Kuyini i-Castleman Disease? Ake sixoxe ngakho kalula!

Wake waphawula ukuthi uthola la maqhubu amancane entanyeni noma emakhwapheni akho? Ngezinye izikhathi ayenzeka uma unomkhuhlane noma umkhuhlane. Kodwa, lokhu akuphathelene nento elula njengaleyo. Lesi yisimo esingavamile kodwa esibalulekile okufanele usiqaphele. Sisibiza ngokuthi yi -Castleman Disease .

Kalula nje, i-Castleman Disease yisimo lapho amasosha omzimba wethu eba matasa kakhulu . Cabanga nje, lapho igciwane elibangela ukugula lingena emzimbeni wethu, amasosha omzimba ethu alwa nalo njengesosha eliya empini. Lapho ukugula sekwelaphekile, leso sosha liyathula futhi. Kodwa kumuntu onesifo i-Castleman Disease, lesi simiso somzimba 'sihlala sivuliwe'. Lokhu kubangela ukuvuvukala isikhathi eside emzimbeni, okungalimaza izitho zethu zomzimba.

Ngalesi sikhathi, ama-lymph node ethu – lawo maqhubu amancane yilokho esiwabiza ngakho – asebenza njengesihlungi. Wonke amagciwane namaseli omzimba ahlungwa ngawo. Ngakho-ke, ngenxa yokuthi isimiso somzimba sokuzivikela sisebenza njalo, la ma-lymph node nawo asebenza isikhathi esengeziwe. Bese amaseli akuwo ekhula ngokweqile, futhi ama-lymph node avuvukala. Hhayi lokhu kuvuvukala kuphela, kodwa naku-Castleman Disease, kukhona ngisho nezinguquko ezicutshini zala ma-lymph node.

Lesi yisifo esingavamile kakhulu. Isibonelo, e-United States, kutholakala amacala amasha angu-4,300 kuya ku-5,200 kuphela unyaka ngamunye. Ngakho odokotela basafunda okuningi ngalokhu. Kodwa-ke, uma utholakala unalesi sifo, uzothola ukwelashwa okuqondile okuqondene nawe.

Yiziphi izinhlobo eziyinhloko ze-Castleman Disease?

Kunezinhlobo ezimbili eziyinhloko zalesi sifo.

Isifo se-Castleman esiyingqayizivele (UCD)

Isifo se-Unicentric Castleman (UCD) siwukukhula kwe-lymph node eyodwa noma ezimbalwa endaweni eyodwa yomzimba wakho . Cabanga ngentamo yakho, noma amakhwapha akho. Cishe izingxenye ezintathu kwezine zeziguli zaseCastleman ziwela kulesi sigaba.

Isifo se-Castleman esihlanganisa izingxenye eziningi (MCD)

Isifo se-Multicentric Castleman (MCD) yisimo lapho ama-lymph node evuvukala ezindaweni eziningi zomzimba, kunokuba kube yinye kuphela. Ukuze kube sobala, odokotela bahlukanisa lolu hlobo lwe-MCD ngalezi zindlela ezilandelayo:

• I-MCD ehlobene nezinkondlo: Izinkondlo ziyisifo segazi esingavamile kakhulu. Ngezinye izikhathi, izinkondlo zingavela kanye ne-MCD.
• I-MCD ehlobene ne-HHV-8: Lolu hlobo lubangelwa ukutheleleka ngegciwane elibizwa ngokuthi i- Human Herpes Virus-8 (HHV-8) . Uma unegciwane le-HIV noma une-immune system ebuthakathaka, kungenzeka kakhulu ukuthi ube nalolu hlobo lwe-MCD.
• I-MCD yembangela engaziwa (i-Idiopathic MCD - iMCD):Lolu uhlobo oluvame kakhulu lwe-MCD. I-'Idiopathic' isho ukuthi imbangela eqondile ayaziwa.

Manje lesi sizathu esingaziwa i-iMCD inezingxenye ezintathu ezincane:

• iMCD ehlotshaniswa ne-TAFRO syndrome: Ngezinye izikhathi iMCD ingahambisana nesimo esibizwa ngokuthi i-TAFRO syndrome. Igama elithi TAFRO lisuselwa ezinhlamvini zokuqala zezimpawu zalo. Lezi yizi: I-Thrombocytopenia (ama-platelet aphansi egazi), i-Anasarca (ukuvuvukala komzimba ngamanzi), i-Fever , ukungasebenzi kahle kwezinso (izinso), kanye ne -Organomegaly (ukukhula kwe-spleen noma isibindi).
• iMCD ene-Idiopathic Plasmacytic Lymphadenopathy (iMCD-IPL): Kulolu hlobo, ama-platelet angase aphakame. Amaseli akho amhlophe egazi angase akhiqize nama-antibodies amaningi kakhulu.
• iMCD engachazwanga ngenye indlela (iMCD-NOS): Imbangela eqondile yalolu hlobo, ebizwa ngokuthi iMCD-NOS, nayo ayaziwa, futhi ayihlobene ne-TAFRO.

Ziyini izimpawu zesifo sikaCastleman?

Izimpawu zesifo sikaCastleman ziyahlukahluka kuye ngohlobo onalo. Ezimweni eziningi, uma unesifo sikaCastleman esiyingqayizivele (UCD), ungase ungabi nazo izimpawu ezinkulu. Ama-lymph node avuvukile angaba yisona kuphela isibonakaliso osibonayo. Kodwa-ke, ngezinye izikhathi izimpawu zingavela uma i-lymph node evuvukile icindezela esinye isitho esiseduze.

Kodwa-ke, uma une-MCD, izimpawu cishe zisobala. Ngaphezu kwama-lymph node avuvukele, ungase ubone izimpawu ezifana nalezi:

• Imfiva
• Ukukhathala (lokhu kungaba uphawu lwe-anemia)
• Ukujuluka ebusuku
• Isicanucanu nokuhlanza
• Ukwehlisa isisindo ngaphandle kwesizathu
• Ukuvuvukala ezinyaweni zakho, emaqakaleni, noma esiswini
• Ubende okhulisiwe (i-splenomegaly) noma isibindi esikhulisiwe (i-hepatomegaly)
• Ukuba ndikindiki ezandleni nasezinyaweni (i-peripheral neuropathy)

Yini ebangela isifo seCastleman?

Imbangela eqondile yesifo se-Castleman esingaphakathi (UCD) ayaziwa okwamanje, kodwa ukutheleleka ngegciwane elibizwa nge-HHV-8 kutholakale kuyimbangela yokuthuthuka kwesifo se-Castleman esingaphakathi (MCD) esingaphakathi.

Ososayensi basaphenya ngezinye izimbangela ezingaba khona ze-UCD kanye ne-iMCD. Isibonelo, babheka izifo ezingezona i-HHV-8, ukuguquka kwezakhi zofuzo , kanye nezimpendulo zokuzivikela komzimba .

Ubani osengozini enkulu yokuthola lesi sifo?

Izici ezithile eziyingozi ze-UCD noma iMCD azikatholakali okwamanje. Kodwa-ke, uma unegciwane lesandulela ngculaza noma esinye isimo esenza amasosha omzimba wakho abe buthaka, usengozini enkulu yokuthola i-HHV-8-associated MCD.

Isifo sikaCastleman singavela kunoma yimuphi ubudala, kodwa sivame ukutholakala kubantu abaneminyaka ephakathi kuka-30 no-60 .

Yiziphi izinkinga ezingaba khona zesifo sikaCastleman?

Isifo sikaCastleman sandisa ingozi yakho yokuba nomdlavuza, ikakhulukazi i-lymphoma .

Futhi, nakuba kungavamile kakhulu, abantu abane-UCD basengozini yokuthola isimo esibi esibizwa ngokuthi i-Paraneoplastic Pemphigus (PNP) . I-MCD iphinde inyuse ingozi yakho yokuthola izifo ezingalimaza izitho zakho zomzimba. Lo monakalo ungaba yingozi empilweni uma ungelashwa kahle.

Ungakhathazeki, udokotela wakho uzohlala eqapha isimo sakho futhi akusize uvimbele izinkinga ezinjalo noma nini lapho kungenzeka.

Odokotela bahlonza kanjani isifo sikaCastleman?

Izimpawu ze-Castleman Disease zingafana nezezinye izifo ezivamile, njengomkhuhlane noma umkhuhlane. Ngakho udokotela wakho uzoqala ngokususa lezo zimo. Ngemuva kwalokho, uzokwenza izivivinyo zokuxilonga i-Castleman Disease bese enquma uhlobo lwayo.

Ukuhlolwa nezinqubo kungafaka:

• Ukuhlolwa kwelebhu: Udokotela wakho uzohlola inani lamaseli akho egazi ukuze athole ukukhubazeka kanye nezinye izimpawu ezincane zesifo sikaCastleman. Bangase futhi bacele ukuhlolwa kwe-HIV . Uma une-MCD ehlobene ne-HHV-8, ukuhlolwa kwakho kwe-HIV kungase kube nomphumela omuhle.
• Ukuhlolwa kwezithombe: Ukuhlolwa okufana ne -CT scans kanye ne-PET scans kungasiza ekutholeni ama-lymph node avuvukele emzimbeni wakho, kanye nokuhlola ezinye izimpawu zesifo sikaCastleman, njengesibindi esikhulu noma uqweqwe.
• Ukuhlolwa kwe-lymph node: Okuwukuphela kwendlela yokwazi ngokuqinisekile ukuthi unesifo sikaCastleman yini ukwenza i-biopsy. Lokhu kuhilela ukuthatha ingxenye encane yezicubu ku-lymph node bese uyihlola ngaphansi kwe-microscope. Lokhu kuzonquma ukuthi zikhona yini izimpawu zesifo.

Isifo sikaCastleman siphathwa kanjani?

Ukwelashwa kwesifo sikaCastleman kuyahlukahluka kuye ngohlobo onalo.

Ukwelashwa kwe-Unicentric Castleman Disease (UCD)

Ukwelashwa okuyinhloko kwe-UCD ukususa ngokuhlinzwa ama-lymph node athintekile. Ngezinye izikhathi ungadinga ukwelashwa ngemisebe noma i-immunotherapy ngaphambi kokuhlinzwa. Lokhu kwelashwa kunciphisa ukukhula kwama-lymph node, okwenza kube lula ukuwasusa.

Kodwa uma odokotela abahlinzayo bengakwazi ukususa i-lymph node ethintekile, futhi wena ungenazo izimpawu, udokotela wakho angase ayiqaphe esikhundleni sokuyelapha. Uma unezimpawu futhi ungakwazi ukuhlinzwa, bangase batuse ukwelashwa kwe-MCD.

Ukwelashwa kwesifo se-Castleman esihlanganisa izingxenye eziningi (i-MCD)

I-MCD inzima kakhulu ukwelapha kune-UCD. Ngenxa yokuthi isakazeka emzimbeni wonke, ukuhlinzwa noma ukwelashwa ngemisebe akuvamile ukusetshenziswa. Kunalokho, ukwelashwa kuncike ekutheni unegciwane le-HHV-8 nokuthi isifo sibi kangakanani.

Udokotela wakho angasebenzisa izindlela zokwelapha ezifana nalezi:

• Ama-Corticosteroids: Le mithi isiza ekunciphiseni ukuvuvukala futhi inikeze impumuzo ezimpawini.
• Imithi yokwelapha ngamakhemikhali: Imithi yokwelapha ngamakhemikhali ilawula ukukhula ngokweqile kwamaseli ohlelweni lwakho lwe-lymphatic. I-Rituximab umuthi ovame ukunqunyelwa i-HHV-8-associated MCD.
• Ukwelashwa Ngezifo Ezingalawuleki: Lokhu kwelashwa kuhilela ukusebenzisa izinto ezifana nama-antibodies e-monoclonal . Lokhu kusiza ukuzola impendulo yomzimba wakho. I-Siltuximab (Sylvant®) ukuphela kokwelashwa okuvunyelwe yi-U.S. Food and Drug Administration (FDA) ye-iMCD.
• Imithi yokulwa namagciwane: Uma une-MCD ehlobene ne-HHV-8, ungase udinge imithi yokulwa namagciwane yokwelapha i-HIV noma ukutheleleka kwe-HHV-8.

Kufanele ngimbone nini udokotela?

Uma ubona iqhubu noma ukukhula entanyeni yakho, emakhwapheni, noma embotsheni, bona udokotela ngokushesha . Futhi, uma unezinye izimpawu ze-Castleman Disease okukhulunywe ngazo ngenhla ezingathuthuki ngemva kwamasonto ambalwa, tshela udokotela wakho ngazo.

Ungaphila isikhathi esingakanani nalesi sifo?

Kubantu abaningi abane-UCD, isibikezelo, noma umbono, muhle kakhulu. Ukususa ama-lymph node athintekile kungelapha lesi sifo. Ngokwelashwa, i-UCD ngokuvamile ayithinti isikhathi sokuphila.

Kodwa umbono wabantu abane-MCD uncike ohlotsheni lwe-MCD abanayo kanye nobunzima besifo. Kwabanye abantu, izimpawu zingavela ngokuzumayo futhi zibe yingozi empilweni. Kwabanye, izimpawu zingahlala isikhathi eside. Phakathi kuka-65% no-75% wabantu abatholakale bene-MCD basaphila ngemva kweminyaka emihlanu . Ngokwelashwa okusha, kunethemba lokuthi lesi simo sizothuthuka.

Azikho izimpendulo ezilula uma kukhulunywa ngesifo sikaCastleman. Okuhlangenwe nakho kwakho kuzoncika ohlotsheni lwesifo sikaCastleman onaso. Izimo ezengeziwe, njenge-HIV, noma izimo ezihlobene, njenge-TAFRO kanye ne-POEMS, nazo zingathinta okuhlangenwe nakho kwakho. Kwezinye izimo, ukuhlinzwa kanye nokubhekisisa kahle kungaba yikho kuphela okudingekayo. Noma, ungadinga ukwelashwa okuhlukahlukene kuyo yonke impilo yakho ukuze uvimbele izinkinga. Buza udokotela wakho ukuthi ukuxilongwa kwakho kuzothinta kanjani ukwelashwa kwakho kanye nombono wakho.

Ngakho-ke, yiziphi izinto ezibaluleke kakhulu okudingeka sizikhumbule kulokhu?

• Isifo sikaCastleman siyisifo esingavamile kodwa esingaba yingozi kakhulu lapho amasosha omzimba ethu eba matasa kakhulu.
• Kunezinhlobo ezimbili eziyinhloko: i-UCD kanye ne-MCD. Ku-UCD, ama-lymph node avuvukala endaweni eyodwa, kanti ku-MCD, avuvukala ezindaweni eziningi.
• Izimpawu ziyahlukahluka. Ngezinye izikhathi kungase kungabi nazimpawu nhlobo, kumane nje kube yiqhubu elivuvukile. Noma kungase kube nezimpawu ezifana nomkhuhlane, ukukhathala, kanye nokwehla kwesisindo.
• Izimbangela azicaci ngaso sonke isikhathi, kodwa igciwane le-HHV-8 liye lahlotshaniswa ne-MCD. Abantu abane-HIV basengozini enkulu.
• Ukutholwa kusenesikhathi kanye nokwelashwa okufanele kuvame ukuholela emiphumeleni emihle. Ngakho-ke uma ubona iqhubu elingavamile, ukuvuvukala, noma ezinye izimpawu ezingavamile emzimbeni wakho, funa iseluleko sezokwelapha ngokushesha. Into ebaluleke kakhulu ukuhlala unolwazi futhi ungesabi.

Isifo sikaCastleman , Ama-Lymph Node, i-Lymphadenopathy, i-Immune System, i-UCD, i-MCD, i-HHV-8, Izimpawu, Ukwelashwa