Okudingeka ukwazi nge-Castleman Disease

Wake waba neqhubu, esilibiza ngokuthi “iqhubu,” entanyeni yakho, emakhwapheni, noma endukwini? Ngezinye izikhathi, amaqhubu anjengalawa ayavuvukala uma unesifo somkhuhlane noma uhlobo oluthile lokutheleleka. Lokho kuvamile. Kodwa uma uneqhubu elivuvukile njengalawa ngaphandle kwesizathu, isikhathi eside, futhi uzizwa ukhathele futhi wehlisa isisindo, kufanele ukhathazeke kancane. Namuhla, sizokhuluma ngesimo esingavamile esingasolwa ngezikhathi ezinjalo. Leso yi-Castleman Disease.

Kalula nje, siyini isifo sikaCastleman?

Cabanga nje ukuthi umzimba wethu ufana nezwe. Khona-ke amasosha ethu omzimba yibutho elivikela lelo zwe. Lapho igciwane (isitha) lingena emzimbeni livela ngaphandle, leli butho liya empini bese linqoba leso sitha. Lapho impi isiphelile, ibutho libuyela ekamu bese lithula. Yilokhu okwenzeka emzimbeni womuntu ophilile.

Kodwa kumuntu onesifo sikaCastleman, kuhlukile. Okwenzekayo lapha ukuthi ibutho elaya empini, isimiso somzimba sokuzivikela, alizoli ngisho nangemva kokuba impi isiphelile. Bahlala bekhuthele. Kufana nokuthi bahlala belungele impi. Lokhu kubangela ukuvuvukala isikhathi eside emzimbeni. Lokhu kungalimaza izitho zethu zomzimba.

Okwamanje, ama-lymph node ethu, lezo zinto ezincane, zifana nezisekelo zempi. Zenza umsebenzi omningi, njengokuhlunga amagciwane nokugcina amangqamuzana omzimba. Manje, ngenxa yokuthi isimiso somzimba sokuzivikela sisebenza njalo, la ma-lymph node nawo asebenza njalo. Ngenxa yalokho, amangqamuzana akuwo aqala ukukhula ngokweqile. Yingakho ama-lymph node evuvukala esifweni sikaCastleman. Akusikho ukuvuvukala nje kuphela, kodwa futhi kubangela nezinguquko ezicutshini zalawo ma-lymph node.

Yiziphi izinhlobo eziyinhloko zesifo sikaCastleman?

Isifo i-Casselman sihlukaniswe ngezinhlobo ezimbili eziyinhloko. Izimpawu kanye nokwelashwa kwaso ngasinye kuhlukile kakhulu. Ngakho-ke, kubalulekile ukuqonda lezi zinhlobo ezimbili ngokunembile.

Ingabe kukhona izinhlobo ezincane zesifo se-Castleman esigxile ezintweni eziningi (MCD)?

Yebo, i-MCD ihlukaniswe kabanzi ngezinhlobo eziningana. Lokhu kuhlukaniswa kwenziwa ngokusekelwe kumbangela kanye nezinye izimo ezenzeka ngayo.

• I-MCD ehlobene nezinkondlo: Izinkondlo ziyisifo segazi esingavamile kakhulu. Ngezinye izikhathi i-MCD ingenzeka kanye nezinkondlo.
• I-HHV-8-associated MCD: Lokhu kubangelwa ukutheleleka ngegciwane elibizwa ngokuthi i-Human Herpes Virus-8 (HHV-8). Lolu hlobo luvame kakhulu kubantu abatheleleke nge-HIV noma abakhubazekile ngenxa yezinye izizathu.
• Idiopathic MCD (iMCD): Igama lezokwelapha elithi " idiopathic " lisho ukuthi "akukho sizathu esitholiwe." Iningi leziguli ze-MCD linalolu hlobo. Lokhu kusho ukuthi imbangela eqondile ayaziwa okwamanje.

Lolu hlobo lwe-iMCD luhlukaniswe ngezigaba ezintathu ezincane:

1. iMCD ehlotshaniswa ne-TAFRO: I-TAFRO igama elisuselwa ekuhlanganisweni kwezimpawu eziningana. Okusho ukuthi, i-Thrombocytopenia (ama-platelet aphansi egazi), i-Anasarca (ukuvuvukala komzimba ngamanzi), i-Fever (umkhuhlane), ukungasebenzi kahle kwezinso (ukuba buthaka komsebenzi wezinso) kanye ne-Organomegaly (ukwanda kwesibindi noma udende) yizimpawu ezingabonakala kulokhu.

2. iMCD ene-idiopathic plasmacytic lymphadenopathy (iMCD-IPL): Kulolu hlobo, inani lama-platelet lingase likhuphuke. Kungase kube nokukhiqizwa ngokweqile kwama-antibodies ngamaseli amhlophe egazi.

3. iMCD, engachazwanga ngenye indlela (iMCD-NOS): Iziguli ezine-iMCD ezingahlobene ne-TAFRO futhi ezingatholakali esinye isizathu esithile ngazo ziwela kulesi sigaba.

Yiziphi izimpawu zalesi sifo?

Izimpawu ziyahlukahluka kuye ngohlobo lwesifo sikaCastleman onaso.

Isifo se-Unicentric Casselman (UCD) ngokuvamile asinazo izimpawu . Uphawu olulodwa kuphela i-lymph node evuvukile (i-adenoid). Ngezinye izikhathi, izimpawu (isib. ubuhlungu, ukuphelelwa umoya) zenzeka kuphela lapho i-node evuvukile icindezela esinye isitho.

Kodwa-ke, kwisifo se-Castleman esihlanganisa i-multicentric (MCD), izimpawu zisobala kakhulu. Ngaphezu kwezindlala ezivuvukele, ungase ubhekane nalokhu:

• Umkhuhlane ovamile
• Ukuzizwa ukhathele kakhulu futhi ukhathele ngaphandle kwesizathu (lokhu kungabangwa yi-anemia)
• Ukujuluka ngokweqile ebusuku
• Isicanucanu nokuhlanza
• Ukwehlisa isisindo okungalindelekile
• Ukuvuvukala kwemilenze, amaqakala, noma isisu
• Ubende okhulisiwe (i-splenomegaly) noma isibindi esikhulisiwe (i-hepatomegaly)
• Ukuba ndikindiki ezandleni nasezinyaweni (i-peripheral neuropathy)

Kungani lesi sifo senzeka? Ingabe zikhona izinto eziyingozi?

Eqinisweni, ososayensi abakayitholi imbangela ethile yesifo se-Castleman esiyingqayizivele (UCD) .

Isifo se-Multicentric Castleman (MCD) sihlotshaniswa negciwane le-HHV-8 elishiwo ngenhla. Kodwa-ke, abacwaningi basaphenya ukuthi ezinye izifo, ukuguquka kwezakhi zofuzo, noma izimpendulo zokuzivikela komzimba kungaba yini imbangela yohlobo olungaziwa lwe-iMCD.

Uma kukhulunywa ngezinto eziyingozi, azikho izinto eziyingozi ezaziwayo ze-UCD kanye ne-iMCD. Kodwa-ke, uma unegciwane lesandulela ngculaza noma esinye isimiso somzimba sokuzivikela esibuthakathaka, usengozini enkulu yokuthola i-HHV-8-associated MCD.

Nakuba lesi sifo singakhula kubantu banoma yimuphi ubudala, sivame kakhulu kubantu abaneminyaka ephakathi kuka-30 no-60 .

Yiziphi izinkinga ezingaba khona ngenxa yalesi sifo?

Isifo sikaCastleman singase sandise kancane ingozi yakho yokuthola ezinye izimo, ikakhulukazi umdlavuza , njenge -lymphoma, umdlavuza wesistimu ye-lymphatic.

Ngaphezu kwalokho, abantu abane-UCD basengozini yokuthola isifo sesikhumba esingavamile kodwa esibi esibizwa ngokuthi i-paraneoplastic pemphigus (PNP). Abantu abane-MCD basengozini enkulu yokuthola izifo ezahlukahlukene. Uma zingelashwa, lezi zifo zingabangela ukulimala kwezitho zomzimba ngisho nokufa.

Kodwa ungakhathazeki, udokotela wakho uzohlala eqapha lesi simo futhi athathe izinyathelo ezidingekayo ukusiza ekuvimbeleni lezi zinkinga ngangokunokwenzeka.

Udokotela usithola kanjani lesi sifo ngokunembile?

Ngenxa yokuthi izimpawu zesifo sikaCastleman zifana nezezinye izifo, njengomkhuhlane ovamile noma umkhuhlane, udokotela uzoqala ahlole izifo ezivamile. Kulapho-ke kuphela lapho bezosola khona isifo sikaCastleman futhi benze izivivinyo ezithile.

Ukuhlolwa okulandelayo kwenziwa kakhulu:

• Ukuhlolwa kwelebhu: Igazi lakho lizohlolwa ngaphansi kwe-microscope ukuze kuhlolwe ukungalingani ekubalweni kwamaseli egazi lakho kanye nokunye ukungalingani. Uma kusolwa ukuthi i-HHV-8-associated MCD, ukuhlolwa kwe-HIV nakho kungenziwa.
• Ukuhlolwa kwezithombe: Ukuskena kwe-CT kanye nokuskena kwe-PET kungasiza ekutholeni ukuthi ama-lymph node avuvukele akuphi emzimbeni. Kungahlola nokukhula kwezitho zomzimba njengesibindi kanye ne-spleen.
• Ukuhlolwa kwe-lymph node biopsy: Lena yindlela ewukuphela kwayo necacile yokuqinisekisa lesi sifo . Lokhu kuhilela ukuthatha ingxenye encane kakhulu yezicubu ezivela ku-lymph node evuvukele bese uyihlola ngaphansi kwe-microscope. Lokhu kungabonisa ngokuqinisekile ukuthi kukhona yini izinguquko ezithile zamaseli ezihlobene nesifo se-Casselman.

Yiziphi izindlela zokwelapha lokhu?

Izindlela zokwelapha ziyahlukahluka ngokuphelele kuye ngohlobo lwesifo.

Ukwelashwa kweSifo se-Unicentric Casselman (UCD)

Ukwelashwa okuyinhloko nokuphumelela kakhulu kwe-UCD ukususwa ngokuhlinzwa kwe-lymph node ethintekile . Ezimweni eziningi, lesi sifo siyaphela ngokuphelele ngemva kwalokhu kuhlinzwa.

Ngezinye izikhathi, uma isimila sikhulu, ukwelashwa ngemisebe noma nge-immunotherapy kunikezwa ngaphambi kokuhlinzwa ukuze kusinciphise futhi kube lula ukusisusa.

Uma iqhubu lisendaweni engenakususwa ngokuhlinzwa, futhi ungenazo izimpawu, udokotela wakho angase anqume ukungaliphathi bese elibheka nje. Kodwa-ke, kumuntu ongeyena umuntu ofanele ukuhlinzwa futhi osenezimpawu, ukwelashwa okufanayo nokwe-MCD kunganikezwa.

Ukwelashwa kwesifo se-Casselman esine-multicentric (MCD)

Ngenxa yokuthi i-MCD yisimo esisakazekela ezingxenyeni eziningi zomzimba, asikwazi ukwelashwa ngokuhlinzwa noma ngokwelashwa ngemisebe. Ngakho-ke, kusetshenziswa ezinye izindlela zokwelapha. Ukwelashwa kuncike ekutheni une-HHV-8 nokuthi isifo sibi kangakanani.

Ukwelashwa okulandelayo kuhlinzekwa kakhulu:

• Ama-Corticosteroids: Le mithi isiza ukulawula izimpawu ngokunciphisa ukuvuvukala emzimbeni.
• Imithi yokwelapha ngamakhemikhali: Le mithi isebenza ngokulawula izinga lapho amaseli ohlelweni lwe-lymphatic akhula ngokushesha kakhulu. I-Rituximab ivame ukusetshenziswa kwi-HHV-8-associated MCD.
• Ukwelashwa Ngezifo Ezingalawuleki: Lokhu kwelashwa kusebenza ngokuzolisa amasosha omzimba wakho asebenza ngokweqile. I-Siltuximab (Sylvant®) iyona kuphela imithi evunyelwe i-iMCD.
• Imithi yokulwa namagciwane: Uma une-HHV-8-associated MCD, unganikezwa le mithi yokulawula i-HIV noma ukutheleleka kwe-HHV-8.

Ngingayibona nini udokotela?

Uma ubona iqhubu noma iqhubu entanyeni yakho, emakhwapheni, noma embotsheni , qiniseka ukuthi utshela udokotela wakho ngakho.

Futhi, uma izimpawu ezishiwo kulesi sihloko, njengomkhuhlane ongachazeki, ukwehla kwesisindo, nokukhathala ngokweqile, ziqhubeka amasonto amaningana, qiniseka ukuthi ufuna iseluleko sezokwelapha.

Kungenzeka yini ukuphila nalesi sifo?

Impendulo yalo mbuzo incike ohlotsheni lweCasselman onalo.

Umuntu one-UCD angalindela imiphumela emihle kakhulu . Uma isimila sisuswa ngokuhlinzwa, lesi sifo sivame ukuphulukiswa ngokuphelele. Ngemva kokwelashwa, lokhu ngeke kuthinte isikhathi sakho sokuphila esijwayelekile.

Ukubikezela isifo kubantu abane-MCD kuyinkimbinkimbi kancane . Kuyahlukahluka kuye ngohlobo kanye nobunzima besifo. Kwabanye, izimpawu zingaba zimbi ngokuzumayo futhi zisongele impilo. Kwabanye, izimpawu zingahlala isikhathi eside, kodwa kukhona ithemba. Phakathi kuka-65% no-75% wabantu abatholakale bene-MCD baphila iminyaka engaphezu kwemihlanu . Lokhu kuyathuthuka ngemithi emisha.

Ayikho impendulo elula, efanela wonke umuntu ngesifo i-Casselman. Kuzoncika kokuhlangenwe nakho kwakho, uhlobo lwesifo onaso, nokuthi unezinye izimo, njenge-HIV, i-TAFRO, noma i-POEMS. Ngezinye izikhathi ukuhlinzwa nokuqapha njalo kwezokwelapha kuzokwanela. Noma kungadingeka ukuthi uthole ukwelashwa okuningi empilweni yakho yonke. Ngakho-ke kungcono ukukhuluma ngokukhululekile nodokotela wakho ngesimo sakho, ukwelashwa kwakho, kanye nekusasa lakho.