I-myeloproliferative neoplasms (i-MPN), ebizwa ngokuthi yi-myeloproliferative disorders, i-group of disorders ibonisa ukukhipha ngokweqile kwesisodwa segazi noma ngaphezulu (amaseli egazi amhlophe, amangqamuzana egazi abomvu, kanye / noma amaplatelets). Nakuba ungase uhlanganise igama elithi neoplasm nomdlavuza, akusikho lokho okucacile.
I-Neoplasm ichazwa njengokukhula okungavamile kwezicubu ezibangelwa ukuguqulwa komzimba futhi zingahle zihlukaniswe njengezinhle (ezingekho komdlavuza), zangaphambi komdlavuza, noma ezinomdlavuza (ezimbi).
Lapho kutholakala ukuthi i-myeloproliferative neoplasms iyingozi kodwa ngokuhamba kwesikhathi ingaba isifo esibi (umdlavuza). Le ngozi yokuthola umdlavuza nalezi zifo zifakazela ukubaluleka kokulandelwa okulandelayo ne-hematologist yakho.
Ukuhlukaniswa kwe-myeloproliferative neoplasms kushintshile kakhulu eminyakeni embalwa edlule kodwa sizobukeza izigaba jikelele lapha.
I-Classic Myeloproliferative Neoplasms
I-"classic" i-myeloproliferative neoplasms ihlanganisa:
- I-Polycythemia vera (PV): imiphumela ye-PV evela ekuguquleni kwezakhi zofuzo okubangela ukwedlula ngokweqile amaseli egazi abomvu. Ngezinye izikhathi i-white cell kanye ne-platelet izibalo nazo ziphakanyisiwe. Lokhu kuphakama kokubala kwegazi kukwandisa ingozi yakho yokuthuthukisa i-clot yegazi. Uma uthola ukuthi une-PV, unethuba elincane lokuthuthukisa i-myelofibrosis noma umdlavuza.
- I-Essential Thrombocythemia (ET): Ukuguqulwa kwezakhi zofuzo ku-ET kwenza kube nokukhiqizwa ngokweqile kwamaplatelets. Inombolo ekhuphukile yamaplatelet ekusakazeni yandisa ingozi yokuthuthukisa i-blood clot. Ingozi yakho yokuthola umdlavuza uma ithola ukuthi i-ET incane kakhulu. I-ET iyingqayizivele phakathi kwama-MPN njengoba kutholakala ukuxoshwa. Lokhu kusho ukuthi udokotela wakho uzolawula ezinye izimbangela zokubala kweplatelet ephakeme ( thrombocytosis ) kuhlanganise namanye ama-MPN.
- I-Primary Myelofibrosis (PMF): I- myelofibrosis eyinhloko ibizwa ngokuthi idiopathic myelofibrosis noma i-agnogenic myeloid metaplasia. Ukuguqulwa kwezakhi zofuzo ku-PMF kubangela ukuhluleka (fibrosis) emnothweni wethambo. Lokhu kutholakala kwenza kube nzima kuwe ngomnkantsha wakho ukwenza amangqamuzana egazi amasha. Ngokuphambene ne-PV, i-PMF ngokuvamile ibangela i-anemia (inani elibomvu lesisindo segazi). Ukubalwa kwamaseli amhlophe wegazi kanye nesamba seplatelet kungandiswa noma kunciphise.
- I-Chronic Myeloid Leukemia (CML) : I-CML ingabuye ibizwe ngokuthi i-leukemia engapheliyo. Imiphumela ye-CML kusukela ekuguqulweni kwezakhi zofuzo okuthiwa i-BCR / ABL1. Lokhu kuguqulwa kubangelwa ukukhiqizwa ngokweqile kwama-granulocyte, uhlobo lwe-white cell cell. Okokuqala, kungenzeka ukuthi awunayo izimpawu futhi i-CML ivame ukutholakala ngokuzenzakalelayo nomsebenzi webhulethi wesikhashana.
I-Atypical Myeloproliferative Neoplasms
Ama-neoplasms "ase-atypical" ase-atypical ahlanganisa:
- I-Juvenile Myleomonocyic Leukemia (JMML): I- JMML ibizwa ngokuthi i-CML yabantwana. Kuyindlela engavamile ye-leukemia eyenzeka ekusaneni nasebuntwaneni. Umnkantsha wamathambo ukhiqiza amangqamuzana egazi amhlophe aseMyeloid, ikakhulukazi okuthiwa i-monocyte (monocytosis). Izingane ezine-neurofibromatosis uhlobo I no-Noonan syndrome zisengozini enkulu yokuthuthukisa i-JMML kunabantwana ngaphandle kwezimo zofuzo.
- I-Neutrophilic Leukemia Esingapheli: I-leukemia engapheliyo ye-neutrophilic iyisifo esingavamile esichazwa ngokweqile kwama-neutrophils, uhlobo lwamaseli amhlophe egazi. Lawa maseli angakwazi ukungenelela ezingxenyeni zakho futhi abangele ukwandiswa kwesibindi nesipere (i-hepatosplenomegaly).
- I-Eosinophilic Leukemia / Hypereosinophilic Syndromes (HES): I-eosinophilic leukemia engapheliyo kanye ne-hypereosinophilc syndromes ibonisa iqembu lezinkinga ezibhekene nenani elikhulayo lama-eosinophils (i-eosinophilia) elidala umonakalo ezithombeni ezihlukahlukene. Isibalo esithile se-HES senza okuningi njengama-neoplasms angu-myleoproliferative (ngakho-ke igama le-eosinophilic leukemia).
- I-Mast Cell Disease: Systemic (okusho yonke umzimba) isifo sesisindo se-mast noma i-mastocytosis yinto ehlanganisa kakhulu nesigaba se-myeloproliferative neoplasms. Izifo zesisindo se-mast zivela ekukhiqizeni ngokweqile kwamangqamuzana omzimba, uhlobo lwesigungu segazi elimhlophe bese lihlasela emnkantsheni wamathambo, umgudu wamathumbu, isikhumba, i-spleen nesibindi. Lokhu kuqhathaniswa ne-mastocytosis enomuthi echaphazela kuphela isikhumba. Amangqamuzana amancane akhipha i-histamine eholele ekutheni inhlobo ye-allergen iphendulwe kanjani.
Imithombo:
URoufosse F, iKlion AD ne-Weller PF. Ama-hyndereosinophilic syndromes: Ukubonakaliswa kwemitholampilo, ukuguqulwa kwamathambo, nokuxilongwa. Ku: Phezulu, I-post TW (Ed), UpToDate, Waltham, MA. (Kufinyelelwe ngoJuni 27, 2016.)
I-Teferri A. Uhlolojikelele lwe-neoplasms ye-myeloproliferative. Ku: Phezulu, I-post TW (Ed), UpToDate, Waltham, MA. (Kufinyelelwe ngoJuni 26, 2016.)
UVan Eatton RA. Ukubonakaliswa kwemitholampilo nokuxilongwa kwe-myeloid leukemia engapheli. Ku: Phezulu, I-post TW (Ed), UpToDate, Waltham, MA. (Kufinyelelwe ngoJuni 28, 2016.)