Iyini i-Evans Syndrome? Ake sixoxe ngayo!

Ingabe ngezinye izikhathi uzizwa ukhathele? Noma ingabe unenkinga yokuyeka ukopha ngisho nasezintweni ezincane kakhulu? Mhlawumbe kungenxa yokuthi amasosha omzimba wakho asebenza ngokumelene nawe. Namuhla sizokhuluma ngesimo esingavamile kodwa esibaluleke kakhulu okufanele sazi ngaso. Leso yi -Evans Syndrome . Ungakhathazeki, sizoxoxa ngakho konke ngamagama alula.

Ngakho-ke, iyini i-Evans Syndrome?

Kalula nje, i-Evans Syndrome iyisifo esibangelwa ukuzivikela komzimba . Okwenzekayo ukuthi amasosha okufanele avikele imizimba yethu, amasosha omzimba, aqala ngephutha ukubhubhisa amanye amangqamuzana ethu egazi. Kufana nebutho lethu lihlasela abantu bakithi. Ngenxa yalokho, imizimba yethu ilahlekelwa inani elidingekayo lamangqamuzana egazi. Lokhu kwehla okungavamile kwamangqamuzana egazi yilokho esikubiza ngokuthi i-cytopenia kwezokwelapha.

Iningi labantu abane-Evans Syndrome liqala ngokuthintwa amaseli abomvu egazi aphansi (isimo esibizwa ngokuthi i-anemia ) kanye nama-platelet (isimo esibizwa ngokuthi i-thrombocytopenia ). Ngaphezu kwakho kokubili, abanye abantu bangase babe nenani eliphansi lohlobo lwamaseli amhlophe egazi abizwa ngokuthi ama-neutrophils (isimo esibizwa ngokuthi i-neutropenia ).

Uma lezi zinhlobo zamaseli egazi ziphansi, izinto eziningi ezibalulekile emzimbeni wethu, njengokuthola umoya-mpilo, ukujiya kwegazi, kanye nokulwa nezifo, ziyathinteka. Yingakho sizizwa sibuthakathaka, sikhathele, siqala ukopha kalula, futhi sigula kaningi .

Lesi simo singathinta abanye abantu kancane kakhulu, kanti abanye bangaba nzima kakhulu. Futhi imiphumela yaso ayihlali isikhathi esifanayo impilo yonke. Into engcono kakhulu ukuthi yize kungekho ukwelashwa kwaso, ukwelashwa kungasiza ekulawuleni izimpawu futhi kunciphise imiphumela .

Ziyini izimpawu ze-Evans Syndrome?

Izimpawu ze-Evans Syndrome zingahluka kakhulu. Kuya ngokuthi hlobo luni lwe-cytopenia onalo nokuthi lubi kangakanani. Ngokuvamile, i-anemia noma i-thrombocytopenia iqala kuqala, kanti ezinye zivele kamuva.

Izimpawu zokuqala zokuntuleka kwamangqamuzana abomvu egazi ( i-anemia ):

Ingabe ukhathele ngisho nangemva kokuhamba ibanga elifushane? Ingabe uzizwa ukhathele futhi ungakwazi ukwenza izinto obuzenza? Mhlawumbe ubuso bakho bubukeka buphaphathekile kancane. Lezi ezinye zezimpawu zokuqala ze -anemia :

• Ukuzizwa nje ukhathele kakhulu.
• Isikhumba esikhanyayo (ubumthubi) .
• Ukungaqondi kahle noma isiyezi .
• Ubunzima bokuphefumula (ukuphelelwa umoya) .
• Ukushaya kwenhliziyo okusheshayo (i-tachycardia) .
• Ukuzwa noma ukuzizwa sengathi inhliziyo yakho ishaya ngendlela engavamile (ukushaya kwenhliziyo) .

Izimpawu zokuqala zokuntuleka kwama-platelet (thrombocytopenia):

Uke waphawula ukuthi ngezinye izikhathi kuvela amachashazi amancane abomvu esikhumbeni sakho? Noma umane uthole imihuzuko emzimbeni wakho wonke? Ingabe uzizwa sengathi izinsini zakho ziphuma igazi kakhulu kunokujwayelekile uma uxubha amazinyo akho? Lezi yizimpawu ezingaba khona zama-platelet aphansi:

• Amabala amancane abomvu okopha (i-petechiae) ngaphansi kwesikhumba.
• Amabala ansundu esikhumbeni (purpura) .
• Ukuphuka (ecchymosis) okwenzeka ngaphandle kwesizathu.
• Ukopha ngokweqile okuvela ezinsinini noma ekhaleni.
• Ukopha okukhulu ngesikhathi sokuya esikhathini kwabesifazane.
• Ukopha ngendle noma ngomchamo.

Izimpawu ze-neutropenia (lezi zingase zenzeke noma zingabi khona kamuva):

Kuvamile ukuthola umkhuhlane owodwa noma emibili ngenyanga. Kodwa ingabe uvame ukugula ngenxa yomkhuhlane? Ingabe uthola izilonda emlonyeni ngaso sonke isikhathi? Ingabe zibonakala zithatha isikhathi eside ukuphola? Nazi ezinye izinto ezingenzeka uma ama-neutrophil akho ephansi:

• Imfiva.
• Izilonda zomlomo.
• Umkhuhlane ovamile, umkhuhlane, kanye nobuhlungu besisu.
• Ukutheleleka kwendlebe okuphindaphindiwe.
• Ukutheleleka kwesikhunta isikhathi eside.
• Ukutheleleka komgudu womchamo (UTIs) .

Ezinye izimpawu ze-Evans Syndrome (lezi zingase zenzeke ngezikhathi ezithile):

• Ama-lymph node avuvukele entanyeni.
• Ubende obukhulu .
• Isibindi esikhulu .
• I-Jaundice .

Kungani kwenzeka i-Evans Syndrome?

Njengoba sishilo ngaphambili, i-Evans Syndrome iyisifo esibangelwa ukuzivikela komzimba . Lokhu kusho ukuthi amasosha omzimba wethu aqaphela ngephutha amanye amangqamuzana egazi ethu "njengesitha" bese eqala ukuwahlasela.

Lo msebenzi wenziwa amaseli e-B, uhlobo olukhethekile lweseli ohlelweni lwethu lokuzivikela komzimba. Lawa maseli e-B enza okuthile okubizwa ngokuthi ama-antibodies . Lawa ma-antibodies ayahamba ayobamba lawo maseli egazi angenacala bese ewabhubhisa. Ngokuvamile, aqala ukuhlasela uhlobo olulodwa lweseli legazi kuqala, bese eqala ukuhlasela ezinye izinhlobo zamaseli egazi.

Le nqubo yiyona egcina ikwenza ube nokuntuleka okungenani kwezinhlobo ezimbili zamaseli egazi (i-cytopenia), futhi ngezinye izikhathi zonke ezintathu. Ukuntuleka kuzo zonke ezintathu kubizwa ngokuthi i-pancytopenia . Izimo ezintathu eziyinhloko lapho la maseli egazi ephansi khona yilezi:

• I-Autoimmune hemolytic anemia (AIHA): Yilapho amangqamuzana abomvu egazi encipha khona. Okusho ukuthi, abhujiswa ngokushesha kunokuba umzimba ungawenza.
• I-immune thrombocytopenia (ITP) : Lesi yisimo lapho kuba nokwehla kwama-platelet. Amasosha omzimba ahlasela futhi asuse ama-platelet egazini.
• I-Autoimmune neutropenia (AIN) : Lesi yisimo lapho inani lama-neutrophil, uhlobo lweseli elimhlophe legazi, lehla khona. Isimiso somzimba sokuzivikela sihlasela futhi sibhubhise ama-neutrophil.

Cabanga ngakho, kungaba nezizathu eziningi zokubalwa kwamaseli egazi aphansi (i-cytopenia). Ngezinye izikhathi kungaba ngoba umnkantsha wethambo awukhiqizi amaseli egazi anele. Kodwa-ke, ku-cytopenia yokuzivikela komzimba, amasosha omzimba abhubhisa amaseli awo egazi.

Yiziphi izimbangela ze-Evans Syndrome?

Ososayensi abakakazi kahle ukuthi kungani lezi zifo zokuzivikela komzimba zikhula. Kodwa baqaphele ukuthi lezi zifo zivame ukuqala ngemva kokugula okubi noma isenzakalo esibeka ingcindezi enkulu ohlelweni lokuzivikela komzimba. Lokhu kubizwa ngokuthi yizimbangela .

Futhi, uma unesifo esisodwa sokuzivikela komzimba, ngokwezibalo kungenzeka kakhulu ukuthi ube nesinye. Kunjengokungathi lesi sifo sokuzivikela komzimba sisakazeka sisuka kwenye ingxenye yomzimba siye kwenye.

I-Evans syndrome ingazenzekela yodwa (lokhu kubizwa ngokuthi i-primary Evans syndrome ), noma ingenzeka njengesimo sesibili esihlotshaniswa nesinye isimo (lokhu kubizwa ngokuthi i-secondary Evans syndrome) . Ezinye zezimo ezingahlotshaniswa ngale ndlela yilezi:

• I-systemic lupus erythematosus (lupus)
• Ukuntuleka kwamasosha omzimba okuvamile
• Ukuntuleka kwe-IgA ekhethiwe
• Isifo sikaSjögren
• I-Non-Hodgkin lymphoma
• I-leukemia ye-lymphocytic engapheli
• I-hepatitis C engapheli
• Ukutheleleka nge-HIV okungapheli

Uma une-primary Evans syndrome, kungenzeka ukuthi yaqala ngemva kokugula kwesikhashana, noma ungase ungabi naso isizathu esicacile. Ososayensi bahlukanisa i-Evans syndrome njengesimo esingajwayelekile . Lokhu kusho ukuthi akukho mbangela eqondile etholakele.

Yiziphi izinkinga ezingaba khona ngenxa ye-Evans Syndrome?

Akuwona wonke umuntu one-Evans Syndrome ozoba nezinkinga. Abanye abantu banezimpawu ezincane kakhulu. Kodwa-ke, ezimweni ezimbi kakhulu, lokhu kuntuleka kwamangqamuzana egazi (cytopenias) kungaba yingozi empilweni. Isibonelo:

• Uma kungekho amangqamuzana abomvu egazi anele ukuthwala umoya-mpilo emzimbeni, akukho umoya-mpilo owanele egazini (i-hypoxemia) . Lokhu kunzima kakhulu enhliziyweni futhi kungaholela ezifweni zenhliziyo kanye nokwehluleka kwenhliziyo.
• Uma ungenawo ama-platelet anele , asiza ukujiya kwegazi lakho, igazi lakho alijiyi kahle. Lokhu kungabangela ukuthi wophe kalula futhi ngokushesha. Lokhu kungaholela ngokushesha ekulahlekelweni kwegazi okukhulu.
• Uma umzimba wakho ulahlekelwa ama-neutrophil , asiza ekulweni nokutheleleka, unamathuba amaningi okuthola izifo kaningi, futhi zingaba zimbi kakhulu futhi zisakazeke emzimbeni wakho wonke. Lokhu kungaholela esimweni esiyingozi esibizwa ngokuthi i-sepsis .

Odokotela bayixilonga kanjani i-Evans Syndrome?

I-Evans Syndrome itholakala ngokuhlolwa kwegazi . Ingatholakala uma uba nezimpawu ze-cytopenia, noma ngesikhathi sokubalwa kwamaseli egazi okwenziwa ngesikhathi sokuhlolwa kwempilo okuvamile.

Uma i-Complete Blood Count yakho (CBC) ikhombisa ukuthi unamaseli abomvu egazi aphansi, ama-platelet, noma ama-neutrophil, ithimba lakho lezokwelapha lizokwenza izivivinyo ezengeziwe. Isibonelo, bazohlola igazi lakho ukuze babone amazinga aphezulu ama-antibodies ahlobene ne-Evans Syndrome. Bazohlola nezinye izimo ezingabangela i-cytopenia, njengokutheleleka okuthile noma umdlavuza.

Odokotela baqinisekisa i-Evans Syndrome ngokukhipha zonke ezinye izimbangela. Ngezinye izikhathi, kungadingeka benze i -CT scan (i-computed tomography scan) noma i-bone marrow biopsy ukuze kutholakale ezinye izimo.

Ingabe ikhona ikhambi eliphelele le-Evans Syndrome? Ilashwa kanjani?

Eqinisweni, ayikho ikhambi eliphelele le-Evans Syndrome, noma esinye isifo sokuzivikela komzimba. Odokotela belapha lezi zifo zokuzivikela komzimba ngokukunikeza imithi enciphisa ukusebenza kwesimiso sakho sokuzivikela komzimba.

Ngaphezu kwalokho, izimpawu zakho zingase zelashwe ngokwehlukana. Isibonelo, uma inani lamaseli egazi lakho liphansi kakhulu, udokotela wakho angase akwazi ukulungisa lokhu ngokumpontshelwa igazi ngezikhathi ezithile.

Njengezifo eziningi zesikhathi eside zokuzivikela komzimba, i-Evans Syndrome ingaba ngcono ngokuhamba kwesikhathi. Ngezinye izikhathi izimpawu zingase zibonakale ziphela (ezibizwa ngokuthi ukuthethelelwa) , kodwa bese zingase zibuye noma zibe zimbi kakhulu (ezibizwa ngokuthi ukuphinda ubuye ).

Kungase kudingeke uzame izindlela zokwelapha ezahlukene empilweni yakho yonke, njengoba impumelelo yazo ingahluka ngokuhamba kwesikhathi. Udokotela wakho uzozama ukukuvikela ezinkingeni ezinkulu kakhulu ze-Evans Syndrome.

Yiziphi izindlela zokwelapha?

Ukwelashwa okujwayelekile kwe-Evans Syndrome imithi yokucindezela amasosha omzimba , futhi uma kudingeka, ukumpontshelwa igazi. Lawa ma-immunosuppressants asebenza ngokunciphisa ukuhlaselwa okungalungile yisimiso sakho somzimba sokuzivikela.

Izinhlobo eziyinhloko zemithi ezinikezwayo:

• Ama-Corticosteroids , njenge -prednisone . Lokhu ngokuvamile ukwelashwa kokuqala okunikezwayo.
• Ukwelashwa nge-intravenous immunoglobulin (IVIg) kuhilela ukunikeza ikhambi eliqukethe ama-antibodies abantu ngomthambo. Lawa ma-antibodies amasha angabopha kuma-antibodies azimele anephutha futhi awakhubaze.

Uma ungasabeli kahle ekwelashweni okuvamile, udokotela wakho angase azame ezinye izindlela zokwelapha. Lokhu kufaka phakathi:

• Ama-antibodies e-monoclonal , njenge -rituximab . Lawa angama-antibodies enziwa elebhu. Ngezinye izikhathi lokhu kungamisa impendulo ye-autoimmune. Udokotela wakho angase akuphakamise lokhu uma eminye imithi ingasebenzi.
• Ukususwa kwe-spleenectomy . Ezimweni ezimbi kakhulu, udokotela wakho angase ancome ukuhlinzwa ukuze kususwe i-spleen yakho. I-spleen yakho yilapho kususwa khona amaseli egazi amadala. Ngakho-ke lapho i-spleen isuswa, inqubo iyancipha kancane.

Ingabe kukhona izinkinga noma imiphumela emibi yokwelashwa?

Uma uthatha imithi yokucindezela amasosha omzimba, impendulo yamasosha omzimba wakho esifweni sakho sokuzivikela komzimba iyancipha. Kodwa-ke, amasosha omzimba wakho ezifweni nawo ayancipha . Ngakho kuzodingeka uthathe izinyathelo zokuphepha ezikhethekile ukuze ugweme ukugula.

Udokotela wakho angase ancome eminye imijovo yokugoma ngaphezu kwaleyo osuyitholile kakade. Uma amasosha omzimba wakho ebuthakathaka (engavikelekile), ungase ube sengozini enkulu yokutheleleka yizifo obungeke uzithole ngokuvamile.

Ingabe ukuhlala ne-Evans Syndrome kuthinta impilo yakho yonke?

I-Evans Syndrome ayithinti ngempela isikhathi sakho sokuphila. Abanye abantu banezimpawu ezincane kakhulu, ezingavamile. Kodwa-ke, ezinye izinkinga, njengokuphuma kwegazi, isifo senhliziyo, kanye nokutheleleka, zingaba yingozi empilweni.

Uma une-secondary Evans syndrome, okusho ukuthi unayo nesinye isifo esingamahlalakhona, lokho kungathinta nokuphila kwakho. Ungase ube nezinkinga ezisongela ukuphila ezihambisana nesinye isimo sakho.

Uyini umbono womuntu one-Evans Syndrome?

Okuhlangenwe nakho kwabantu abane-Evans Syndrome kuyahlukahluka kakhulu. Izimpawu zingaba zincane noma zibe zimbi kakhulu. Abanye abantu bazizwa sengathi izimpawu zabo ziyaphela ngokuphelele (ukuxolelwa). Abanye baphinde bazizwe kamuva (ukubuyela emuva).

Abantu abaningi badinga uhlobo oluthile lokwelashwa kanye nokuhlolwa njalo kwezokwelapha. Ukwelashwa kuvame ukusebenza kahle, kodwa hhayi njalo. Ngezinye izikhathi, ukusebenza kahle kokwelashwa kungase kubonakale kuncipha ngokuhamba kwesikhathi. Kungase kudingeke uzame ezinye izindlela zokwelapha.

Ngizinakekela kanjani ngesikhathi ngiphila ne-Evans Syndrome?

Uma une-Evans Syndrome, kubalulekile ukuqhubeka nokuhlolwa kwakho njalo kwezokwelapha . Ngisho noma uzizwa uphilile, udokotela wakho uzofuna ukuqapha isimo sakho futhi asithole kusenesikhathi uma siba sibi kakhulu.

Futhi, kufanele uziqaphele njalo futhi uqaphele noma yiziphi izimpawu ezintsha. Uma unomkhuhlane ongapholi, noma uma unesifo sokopha esingalawuleki, shayela udokotela wakho ngokushesha .

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Ungase ufune ukubuza udokotela wakho imibuzo efana nale:

• Yiziphi izinhlobo zamaseli egazi engintula zona?
• Zinciphe kangakanani?
• Yiziphi izimpawu noma izimpawu eziyisixwayiso okufanele ngiziqaphele?
• Ngingazi kanjani ukuthi ukwelashwa kuyasebenza noma cha?
• Kwenzekani uma ukwelashwa kungasebenzi?
• Kufanele ngibhukhe nini isikhathi sami esilandelayo sokubonana nodokotela?

I-Evans Syndrome yisimo esingavamile okungenzeka ukuthi awukaze uzwe ngaso ngaphambili. Izimpawu zingaba yinqaba futhi ziphazamise. Ukuqonda ukuthi kwenzekani nokuthi kusho ukuthini kungaba inqubo eyinkimbinkimbi.

Nakuba i-Evans Syndrome iyisimo sokuphila konke, ayifani ngaso sonke isikhathi. Gcina ithemba lokuthi uzozizwa ungcono, okungenani ngezinye izikhathi. Kungakhathaliseki ukuthi uzizwa kanjani, ithimba lakho lezokwelapha lizobe likhona ukukunikeza ukunakekelwa okudingayo.

Umyalezo Wokugcina Wokuya Nasekhaya

Kulungile, ngakho ake sikukhumbuze ezinye zezinto ezibaluleke kakhulu esixoxe ngazo.

• Iyini i-Evans Syndrome?Isifo esingavamile lapho amasosha omzimba wakho ehlasela khona amangqamuzana akho egazi.
• Lokhu kungaholela ekunciphiseni kwamangqamuzana abomvu egazi ( i-anemia ), ama-platelet (i-thrombocytopenia), kanye nama-neutrophils (i-neutropenia) .
• Izimpawu ezinjengokukhathala ngokweqile, ukuphaphatheka, ukuphuka kalula, ukopha njalo, kanye nokutheleleka njalo kungase kube khona.
• Nakuba kungekho ikhambi eliphelele lalokhu, izimpawu zingalawulwa ngokwelashwa .
• Kubaluleke kakhulu ukulandela iseluleko sezokwelapha njalo nokunaka izimpawu.
• Awuwedwa. Odokotela bakho kanye nabantu obathandayo bazokusiza. Kubaluleke kakhulu futhi ukuhlala unethemba.

Ngiyethemba ukuthi lolu lwazi luzokusiza. Uma unemibuzo, ungangabazi ukukhuluma nodokotela wakho.

I -Evans syndrome, isifo sokuzivikela komzimba, i-cytopenia, i-anemia, i-thrombocytopenia, i-neutropenia, izinkinga zegazi