Zomwe Muyenera Kudziwa Zokhudza Matenda a Castleman

Kodi munayamba mwakhala ndi chotupa, chomwe timachitcha "chotupa," pakhosi panu, m'khwapa, kapena m'mimba mwanu? Nthawi zina, zotupa ngati izi zimatupa mukakhala ndi chimfine kapena matenda enaake. Zimenezo ndi zachilendo. Koma ngati muli ndi zotupa ngati izi popanda chifukwa, kwa nthawi yayitali, ndipo mukumva kutopa komanso kutaya thupi, muyenera kuda nkhawa pang'ono. Lero, tikambirana za vuto losowa lomwe lingaganiziridwe kuti ndi vuto nthawi zina. Ndi matenda a Castleman.

Mwachidule, kodi matenda a Castleman ndi chiyani?

Tangoganizani kuti thupi lathu lili ngati dziko. Ndiye chitetezo chathu cha mthupi ndi gulu lankhondo lomwe limateteza dzikolo. Pamene kachilombo (mdani) kalowa m'thupi kuchokera kunja, gulu lankhondoli limapita kunkhondo ndikugonjetsa mdaniyo. Nkhondoyo ikatha, gulu lankhondo limabwerera ku malo osungiramo zida ndipo limakhala chete. Izi ndi zomwe zimachitika m'thupi la munthu wathanzi.

Koma ndi munthu amene ali ndi matenda a Castleman, n’zosiyana. Chomwe chimachitika apa n’chakuti asilikali omwe anapita kunkhondo, chitetezo cha m’thupi, samakhala chete ngakhale nkhondoyo itatha. Amapitirizabe kugwira ntchito. Zili ngati kuti nthawi zonse amakhala okonzeka nkhondo. Izi zimayambitsa kutupa kwa nthawi yayitali m’thupi. Izi zitha kuwononga ziwalo zathu.

Pakadali pano, tinthu tating'onoting'ono ta lymph, tinthu tating'onoting'ono timeneti, tili ngati malo ankhondo. Timagwira ntchito zambiri, monga kusefa majeremusi ndikusunga maselo oteteza chitetezo chamthupi. Tsopano, chifukwa chitetezo chamthupi chimagwira ntchito nthawi zonse, tinthu ta lymph timeneti timagwiranso ntchito nthawi zonse. Zotsatira zake, maselo omwe ali mkati mwake amayamba kukula kwambiri. Ndicho chifukwa chake tinthu ta lymph timatupa mu matenda a Castleman. Sikuti ndi kutupa kokha, komanso kumayambitsa kusintha kwa minofu ya tinthu ta lymph timeneti.

Kodi mitundu ikuluikulu ya matenda a Castleman ndi iti?

Matenda a Casselman amagawidwa m'magulu awiri akuluakulu. Zizindikiro ndi mankhwala a matenda aliwonse ndi osiyana kwambiri. Chifukwa chake, ndikofunikira kumvetsetsa mitundu iwiriyi molondola.

Kodi pali mitundu yosiyanasiyana ya matenda a Castleman (MCD)?

Inde, MCD imagawidwanso m'magulu angapo. Kugawa kumeneku kumachitika kutengera chomwe chimayambitsa ndi zina zomwe zimachitika chifukwa cha matendawa.

• MCD yogwirizana ndi NDANDANDA YA MASAMBA: NDANDANDA YA MASAMBA ndi matenda osowa kwambiri a magazi. Nthawi zina MCD imatha kuchitika limodzi ndi NDANDANDA YA MASAMBA.
• MCD yogwirizana ndi HHV-8: Izi zimachitika chifukwa cha kachilombo kotchedwa Human Herpes Virus-8 (HHV-8). Mtundu uwu umapezeka kwambiri mwa anthu omwe ali ndi kachilombo ka HIV kapena omwe ali ndi chitetezo chamthupi chofooka pazifukwa zina.
• Idiopathic MCD (iMCD): Mawu azachipatala akuti " idiopathic " amatanthauza "palibe chifukwa chomwe chapezeka." Odwala ambiri a MCD ali ndi mtundu uwu. Izi zikutanthauza kuti chifukwa chenicheni sichinadziwikebe.

Mtundu uwu wa iMCD umagawidwanso m'magulu atatu ang'onoang'ono:

1. iMCD yogwirizana ndi TAFRO: TAFRO ndi dzina lochokera ku kuphatikiza zizindikiro zingapo. Izi zikutanthauza kuti, Thrombocytopenia (ma platelet otsika m'magazi), Anasarca (kutupa kwa thupi ndi madzi), Malungo (malungo), Kulephera kugwira ntchito bwino kwa impso (kufooka kwa ntchito ya impso) ndi Organomegaly (kukula kwa chiwindi kapena ndulu) ndi zizindikiro zomwe zingawonekere pamenepa.

2. iMCD yokhala ndi idiopathic plasmacytic lymphadenopathy (iMCD-IPL): Mu mtundu uwu, kuchuluka kwa ma platelet kumatha kukwera. Pakhozanso kukhala kupanga kwakukulu kwa ma antibodies ndi maselo oyera amagazi.

3. iMCD, yomwe sinatchulidwe mwanjira ina (iMCD-NOS): Odwala omwe ali ndi iMCD omwe si achibale a TAFRO ndipo omwe palibe chifukwa china chomwe chingapezeke ali m'gululi.

Kodi zizindikiro za matendawa zingakhale zotani?

Zizindikiro zimasiyana malinga ndi mtundu wa matenda a Castleman omwe muli nawo.

Matenda a Unicentric Casselman (UCD) nthawi zambiri samakhala ndi zizindikiro . Chizindikiro chokhacho ndi kutupa kwa lymph node (adenoid). Nthawi zina, zizindikiro (monga kupweteka, kupuma movutikira) zimachitika pokhapokha ngati node yotupayo ikakankhira chiwalo china.

Komabe, mu matenda a Castleman (MCD) omwe ali ndi zizindikiro zambiri, zizindikiro zake zimakhala zoonekeratu. Kuwonjezera pa kutupa kwa glands, mungakumane ndi izi:

• Malungo obwerezabwereza
• Kumva kutopa kwambiri komanso kutopa popanda chifukwa (izi zitha kukhalanso chifukwa cha kuchepa kwa magazi m'thupi)
• Thukuta kwambiri usiku
• Kusanza ndi kusanza
• Kuchepetsa thupi mosayembekezereka
• Kutupa kwa miyendo, akakolo, kapena mimba
• Nkhumba yokulirapo (splenomegaly) kapena chiwindi chokulirapo (hepatomegaly)
• Kusanza m'manja ndi m'mapazi (peripheral neuropathy)

N’chifukwa chiyani matendawa amapezeka? Kodi pali zinthu zina zomwe zingachititse kuti matendawa ayambe?

Ndipotu, asayansi sanapezebe chifukwa chenicheni cha matenda a Castleman (UCD) omwe ali m'gulu limodzi .

Matenda a Castleman ambiri (MCD) amagwirizanitsidwa ndi kachilombo ka HHV-8 komwe katchulidwa pamwambapa. Komabe, ofufuza akufufuzabe ngati matenda ena, kusintha kwa majini, kapena mayankho a autoimmune angakhale omwe amachititsa mtundu wosadziwika wa iMCD.

Ponena za zinthu zomwe zingakubweretsereni chiopsezo, palibe zinthu zomwe zimadziwika kuti zimayambitsa matenda a UCD ndi iMCD. Komabe, ngati muli ndi kachilombo ka HIV kapena chitetezo china chofooka cha mthupi, muli pachiwopsezo chachikulu chotenga matenda a HHV-8-associated MCD.

Ngakhale kuti matendawa amatha kufalikira mwa anthu azaka zilizonse, amapezeka kwambiri mwa anthu azaka zapakati pa 30 ndi 60 .

Kodi ndi mavuto otani omwe angayambitse matendawa?

Matenda a Castleman angakulitse pang'ono chiopsezo chanu chokhala ndi matenda ena, makamaka khansa , monga lymphoma, khansa ya lymphatic system.

Kuphatikiza apo, anthu omwe ali ndi UCD ali pachiwopsezo chotenga matenda a pakhungu osowa koma oopsa otchedwa paraneoplastic pemphigus (PNP). Anthu omwe ali ndi MCD ali pachiwopsezo chachikulu chotenga matenda osiyanasiyana. Ngati sanalandire chithandizo, matendawa amatha kuwononga ziwalo zawo ngakhale kufa.

Koma musadandaule, dokotala wanu nthawi zonse aziyang'anira vutoli ndikuchitapo kanthu kuti apewe mavutowa momwe angathere.

Kodi dokotala amazindikira bwanji matendawa molondola?

Popeza zizindikiro za matenda a Castleman zimafanana ndi za matenda ena, monga chimfine kapena fuluwenza, dokotala amayamba kaye wafufuza matenda ofala. Pokhapokha akamakayikira matenda a Castleman ndikuchita mayeso enaake.

Mayeso otsatirawa amachitika makamaka:

• Mayeso a labu: Magazi anu adzawunikidwa pogwiritsa ntchito maikulosikopu kuti awone ngati pali zolakwika pa kuchuluka kwa maselo amagazi anu ndi zolakwika zina. Ngati mukukayikira kuti pali MCD yogwirizana ndi HHV-8, mayeso a HIV angachitikenso.
• Mayeso ojambulira: Kujambula kwa CT ndi PET scan zingathandize kuzindikira komwe kuli ma lymph nodes otupa m'thupi. Angathenso kuyang'ana kukula kwa ziwalo monga chiwindi ndi ndulu.
• Kufufuza za lymph node biopsy: Iyi ndiyo njira yokhayo komanso yotsimikizika yotsimikizira matendawa . Izi zimaphatikizapo kutenga chidutswa chaching'ono kwambiri cha minofu kuchokera ku lymph node yotupa ndikuchiyang'ana pansi pa maikulosikopu. Izi zimatha kudziwa ngati pali kusintha kulikonse kwa maselo komwe kumakhudzana ndi matenda a Casselman.

Kodi mankhwala ochizira izi ndi otani?

Njira zochiritsira zimasiyana kwambiri malinga ndi mtundu wa matenda.

Chithandizo cha Matenda a Casselman Osakhazikika (UCD)

Chithandizo chachikulu komanso chopambana kwambiri cha UCD ndi kuchotsa lymph node yomwe yakhudzidwa ndi opaleshoni . Nthawi zambiri, matendawa amatha kuchiritsidwa kwathunthu pambuyo pa opaleshoniyi.

Nthawi zina, ngati chotupacho ndi chachikulu, chithandizo cha radiation kapena immunotherapy chimaperekedwa musanachite opaleshoni kuti chichepetsedwe ndikupangitsa kuti chichotsedwe mosavuta.

Ngati chotupacho chili pamalo omwe sichingachotsedwe ndi opaleshoni, ndipo mulibe zizindikiro, dokotala wanu angasankhe kuti asachichiritse ndikungochiyang'ana. Komabe, kwa munthu amene sali woyenera opaleshoni ndipo ali ndi zizindikiro, chithandizo chofanana ndi cha MCD chingaperekedwe.

Chithandizo cha matenda a Casselman ambiri (MCD)

Popeza MCD ndi matenda omwe amafalikira m'malo osiyanasiyana a thupi, sangachiritsidwe ndi opaleshoni kapena chithandizo cha radiation. Chifukwa chake, njira zina zochiritsira zimagwiritsidwa ntchito. Chithandizo chimadalira ngati muli ndi HHV-8 komanso momwe matendawa alili oopsa.

Mankhwala otsatirawa amaperekedwa makamaka:

• Corticosteroids: Mankhwalawa amathandiza kuchepetsa zizindikiro mwa kuchepetsa kutupa m'thupi.
• Mankhwala a Chemotherapy: Mankhwalawa amagwira ntchito poletsa kuchuluka kwa maselo m'thupi lomwe limakula mofulumira kwambiri. Rituximab imagwiritsidwa ntchito kwambiri pa HHV-8-associated MCD.
• Chithandizo cha Immunotherapy: Mankhwalawa amagwira ntchito pochepetsa mphamvu ya chitetezo cha mthupi yomwe imagwira ntchito mopitirira muyeso. Siltuximab (Sylvant®) ndiye mankhwala okhawo ovomerezeka a iMCD.
• Mankhwala oletsa mavairasi: Ngati muli ndi HHV-8-associated MCD, mungapatsidwe mankhwala awa kuti muchepetse kachilombo ka HIV kapena HHV-8.

Ndiyenera kupita kwa dokotala nthawi yanji?

Ngati muwona chotupa kapena chotupa pakhosi panu, m'khwapa, kapena m'chifuwa mwanu chomwe mungamve, onetsetsani kuti mwauza dokotala wanu za icho.

Komanso, ngati zizindikiro zomwe zatchulidwa m'nkhaniyi, monga kutentha thupi kosadziŵika bwino, kuchepa thupi, ndi kutopa kwambiri, zikupitirira kwa milungu ingapo, onetsetsani kuti mwapeza upangiri kwa dokotala.

Kodi n'zotheka kukhala ndi matendawa?

Yankho la funsoli limadalira mtundu wa Casselman womwe muli nawo.

Munthu amene ali ndi UCD angayembekezere zotsatira zabwino kwambiri . Chotupa chikachotsedwa opaleshoni, nthawi zambiri matendawa amachira kwathunthu. Pambuyo pa chithandizo, izi sizingakhudze moyo wanu watsiku ndi tsiku.

Kuyezetsa matenda kwa anthu omwe ali ndi MCD n'kovuta pang'ono . Zimasiyana malinga ndi mtundu ndi kuopsa kwa matendawa. Kwa ena, zizindikiro zimatha kukhala zoopsa mwadzidzidzi komanso zoopsa kwa moyo. Kwa ena, zizindikiro zimatha kukhala nthawi yayitali, koma pali chiyembekezo. Pakati pa 65% ndi 75% ya anthu omwe apezeka ndi MCD amakhala ndi moyo kwa zaka zoposa zisanu . Izi zikuyenda bwino ndi mankhwala atsopano.

Palibe yankho losavuta komanso lofanana ndi la matenda a Casselman. Zimadalira zomwe mwakumana nazo, mtundu wa matenda omwe muli nawo, komanso ngati muli ndi matenda ena, monga HIV, TAFRO, kapena POEMS. Nthawi zina opaleshoni ndi kuyang'aniridwa nthawi zonse ndi dokotala zidzakhala zokwanira. Kapena mungafunike kulandira chithandizo chamankhwala kangapo pa moyo wanu wonse. Chifukwa chake ndi bwino kukambirana momasuka ndi dokotala wanu za matenda anu, chithandizo chanu, komanso tsogolo lanu.