala ECD omwe ali ndi kusintha kwa majini a BRAF, ndi mankhwala otchedwa Cobimetinib kwa omwe ali ndi kusintha kwa majini a MEK. Kutengera mtundu wa kusintha kwa maselo anu, dokotala wanu angakulangizeni mankhwala awa kapena ena omwe akuyang'aniridwa kapena kuphatikiza mankhwala.
• Chithandizo cha Immunotherapy: Mankhwalawa amathandiza chitetezo chathu cha mthupi kuzindikira bwino ndikulimbana ndi maselo a khansa (pankhaniyi, ma histiocytes osazolowereka). Interferon-alpha ndi mankhwala ochiritsira matenda a ECD omwe amagwiritsidwa ntchito kwambiri.
• Chemotherapy: Chemotherapy imagwiritsa ntchito mankhwala ophera maselo a khansa (maselo osazolowereka) m'thupi lonse ndikuletsa kukula kwa zotupa. Mankhwala odziwika bwino a chemotherapy omwe amagwiritsidwa ntchito pa ECD ndi cladribine . Komabe, dokotala wanu angakulangizeni mankhwala ena a chemotherapy kapena kuphatikiza mankhwala.

Kuwonjezera pa mankhwala akuluakulu awa, dokotala wanu angakulangizeni njira zina zothandizira kuchepetsa zizindikiro zanu. Ngakhale kuti mankhwala awa saletsa ma histiocyte kulowa m'thupi lanu, angathandize kuchepetsa kusasangalala komwe mukumva.

• Opaleshoni: Opaleshoni ingafunike kuti ikonze zina mwa zinthu zomwe zawonongeka chifukwa cha ECD. Mwachitsanzo, kutupa ndi minofu yowonongeka zimatha kutseka machubu omwe amanyamula mkodzo (ureters). Ngati izi zitachitika, opaleshoni ingafunike kuti ikonze.
• Chithandizo cha radiation:Chithandizo cha radiation chingalimbikitsidwe kuti chiwononge maselo osazolowereka m'dera linalake la thupi ndikuchepetsa zizindikiro zosasangalatsa zomwe zimayambitsa.
• Corticosteroids: Mankhwalawa amatha kuchepetsa kutupa komwe kumachitika chifukwa cha kulowa kwa ma histiocytes m'thupi.

Mungakhalenso ndi mwayi wochita nawo mayeso azachipatala . Mayeso azachipatala ndi kafukufuku amene amayesa chitetezo ndi mphamvu ya mankhwala atsopano komanso kuphatikiza mankhwala atsopano. Funsani dokotala wanu ngati mungathe kutenga nawo mbali mu mayeso azachipatala a matenda a Erdheim-Chester (ECD).

Kodi izi zingapewedwe?

Mwatsoka, palibe njira yopewera matenda a Erdheim-Chester (ECD). Komabe, matendawa amatha kuchiritsidwa kwambiri ndi mankhwala.

Kodi mungakhale ndi matendawa kwa nthawi yayitali bwanji?

Kuyembekeza kwanu kumadalira mbali za thupi lanu zomwe zakhudzidwa ndi ma histiocyte ndi momwe mumayankhira chithandizo. Komabe, chifukwa cha chitukuko cha mankhwala atsopano, monga chithandizo cholunjika, zotsatira za kupulumuka zokhudzana ndi ECD tsopano zasintha kwambiri.

Tangoganizirani, mu 1996, chiwerengero cha anthu opulumuka zaka zisanu kwa odwala ECD chinali 43% yokha. Komabe, malinga ndi kafukufuku waposachedwapa, chiwerengero chimenecho chawonjezeka kufika pa 83%!

Lankhulani ndi dokotala wanu za momwe matenda anu adzakhalire mtsogolo kutengera momwe mulili komanso momwe chithandizo chidzachitikire.

Kodi muyenera kupita kwa dokotala liti?

ECD imafuna chithandizo ndi kuyang'aniridwa nthawi zonse . Dokotala wanu adzakulangizani kangati komwe muyenera kupita kwa dokotala wanu, komanso ngati muyenera kuyesedwa magazi ndi kuyesedwa magazi.

Mankhwala ambiri a ECD angayambitse zotsatira zoyipa. Kuwonjezera pa gulu lanu la chithandizo cha ECD, kupeza thandizo kuchokera ku gulu lothandizira odwala kungakuthandizeni kuthana ndi zotsatira zoyipa za mankhwalawa ndikuthana ndi nkhawa zomwe zimabwera chifukwa cha matenda.

Pomaliza, chinthu chofunikira kwambiri (Uthenga Wopita Nawo Kunyumba)

Matenda a Erdheim-Chester (ECD) ndi matenda omwe amakhudza munthu aliyense mosiyana, omwe amayamba chifukwa cha kuchuluka kwa ma histiocytes, omwe amawononga minofu. Zizindikiro za matendawa zimasiyana malinga ndi munthu. Izi zingakhudze zomwe mukukumana nazo, kuphatikizapo mankhwala omwe alipo.

Koma mwamwayi, ndi kupita patsogolo kwaposachedwa, asayansi apeza njira zothandizira zomwe zimathandizira kuti ECD iyambe kugwira ntchito.Lankhulani momasuka ndi dokotala wanu za njira zochiritsira zomwe zili zoyenera kwa inu komanso zotsatira zomwe mungayembekezere, kutengera mawonekedwe enieni a vuto lanu. Kumbukirani, ngakhale muzochitika zachilendo izi, mutha kupita patsogolo mwamphamvu ndi upangiri ndi chithandizo choyenera chamankhwala.

Matenda a Erdheim -Chester, ECD, histiocytosis, matenda osowa, kusintha kwa majini, BRAF, zizindikiro, chithandizo