I-Evans Syndrome iyinkimbinkimbi yezifo ezimbili ze-immune hematological lapho amasosha omzimba wakho ehlasela amangqamuzana egazi amhlophe, amangqamuzana egazi abomvu kanye / noma amaplatelet. Lokhu kufaka i- immune thrombocytopenia (i-ITP) , i- autoimmune anemia ye-hemolytic (AIHA) , kanye / noma i-autoimmune neutropenia (AIN). Lezi zifo zingase zenzeke ngesikhathi esisodwa kodwa zingase zenzeke nesiguli esifanayo ngezikhathi ezimbili ezihlukene.
Isibonelo, uma uthola ukuthi une-ITP futhi ngemva kweminyaka emibili kuthiwa u-AIHA, uzoba ne-Evans Syndrome.
Izimpawu
Ezimweni eziningi, usuvele uthola ukuthi unesifo esisodwa: ITP, AIHA, noma i-AIN. I-Evans Syndrome iveza njengenye yezinkinga zomuntu ngamunye. Izimpawu zingabandakanya:
Ukubalwa kweplatelet ephansi (thrombocytopenia) kuvame kakhulu:
- Ukulimala okwandisiwe
- Ukuphuma kwegazi: i-blood blisters emlonyeni, i-nosebleeds, i-gum ukuphuma, igazi emcimbini noma esitokisini
- Amachashazi amancane obomvu esikhumbeni esibizwa ngokuthi i-petechiae
I-anemia:
- Ukukhathala noma ukukhathala
- Ukuphelelwa umoya
- Inhliziyo yenhliziyo ephakeme (i-tachycardia)
- I-yellowing yesikhumba (i-jaundice) noma amehlo (i-icterus yesikhumba)
- Umchamo omnyama (itiye noma i-coca-cola enemibala)
Ukubalwa kwe-neutrophil ephansi (neutropenia):
- Fever
- Izifo zesikhumba noma ngomlomo
- Ngokuvamile azikho izimpawu
Kungani I-Evans Syndrome Igaxa Igazi Lami Liba Nciphisa?
I-Evans Syndrome yisifo esizimele.
Ngezinye izizathu ezingaziwa, isimiso sakho somzimba sokuvikela omzimba singabonakali kahle amangqamuzana akho abomvu wegazi, amaplatelets, kanye / noma i-neutrophils "njengangaphandle" futhi ibhubhise. Akuqondwa ngokuphelele ukuthi kungani abanye abantu benomzimba owodwa kuphela wegazi, njengoba ku-ITP, AIHA, noma i-AIN, ngokumelene nokungaphezu kweyodwa ku-Evans Syndrome.
Ukuxilongwa
Njengoba abantu abaningi abane-Evans Syndrome sebevele bephethe esinye sezifo, isethulo somunye silingana no-Evans Syndrome. Isibonelo, uma uthola ukuthi une-ITP futhi uthuthukisa i-anemia, udokotela wakho uzodinga ukunquma imbangela ye-anemia yakho. Uma i-anemia yakho itholakale ukuthi ingenxa ye-AIHA, uzothola ukuthi une-Evans Syndrome.
Ngenxa yokuthi lezi ziphazamiso zithinta inani lakho legazi, inani eliphelele legazi (CBC), yisinyathelo sokuqala emsebenzini. Udokotela wakho ubheka ubufakazi be- anemia (i-hemoglobin ephansi), i- thrombocytopenia (isibalo esiphansi se-platelet) noma i- neutropenia (isibalo esincane se-neutrophil, uhlobo lwe-white cell cell). Igazi lakho lizohlolwa ngaphansi kwe-microscope ukuzama ukubona imbangela. I-ITP ne-AIN ixilongwa ngokushiya okushiwo ngaphandle kokuhlolwa kokuhlola okuqondile. Udokotela wakho kumele alawule ezinye izimbangela kuqala. I-AI iqinisekiswa yizivivinyo eziningi, ikakhulukazi ukuhlolwa okubizwa ngokuthi i-DAT (ukuqondisa ukuhlolwa kwe-antiglobulin). I-DAT ibukeka ubufakazi bokuthi amasosha omzimba ahlasele amangqamuzana egazi abomvu.
Ukwelapha
Kukhona uhlu olude lwezokwelapha okungenzeka. Ukwelashwa kuqondiswa esitokisini segazi esithintekayo futhi ngabe unayo yini izimpawu (ukuphuma kwegazi okuqhubekayo, ukuphefumula, ukuphakama kwenhliziyo ephezulu, ukutheleleka):
- I-Steroids: Imithi efana ne-prednisone isetshenziselwe iminyaka ezinkingeni ezihlukahlukene ze-autoimmune. Ziyindlela yokwelapha yokuqala ye-AIHA futhi iyasetshenziswa ku-ITP. Ngeshwa, uma u-Evans Syndrome, ungadinga i-steroids isikhathi esithile esingabangela ezinye izindaba ezifana nomfutho wegazi ophezulu (umfutho wegazi ophezulu) kanye neshukela ephezulu yegazi (isifo sikashukela). Ngenxa yalesi sizathu, udokotela angase afune ezinye izindlela zokwelapha.
- I-immunoglobulin engenayo i-intravenous (IVIG): I- IVIG iyindlela yokwelapha yokuqala ye-ITP. Ngokuyinhloko, i-IVIG iyaphazamisa isimiso somzimba wakho wamasosha omzimba okwesikhashana ukuze amaplatelets achithe ngokushesha. I-IVIG ayiyona ephumelelayo ku-AIHA noma i-AIN.
- I-Splenectomy: I-spleen iyindawo enkulu yokubhujiswa kwamaseli abomvu egazi, amaplatelets, nama-neutrophils ku-Evans Syndrome. Kwezinye iziguli, ukususwa kokuhlinzwa kwe-spleen kungahle kuthuthukise ukubala kwegazi, kodwa lokhu kungase kube okwesikhashana kuphela.
- I-Rituximab: I- Rituximab emithi ebizwa ngokuthi i-antibody monoclonal. Iyanciphisa i-L-lymphocytes yakho (iseli elimhlophe legazi elenza imishanguzo), engenza ngcono ukubala kwegazi lakho.
- I-G-CSF (i-filgrastim): I- G-CSF imithi yakhe esetshenziselwa ukugqugquzela umnkantso wethambo ukwenza i-neutrophils engaphezulu. Ngezinye izikhathi kusetshenziselwa ukwandisa inani le-neutrophil ku-AIN, ikakhulu uma unesifo.
- Imithi ye-immunosuppressive: Le mithi ivikela isimiso somzimba sokuzivikela. Lezi zihlanganisa imithi efana ne-mycophenolate motel (MMF), i-azathioprin (Immuran), i-tacrolimus (i-Prograf). A
Iyini Ingqondo Engizokuphiliswa?
Nakuba iziguli zingase zamukele izindlela zokwelashwa ngabanye ngokuthuthukiswa kwezibalo zegazi, le mpendulo uma ngokuvamile yesikhashana idinga ukwelashwa okwengeziwe.
> Imithombo:
> Schrier SL. I-Autoemmune i-Hemolytic Anemia: Ukulashwa ku-UpToDatePost TW (Ed), UpToDate, Waltham, MA.