I-Evans Syndrome iyintlangano yezifo ezimbini okanye ngaphezulu ze-hematological ezikhuselweyo zokulwa nomzimba apho i-immune system yakho ihlasela amangqamuzana egazi amhlophe, amangqamuzana egazi abomvu kunye / okanye iiplatelet. Ezi zibandakanya i- immune thrombocytopenia (i-ITP) , i- hemolytic anemia (AIHA) , kunye / okanye i-neimropenia ye-autoimmune (AIN). Ezi zifo ziyakwenzeka ngexesha elifanayo kodwa zinokuthi zenzeke kwisigulane esifanayo kumaxesha amabini ahlukeneyo.
Ngokomzekelo, ukuba ufumene i-ITP kwaye emva kweminyaka emibini ufunyaniswa ne-AIHA, uya kuba ne-Evans Syndrome.
Iimpawu
Kwiimeko ezininzi, sele ufumene ukuba unesifo sengxaki esisodwa: ITP, AIHA, okanye i-AIN. I-Evans Syndrome ifana naluphi na olunye lwezifo. Iimpawu zibandakanya:
I-platelet ephantsi ibala (thrombocytopenia) ixhaphake kakhulu:
- Ukunyuka kwamanani
- Ukuphuma kwegazi: i-blood blisters emlonyeni, i-nosebleeds, igazi eliphuma kumanzi, igazi kumchamo okanye kwisitonsi
- Amacatswana amancinci amancinci kwesikhumba esibizwa nge-petechiae
IAnemia:
- Ukukhathala okanye ukhathala
- Ukuqhawukelwa ngumphefumlo
- Inqanaba lentliziyo ephakamileyo (tachycardia)
- Ukuqhwala kwesikhumba (jaundice) okanye amehlo (i-icterus yesikhala)
- Umchamo omnyama (itiye okanye i-coca-cola enebala)
Ubuninzi be-neutrophil count (neutropenia):
- Fever
- Izifo zolusu okanye umlomo
- Ngokuqhelekileyo akukho zibonakaliso
Kutheni I-Evans Syndrome Isisombululo Igazi Lami Linokuthi Linciphise?
I-Evans Syndrome iyisifo esingenanto.
Ngesizathu esingaziwayo, isistim somzimba sakho sokuzivikela sisichaze ngokuchanekileyo amaseli akho abomvu, iiplatelets, kunye / okanye i-neutrophils nje "yangaphandle" kwaye ibatshabalalise. Akuqondi kakuhle ukuba kutheni abantu abathile banegazi elilodwa elichaphazelekayo, njenge-ITP, AIHA, okanye i-AIN, ngokumalunga nomnye kwi-Evans Syndrome.
Ukuxilongwa
Njengoko abaninzi abantu abane-Evans Syndrome sele bephethe enye yezifo, ukunikezwa komnye kufana ne-Evans Syndrome. Ngokomzekelo, ukuba unikwe i-ITP kwaye uhlakulela i-anemia, ugqirha wakho uya kufuneka ukuba aqinisekise isizathu sokuba unemia yakho. Ukuba i-anemia yakho ifumaneka ngenxa ye-AIHA, uya kuthi ufumane i-Evans Syndrome.
Ngenxa yokuba ezi ngxaki zichaphazela ukubala kwegazi lakho, inani elipheleleyo legazi (CBC), liyinyathelo lokuqala ekusebenzeni. Ugqirha wakho ukhangele ubungqina bendalo ye- anemia (i-hemoglobin ephantsi), i- thrombocytopenia (isibalo esiphantsi seplatelet) okanye i- neutropenia ( inani eliphantsi le-neutrophil, uhlobo lwe-cell white white). Igazi lakho liza kuhlolwa phantsi kwe-microscope ukuzama ukufumanisa isizathu. I-ITP kunye ne-AIN zixilongwa ngentsingiselo yokungabikho ngaphandle kovavanyo oluthile lokuhlola. Udokotela wakho kufuneka alawule ezinye izizathu kuqala. I-AI iqinisekiswa yimvavanyo emininzi, ngokuthe ngqo uvavanyo olubizwa ngokuba yi-DAT (uvavanyo lwe-antiglobulin ngqo). I-DAT ibheka ubungqina bokuba i-immune system ihlasele iiseli ezibomvu zegazi.
Unyango
Kukho uluhlu olude lwezo kunyango. Unyango lujoliswe kwiseli echaphazelekayo yegazi kunye nokuba unayo nayiphi na impawu (ukuphuma kwamanzi, ukuphefumula okufutshane, izinga lentliziyo ephakamileyo, ukusuleleka):
- I-Steroids: Imithi efana ne-prednisone isetyenziswe iminyaka kwimingcipheko eyahlukeneyo. Ziyinkqubo yokuqala ye-AIHA kwaye isetyenziswe kwi-ITP. Ngelishwa, ukuba unayo i-Evans Syndrome, unokufuna i-steroids ixesha elide elinokubangela ezinye izinto ezinjengexinzelelo eliphezulu legazi (i-blood pressure) kunye ne-sugar sugar (sophisticated blood sugar). Ngenxa yoko, ugqirha unokujonga ezinye iindlela zokwelapha.
- Intravenous immunoglobulin (IVIG): I- IVIG yonyango lokuqala lwe-ITP. Okubaluleke kakhulu, i-IVIG iphazamisa isistim somzimba wakho wexeshana ukuze iiplatelet zingonakali ngokukhawuleza. I-IVIG ayisebenzisiyo e-AIHA okanye kwi-AIN.
- I-Splenectomy: I-spleen yindawo ephezulu yokutshatyalaliswa kweeseli ezibomvu zegazi, iplatelets, kunye neutrophils kwi-Evans Syndrome. Kwezinye izigulane, ukutshatyalaliswa kokutshatyalaliswa kwepen kunokuphucula ukubala kwegazi, kodwa oku kungenokwesikhashana.
- I-Rituximab: I- Rituximab kwipilisi ebizwa ngokuba yi-antioclonal antibody. Iyanciphisa i-L-lymphocyte yakho (iseli egazini legazi elenza izikhuhlane), ezinokuphucula ukubala kwegazi lakho.
- G-CSF (filgrastim): I- G-CSF yile myeza esetyenziselwa ukuvuselela umongo wethambo ukwenza i-neutrophils engaphezulu. Ngamanye amaxesha kusetyenziswa ukunyusa inani le-neutrophil kwi-AIN, ngakumbi xa unesifo.
- Amachiza omzimba: Amachiza ayawavimbela isistim somzimba. Ezi ziza kubandakanya imithi efana ne-mycophenolate motel (MMF), i-azathioprin (Immuran), i-tacrolimus (i-Prograf). A
Ubunjani Ukuba Ndiza Kuphiliswa?
Nangona izigulane zingaphendula kwizonyango zodwa kunye nokuphucula kwiibalo zegazi, le mpendulo ukuba ngaba ixeshana ifuna ixesha elongezelelweyo lonyango.
> Imithombo:
> Schrier SL. I-Hemolytic Anemia: Ukunyanga kwi-UpToDatePost TW (Ed), UpToDate, Waltham, MA.