Kupenda kwa Zizindikiro, Kuzindikira, ndi Kuchiza Mankhwala a Hypereosinophilic Syndromes
Mwachidule
Matenda a hypereosinophilic (HES) ndi magulu osowa kwambiri omwe amadziwika ndi eosinophils (eosinophilia) omwe amachititsa kuwonongeka kwa ziwalo (khungu, mapapo, mtima, m'mimba). Ambiri mwa anthu ali ndi zaka zoposa makumi asanu ndi ziwiri (20) omwe amawazindikira koma akhoza kuchitika kwa ana. Ambiri a HES amaonedwa kuti ndi oopsa (osati a khansa) koma kachigawo kakang'ono kamakhala ngati myeloproliferative neoplasms yomwe ingasinthe kukhala khansara.
Tanthauzo
Hypereosinophilia imatanthauzidwa ngati nthenda yeniyeni ya eosinophil yoposa 1500 maselo / microliter pazigawo ziwiri zokha za magazi (CBC) pazigawo ziwiri zosiyana zomwe zimasiyanitsidwa ndi mwezi umodzi kapena / kapena kutsimikiziridwa kwa minofu ya hypereosinophilia pa chiwopsezo. Hypereosinophilia mu ziwalozi zimatanthauzidwa molingana ndi malo a chidziwitso. Pa nthenda ya mafupa, hypereosinophilia imatanthauzira pamene opitirira 20 peresenti ya maselo a nuclein ndi eosinophils. Pazinthu zina, zimatanthauzidwa kukhala "wambiri" kulowerera m'matumbo mwa lingaliro la wodwala matenda (dokotala akuyang'ana zokhudzana ndi vutoli).
Zambiri zingayambitse ma eosinophilia koma izi sizimayambitsa kuwonongeka kwa minofu yomwe imapezeka mu HES. Eosinophilia ikhoza kuphwasulidwa m'magulu atatu: ofatsa (500 mpaka 1500 eosinophils / microliter), ochepa (1500 mpaka 5000 eosinophils / microliter), ndi oopsa (oposa 5000 eosinophils / microliter.
Mitundu
HES ikhoza kugawidwa m'magulu atatu akuluakulu: oyambirira (kapena neoplastic), wachiwiri (kapena otetezeka), ndi adiopathic. Idiopathic HES ndi matenda omwe amadziwika kuti palibe chifukwa china chomwe chimayambitsa eosinophilia. Pachiyambi cha HES, kusintha kwa majeremusi kumapangitsa mafupa kuti athandize kupanga zofanana ndi polycythemia vera kapena thrombocythemia yofunikira .
Mu HES yachiwiri, vuto lalikulu (matenda a tizilombo toyambitsa matenda, lymphoma, etc.) amapanga mapuloteni (otchedwa cytokines) omwe amachititsa kupanga eosinophil.
Zizindikiro
Zizindikiro za HES zimachokera m'deralo ndipo zingaphatikizepo izi:
- Khungu: dzuŵa, kuphulika khungu, ming'oma,
- Maungulo: chifuwa, kupuma pang'ono, kupuma
- Mtima: kupweteka pamtima, kupuma kovuta,
- Matenda a Neurologic
- Magazi a magazi (thrombosis)
- Maso: Masomphenya opunduka
- Matenda a m'mimba (GI): kutaya kwa thupi, kupweteka m'mimba, kusanza, kutsegula m'mimba
Kudziwa
Monga tafotokozera pamwambapa, mitundu yambiri ya magazi oyera (neutrophils, lymphocytes, monocytes, eosinophils ndi basophils). Chiyeso choyambirira choyezetsa matenda ndi chiwerengero chathunthu cha magazi. Ma eosinophil amawerengera oposa 1500 maselo / microliter pafupipafupi maulendo awiri amachititsa kuti ayesedwe. Poyamba, zifukwa zambiri zomwe zimayambitsa eosinophilia ziyenera kuthetsedwa.
Pomwe HES akudandaula, kufufuza kumayang'anitsitsa kuyang'ana chigwirizano cha bungwe. Kufufuza mtima kungaphatikizepo electrocardiogram (ECG) ndi echocardiogram (ultrasound of the heart). Kuyezetsa ntchito kumayenera kuchitidwa. Tomato yamtundu (CT) idzagwiritsidwa ntchito kuyang'ana zizindikiro zirizonse za HES m'mapapu kapena m'mimba.
Kuyezetsa kwina kudzayang'anitsitsa pakuwona ngati pali umboni uliwonse wa myeloproliferative neoplasm. Izi ziphatikizapo ntchito ya magazi ndi aspirate / fupa la mafupa . Minofu ya fupa la mafupa idzayesedwa kuti kusintha kwa majini kumayenderana ndi HES. Ngati pali kuchuluka kwa maselo a masi, kuyesedwa kudzatumizidwa kudzayang'ana machitidwe a mastocytosis (mtundu wina wa myeloproliferative neoplasm).
Chithandizo
Mankhwala anu adzatsimikiziridwa ndi mtundu wanu wa hypereosinophilic syndrome ndi kuopsa kwa zizindikiro zanu. Si zachilendo kufunikira chithandizo pakadali pano, koma nthawi zambiri HES ingafunike kuchipatala mwamsanga.
- Steroids: Steroids monga prednisone ndi methylprednisolone ingagwiritsidwe ntchito.
- Imatinib: Imatinib ndi mankhwala opatsirana pogwiritsa ntchito khansa yotchedwa tyrosine kinase inhibitor.
- Vincristine: Vincristine ndi mankhwala osokoneza bongo (IV) omwe angagwiritsidwe ntchito. Ngati mutayankha, chiwerengero chanu cha eosinophil chingachepetse mkati mwa maola otsogolera.
- Hydroyxurea: Hydroxyurea ndi mankhwala omwe amamwa mankhwala am'thupi. Zitha kutenga masabata awiri kuti muwone yankho la mankhwalawa.
Ngati pali zizindikiro za myeloproliferative neoplasm (mavitamini B12 owonjezereka, nthonje yofutukuka, mapuloteni otchuka, etc.), mankhwala oyamba ndi imatinib. Ngati pali kukanika kwa mtima, steroids ndizowonjezeredwa. Ngati imatinib siilibwino, pali mankhwala ambiri omwe angagwiritsidwe ntchito. Ambiri mwa anthu omwe ali ndi HES safuna thandizo la mankhwala koma amafunika kuyang'anitsitsa mosamala zowononga thupi, kupititsa patsogolo magazi (thrombosis), ndi kupititsa patsogolo matenda. Ngati pali kugwirizana kwa thupi, steroids ndi mankhwala oyamba. Chifukwa chakuti nthawi yayitali steroid imagwiritsidwa ntchito ndi zotsatira zambiri, pamene zizindikiro zatha, mukhoza kusintha mankhwala ena monga hydroxyurea, alpha interferon, kapena mettretrexate kuti mupitirize kuchipatala.
Monga taonera pamwambapa, steroids ndi mankhwala opambana. Musanayambe steroids ndikofunika kudziwa ngati muli pachiopsezo cha matenda opatsirana otchedwa Strongyloides. Steroids ingawononge kwambiri Strongyloides matenda ambiri. Ngati mukufunikira chithandizo chamankhwala ndipo pali vuto lililonse kuti muli ndi matenda a Strongyloides, mudzatengedwera matenda (kumwa mankhwala kwa masiku awiri).
Mawu Ochokera
Kuphunzira kuti muli ndi hypereosinophilic syndrome kungakhale koopsa. Mwamwayi, anthu ambiri omwe ali ndi HES sadzafuna chithandizo. Kwa iwo omwe amachitapo, pali njira zingapo ndipo ngakhale panopa mukuphunzira.
> Zosowa
> Roufosse F, Klion AD, Weller PF. Matenda a hypereosinophilic: Mankhwala owonetserako matenda, matenda opatsirana, ndi matenda ndi Hypereosinophilic syndromes: Chithandizo. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Yapezeka pa July 5, 2016.)