Evans Syndrome ke motsoako oa maloetse a mabeli kapa a mangata a immune a hemophilia moo sesole sa hau sa 'mele se hlaselang lisele tsa hao tse tšoeu tsa mali, lisele tse khubelu tsa mali le / kapa liplatelete. Tsena li kenyeletsa lefu la immune thrombocytopenia (ITP) , phokolo ea mali ea hemolytic (AIHA) ea autoimmune, le / kapa autoimmune neutropenia (AIN). Ts'oaetso ena e ka 'na ea etsahala ka nako e le' ngoe empa e ka boela ea hlaha mokuli a le mong ka makhetlo a mabeli a fapaneng.
Ka mohlala, haeba u fumanoa u e-na le ITP 'me lilemo tse peli hamorao li fumanoa u e-na le AIHA, u tla ba le Evans Syndrome.
Matšoao
Maemong a mangata, u se u fumanoe u e-na le bothata ba motho ka mong: ITP, AIHA, kapa AIN. Evans Syndrome e hlahisa mofuta leha e le ofe oa mathata a motho ka mong. Matšoao a ka kenyelletsa:
Litebelisoa tse tlase tsa platelet (thrombocytopenia) li atile haholo:
- Ho senyeha ho eketsehileng
- Ho tsoa mali a mangata: maling a mali molomong, nosebleeds, mali a mali, mali a motsoako kapa setulo
- Matheba a mabeli a khubelu letlalong le bitsoang petechiae
Anemia:
- Ho khathala kapa mokhathala
- Phefumoloho e khutšoanyane
- Nako e phahameng ea pelo (tachycardia)
- Ho khanya ha letlalo (jaundice) kapa mahlo (scleral icterus)
- Motso o lefifi (tee kapa coca-cola mebala)
Palo e tlaase ea neutrophile (neutropenia):
- Feberu
- Matšoao a letlalo kapa molomo
- Hangata ha ho na matšoao
Ke hobane'ng ha Evans Syndrome e le Lebaka la Hore Mali a ka a Hlōle?
Evans Syndrome ke boloetse bo ikemetseng.
Bakeng sa mabaka a mang a sa tsejoeng, tsamaiso ea 'mele ea hau ea' mele ea ho itšireletsa mafung e bontša ka mokhoa o fosahetseng lisele tse khubelu tsa mali, liplatelete, le / kapa li-neutrophils e le "basele" 'me li li senya. Ha ho utloisisoe ka ho feletseng hore na ke hobane'ng ha batho ba bang ba e-na le sele e 'ngoe feela ea mali e amehang, joalo ka ITP, AIHA, kapa AIN, khahlanong le e fetang e' ngoe ho Evans Syndrome.
Tsejoa
Ha batho ba bangata ba nang le Evans Syndrome ba se ba ntse ba e-na le e 'ngoe ea lisebelisoa, ho hlahisa e' ngoe ho tšoana le Evans Syndrome. Ka mohlala, haeba u fumanoe u e-na le ITP mme u hlaolela phokolo ea mali, ngaka ea hau e tla hloka ho tseba hore na sesosa sa mali a hau ke afe. Haeba mali a hao a fumanoa a bakoa ke AIHA, u tla fumanoa u e-na le Evans Syndrome.
Hobane mathata ana a ama dipalo tsa mali, tekanyo e feletseng ea mali (CBC), ke mohato oa pele mosebetsing. Ngaka ea hau e bona bopaki ba mali a phofshoana (lower hemoglobin), thrombocytopenia (palo e tlaase ea platelet) kapa neutropenia (palo e tlaase ea neutrophil, mofuta oa sele e tšoeu ea mali). Mali a hau a tla hlahlojoa ka tlas'a microscope ho leka ho khetholla sesosa. ITP le AIN li fumanoa ka moelelo oa ho ikhetholla ha ho na teko e hlakileng ea ho hlahloba. Ngaka ea hau e tlameha ho laola lisosa tse ling pele. AIHA e tiisoa ke liteko tse ngata, haholo-holo tlhahlobo e bitsoang DAT (e tobileng tlhahlobo ea antiglobulin). DAT e batla bopaki ba hore tsamaiso ea 'mele ea ho itšireletsa mafung e hlasela lisele tse khubelu tsa mali.
Litlhare
Ho na le lenane le lelelele la mekhoa ea phekolo. Kalafo e lebisitsoe ho sele e itseng ea mali e amehileng le hore na o na le matšoao (ho tsoa mali a phetseng hantle, phefumoloho e khutšoanyane, tekanyo ea pelo e phahameng, tšoaetso):
- Steroids: Meriana e kang prednisone e 'nile ea sebelisoa ka lilemo tse ngata mathateng a fapaneng a ho itšireletsa. Ke eona phekolo ea pele ea AIHA mme e boetse e sebelisoa ho ITP. Ka bomalimabe, haeba u e-na le Evans Syndrome, u ka 'na ua hloka steroids nako e telele e ka' nang ea fella ka lintlha tse ling tse kang khatello ea mali e phahameng (ts'oaetso ea mali) le tsoekere e phahameng ea mali (lefu la tsoekere). Ka lebaka lena, ngaka e ka batla litsela tse ling tsa phekolo.
- In immunoglobulin (IVIG): Inthaneteng ke phekolo ea pele ea ITP. Ha e le hantle, IVIG e senya tsamaiso ea hau ea 'mele ea ho itšireletsa mafung ka nakoana e le hore liplatelete li se ke tsa senngoa kapele. IVIG ha e sebetse ho AIHA kapa AIN.
- Splenectomy: Spleen ke sebaka se ka sehloohong sa ho timetsoa ha lisele tse khubelu tsa mali, liplatelete le li-neutrophils ho Evans Syndrome. Bakeng sa bakuli ba bang, ho tlosoa ho buuoa ha spleen ho ka 'na ha ntlafatsa litekanyetso tsa mali, empa sena se ka ba sa nakoana feela.
- Rituximab: Rituximab ka meriana e bitsoang monoclonal antibody. E fokotsa li-lymphocyte tsa hau (lisele tse tšoeu tsa mali tse etsang li-antibodies), tse ka ntlafatsang lintlha tsa mali.
- G-CSF (filgrastim): G-CSF ke eona meriana e sebelisetsoang ho susumelletsa mokoallo hore a etse li-neutrophils tse eketsehileng. Ka linako tse ling ho sebelisoa ho eketsa palo ea neutrophil ho AIN, haholo-holo haeba u na le tšoaetso.
- Meriana ea ho itšireletsa mafung : Meriana ena e thibela ho itšireletsa mafung. Tsena li tla kenyelletsa meriana e kang mycophenolate motel (MMF), azathioprine (Immuran), tacrolimus (Prograf). A
Monyetla oa Hore ke Pholisoe ke Ofe?
Le hoja bakuli ba ka arabela kalafo ka bomong ka ntlafatso ea lipalo tsa mali, karabo ena haeba hangata e hloka nakoana kalafo ea phekolo.
> Mehloli:
> Schrier SL. Hemolytic Hemolytic Anemia e futhumetseng: Ho phekola ho Holimo ho Pati TW (Ed), UpToDate, Waltham, MA.