Evans Syndrome inobatanidza zvirwere zviviri kana zvitatu zvema immune hematological umo masimba ako ezvirunha anorwisa masero machena eropa, masero matsvuku eropa uye / kana maplatelet. Izvi zvinosanganisira immune thrombocytopenia (ITP) , autoimmune hemolytic anemia (AIHA) , uye / kana autoimmune neutropenia (AIN). Izvi zvinogona kuitika panguva imwechete asi zvinogonawo kuitika murwere mumwechete panguva dzakasiyana.
Somuenzaniso, kana iwe ukaonekwa kuti une ITP uye makore maviri gare gare unorwara neAIHA, unenge uine Evans Syndrome.
Zviratidzo
Munzvimbo dzakawanda, iwe watoona kuti une imwe yezvinetso zvakasiyana-siyana: ITP, AIHA, kana AIN. Evans Syndrome inoratidzira seimwe yezvinetso zveumwe munhu. Zviratidzo zvinosanganisira:
Low platelet inonzi (thrombocytopenia) inowanzoonekwa:
- Kuwedzera kuputika
- Kuwedzerwa kweropa: ropa blister mumuromo, nosebleeds, gum ropa, ropa mumurini kana chigaro
- Zviduku zviduku zvitsvuku paganda rinonzi petechiae
Anemia:
- Kuneta kana kuneta
- Kupfupika kwemhepo
- Yakakura mwoyo mwoyo (tachycardia)
- Kupenya kweganda (jaundice) kana maziso (scleral icterus)
- Misi yakasviba (tei kana coca-cola yakavara)
Low neutrophil count (neutropenia):
- Fever
- Zvirwere zveganda kana muromo
- Kakawanda hapana zviratidzo
Sei Evans Syndrome Nemhaka Yekuti Ropa Rangu Rinoita Kuti Rive Rakaderera?
Evans Syndrome chirwere chekuzvimirira.
Nokuda kwechimwe chikonzero chisingazivikanwi, maune immune system haatauri masero matsvuku eropa, platelets, uye / kana neutrophils se "vatorwa" uye anovaparadza. Haisi kunzwisiswa zvachose nei vamwe vanhu vachiine ropa rimwe chete rakabatwa, sezvakaitwa muItP, AIHA, kana AIN, inopesana nevanopfuura rimwe muEvans Syndrome.
Kuziva
Sezvo vanhu vakawanda vane Evans Syndrome vakatotakura imwe yezviongorori, mharidzo yeimwe inofanana neEvans Syndrome. Semuenzaniso, kana wakamboonekwa kuti une ITP uye unoita kuti chirwere chemaanti chiwedzere, chiremba wako achada kuziva chikonzero chekushaya ropa. Kana kushaya ropa kwako kunowanikwa kuve nekuda kweAIHA, uchazoonekwa neEvans Syndrome.
Nokuti kushungurudzwa uku kunobatanidza ropa rako, nhamba yakakwana yeropa (CBC), ndiyo danho rokutanga mubasa. Chiremba wako anotarisa uchapupu hwekushaya ropa (low hemoglobin), thrombocytopenia (low platelet count) kana neutropenia (pasi neutrophil count, rudzi rwe white white cell). Ropa rako richaongororwa pasi pe microscope kuedza kuziva chikonzero. ITP neAIN vari kuongororwa kwekuregererwa zvinoreva kuti hakuna munhu anoongorora kuongorora. Chiremba wako anofanira kutonga kunze kwezvimwe zvinokonzera kutanga. AIHA inotsigirwa nekuongororwa kwakawanda, kunyanya kuedzwa kunonzi DAT (kurondedzera antiglobulin test). DAT inotarisa uchapupu hwokuti maitiro ezvirwere zvemuviri ari kurwisa masero matsvuku eropa.
Mishonga
Pane rurefu rurefu rwezvinogona kurapwa. Mishonga inotungamirirwa kune rimwe ropa rinoshandiswa uye kana iwe une zviratidzo (kushingaira kunopa ropa, kufema kwepemuviri, kukwirira kwemwoyo, utachiona):
- Steroids: Mishonga yakadai se prednisone yakashandiswa kwemakore mumararamiro akasiyana-siyana ekudzivirira zvirwere. Ndiyo yekutanga kurapa kweAIHA uye inoshandiswawo muItP. Zvinosuruvarisa, kana iwe uine Evans Syndrome, unogona kutora steroidhi kwenguva yakareba iyo inogona kukonzera dzimwe nyaya dzakadai sepamusoro weupemha (hypertension) uye yakakosha sutu yeropa (chirwere cheshuga). Nokuda kwechikonzero ichi, iwe chiremba unogona kutsvaga mamwe maitiro ekurapa.
- Intravenous immunoglobulin (IVIG): IVIG ndiyo mushonga wokutanga weTP. Zvinonyanya kukosha, iyo IVIG inoshandura muviri wako wekuzvidzivirira kwenguva pfupi kuitira kuti maplatelets arege kuparadzwa nokukurumidza. IVIG haisi iyo inoshanda muAIHA kana AIN.
- Splenectomy: The spleen ndiyo nzvimbo huru yekuparadzwa kwemasero matsvuku eropa, maplatelets, uye neutrophils muEvans Syndrome. Mune vamwe varwere, kubviswa kushandiswa kwepleen kunogona kuwedzera kuwedzerwa kweropa, asi izvi zvingava zvenguva pfupi.
- Rituximab: Rituximab mune mushonga unonzi monoclonal antibody. Iyo inoderedza B-lymphocytes yako (iyo yeropa rega rega iro rinogadzira zvirwere), izvo zvinogona kuvandudza ropa rako.
- G-CSF (filgrastim): G-CSF imishonga inoshandiswa kukurudzira marora emapfupa kuita neutrophils akawanda. Iko dzimwe nguva inoshandiswa kuwedzera kuverenga neutrophil muAIN, kunyanya kana iwe une utachiona.
- Immunosuppressive mishonga: Mishonga iyi inodzivirira zvirwere zvemuviri. Izvi zvinosanganisira mishonga se mycophenolate motel (MMF), azathioprin (Immuran), tacrolimus (Prograf). A
Chii Chingaita Kuti Ndichaporeswa?
Kunyange zvazvo varwere vanogona kupindura kune dzimwe nzira dzokurapa nekunatsiridza muhuwandu hweropa, mhinduro iyi kana kazhinji kwenguva pfupi ichida mishonga yakawedzerwa.
> Sources:
> Schrier SL. Kushamwaridzana nekudziya kweHemolytic Anemia: Kurapa muToDatePost TW (Ed), UpToDate, Waltham, MA.