Yintoni i-Evans Syndrome? Makhe sithethe ngayo!

Ngaba ngamanye amaxesha uziva udiniwe? Okanye ngaba unengxaki yokuyeka ukopha nokuba zizinto ezincinci? Mhlawumbi kungenxa yokuba amasosha omzimba wakho asebenza ngokuchasene nawe. Namhlanje siza kuthetha ngesimo esingaqhelekanga kodwa esibaluleke kakhulu ekufuneka sazi ngaso. Leyo yi -Evans Syndrome . Ungakhathazeki, siza kuthetha ngayo yonke into ngokulula.

Ngoko ke, yintoni i-Evans Syndrome?

Ngamafutshane, i-Evans Syndrome sisifo esibangelwa kukuzikhusela komzimba . Okwenzekayo kukuba amajoni afanele ukukhusela imizimba yethu, inkqubo yomzimba yokuzikhusela, ngempazamo aqala ukutshabalalisa ezinye zeeseli zethu zegazi. Kufana nomkhosi wethu ohlasela abantu bethu. Ngenxa yoko, imizimba yethu ilahlekelwa sisixa esifunekayo seeseli zegazi. Oku kuncipha okungaqhelekanga kweeseli zegazi yinto esiyibiza ngokuba yi-cytopenia kwezonyango.

Uninzi lwabantu abane-Evans Syndrome luqala ngokuchaphazeleka ziiseli ezibomvu zegazi ezisezantsi (imeko ebizwa ngokuba yi-anemia ) kunye neeplatelets (imeko ebizwa ngokuba yi -thrombocytopenia ). Ukongeza kuzo zombini, abanye abantu banokuba nenani eliphantsi lohlobo lweeseli ezimhlophe zegazi ezibizwa ngokuba yi-neutrophils (imeko ebizwa ngokuba yi -neutropenia ).

Xa ezi ntlobo zeeseli zegazi ziphantsi, izinto ezininzi ezibalulekileyo emzimbeni wethu, ezinje ngokufumana ioksijini, ukujiya kwegazi, kunye nokulwa nezifo, ziyachaphazeleka. Yiyo loo nto siziva sibuthathaka, sidiniwe, siqala ukopha lula, kwaye sigula rhoqo .

Le meko inokuchaphazela abanye abantu kancinci kakhulu, ngelixa abanye benokuba nzima kakhulu. Kwaye iziphumo zayo azihlali zifana ubomi bonke. Eyona nto ilungileyo kukuba nangona kungekho nyango lwayo, unyango lunokunceda ukulawula iimpawu kunye nokunciphisa iziphumo .

Zithini iimpawu ze-Evans Syndrome?

Iimpawu ze-Evans Syndrome zinokwahluka kakhulu. Kuxhomekeke ekubeni loluphi uhlobo lwe-cytopenia onayo kunye nokuba inzima kangakanani. Rhoqo, i-anemia okanye i-thrombocytopenia iqala kuqala, ize eminye ivele kamva.

Iimpawu zokuqala zokungabikho kweeseli ezibomvu zegazi ( i-anemia ):

Ngaba udiniwe nasemva kokuhamba umgama omfutshane? Ngaba uziva udiniwe kwaye awukwazi ukwenza izinto obuqhele ukuzenza? Mhlawumbi ubuso bakho bubonakala buphaphathekile kancinci. Nazi ezinye zeempawu zokuqala ze- anemia :

• Ndiziva ndidiniwe kakhulu.
• Ulusu oluphaphathekileyo (olumnyama) .
• Ukungakhathali okanye isiyezi .
• Ubunzima bokuphefumla (ukuphelelwa ngamandla) .
• Ukubetha kwentliziyo ngokukhawuleza (i-tachycardia) .
• Ukuziva okanye ukuziva ngathi intliziyo yakho ibetha ngendlela engaqhelekanga (ukubetha kwentliziyo) .

Iimpawu zokuqala zokungabikho kweeplatelet (thrombocytopenia):

Ngaba ukhe waqaphela ukuba ngamanye amaxesha kuvela amachaphaza amancinci abomvu eluswini lwakho? Okanye ufumana nje imivumbo emzimbeni wonke? Ngaba uziva ngathi iintsini zakho ziphuma igazi ngaphezu kwesiqhelo xa uhlamba amazinyo akho? Nazi iimpawu ezinokubakho zeeplatelets ezisezantsi:

• Amabala amancinci abomvu okopha (i-petechiae) phantsi kolusu.
• Amabala amfusa eluswini (i-purpura) .
• Ukudumba (ecchymosis) okwenzeka ngaphandle kwesizathu.
• Ukopha kakhulu kwiintsini okanye empumlweni.
• Ukopha kakhulu ngexesha lokuya exesheni kwabasetyhini.
• Ukopha ngenxa yendle okanye umchamo.

Iimpawu ze-neutropenia (ezi zinokwenzeka okanye zingenzeki kamva):

Kuqhelekile ukuba nomkhuhlane omnye okanye emibini ngenyanga. Kodwa ngaba uhlala ugula ngumkhuhlane? Ngaba usoloko ufumana izilonda emlonyeni? Ngaba zibonakala zithatha ixesha elide ukuphola? Nazi ezinye izinto ezinokwenzeka xa ii-neutrophils zakho ziphantsi:

• Ifiva.
• Izilonda zomlomo.
• Umkhuhlane oqhelekileyo, umkhuhlane, kunye nesisu esibuhlungu.
• Usulelo lwendlebe oluphindaphindayo.
• Usulelo lwefungus oluhlala ixesha elide.
• Usulelo lwendlela yomchamo (UTIs) .

Ezinye iimpawu ze-Evans Syndrome (ezi zinokwenzeka ngamanye amaxesha):

• Ii-lymph nodes ezidumbileyo entanyeni.
• I-spleen ekhulisiweyo .
• Isibindi esikhulu .
• I-Jaundice .

Kutheni i-Evans Syndrome isenzeka?

Njengoko besitshilo ngaphambili, i-Evans Syndrome sisifo esibangelwa kukuzikhusela komzimba . Oku kuthetha ukuba inkqubo yomzimba yethu yokuzikhusela iyaziqonda ngempazamo ezinye zeeseli zethu zegazi "njengotshaba" ize iqale ukuzihlasela.

Lo msebenzi wenziwa zii -B cells, uhlobo olukhethekileyo lweeseli kwinkqubo yethu yokuzikhusela komzimba. Ezi B cells zenza into ebizwa ngokuba zii-antibodies . Ezi antibodies ziyahamba zibambe ezo seli zegazi zingenatyala zize zizitshabalalise. Ngokwesiqhelo, ziqala ngokuhlasela uhlobo olunye lweeseli zegazi kuqala, emva koko ziqale ukuhlasela ezinye iintlobo zeeseli zegazi.

Le nkqubo yiyo ekugqibeleni ebangela ukuba ube nokusilela ubuncinane kwiintlobo ezimbini zeeseli zegazi (i-cytopenia), kwaye ngamanye amaxesha zonke ezintathu. Ukusilela kuzo zontathu kubizwa ngokuba yi-pancytopenia . Iimeko ezintathu eziphambili apho ezi seli zegazi ziphantsi khona zezi:

• I-Autoimmune hemolytic anemia (AIHA): Kulapho iiseli ezibomvu zegazi zincipha khona. Oko kukuthi, zitshatyalaliswa ngokukhawuleza kunokuba umzimba unokuzenza.
• I-immune thrombocytopenia (ITP) : Le yimeko apho kukho ukwehla kwee-platelet. Inkqubo yokuzikhusela komzimba ihlasela kwaye isuse ii-platelet egazini.
• I-Autoimmune neutropenia (AIN) : Le yimeko apho inani lee-neutrophils, uhlobo lweseli emhlophe yegazi, liyehla. Inkqubo yomzimba yokuzikhusela ihlasela kwaye itshabalalise ii-neutrophils.

Cinga ngayo, zininzi izizathu zokuba inani leeseli zegazi liphantsi (i-cytopenia). Ngamanye amaxesha kungenxa yokuba umongo wethambo awuvelisi iiseli zegazi ezaneleyo. Nangona kunjalo, kwi-autoimmune cytopenia, inkqubo yomzimba yokuzikhusela itshabalalisa iiseli zayo zegazi.

Zithini izinto ezibangela i-Evans Syndrome?

Izazinzulu azikayazi kakuhle ukuba kutheni ezi zifo zokuzikhusela komzimba zikhula. Kodwa ziye zaphawula ukuba ezi zifo zihlala ziqala emva kokugula kakhulu okanye isiganeko esibeka uxinzelelo olukhulu kwinkqubo yomzimba yokuzikhusela. Ezi zibizwa ngokuba zizinto ezibangela ukugula .

Kwakhona, ukuba unesifo esinye sokuzikhusela komzimba, ngokwezibalo unamathuba amaninzi okufumana esinye. Kungathi esi sifo sokuzikhusela komzimba sisasazeka ukusuka kwelinye icala lomzimba ukuya kwelinye.

I-Evans syndrome ingenzeka yodwa (oku kubizwa ngokuba yi -primary Evans syndrome ), okanye ingenzeka njengemeko yesibini enxulunyaniswa nenye imeko (oku kubizwa ngokuba yi -secondary Evans syndrome) . Ezinye zeemeko ezinokunxulunyaniswa ngale ndlela zezi:

• I-systemic lupus erythematosus (lupus)
• Ukungakwazi ukuzikhusela komzimba okuguquguqukayo okuqhelekileyo
• Ukunqongophala kwe-IgA ekhethiweyo
• Isifo sikaSjögren
• I -lymphoma engeyiyo iHodgkin
• I-leukemia engapheliyo ye-lymphocytic
• I-hepatitis C engapheliyo
• Usulelo lwe-HIV olungapheliyo

Ukuba une-primary Evans syndrome, kusenokwenzeka ukuba ivele emva kokugula okwethutyana, okanye usenokungabi nasizathu sicacileyo. Izazinzulu zibeka i-Evans syndrome njengemeko engaqhelekanga . Oku kuthetha ukuba akukho sizathu sithe ngqo sifunyenweyo.

Ziziphi iingxaki ezinokubakho ze-Evans Syndrome?

Ayinguye wonke umntu one-Evans Syndrome oza kuba neengxaki. Abanye abantu baneempawu ezincinci kakhulu. Nangona kunjalo, kwiimeko ezinzima, ezi ngxaki zokungabikho kweeseli zegazi (cytopenias) zinokuba yingozi ebomini. Umzekelo:

• Xa iiseli ezibomvu zegazi zinganeli ukuhambisa ioksijini emzimbeni, akukho oksijini yaneleyo egazini (i-hypoxemia) . Oku kunzima kakhulu entliziyweni kwaye kunokukhokelela kwisifo sentliziyo kunye nokungasebenzi kakuhle kwentliziyo.
• Xa ungenazo iiplatelets ezaneleyo , ezinceda ukujiya kwegazi lakho, igazi lakho alijiyi kakuhle. Oku kunokubangela ukuba wophe igazi ngokulula nangokukhawuleza. Oku kunokukhokelela ngokukhawuleza kwi-anemia enzima.
• Xa umzimba wakho ulahlekelwa zii-neutrophils , ezinceda ekulweni nosulelo, unamathuba amaninzi okufumana usulelo rhoqo, kwaye lunokuba nzima kwaye lusasazeke emzimbeni wakho wonke. Oku kunokukhokelela kwimeko eyingozi ebizwa ngokuba yi-sepsis .

Oogqirha bayixilonga njani i-Evans Syndrome?

I-Evans Syndrome ifunyaniswa ngovavanyo lwegazi . Ingafunyaniswa ukuba ubonakalisa iimpawu ze-cytopenia, okanye ngexesha lokubalwa kweeseli zegazi okwenziwe ngexesha lokuhlolwa kwempilo rhoqo.

Ukuba i-Complete Blood Count yakho (CBC) ibonisa ukuba uneeseli ezibomvu zegazi ezisezantsi, iiplatelets, okanye ii-neutrophils, iqela lakho lezonyango liza kwenza uvavanyo olongezelelekileyo. Umzekelo, baza kujonga igazi lakho ukuba lifuna amanqanaba aphezulu ee-antibodies ezinxulumene ne-Evans Syndrome. Baza kujonga nezinye iimeko ezinokubangela i-cytopenia, ezifana nosulelo oluthile okanye umhlaza.

Oogqirha bayaqinisekisa ukuba i-Evans Syndrome ayizizo zonke ezinye izizathu. Ngamanye amaxesha, banokufuna ukwenza i -CT scan (i-computed tomography scan) okanye i-bone marrow biopsy ukuze baqinisekise ukuba azikho ezinye iimeko.

Ngaba ikho indlela yokunyanga i-Evans Syndrome ngokupheleleyo? Inyangwa njani?

Enyanisweni, akukho nyango lupheleleyo lwe-Evans Syndrome, okanye nasiphi na esinye isifo sokuzikhusela komzimba. Oogqirha banyanga ezi zifo zokuzikhusela komzimba ngokukunika amayeza anciphisa ukusebenza kwenkqubo yakho yokuzikhusela komzimba.

Ukongeza, iimpawu zakho zinokunyangelwa ngokwahlukeneyo. Umzekelo, ukuba inani leeseli zakho zegazi liphantsi kakhulu, ugqirha wakho unokukwazi ukulungisa oku ngokutofela igazi ngamanye amaxesha.

Njengezifo ezininzi ezihlala ixesha elide ezibangelwa kukungasebenzi kakuhle komzimba, i-Evans Syndrome inokuphucula ngokuhamba kwexesha. Ngamanye amaxesha iimpawu zinokubonakala ngathi ziyaphela (ezibizwa ngokuba yi -remission) , kodwa emva koko zinokubuya okanye zibe mandundu (ezibizwa ngokuba yi-relapse ).

Kusenokufuneka uzame unyango olwahlukeneyo ebomini bakho bonke, njengoko impumelelo yalo inokwahluka ngokuhamba kwexesha. Ugqirha wakho uza kuzama ukukukhusela kwiingxaki ezinkulu ze-Evans Syndrome.

Zithini iindlela zonyango?

Unyango oluqhelekileyo lwe-Evans Syndrome ngamayeza okunciphisa amasosha omzimba , kwaye ukuba kuyimfuneko, utofelo-gazi. La mayeza okunciphisa amasosha omzimba asebenza ngokunciphisa uhlaselo olungalunganga olubangelwa yinkqubo yakho yokuzikhusela komzimba.

Iintlobo eziphambili zamayeza anikiweyo:

• Ii-corticosteroids , ezifana ne -prednisone . Olu lunyango lokuqala olunikwayo.
• Unyango lwe-immunoglobulin olufakwa kwimithambo yegazi (IVIg) lubandakanya ukunika isisombululo esinee-antibodies zabantu ngomthambo. Ezi antibodies zintsha zinokubophelela kwii-antibodies ezineempazamo kwaye zizenze zingabi namandla.

Ukuba awusabeli kakuhle kunyango oluqhelekileyo, ugqirha wakho angazama ezinye iindlela zonyango. Ezi ziquka:

• Ii-antibodies ze-Monoclonal , ezifana ne -rituximab . Ezi zii-antibodies ezenziwe kwilebhu. Ngamanye amaxesha ezi zinokumisa impendulo ye-autoimmune. Ugqirha wakho unokucebisa ukuba ezinye amayeza azisebenzi.
• Utyando lwe-Splenectomy . Kwiimeko ezinzima, ugqirha wakho unokucebisa utyando lokususa i-spleen yakho. I-spleen yakho yindawo apho kususwa khona iiseli zegazi ezindala. Ngoko ke xa i-spleen isuswa, inkqubo iyehla kancinci.

Ngaba kukho naziphi na iingxaki okanye iziphumo ebezingalindelekanga zonyango?

Xa usela amayeza okunciphisa amasosha omzimba, impendulo yomzimba wakho kwisifo sakho sokuzikhusela komzimba iyancitshiswa. Nangona kunjalo, amandla akho okuzikhusela kwizifo nawo ayancitshiswa . Ngoko ke kuya kufuneka uthathe amanyathelo akhethekileyo okuzikhusela ukuze ungaguli.

Ugqirha wakho unokucebisa ezinye izitofu zokugonya ukongeza kwezo sele uzifumene. Xa inkqubo yakho yomzimba yokuzikhusela ibuthathaka (ingasebenzi kakuhle), usenokuba sesichengeni sokufumana izifo obungenakuzifumana ngokuqhelekileyo.

Ngaba ukuhlala ne-Evans Syndrome kuyayichaphazela indlela umntu aphila ngayo?

I-Evans Syndrome ayichaphazeli ubomi bakho. Abanye abantu baneempawu ezibuthathaka kakhulu, ngamanye amaxesha. Nangona kunjalo, ezinye iingxaki, ezinje ngokopha, isifo sentliziyo, kunye nosulelo, zinokuba yingozi ebomini.

Ukuba une-secondary Evans syndrome, oko kuthetha ukuba unayo nesinye isifo esinganyangekiyo, oko kunokuchaphazela nobomi bakho. Usenokuba neengxaki ezisongela ubomi ezihambisana nesinye isifo sakho.

Injani imbono yomntu one-Evans Syndrome?

Amava abantu abane-Evans Syndrome ahluka kakhulu. Iimpawu zinokuba zincinci okanye zibe nzima. Abanye abantu baziva ngathi iimpawu zabo ziyaphela ngokupheleleyo (ukuxolelwa). Abanye bazifumana kwakhona kamva (ukubuyela umva).

Abantu abaninzi bafuna uhlobo oluthile lonyango kunye nokuhlolwa rhoqo kwezonyango. Unyango luhlala lusebenza kakuhle, kodwa alusoloko lusebenza. Ngamanye amaxesha, ukusebenza kakuhle konyango kunokubonakala kuncipha ngokuhamba kwexesha. Kusenokufuneka uzame ezinye iindlela zonyango.

Ndingazinyamekela njani ngelixa ndiphila ne-Evans Syndrome?

Ukuba une-Evans Syndrome, kubalulekile ukuba uhlale uhlola rhoqo . Nokuba uzive uphilile, ugqirha wakho uya kufuna ukujonga imeko yakho aze ayifumane kwangethuba ukuba imeko iba mandundu.

Kwakhona, kufuneka uhlale uziqaphele kwaye uqaphele naziphi na iimpawu ezintsha. Ukuba unomkhuhlane ongapheliyo, okanye ukuba ukopha kwakho akulawuleki, tsalela umnxeba ugqirha wakho ngoko nangoko .

Ndingayibuza yiphi imibuzo ugqirha wam?

Usenokubuza ugqirha wakho imibuzo efana nale:

• Zeziphi iintlobo zeeseli zegazi endingazifumaniyo?
• Zinciphe kangakanani?
• Zeziphi iimpawu okanye iimpawu ezilumkisayo ekufuneka ndiziqaphele?
• Ndingazi njani ukuba unyango luyasebenza okanye alusebenzi?
• Kuza kwenzeka ntoni ukuba unyango alusebenzi?
• Ndifanele ndibhukishe nini ixesha lam elilandelayo lokubonana nogqirha?

I-Evans Syndrome yimeko engaqhelekanga onokuthi awukaze uve ngayo ngaphambili. Iimpawu zinokuba zingaqhelekanga kwaye ziphazamise. Ukuqonda okwenzekayo kunye nentsingiselo yayo kunokuba yinkqubo eyoyikisayo.

Nangona i-Evans Syndrome iyinto ehlala ihleli, ayisoloko ifana. Hlala unethemba lokuba uza kuziva ungcono, ubuncinane ngamanye amaxesha. Nokuba uziva njani, iqela lakho lezonyango liya kuba lapho ukukunika unyango olufunayo.

Umyalezo Wokugqibela Wokuya Ekhaya

Kulungile, masikukhumbuze ngezinye zezona zinto zibalulekileyo esikhe sathetha ngazo.

• Yintoni i-Evans Syndrome?Isifo esingaqhelekanga apho amajoni akho omzimba ahlasela khona iiseli zakho zegazi.
• Oku kunokukhokelela ekunciphiseni kweeseli ezibomvu zegazi ( i-anemia ), iiplatelets (i-thrombocytopenia), kwaye mhlawumbi nee-neutrophils (i-neutropenia) .
• Iimpawu ezinje ngokudinwa kakhulu, ukuphaphatheka, ukukrweleka lula, ukopha rhoqo, kunye nosulelo oluqhelekileyo lunokubakho.
• Nangona kungekho nyango lupheleleyo loku, iimpawu zinokulawulwa ngonyango .
• Kubaluleke kakhulu ukulandela iingcebiso zonyango rhoqo kunye nokuqwalasela iimpawu.
• Awuwedwa. Oogqirha bakho kunye nabantu obathandayo baya kukunceda. Kubaluleke kakhulu ukuhlala unethemba.

Ndiyathemba ukuba olu lwazi luza kukunceda. Ukuba unemibuzo, ungathandabuzi ukuthetha nogqirha wakho.

I- Evans syndrome, isifo sokuzikhusela komzimba, i-cytopenia, i-anemia, i-thrombocytopenia, i-neutropenia, iingxaki zegazi