Ukubuyekezwa Kwezibonakaliso, Ukuxilongwa, Nokunakekelwa Kwe-Hypereosinophilic Syndromes
Sibutsetelo
Ama-hyndereosinophilic syndromes (HES) yiqembu lezinkinga ezingavamile ezibhekwa ngamazinga aphakeme ama-eosinophils (eosinophilia) abangela ukulimala kwezitho (isikhumba, amaphaphu, inhliziyo, isisu). Iningi labantu lineminyaka engaphezu kwengu-20 ekuxilongweni kodwa kungenzeka ezinganeni. Iningi le-HES libhekwa njengezimo ezimbi (ezingekho komdlavuza) kodwa i-subset ibhekwa njenge- myeloproliferative neoplasms engaba ngumdlavuza.
Incazelo
I-Hypereosinophilia ichazwa njengesibalo esiphezulu se-eosinophil ngaphezu kwamakhulu angu-1500 amaseli / microliter kokubili okuphelele kwegazi (CBC) ngezikhathi ezimbili ezihlukene ezahlukaniswa okungenani inyanga eyodwa kanye / noma ukuqinisekiswa kwezicubu ze-tissue hypereosinophilia kwi-biopsy. I-hypereosinophilia kulezi zicubu zichazwa ngokusekelwe endaweni ye-biopsy. Ngomnkantsha we-bone, i-hypereosinophilia ichazwa uma amaphesenti angaphezu kuka-20 wamaseli ase-nucleated eosinophils. Kwezinye izicubu, kuchazwe ngokuthi "ukujula" kokungena kwe-eosinophils kuya emthonjeni ngokombono we-pathologist (udokotela ohlola i-biopsy).
Izimo eziningana zingabangela i-eosinophilia kodwa lokhu okungajwayelekile ukuthi kubangele ukulimala kwezicubu ezitholakala ku-HES. I-Eosinophilia ingahle ihlukaniswe zibe yizigaba ezintathu: ezinomusa (500 kuya ku-1500 i-eosinophils / microliter), ngokulinganisela (ama-eosinophils / microliter angu-1500 kuya ku-5000), futhi ezinzima (ngaphezulu kuka-5000 eosinophils / microliter.
Izinhlobo
I-HES ingahlukaniswa ngezigaba ezintathu ezinkulu: okuyinhloko (noma i-neoplastic), eyesibili (noma esebenzayo), ne-idiopathic. Idiopathic HES ixilongwa ngokukhipha, okusho ukuthi akekho enye imbangela ye-eosinophilia engabonakala. Ku-HES eyinhloko, ushintsho lwezakhi zofuzo luvuselela umongo we-bone ukusheshisa ukukhiqizwa kwe-eosinophil efana ne- polycythemia vera noma i- thrombocythemia ebalulekile .
E-HES yesibili, isimo esiwumgogodla (ukutheleleka kwegciwane, i-lymphoma, njll) kuveza amaprotheni (okuthiwa ama-cytokines) avuselela ukukhiqizwa kwe-eosinophil.
Izimpawu
Izimpawu ze-HES zisekelwe endaweni ezithintekile futhi zingabandakanya:
- Isikhumba: i-eczema, ukuqina kwesikhumba, imifuba,
- Imifula: ukukhwehlela, ukuphefumula, ukushaya umoya
- Inhliziyo: ubuhlungu besifuba, ukuphefumula kanzima,
- Izifo ze-neurologic
- Ama-blood clots (thrombosis)
- Amehlo: Umbono ophukile
- Isisu sezinsipho (GI): ukulahlekelwa isisindo, ubuhlungu besisu, ukuhlanza, isifo sohudo
Ukuxilongwa
Njengoba kushiwo ngenhla, i-eosinophils ingenye yezinhlobo ezinhlanu zamangqamuzana amhlophe egazi (neutrophils, i-lymphocytes, i-monocytes, i-eosinophils kanye ne-basophils). Isivivinyo sokuqala sokuxilonga yisilinganiso esiphelele segazi. Ukubalwa kwama-eosinophil kunama-cell angu-1500 / microliter ngezikhathi ezimbili kuvumela ukuhlolwa okuqhubekayo Ekuqaleni, izimbangela ezivame kakhulu ze- eosinophilia kufanele zisuswe.
Uma sekusolakala ukuthi i-HES, ukuhlolwa kugxile ekubhekeleni ukubandakanyeka komzimba. Ukuhlola kwenhliziyo kungafaka i-electrocardiogram (ECG) kanye ne-echocardiogram (i-ultrasound yenhliziyo). Ukuhlolwa komsebenzi we-Lung kufanele kwenziwe. I-tomography ehlanganisiwe (CT) izosetshenziselwa ukubheka noma yiziphi izimpawu ze-HES emaphashini noma esiswini.
Ukuhlolwa okungeziwe kuzokugxila ekunqumeni ukuthi kukhona yini ubufakazi bokuthi i-neoplasm ye-myeloproliferative ikhona. Lokhu kuzobandakanya umsebenzi wegazi kanye ne- aspirate / biopsy yamarrow . Amathisheka omnyofu we-bone azohlolelwa izinguquko zofuzo ezihlobene ne-HES. Uma kukhona inombolo ekhuphukile yamaseli omzimba, ukuhlolwa kuzothunyelwa ukuyohlola amastocytosis ohlelweni (olunye uhlobo lwe-neoplasm ye-myeloproliferative).
Ukwelapha
Ukwelashwa kwakho kuyonqunywa uhlobo lwakho lwe-hypereosinophilic syndrome kanye nobukhulu bezimpawu zakho. Kuyinto engavamile ukudinga ukwelashwa lapho kutholakala ukuxilongwa, kepha i-HES engavamile kakhulu ingadinga ukwelashwa ngokushesha.
- I-Steroids: I-steroids efana ne-prednisone ne-methylprednisolone ingasetshenziswa.
- I-Imatinib: Imatinib imithi yomlomo esetshenziselwa ukwelapha umdlavuza obizwa ngokuthi i-tyrosine kinase inhibitor.
- U-Vincristine: UVincristine uyimithi yamakhemikhali e-intravenous (IV) engasetshenziswa. Uma uphendula, inani lakho le-eosinophil lingancipha phakathi namahora wokuphatha.
- I-Hydroyxurea: I-Hydroxyurea yimithi yokwelapha ngomlomo. Kungathatha amasonto amabili ukuze ubone impendulo kulo muthi.
Uma kukhona izici ze-neoplasm ye-myeloproliferative (amazinga e-vitamin B12 aphakanyisiwe, i-pleen ekhulisiwe, ama-eosinophils ase-atypical, njll.), Ukwelashwa kokuqala i-imatinib. Uma kukhona ukubandakanya inhliziyo, i-steroids iyanezelwa. Uma i-imatinib ingasebenzi, kunemithi eminingi efana nayo engasetshenziswa. Iningi labantu abane-HES ayidingi ukwelashwa kodwa kudinga ukuqapha ngokucophelela ukulimala komzimba, ukuthuthukiswa kwamagundane egazi (thrombosis), nokuqhubeka kwezifo. Uma kukhona ukubandakanyeka komzimba, i-steroids iyindlela yokwelapha yokuqala. Ngenxa yokuthi ukusetshenziswa kwe-steroid isikhathi eside kuhlotshaniswa nemiphumela emibi eminingi, uma izimpawu zilawulwa, ungadluliselwa kwenye imithi efana ne-hydroxyurea, i-alpha interferon, noma i-methotrexate yokunakekelwa okuqhubekayo.
Njengoba kubonwe ngenhla, i-steroids iyindlela yokwelashwa okuyisisekelo. Ngaphambi kokuqala i-steroids kubalulekile ukucacisa ukuthi usengozini yokutheleleka kwegciwane elibizwa nge-Strongyloides. I-steroid ingadala kakhulu ukutheleleka kweStongyloides kakhulu. Uma udinga ukwelashwa okuphuthumayo futhi kukhona ukukhathazeka ukuthi une-infection ye-Strongyloides, uzokwelapha ukutheleleka (imithi ngomlomo izinsuku ezimbili).
Izwi elivela
Ukufunda ukuthi une-hypereosinophilic syndrome ingasabisa. Ngenhlanhla, iningi labantu abane-HES ngeke lidinga ukwelashwa. Kulabo abenzayo, kunezinketho eziningana futhi ngisho nakakhulu njengamanje befundelwa.
> Imithombo
> Roufosse F, Klion AD, i-Weller PF. Ama-hyndereosinophilic syndromes: Ukubonakaliswa kwemitholampilo, ukuphazamiseka kwemithi, nokuxilongwa kanye ne-Hypereosinophilic syndromes: Ukwelashwa. Ku: Phezulu, I-post TW (Ed), UpToDate, Waltham, MA. (Kufinyelelwe ngo-Julayi 5, 2016.)