Yintoni iCastleman Disease? Masithethe ngayo ngokulula!

Ngaba wakha waphawula ukuba ufumana la maqhuqhuva amancinci entanyeni okanye emakhwapheni? Ngamanye amaxesha ayenzeka xa unomkhuhlane okanye umkhuhlane. Kodwa, oku akuyonto ilula ngolu hlobo. Le yimeko engaqhelekanga kodwa ebalulekileyo ekufuneka uyiqaphele. Siyibiza ngokuba yiCastleman Disease .

Ngamafutshane, iCastleman Disease yimeko apho amasosha omzimba wethu esebenza kakhulu . Khawuthelekelele, xa intsholongwane ebangela ukugula ingena emzimbeni wethu, amasosha omzimba ethu ayalwa nayo njengejoni eliya emfazweni. Xa ukugula kuphelile, loo joni liyathula kwakhona. Kodwa kumntu onesifo sikaCastleman, le amasosha omzimba ahlala 'evuliwe'. Oku kubangela ukudumba okuhlala ixesha elide emzimbeni, okunokonakalisa amalungu ethu omzimba.

Ngeli xesha, ii-lymph nodes zethu – ezo ziqhuma zincinci yile nto sizibiza ngayo – zisebenza njengesihluzo. Zonke iintsholongwane kunye neeseli zomzimba ziyahluzwa kuzo. Ngoko ke, ngenxa yokuba inkqubo yomzimba yokuzikhusela isebenza rhoqo, ezi lymph nodes nazo zisebenza ixesha elide. Emva koko iiseli ezikuzo ziyakhula kakhulu, kwaye ii-lymph nodes ziyadumba. Akukuphela nje oku kudumba, kodwa nakwiCastleman Disease, kukho notshintsho kwizicubu zezi lymph nodes.

Esi sisifo esingaqhelekanga . Umzekelo, eMelika, kuphela amatyala amatsha angama-4,300 ukuya kuma-5,200 afunyaniswa minyaka le. Ngoko ke oogqirha basafunda lukhulu ngale nto. Nangona kunjalo, ukuba ufunyaniswa unesi sifo, uya kufumana unyango oluthile oluhambelana nawe.

Ziziphi iintlobo eziphambili zeSifo sikaCastleman?

Kukho iintlobo ezimbini eziphambili zesi sifo.

Isifo seCastleman esiNye (UCD)

Isifo seCastleman esibizwa ngokuba yi-Unicentric (UCD) sibangelwa kukwanda kwe-lymph nodes kwindawo enye yomzimba wakho . Cinga nje ngentamo yakho, okanye ngamakhwapha akho. Malunga nesithathu kwisine sezigulane zaseCastleman ziwela kolu luhlu.

Isifo seCastleman esiNxulumene neMininzi (MCD)

Isifo se-Multicentric Castleman (MCD) sisifo apho ii-lymph nodes zidumbile kwiindawo ezininzi zomzimba, endaweni yokuba zibe kwenye. Ukuchaneka, oogqirha bahlula olu hlobo lwe-MCD lube:

• I-MCD enxulumene neembongi: Iimbongi sisifo segazi esingaqhelekanga. Ngamanye amaxesha, iimbongi zinokwenzeka kunye ne-MCD.
• I-HHV-8-associated MCD: Olu hlobo lubangelwa kusulelo lwentsholongwane ebizwa ngokuba yiHuman Herpes Virus-8 (HHV-8) . Ukuba unosulelo lwe-HIV okanye unesifo sokuzikhusela komzimba, unamathuba amaninzi okufumana olu hlobo lwe-MCD.
• I-MCD yesizathu esingaziwayo (i-Idiopathic MCD - iMCD):Olu lolona hlobo luqhelekileyo lwe-MCD. 'Idiopathic' ithetha ukuba unobangela ochanekileyo awaziwa.

Ngoku le nto ingaziwayo i-iMCD ikwanazo neenxalenye ezintathu ezingaphantsi:

• iMCD enxulumene ne-TAFRO syndrome: Ngamanye amaxesha iMCD inokuhamba kunye nemeko ebizwa ngokuba yi-TAFRO syndrome. Igama elithi TAFRO livela kwiileta zokuqala zeempawu zayo. Ezi zezi: I-Thrombocytopenia (iiplatelets ezisezantsi zegazi), i-Anasarca (ukudumba komzimba ngamanzi), i-Fever , ukungasebenzi kakuhle kwezintso (izintso), kunye ne -Organomegaly (ukwanda kwepleen okanye isibindi).
• iMCD ene-Idiopathic Plasmacytic Lymphadenopathy (iMCD-IPL): Kule ntlobo, iiplatelets zinokuphakama. Iiseli zakho ezimhlophe zegazi nazo zinokuvelisa ii-antibodies ezininzi kakhulu.
• iMCD ayichazwanga ngenye indlela (iMCD-NOS): Unobangela ochanekileyo wolu hlobo, obizwa ngokuba yi-iMCD-NOS, nawo awaziwa, kwaye awunxulumananga ne-TAFRO.

Zithini iimpawu zesifo sikaCastleman?

Iimpawu zesifo sikaCastleman ziyahluka ngokuxhomekeke kuhlobo onalo. Kwiimeko ezininzi, ukuba unesifo sikaCastleman esizimeleyo (UCD), usenokungabi nazo naziphi na iimpawu ezinkulu. Ii-lymph nodes ezidumbileyo zisenokuba lolona phawu kuphela olubonayo. Nangona kunjalo, ngamanye amaxesha iimpawu zinokwenzeka ukuba i-lymph node edumbileyo icinezele elinye ilungu elikufutshane.

Nangona kunjalo, ukuba une-MCD, iimpawu zibonakala ngakumbi. Ukongeza kwii-lymph nodes ezivuvukileyo, unokubona iimpawu ezifana nezi:

• Ifiva
• Ukudinwa (oku kungaba luphawu lwe-anemia)
• Ukubila ebusuku
• Isicaphucaphu nokuhlanza
• Ukunciphisa umzimba ngaphandle kwesizathu
• Ukudumba ezinyaweni zakho, emaqatheni, okanye esiswini
• I-spleen ekhulisiweyo (i-splenomegaly) okanye isibindi esikhulisiweyo (i-hepatomegaly)
• Ukuba ndindisholo ezandleni nasezinyaweni (peripheral neuropathy)

Yintoni ebangela isifo sikaCastleman?

Isizathu esichanekileyo sesifo seCastleman esingaphakathi (UCD) asikaziwa okwangoku, kodwa ukosuleleka yintsholongwane ebizwa ngokuba yiHHV-8 kufunyenwe kuyinxalenye yophuhliso lwesifo seCastleman esiphakathi (MCD).

Izazinzulu zisaphanda ezinye izizathu ezinokubakho ze-UCD kunye ne-iMCD. Umzekelo, zijonga izifo ezingezizo i-HHV-8, utshintsho lwezakhi zofuzo , kunye neempendulo ze-autoimmune .

Ngubani osengozini enkulu yokuhlaselwa sesi sifo?

Izinto ezithile ezibangela umngcipheko we-UCD okanye iMCD azikachongwa okwangoku. Nangona kunjalo, ukuba unentsholongwane kaGawulayo okanye enye imeko ebuthathaka amasosha omzimba wakho, usemngciphekweni omkhulu wokufumana i-HHV-8-associated MCD.

Isifo sikaCastleman sinokubakho nakweyiphi na iminyaka, kodwa sixhaphake kakhulu kubantu abaphakathi kweminyaka engama-30 ukuya kwengama-60 ubudala .

Ziziphi iingxaki ezinokubakho zesifo sikaCastleman?

Isifo sikaCastleman sandisa umngcipheko wokuba nomhlaza, ingakumbi i-lymphoma .

Kwakhona, nangona kunqabile kakhulu, abantu abane-UCD basengozini yokuhlaselwa sisifo esibi esibizwa ngokuba yiParaneoplastic Pemphigus (PNP) . I-MCD ikwanyusa umngcipheko wakho wokufumana usulelo olunokonakalisa amalungu akho omzimba. Lo monakalo unokuba yingozi ebomini ukuba awunyangwa ngokufanelekileyo.

Ungakhathazeki, ugqirha wakho uya kuhlala ejonga imeko yakho aze akuncede uthintele iingxaki ezinjalo nanini na xa kunokwenzeka.

Oogqirha bayixilonga njani iCastleman Disease?

Iimpawu zeSifo sikaCastleman zinokufana nezezinye izifo eziqhelekileyo, ezifana nomkhuhlane okanye umkhuhlane. Ngoko ke ugqirha wakho uya kuqala angazifumani ezo meko. Emva koko, uya kwenza uvavanyo lokufumanisa isifo sikaCastleman aze amisele uhlobo lwaso.

Uvavanyo kunye neenkqubo zingabandakanya:

• Uvavanyo lwelebhu: Ugqirha wakho uza kujonga inani leeseli zakho zegazi ukuze ajonge izinto ezingaqhelekanga kunye nezinye iimpawu ezincinci zesifo sikaCastleman. Banokuyalela uvavanyo lwe-HIV . Ukuba une-HHV-8-related MCD, uvavanyo lwakho lwe-HIV lusenokuba luchanekile.
• Uvavanyo lwemifanekiso: Uvavanyo olufana ne -CT scans kunye ne-PET scans lunokunceda ekufumaneni ii-lymph nodes ezidumbileyo emzimbeni wakho, kunye nokujonga ezinye iimpawu zesifo sikaCastleman, ezifana nesibindi esikhulu okanye i-spleen.
• I-lymph node biopsy: Eyona ndlela yokwazi ngokuqinisekileyo ukuba unesifo sikaCastleman kukwenza i-biopsy. Oku kuquka ukuthatha iqhekeza elincinci lesicwili kwi-lymph node uze ulihlole phantsi kwe-microscope. Oku kuya kuqinisekisa ukuba kukho naziphi na iimpawu zesifo.

Iphathwa njani iCastleman Disease?

Unyango lwesifo sikaCastleman luyahluka ngokuxhomekeke kuhlobo onalo.

Unyango lweSifo seCastleman esiNgqongqo (UCD)

Unyango oluphambili lwe-UCD kukususa ii-lymph nodes ezichaphazelekayo ngotyando . Ngamanye amaxesha unokufuna unyango lwe-radiation okanye i-immunotherapy ngaphambi kotyando. Olu nyango lunciphisa ukukhula kwee-lymph nodes, okwenza kube lula ukuzisusa.

Kodwa ukuba oogqirha botyando abakwazi ukususa i-lymph node echaphazelekayo, kwaye wena awunazo iimpawu, ugqirha wakho unokuyijonga endaweni yokuyinyanga. Ukuba uneempawu kwaye awukwazi ukwenza utyando, banokucebisa unyango lwe-MCD.

Unyango lwesifo seCastleman esineenkalo ezininzi (MCD)

I-MCD inzima kancinci ukuyinyanga kune-UCD. Ngenxa yokuba isasazeka emzimbeni wonke, unyango lotyando okanye unyango lwemitha alusetyenziswa rhoqo. Endaweni yoko, unyango luxhomekeke ekubeni unosulelo lwe-HHV-8 na kwaye isifo sibi kangakanani.

Ugqirha wakho angasebenzisa unyango olufana nolu:

• IiCorticosteroids: La mayeza anceda ekunciphiseni ukudumba kwaye anike isiqabu kwiimpawu.
• Amayeza e-Chemotherapy: Amayeza e-Chemotherapy alawula ukukhula okugqithisileyo kweeseli kwinkqubo yakho ye-lymphatic. I-Rituximab liyeza eliqhele ukumiselwa kwi-HHV-8-associated MCD.
• Unyango lwe-Immunotherapy: Olu nyango lubandakanya ukusebenzisa izinto ezifana nee-antibodies ze-monoclonal . Ezi zinceda ukuzola impendulo yomzimba wakho. I-Siltuximab (Sylvant®) lolona nyango luvunyiweyo yi-U.S. Food and Drug Administration (FDA) ye-iMCD.
• Amayeza okulwa neentsholongwane: Ukuba une-HHV-8-related MCD, unokufuna amayeza okulwa neentsholongwane ukunyanga i-HIV okanye usulelo lwe-HHV-8.

Ndifanele ndimbone nini ugqirha?

Ukuba ubona iqhuma okanye ukukhula entanyeni, emakhwapheni, okanye emqolo, bonana nogqirha ngokukhawuleza . Kwakhona, ukuba unezinye iimpawu zeSifo sikaCastleman ezikhankanyiweyo apha ngasentla ezingaphucukiyo emva kweeveki ezimbalwa, xelela ugqirha wakho ngazo.

Ungaphila ixesha elingakanani nale sifo?

Kubantu abaninzi abane-UCD, i-prognosis, okanye umbono, ilungile kakhulu. Ukususa ii-lymph nodes ezichaphazelekayo kunokunyanga esi sifo. Ngonyango, i-UCD ayidli ngokuchaphazela ixesha lokuphila.

Kodwa imbono yabantu abane-MCD ixhomekeke kuhlobo lwe-MCD abanayo kunye nobunzima besifo. Kwabanye abantu, iimpawu zinokuvela ngequbuliso kwaye zibe yingozi ebomini. Kwabanye, iimpawu zinokuhlala ixesha elide. Phakathi kwama-65% kunye nama-75% abantu abafunyaniswe bene-MCD basaphila emva kweminyaka emihlanu . Ngonyango olutsha, kukho ithemba lokuba le meko iya kuphucuka.

Akukho zimpendulo zilula xa kufikwa kwisifo sikaCastleman. Amava akho aya kuxhomekeka kuhlobo lwesifo sikaCastleman onaso. Ezinye iimeko, ezifana ne-HIV, okanye iimeko ezinxulumene noko, ezifana ne-TAFRO kunye ne-POEMS, nazo zinokuchaphazela amava akho. Kwezinye iimeko, utyando kunye nokujonga ngokusondeleyo kunokuba yimfuneko kuphela. Okanye, unokufuna unyango olwahlukeneyo ebomini bakho ukuthintela iingxaki. Buza ugqirha wakho ukuba ukuxilongwa kwakho kuya kuyichaphazela njani unyango lwakho kunye nendlela ocinga ngayo.

Ngoko ke, zeziphi izinto ezibalulekileyo ekufuneka sizikhumbule koku?

• Isifo sikaCastleman sisifo esingaqhelekanga kodwa esinokuba yingozi kakhulu apho amajoni ethu omzimba eba namandla kakhulu.
• Kukho iintlobo ezimbini eziphambili: i-UCD kunye ne-MCD. Kwi-UCD, ii-lymph nodes ziyadumba kwindawo enye, ngelixa kwi-MCD, ziyadumba kwiindawo ezininzi.
• Iimpawu ziyahluka. Ngamanye amaxesha kusenokungabikho zimpawu konke konke, kusenokuba liqhuma elidumbileyo. Okanye kusenokuba kukho iimpawu ezifana nomkhuhlane, ukudinwa, kunye nokwehla kobunzima.
• Izizathu azicaci rhoqo, kodwa intsholongwane yeHHV-8 inxulunyaniswe ne-MCD. Abantu abane-HIV basengozini enkulu.
• Ukufunyanwa kwangoko kunye nonyango olufanelekileyo kunokubangela iziphumo ezilungileyo. Ngoko ke ukuba ubona iqhuma elingaqhelekanga, ukudumba, okanye ezinye iimpawu ezingaqhelekanga emzimbeni wakho, funa ingcebiso yezonyango ngokukhawuleza. Eyona nto ibalulekileyo kukuhlala unolwazi kwaye ungoyiki.

Isifo sikaCastleman , iiLymph Nodes, iLymphadenopathy, iSystem Immune, i-UCD, iMCD, iHHV-8, Iimpawu, Unyango