Evans Syndrome ndi kuphatikiza matenda awiri kapena ochuluka omwe amateteza thupi lanu kumagazi anu oyera, maselo ofiira ndi / kapena mapulateletti. Izi zikuphatikizapo chitetezo cha chitetezo cha mthupi (ITP) , kuchepa kwa magazi m'thupi (AIHA) , ndi / kapena autoimmune neutropenia (AIN). Matendawa akhoza kuchitika panthawi imodzimodzi komanso amatha kupezeka mliri yemweyo nthawi ziwiri.
Mwachitsanzo, ngati mwapezeka ndi ITP ndipo patatha zaka ziwiri mutapezeka ndi AIHA, mudzakhala ndi Evans Syndrome.
Zizindikiro
Nthawi zambiri, mwapezeka kuti muli ndi vuto limodzi: ITP, AIHA, kapena AIN. Evans Syndrome ikuwoneka ngati chimodzi mwazovuta zaumwini. Zizindikiro zingakhalepo:
Ma plate platelet (thrombocytopenia) amapezeka kwambiri:
- Kuwonjezereka koopsa
- Kuchulukanso kwa magazi: mitsempha ya magazi m'kamwa, nosebleeds, magazi amagazi, magazi mumtsuko kapena chotupa
- Madontho aang'ono ofiira pa khungu lotchedwa petechiae
Matenda a magazi:
- Kutopa kapena kutopa
- Kupuma pang'ono
- Kuthamanga mtima kwa mtima (tachycardia)
- Khungu la khungu (jaundice) kapena maso (scleral icterus)
- Msuzi wamdima (tiyi kapena koka-cola wachikuda)
Kuwerengera kochepa kwa neutrophil (neutropenia):
- Fever
- Matenda a khungu kapena pakamwa
- Kawirikawiri palibe zizindikiro
Chifukwa chiyani Evans Syndrome Chifukwa Magazi Anga Amakhala Ochepa?
Evans Syndrome ndi matenda okhaokha.
Pazifukwa zosadziwika, chitetezo chanu cha mthupi sichimazindikiritsa maselo ofiira a magazi, mapulateletti, ndi / kapena neutrophils ngati "akunja" ndikuwawononga. Sizimvetsetsa bwino chifukwa chake anthu ena ali ndi kachilombo kamodzi kamodzi kokha, monga mu ITP, AIHA, kapena AIN, poyerekeza ndi oposa a Evans Syndrome.
Kudziwa
Pamene anthu ambiri omwe ali ndi Evans Syndrome ali kale ndi matendawa, kufotokoza kwa wina kumakhala ngati Evans Syndrome. Mwachitsanzo, ngati mwapezeka kuti muli ndi ITP ndipo mumakhala ndi kuchepa kwa magazi, dokotala wanu ayenera kudziwa chifukwa chake mukudwala magazi m'thupi. Ngati magazi anu akupezeka chifukwa cha AIHA, mudzapezeka ndi Evans Syndrome.
Chifukwa zovuta izi zimakhudza kuchuluka kwa magazi, kuchuluka kwa magazi (CBC), ndilo gawo loyamba kuntchito. Dokotala wanu akuwonetsa umboni wa kuchepa kwa magazi m'thupi (low hemoglobin), thrombocytopenia (kuwerengeka kochepa pamatope) kapena neutropenia (chiwerengero chochepa cha neutrophil, mtundu wa selo loyera la magazi). Magazi anu adzafufuzidwa pansi pa microscope kuyesa kuzindikira chomwe chimayambitsa. ITP ndi AIN amadziwidwa kuti asatanthauzire kutanthauza kuti palibe mayeso omwe ali nawo. Dokotala wanu ayenera kuletsa zifukwa zina poyamba. AIHA imatsimikiziridwa ndi mayesero ambiri, makamaka mayeso omwe amatchedwa DAT (kulongosola mayeso a antitiglobulin). DAT imafuna umboni wakuti chitetezo cha mthupi chikuyambitsa maselo ofiira a magazi.
Kuchiza
Pali mndandanda wautali wa mankhwala otheka. Kuchiza kumayang'aniridwa ndi maselo enieni a m'magazi komanso ngati muli ndi zizindikiro (kuthamanga kwa magazi, kupuma pang'ono, kuthamanga kwa mtima, matenda):
- Steroids: Mankhwala monga prednisone akhala akugwiritsidwa ntchito kwa zaka zosiyanasiyana m'maganizo osiyanasiyana. Iwo ndiwo mankhwala oyamba a AIHA ndipo amagwiritsidwanso ntchito mu ITP. Mwamwayi, ngati muli ndi Evans Syndrome, mungafune steroids kwa nthawi yaitali zomwe zingayambitse mavuto ena monga kuthamanga kwa magazi (shuga) ndi shuga yambiri ya shuga (shuga). Pa chifukwa ichi, dokotala angafune njira zochiritsira zosagwiritsidwe ntchito.
- Intravenous immunoglobulin (IVIG): IVIG ndi mankhwala oyamba a ITP. Kwenikweni, IVIG imasokoneza chitetezo cha mthupi mwanu kuti mapulaneti asawonongeke mofulumira. IVIG siyothandiza mu AIHA kapena AIN.
- Splenectomy: Mphuno ndi malo enieni a kuwonongeka kwa maselo ofiira, mapiritsi, ndi neutrophils ku Evans Syndrome. Kwa odwala ena, kuchotsa opaleshoni ya nthenda kungathandize kuti magazi asinthe, koma izi zingakhale zanthawi chabe.
- Rituximab: Rituximab mu mankhwala otchedwa antioclonal antibody. Zimachepetsa B-lymphocytes yanu (selo loyera la magazi lomwe limapangitsa tizilombo toyambitsa matenda), zomwe zingasinthe kuchuluka kwa magazi.
- G-CSF (filgrastim): G-CSF ndi mankhwala omwe amachititsa kuti mafupa apange ma neutrophils ambiri. Nthawi zina amagwiritsidwa ntchito kuonjezera chiwerengero cha neutrophil mu AIN, makamaka ngati muli ndi matenda.
- Mankhwala osokoneza bongo: Mankhwala awa amaletsa chitetezo cha mthupi. Izi ziphatikizapo mankhwala monga mycophenolate motel (MMF), azathioprine (Immuran), tacrolimus (Prograf). A
Kodi Ndingatani Kuti Ndichiritsidwe?
Ngakhale odwala angayankhe njira zochiritsira zokhazokha m'magazi, yankho limeneli ngati kawirikawiri likufuna chithandizo china.
> Zotsatira:
> Schrier SL. Kutentha kwapadera kwa Hemolytic Anemia: Chithandizo mu UpToDatePost TW (Ed), UpToDate, Waltham, MA.