I-Myelodysplastic syndrome yisifo segazi. Le pathology idala ukwehla kwenani lamaseli egazi ajikelezayo. Lesi sifo sibizwa nangokuthi: i-myelodysplasia.

Emzimbeni "onempilo", umnkantsha ukhiqiza izinhlobo ezahlukene zamangqamuzana egazi:

- amangqamuzana egazi abomvu, avumela ukunikezwa komoya-mpilo kuwo wonke umzimba;

- amangqamuzana amhlophe egazi, avumela umzimba ukuba ulwe nama-exogenous agents futhi ngaleyo ndlela ugweme ingozi yokutheleleka;

- ama-platelet, avumela amahlule egazi ukuthi akheke futhi angene ohlelweni lwe-coagulation.

Endabeni yeziguli ezine-myelodysplastic syndrome, umnkantsha awusakwazi ukukhiqiza la mangqamuzana abomvu egazi, amangqamuzana egazi amhlophe namaplatelet ngendlela evamile. Amangqamuzana egazi akhiqizwa ngendlela engavamile okuholela ekukhuleni kwawo okungaphelele. Ngaphansi kwalezi zimo ezikhulayo, umnkantsha uqukethe iqoqo lamangqamuzana egazi angavamile abese esakazwa kuwo wonke umgudu wegazi.

Lolu hlobo lwe-syndrome lungakhula kancane noma luthuthuke kakhulu.

 Kunezinhlobo eziningana zesifo: (2)

• i-refractory anemia, kulokhu, kuphela ukukhiqizwa kwamangqamuzana abomvu egazi kuthinteka;
• i-refractory cytopenia, lapho wonke amangqamuzana (amangqamuzana egazi abomvu, amangqamuzana egazi amhlophe namaplatelet) ethinteka;
• i-refractory anemia eqhuma ngokweqile, iphinde ithinte amangqamuzana egazi abomvu, amangqamuzana amhlophe egazi nama-platelet futhi okuholela engcupheni eyengeziwe yokuba ne-acute leukemia.

I-Myelodysplastic syndrome ingathinta abantu bayo yonke iminyaka. Kodwa-ke, izihloko ezithinteka kakhulu ziphakathi kweminyaka engama-65 nengama-70 ubudala. Isiguli esisodwa kuphela kweziyisihlanu ezineminyaka engaphansi kuka-50 singathinteka yilesi sifo. (2)

Iningi labantu abanalesi sifo liba nezimpawu ezithambile kuya kwezincane ekuqaleni. Lokhu kubonakaliswa komtholampilo kuba nzima kamuva.

Izimpawu zalesi sifo zihlotshaniswa nezinhlobo ezahlukene zamangqamuzana egazi athintekayo.

Uma kwenzeka ukuthi amangqamuzana abomvu egazi ethintekile, izimpawu ezihambisanayo yilezi:

• ukukhathala;
• ubuthakathaka;
• ubunzima bokuphefumula.

Esimweni lapho amangqamuzana amhlophe egazi ethinteka, ukubonakaliswa komtholampilo kubangela:

• ukwanda kwengozi yokutheleleka okuhlobene nokuba khona kwamagciwane (amagciwane, amabhaktheriya, ama-parasites, njll.).

Lapho ukuthuthukiswa kweplatelet kuthinteka, sivame ukuqaphela:

• ukopha kakhulu kanye nokubonakala kokulimaza ngaphandle kwesizathu esiyisisekelo.

Ezinye izinhlobo ze-myelodysplastic syndrome zifana nokubonakaliswa komtholampilo ezikhula ngokushesha kunezinye.

Ngaphezu kwalokho, ezinye iziguli zingase zingabonakali ngezimpawu zesici. Ngakho-ke ukuhlonzwa kwalesi sifo kwenziwa ngemva kokuhlolwa kwegazi, okubonisa izinga eliphansi ngokungavamile lamangqamuzana egazi ajikelezayo kanye nokukhubazeka kwawo.

Izimpawu zesifo zihlotshaniswa ngqo nohlobo lwaso. Ngempela, esimweni se-refractory anemia, izimpawu ezizovela ziyoba ukukhathala, imizwa yobuthakathaka kanye nokuba nokwenzeka kobunzima bokuphefumula. (2)

Abanye abantu abane-myelodysplastic syndrome bangaba ne-acute myeloid leukemia. Kungumdlavuza wamangqamuzana amhlophe egazi.

Umsuka oqondile we-myelodysplastic syndrome awukaziwa ngokugcwele.

Kodwa-ke, ubudlelwano bembangela nomphumela bubekwe phambili ukuze kutholakale izinhlanganisela zamakhemikhali ezithile, njenge-benzene, kanye nokuthuthukiswa kwe-pathology. Le nto yamakhemikhali, ehlukaniswa njenge-carcinogenic kubantu, itholakala kakhulu embonini yokwenza amapulasitiki, irabha noma imboni ye-petrochemical.

Ezimweni ezingavamile, ukuthuthukiswa kwalesi sifo kungahlotshaniswa ne-radiotherapy noma i-chemotherapy. Lezi izindlela ezimbili ezisetshenziswa kakhulu ekwelapheni umdlavuza. (2)

Izici eziyingozi zesifo yilezi:

- ukuchayeka kumakhemikhali athile, njenge-benzene;

- Ukwelashwa okuyisisekelo nge-chemotherapy kanye / noma i-radiotherapy.

Ukuxilongwa kwe-myelodysplastic syndrome kuqala ngokuhlolwa kwegazi kanye nokuhlaziywa kwamasampula omnkantsha. Lokhu kuhlola kusiza ukunquma inani lamaseli egazi avamile nangavamile.

Ukuhlaziywa komnkantsha wethambo kwenziwa ngaphansi kwe-anesthesia yendawo. Isampula layo livamise ukuthathwa okhalweni loxoxwayo bese lihlaziywa ngesibonakhulu elabhorethri.

Ukwelashwa kwalesi sifo kuncike ngqo ohlotsheni lwesifo kanye nezimo eziqondene nomuntu ngamunye.

Inhloso yokwelashwa ukubuyisela izinga elijwayelekile lamangqamuzana egazi ajikelezayo kanye nokuma kwawo.

Esimeni lapho isiguli sethula uhlobo lwesifo esinengozi ephansi yokushintsha sibe umdlavuza, incwadi kadokotela yokwelashwa okuthile ngeke ngempela isebenze kodwa izodinga kuphela ukuqapha okuvamile ngokuhlolwa kwegazi.

 Ukwelashwa kwezinhlobo ezithuthuke kakhulu zesifo yilezi:

• ukumpontshelwa igazi;
• izidakamizwa zokulawula insimbi egazini, ngokuvamile ngemva kokumpontshelwa igazi;
• ukujova izici zokukhula, njenge-erythropoietin noma i-G-CSFs, ukuze kukhuliswe ukukhula kwamangqamuzana egazi futhi kusize umnkantsha wamathambo ukhiqize amangqamuzana egazi;
• ama-antibiotics, ekwelapheni izifo ezibangelwa ukuntuleka kwamangqamuzana amhlophe egazi.

Ngaphezu kwalokho, izidakamizwa zohlobo: i-anti-thymocyte immunoglobulins (ATG) noma i-cyclosporine, inciphisa umsebenzi wesimiso sokuzivikela komzimba esivumela umnkantsha ukuba ukhiqize amangqamuzana egazi.

Ezifundweni ezisengozini enkulu yokuba nomdlavuza, i-chemotherapy ingase inqunywe noma ngisho nokufakelwa kwe-stem cell.

I-Chemotherapy ibhubhisa amangqamuzana egazi angavuthiwe ngokumisa ukukhula kwawo. Inganqunywa ngomlomo (amaphilisi) noma ngomjovo.

Lokhu kwelashwa kuvame ukuhlotshaniswa nalokhu:

- i-cytarabine;

- i-fludarabine;

- i-daunorubicine;

- clofarabine;

- i-azacitidine.

Ukufakelwa kwe-stem cell kusetshenziswa ngendlela enzima yesifo. Kulo mongo, ukufakelwa kabusha kwamaseli we-stem kwenziwa kangcono ezifundweni ezincane.

Lokhu kwelashwa kuvame ukuhlanganiswa ne-chemotherapy kanye / noma i-radiotherapy yangaphambi kwesikhathi. Ngemuva kokubhujiswa kwamangqamuzana egazi athintwe yi-syndrome, ukufakelwa kwamaseli anempilo kungasebenza. (2)