I-Myelofibrosis: kuyini, izimpawu, izimbangela kanye nokwelashwa

-Delile

• Izimpawu ze-Myelofibrosis
• Kungani kwenzeka
• Ukuxilongwa kwe-myelofibrosis
• Yelashwa kanjani

I-Myelofibrosis uhlobo lwesifo esingajwayelekile olwenzeka ngenxa yezinguquko eziholela ekuguqulweni komnkantsha wamathambo, okuholela ekuphazamisekeni ngenkathi yokwanda kwamaseli nokusayina. Njengomphumela wokuguquka, kukhona ukwanda ekukhiqizweni kwamaseli angajwayelekile aholela ekwakhekeni kwezibazi emnkantsheni wethambo ngokuhamba kwesikhathi.

Ngenxa yokwanda kwamaseli angajwayelekile, i-myelofibrosis iyingxenye yeqembu lezinguquko ze-hematological eyaziwa ngokuthi i-myeloproliferative neoplasia. Lesi sifo sinokuvela okuhamba kancane futhi, ngakho-ke, izimpawu nezimpawu zivela kuphela ezigabeni ezithuthuke kakhulu zesifo, kepha kubalulekile ukuthi ukwelashwa kuqalwe ngokushesha nje lapho kutholakala ukuxilongwa ukuvimbela ukuqhubeka kwesifo nokwanda isibonelo, i-leukemia.

Ukwelashwa kwe-myelofibrosis kuya ngeminyaka yomuntu nezinga le-myelofibrosis, futhi kungadingeka ukwenza ukufakelwa umnkantsha ukuze welaphe lowo muntu, noma ukusetshenziswa kwemithi esiza ukuqeda izimpawu nokuvikela ukuqhubeka kwesifo.

Izimpawu ze-Myelofibrosis

I-Myelofibrosis yisifo sokuziphendukela kwemvelo okuhamba kancane futhi, ngakho-ke, asiholeli ekubonakaleni kwezimpawu nasezimpawu ekuqaleni kwezifo. Izimpawu zivame ukuvela lapho isifo sesithuthuke kakhulu, futhi kungahle kube khona:

• Ukushoda kwegazi;
• Ukukhathala ngokweqile nobuthakathaka;
• Ukuphefumula okufishane;
• Isikhumba esiphaphathekile;
• Ukungakhululeki esiswini;
• Imfiva;
• Umjuluko wasebusuku;
• Ukutheleleka njalo;
• Ukwehla kwesisindo nokudla;
• Isibindi esikhulisiwe nobende;
• Ubuhlungu emathanjeni nasemalungeni.

Njengoba lesi sifo sinokuguquguquka okuhamba kancane futhi singenazo izimpawu zesici, ukuxilongwa kuvame ukwenziwa lapho umuntu eya kudokotela ukuze aphenye ukuthi kungani evame ukuzizwa ekhathele futhi, kusukela ekuhlolweni okwenziwe, kungenzeka ukuqinisekisa ukuxilongwa.

Kubalulekile ukuthi ukuxilongwa nokwelashwa kuqalwe ezigabeni zokuqala zesifo ukuze kugwenywe ukuvela kwesifo kanye nokwakhiwa kwezinkinga, njengokuvela kwe-leukemia enamandla nokuhluleka komzimba.

Kungani kwenzeka

I-Myelofibrosis yenzeka njengomphumela wezinguquko ezenzeka kwi-DNA futhi eziholela ezinguquko enqubeni yokukhula kwamangqamuzana, ukwanda nokufa.Lezi zinguquko ziyatholakala, okungukuthi, azizalwanga ngofuzo ngakho-ke, ingane yomuntu one-myelofibrosis ngeke ibe nalesi sifo. Ngokuya ngemvelaphi yayo, i-myelofibrosis ingahlukaniswa nge:

• I-myelofibrosis eyinhloko, engenasizathu esithile;
• I-myelofibrosis yesibili, okuwumphumela wokuvela kwezinye izifo ezifana nomdlavuza we-metastatic kanye ne-thrombocythemia ebalulekile.

Cishe ama-50% wamacala we-myelofibrosis alungile ekushintsheni kohlobo lukaJanus Kinase (JAK 2), olubizwa nge-JAK2 V617F, lapho, ngenxa yokuguquka kwalolu hlobo, kuba noguquko enqubweni yokutshengiswa kwamaseli, okuholela kokutholakele kwesifo kwaselebhu. Ngaphezu kwalokho, kwatholakala ukuthi abantu abane-myelofibrosis nabo babene-MPL gene mutation, nayo ehlobene nezinguquko kwinqubo yokwanda kwamaseli.

Ukuxilongwa kwe-myelofibrosis

Ukuxilongwa kwe-myelofibrosis kwenziwa yi-hematologist noma i-oncologist ngokuhlola izimpawu nezimpawu ezethulwe ngumuntu nomphumela wezivivinyo eziceliwe, ikakhulukazi ukubalwa kwegazi nokuhlolwa kwamangqamuzana ukukhomba ukuguquka okuhlobene nalesi sifo.

Ngesikhathi sokuhlolwa kwezimpawu nokuhlolwa komzimba, udokotela angabona futhi i-splenomegaly ethintekayo, ehambelana nokwandiswa kwepeni, okuyilungu elibhekele ukubhujiswa nokukhiqizwa kwamaseli egazi, kanye nomnkantsha wamathambo. Kodwa-ke, njengakwi-myelofibrosis umnkantsha ukhubazekile, ukugcwala ngokweqile kobende kuyaphela, kuholele ekukhulisweni kwawo.

Isibalo segazi lomuntu one-myelofibrosis sinezinguquko ezithile ezichaza izimpawu ezethulwe ngumuntu futhi zikhombisa izinkinga emnkantsheni wethambo, njengokunyuka kwenani lama-leukocyte nama-platelets, ubukhona bama-platelet amakhulu, ukwehla kwenani kwamangqamuzana egazi abomvu, ukwanda kwenani lama-erythroblast, okungamaseli abomvu angavuthwa, nokuba khona kwama-dacryocyte, okungamaseli abomvu egazi asesimweni sedonsi futhi ngokuvamile abonakala ejikeleza egazini lapho kukhona izinguquko emnkantsheni. Funda kabanzi ngama-dacryocyte.

Ngaphezu kokubalwa kwegazi, kwenziwa ukuhlolwa kwe-myelogram nokuhlolwa kwamangqamuzana ukuqinisekisa ukuxilongwa. I-myelogram ihlose ukukhomba izimpawu ezikhombisa ukuthi umnkantsha uthikamezekile, ezimweni lapho kuba khona izimpawu ezibonisa i-fibrosis, i-hypercellularity, inani elikhulu lamaseli avuthiwe emnkantsheni wethambo nokwanda kwenani lama-megakaryocyte, okuyizimbangela zangaphambi ngamaplatelet. I-myelogram ukuhlolwa okungafuneki futhi, ukuze kwenziwe, kuyadingeka ukufaka i-anesthesia yendawo, njengoba kusetshenziswa inaliti ewugqinsi ekwazi ukufinyelela engxenyeni yangaphakathi yethambo nokuqoqa izinto zomnkantsha. Qonda ukuthi i-myelogram yenziwa kanjani.

Ukuxilongwa kwamangqamuzana kwenzelwa ukuqinisekisa lesi sifo ngokukhomba ukuguqulwa kwe-JAK2 V617F ne-MPL, okuyizinkomba ze-myelofibrosis.

Yelashwa kanjani

Ukwelashwa kwe-myelofibrosis kungahluka ngokuya ngobukhulu besifo nobudala bomuntu, futhi kokunye kunganconywa ukusetshenziswa kwezidakamizwa ze-JAK inhibitor, ukuvimbela ukuqhubeka kwesifo nokuqeda izimpawu.

Ezimweni zobungozi obuphakathi nendawo nobukhulu, ukufakelwa umnkantsha wethambo kunconywa ukuze kukhuthazwe umsebenzi ofanele womnkantsha futhi, ngakho-ke, kungenzeka ukukhuthaza ukuthuthuka. Yize kuwuhlobo lwezokwelapha olukwaziyo ukukhuthaza ukwelashwa kwe-myelofibrosis, ukufakelwa umnkantsha wethambo kunolaka futhi kuhlotshaniswa nezinkinga eziningana. Bona okuningi mayelana nokufakelwa komnkantsha kanye nezinkinga.