Kuongorora kwezviratidzo, kuongororwa, nekurapa kweHypereosinophilic Syndromes
Overview
Hypereosinophilic syndromes (HES) iboka rezvirwere zvisingaoneki zvinoratidzirwa nemazinga akareba eosinophils (eosinophilia) anokonzera kukanganisa nhengo (ganda, mapapu, mwoyo, matumbo). Ruzhinji rwevanhu vanopfuura makore makumi maviri ekuongorora chirwere asi zvinogona kuitika kuvana. Ruzhinji rweHES hunoonekwa seruna (kwete-kanhara) mamiriro asi sarsette inonzi myeloproliferative neoplasms inogona kuchinja kuva kenza.
Tsanangudzo
Hypereosinophilia inotsanangurwa seyero yakazara eosinophil inowedzera kupfuura 1500 masero / microliter pazvikamu zviviri zvakakwana zveropa (CBC) panguva mbiri dzakasiyana dzakaparadzaniswa nemwedzi umwechete uye / kana kusimbisa kwemashuga hypereosinophilia pane biopsy. Hypereosinophilia mumishutu inotsanangurwa zvichienderana nenzvimbo yehupopi. Pakafupa remapfupa rinoputika, hypereosinophilia inotsanangurwa apo zvinopfuura makumi maviri zvezana zvemasero euclein eosinophils. Pane mamwe matukisi, rinotsanangurwa se "kuwedzera" kupinda mukati mezvinyorwa muhutano mukufunga kwechiremba (chiremba anoongorora chirevo che).
Mamiriro ezvinhu mazhinji anogona kukonzera eosinophilia asi izvi hazviwanzokonzera kukanganisa kwemuviri kunoonekwa muHES. Eosinophilia inogona kuputsika muzvikamu zvitatu: nyoro (500 kusvika 1500 eosinophils / microliter), inonyoresa (1500 kusvika 5000 eosinophils / microliter), uye yakaoma (inopfuura 5000 eosinophils / microliter.
Aina
HES inogona kugoverwa muzvikamu zvitatu zvakakura: primary (kana neoplastic), yechipiri (kana kuti inoshanda), uye idiopathic. Idiopathic HES ndiko kuongororwa kwekuregererwa, zvinoreva kuti hapana chimwe chinokonzera eosinophilia chinogona kuonekwa. Muchikamu chekutanga cheHES, kuchinja kwechimiro kunokonzera marora emapfupa kuti awedzere maitiro eosinophil zvakafanana ne polycythemia vera kana kuti hutachiona hunokosha .
Muchikamu chechipiri HES, mamiriro ezvinhu (chirwere chepachirwere, lymphoma, etc) chinobereka mapurotini (anonzi cytokines) anokonzera eosinophil kugadzirwa.
Zviratidzo
Zviratidzo zveHES zvinowanikwa panzvimbo yakabatwa uye zvinogona kusanganisira:
- Ganda: eczema, kuoma kweganda, mikoko,
- Mapungiro: kukwezva, kupererwa kwefemhepo, kuruka
- Mwoyo: kurwadziwa kwepfupa, kuoma kufema,
- Neurologic disease
- Ropa rega (thrombosis)
- Maziso: Maziso akasvinudzwa
- Gestrointestinal (GI) tract: kuora muviri, kurwadziwa mumimba, kurutsa, chirwere
Kuziva
Sezvataurwa pamusoro apa, eosinophils ndeimwe yemhando shanu dzemasero machena (neutrophils, lymphocytes, monocytes, eosinophils uye basophils). Chiyero chekutanga chekuongorora ndechokuzara kweropa. An eosinophil count inopfuura 1500 masero / microliter pane zviitiko zviviri zvinoruramisa kuongororwa zvakare Pakutanga, zvinokonzera zvimwe zvinokonzera eosinophilia zvinofanira kubviswa.
Pane imwe nguva HES inofungidzirwa, kuongororwa kunotarisa pakutsvaga kubatanidzwa kwechikwata. Kuongorora kwemoyo kunosanganisira electrocardiogram (ECG) uye echocardiogram (ultrasound of the heart). Muchengetedza basa rinoedzwa rinofanira kuitwa. Computed tomography (CT) ichashandiswa kutarisa chero zviratidzo zveHES mumapapu kana mudumbu.
Kuwedzerwa kuongororwa kuchaiswa pakuona kana kune humwe uchapupu hwemyeloproliferative neoplasm. Izvi zvinosanganisira kushanda kweropa uye mapfupa emafuta aspirate / biopsy . Mucheka wepfupa remapfupa ichaongororwa kuchinja kwemajini kunobatanidza neHES. Kana paine nhamba yakawedzera yemasero, kuongororwa kunotumirwa kunotsvaka systemic mastocytosis (imwe mhando ye myeloproliferative neoplasm).
Kurapa
Mushonga wako uchagadziriswa nehuwandu hwe hypereosinophilic syndrome nekuoma kwezviratidzo zvako. Hazvishamisi kutsvaga kurapwa zvakanaka paanoongororwa, asi kazhinji hazvo HES yakaoma inogona kukurumidza kurapwa.
- Steroid: Steroid yakafanana ne prednisone uye methylprednisolone inogona kushandiswa.
- Imatinib: Imatinib inorapwa mishonga inoshandiswa kurapa kenza inonzi tyrosine kinase inhibitor.
- Vincristine: Vincristine inobata (IV) chemotherapy inogona kushandiswa. Kana iwe ukapindura, yako eosinophil count inogona kuderera mukati meawa ekutonga.
- Hydroyxurea: Hydroxyurea inorapwa chemotherapy. Zvinogona kutora kusvika masvondo maviri kuti uone mhinduro kumushonga uyu.
Kana pane zviitiko zve myeloproliferative neoplasm (yakakwirira yevhitamini B12, maruva akawedzerwa, eyinophils inypical, nezvimwewo), kutanga kurapa imatinib. Kana pane kuve nekushanda kwemwoyo, steroids inowanikwa. Kana imatinib isingashandi, kune mishonga yakawanda yakafanana inogona kushandiswa. Ruzhinji rwevanhu vane HES harudi kurapwa asi runoda kuchenjerera kuongororwa kwechigadziko chemuviri, kukura kweropa (thrombosis), uye kufambira mberi kwezvirwere. Kana pane chikwata ichibatanidza, steroido inotanga kurapa. Nokuti kwenguva yakareba steroid inoshandiswa inobatanidza nemigumisiro yakawanda, kana zviratidzo zviri pasi pesimba, unogona kuchinjwa kune imwe mishonga se hydroxyurea, alpha interferon, kana methotrexate kuti urambe uchirapwa.
Sezvakaratidzwa pamusoro, steroids inonyanya kushandiswa. Usati watanga steroidhi zvakakosha kuziva kuti iwe uri pangozi yehutachiona hwetachiona hunonzi Strongyloides. Steroid inogona kukuvadza chirwere cheStongyloides zvikuru. Kana iwe uchida kurapwa nokukurumidza uye kune chinetso chekuti une Strongyloides utachiona, iwe uchabatwa nehutachiona (muromo wemishonga kwemazuva maviri).
Shoko Rinobva
Kudzidza kuti une hypereosinophilic syndrome inogona kutyisa. Nenzira yakanaka, ruzhinji rwevanhu vane HES haruchadi kurapwa. Kune avo vanoita, kune zvakawanda zvingasarudzwa uye kunyanya zvatiri kudzidza iye zvino.
> Sources
> Roufosse F, Klion AD, Weller PF. Hypereosinophilic syndromes: Clinical zviratidzo, pathophysiology, uye kuongororwa uye Hypereosinophilic syndromes: Kuchirwa. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Yakasvika musi waJuly 5, 2016.)