Oko kufuneka ukwazi ngesifo sikaCastleman

Ngaba wakha waba neqhuma, esilibiza ngokuba "yiqhuma," entanyeni yakho, ekhwapheni, okanye emqolo? Ngamanye amaxesha, amaqhuma anje ayadumba xa unesifo somkhuhlane okanye uhlobo oluthile losulelo. Yinto eqhelekileyo leyo. Kodwa ukuba unamaqhuma anje avuvukileyo ngaphandle kwesizathu, ixesha elide, kwaye uzive udiniwe kwaye wehla emzimbeni, kufuneka ukhathazeke kancinci. Namhlanje, siza kuthetha ngesimo esingaqhelekanga esinokukrokrelwa ngamaxesha anjalo. Yiyo loo nto iCastleman Disease.

Ngamafutshane, yintoni iCastleman Disease?

Khawucinge nje ukuba imizimba yethu ifana nelizwe. Ngoko ke inkqubo yethu yokuzikhusela komzimba ngumkhosi okhusela elo lizwe. Xa intsholongwane (utshaba) ingena emzimbeni isuka ngaphandle, lo mkhosi uya edabini uze woyise olo tshaba. Xa idabi liphelile, umkhosi ubuyela enkampini uze uthule. Oku kwenzeka emzimbeni womntu ophilileyo.

Kodwa kumntu onesifo sikaCastleman, kwahlukile. Okwenzekayo apha kukuba umkhosi owaya emfazweni, inkqubo yomzimba yokuzikhusela, awuzoli nasemva kokuba imfazwe iphelile. Bahlala besebenza. Kufana nokuba bahlala belungele imfazwe. Oku kubangela ukudumba okuhlala ixesha elide emzimbeni. Oku kungonakalisa amalungu ethu omzimba.

Okwangoku, ii-lymph nodes zethu, ezo zinto zincinci, zifana neziseko zomkhosi. Zenza umsebenzi omninzi, njengokucoca iintsholongwane nokugcina iiseli zomzimba. Ngoku, ngenxa yokuba inkqubo yomzimba yokuzikhusela isebenza rhoqo, ezi lymph nodes nazo zisebenza rhoqo. Ngenxa yoko, iiseli ezikuzo ziqala ukukhula kakhulu. Yiyo loo nto ii-lymph nodes zidumba kwisifo sikaCastleman. Asikuko ukudumba nje kuphela, kodwa sibangela notshintsho kwizicubu zezo lymph nodes.

Ziziphi iintlobo eziphambili zesifo sikaCastleman?

Isifo sikaCasselman sahlulwe saba ziintlobo ezimbini eziphambili. Iimpawu kunye nonyango lwaso ngasinye zahlukile kakhulu. Ke ngoko, kubalulekile ukuqonda ezi ntlobo zimbini ngokuchanekileyo.

Ngaba kukho iintlobo ngeentlobo zesifo seCastleman esibizwa ngokuba yi-multicentric (MCD)?

Ewe, i-MCD yahlulwe ngakumbi yaba ziintlobo ngeentlobo ezahlukeneyo. Olu hlu lwenziwa ngokusekelwe kwisizathu kunye nezinye iimeko ezenzeka ngayo.

• I-MCD enxulumene neeMBONGO: IMBONGO sisifo segazi esingaqhelekanga. Ngamanye amaxesha i-MCD inokwenzeka kunye neeMBONGO.
• I-HHV-8-associated MCD: Oku kubangelwa kusulelo lwentsholongwane ebizwa ngokuba yiHuman Herpes Virus-8 (HHV-8). Olu hlobo luxhaphake kakhulu kubantu abosulelwe yiHIV okanye abo bangenawo amasosha omzimba ngenxa yezinye izizathu.
• Idiopathic MCD (iMCD): Igama lezonyango elithi " idiopathic " lithetha "akukho sizathu sifunyenweyo." Uninzi lwezigulane ze-MCD zinolu hlobo. Oku kuthetha ukuba unobangela ochanekileyo awukaziwa okwangoku.

Olu hlobo lwe-iMCD lwahlulwe ngakumbi kwiindidi ezintathu ezincinci:

1. iMCD enxulunyaniswa neTAFRO: I-TAFRO ligama elivela kudibaniso lweempawu ezahlukeneyo. Oko kukuthi, iThrombocytopenia (iiplatelets ezisezantsi egazini), i-Anasarca (ukudumba komzimba ngamanzi), iFever (umkhuhlane), ukungasebenzi kakuhle kwezintso (ukuba buthathaka komsebenzi wezintso) kunye ne-Organomegaly (ukwanda kwesibindi okanye ipleen) ziimpawu ezinokubonakala koku.

2. iMCD ene-idiopathic plasmacytic lymphadenopathy (iMCD-IPL): Kolu hlobo, inani leeplatelet lisenokuphakama. Kusenokubakho nokuveliswa kakhulu kwee-antibodies ziiseli ezimhlophe zegazi.

3. iMCD, engachazwanga ngenye indlela (iMCD-NOS): Izigulane ezine-iMCD ezinganxulumananga ne-TAFRO kwaye ezingafumanekiyo esinye isizathu esithile ziwela kolu didi.

Zingaba yintoni iimpawu zesi sifo?

Iimpawu ziyahluka ngokuxhomekeke kuhlobo lwesifo sikaCastleman onaso.

Isifo seCasselman esingaqhelekanga (UCD) asisoloko sinazo iimpawu . Uphawu kuphela yi-lymph node evuvukileyo (i-adenoid). Ngamanye amaxesha, iimpawu (umz., iintlungu, ukuphefumla kancinci) zenzeka kuphela xa i-node evuvukileyo icinezela elinye ilungu lomzimba.

Nangona kunjalo, kwisifo seCastleman esibizwa ngokuba yi-multicentric (MCD), iimpawu zibonakala ngakumbi. Ukongeza kwiindlala ezidumbileyo, unokufumana oku kulandelayo:

• Umkhuhlane oqhelekileyo
• Ukuziva udiniwe kakhulu kwaye udiniwe ngaphandle kwesizathu (oku kusenokuba kungenxa ye-anemia)
• Ukubila kakhulu ebusuku
• Isicaphucaphu nokuhlanza
• Ukunciphisa umzimba ngokungalindelekanga
• Ukudumba kwemilenze, amaqatha, okanye isisu
• I-spleen ekhulisiweyo (i-splenomegaly) okanye isibindi esikhulisiweyo (i-hepatomegaly)
• Ukuba ndindisholo ezandleni nasezinyaweni (peripheral neuropathy)

Kutheni esi sifo senzeka? Ngaba kukho izinto ezinokubangela umngcipheko?

Enyanisweni, izazinzulu azikafumani unobangela othile wesifo seCastleman esingaphakathi (UCD) .

Isifo se-Multicentric Castleman (MCD) sinxulunyaniswa nentsholongwane ye-HHV-8 ekhankanyiweyo ngaphambili. Nangona kunjalo, abaphandi basaphanda ukuba ezinye izifo, utshintsho lwezakhi zofuzo, okanye iimpendulo ze-autoimmune zinokuba negalelo kuhlobo olungaziwayo lwe-iMCD.

Xa kufikwa kwizinto ezinobungozi, akukho zinto zibalulekileyo ezibangela umngcipheko kwi-UCD nakwi-iMCD. Nangona kunjalo, ukuba une-HIV okanye enye inkqubo yomzimba yokuzikhusela ebuthathaka, usemngciphekweni omkhulu wokufumana i-HHV-8-associated MCD.

Nangona esi sifo sinokukhula nakubantu banoma luphi na ubudala, sixhaphake kakhulu kubantu abaphakathi kweminyaka engama-30 ukuya kwengama-60 ubudala .

Ziziphi iingxaki ezinokubakho zesi sifo?

Isifo sikaCastleman sinokunyusa kancinci umngcipheko wakho wokuba nezinye izifo, ingakumbi umhlaza , njenge- lymphoma, umhlaza wenkqubo ye-lymphatic.

Ukongeza, abantu abane-UCD basengozini yokufumana imeko yesikhumba engaqhelekanga kodwa enzulu ebizwa ngokuba yi-paraneoplastic pemphigus (PNP). Abantu abane-MCD basengozini enkulu yokufumana usulelo olwahlukeneyo. Ukuba alunyangwa, olu sulelo lunokubangela umonakalo kumalungu omzimba kwanokufa.

Kodwa ungakhathazeki, ugqirha wakho uya kuhlala ejonga le meko aze athathe amanyathelo afunekayo ukunceda ukuthintela ezi ngxaki kangangoko kunokwenzeka.

Ugqirha uluchonga njani ngokuchanekileyo olu sifo?

Ngenxa yokuba iimpawu zesifo sikaCastleman zifana nezezinye izifo, ezifana nomkhuhlane oqhelekileyo okanye umkhuhlane, ugqirha uya kuqala ahlole izifo eziqhelekileyo. Kulapho ke baya kuthi bakrokre ukuba sisifo sikaCastleman baze benze uvavanyo oluthile.

Olu vavanyo lulandelayo lwenziwa ikakhulu:

• Uvavanyo lwelebhu: Igazi lakho liza kuhlolwa phantsi kwe-microscope ukujonga ukungalingani kwinani leeseli zakho zegazi kunye nezinye izinto ezingaqhelekanga. Ukuba kurhanelwa ukuba kukho i-HHV-8-associated MCD, uvavanyo lwe-HIV nalo lunokwenziwa.
• Uvavanyo lwemifanekiso: Ii-CT scans kunye nee-PET scans zinokunceda ekufumaneni apho ii-lymph nodes ezivuvukileyo zikhoyo emzimbeni. Zinokujonga nokwanda kwamalungu omzimba anjengesibindi kunye ne-spleen.
• I-lymph node biopsy: Le yindlela ekuphela kwayo necacileyo yokuqinisekisa isifo . Oku kuquka ukuthatha iqhekeza elincinci kakhulu lesicwili kwi-lymph node evuvukileyo uze ulihlolisise phantsi kwe-microscope. Oku kunokubonisa ngokuqinisekileyo ukuba kukho naluphi na utshintsho oluthile kwiseli olunxulumene nesifo sikaCasselman.

Zithini iindlela zonyango zoku?

Iindlela zonyango ziyahluka ngokupheleleyo kuxhomekeke kuhlobo lwesifo.

Unyango lweSifo seCasselman esiNye (UCD)

Unyango oluphambili noluphumelele kakhulu lwe-UCD kukususwa kwe-lymph node echaphazelekayo ngotyando . Kwiimeko ezininzi, esi sifo siyanyangeka ngokupheleleyo emva kolu tyando.

Ngamanye amaxesha, ukuba ithumba likhulu, unyango lwemitha okanye i-immunotherapy lunikwa ngaphambi kotyando ukuze lincitshiswe kwaye kube lula ukulisusa.

Ukuba iqhuma likwindawo engenakususwa ngotyando, kwaye awunazo iimpawu, ugqirha wakho unokugqiba ekubeni angalinyangi kodwa alijonge nje. Nangona kunjalo, kumntu ongengomntu oza kutyandwa kodwa oseneempawu, unyango olufanayo nolwe-MCD lunokunikwa.

Unyango lwesifo seCasselman esineenkalo ezininzi (MCD)

Ngenxa yokuba i-MCD yimeko esasazeka kwiindawo ezininzi zomzimba, ayinakunyangwa ngotyando okanye ngonyango lwemitha. Ke ngoko, kusetyenziswa ezinye iindlela zonyango. Unyango luxhomekeke ekubeni unayo na i-HHV-8 kunye nokuba isifo sibi kangakanani.

Olu nyango lulandelayo lunikezelwa ikakhulu:

• IiCorticosteroids: La mayeza anceda ukulawula iimpawu ngokunciphisa ukudumba emzimbeni.
• Amayeza e-chemotherapy: La mayeza asebenza ngokulawula isantya apho iiseli kwinkqubo ye-lymphatic zikhula ngokukhawuleza kakhulu. I-Rituximab isetyenziswa kakhulu kwi-HHV-8-associated MCD.
• Unyango lwe-Immunotherapy: Olu nyango lusebenza ngokuzolisa inkqubo yomzimba wakho yokuzikhusela esebenza kakhulu. I-Siltuximab (Sylvant®) yeyona yodwa iyeza elivunyiweyo kwi-iMCD.
• Amayeza okulwa neentsholongwane: Ukuba une-HHV-8-associated MCD, unganikwa la mayeza okulawula usulelo lwe-HIV okanye lwe-HHV-8.

Ndifanele ndibonane nogqirha nini?

Ukuba ubona iqhuma okanye iqhuma entanyeni, ekhwapheni, okanye emqolo onokuthi ulive, qiniseka ukuba uxelele ugqirha wakho ngalo.

Kwakhona, ukuba iimpawu ezikhankanywe kweli nqaku, ezinje ngomkhuhlane ongachazwanga, ukwehla kobunzima, kunye nokudinwa kakhulu, zihlala iiveki ezininzi, qiniseka ukuba ufuna ingcebiso kagqirha.

Ngaba kunokwenzeka ukuphila nesi sifo?

Impendulo yalo mbuzo ixhomekeke kuhlobo lweCasselman onayo.

Umntu one-UCD unokulindela iziphumo ezilungileyo kakhulu . Xa ithumba lisusiwe ngotyando, isifo sidla ngokunyangwa ngokupheleleyo. Emva konyango, oku akuyi kuchaphazela ubomi bakho obuqhelekileyo.

Ingqikelelo yabantu abane-MCD iyinkimbinkimbi kancinci . Iyahluka ngokuxhomekeke kuhlobo kunye nobunzima besifo. Kwabanye, iimpawu zinokuba nzima ngequbuliso kwaye zibeke ubomi esichengeni. Kwabanye, iimpawu zinokuhlala ixesha elide, kodwa kukho ithemba. Phakathi kwama-65% kunye nama-75% abantu abafunyaniswe bene-MCD baphila iminyaka engaphezu kwemihlanu . Oku kuyaphucuka ngonyango olutsha.

Akukho mpendulo ilula, ehambelana nayo yonke into kwisifo sikaCasselman. Kuya kuxhomekeka kumava akho, uhlobo lwesifo onaso, nokuba unezinye izifo, ezifana ne-HIV, i-TAFRO, okanye ii-POEMS. Ngamanye amaxesha utyando kunye nokuhlolwa rhoqo kwezonyango kuya kwanele. Okanye unokufuna unyango oluninzi ebomini bakho bonke. Ngoko ke kungcono ukuthetha ngokukhululekileyo nogqirha wakho malunga nemeko yakho, unyango lwakho, kunye nekamva lakho.