Myelofibrosis: yintoni, iimpawu, oonobangela kunye nonyango

Umxholo

• Iimpawu zeMyelofibrosis
• Kutheni le nto isenzeka
• Uxilongo lwe-myelofibrosis
• Unyango lwenziwa njani

I-Myelofibrosis luhlobo olunqabileyo lwesifo esenzeka ngenxa yotshintsho olukhokelela kutshintsho kumongo wethambo, okhokelela kukuphazamiseka kwinkqubo yokwanda kweseli kunye nokusayina. Njengomphumo wokuguquka, kukho ukwanda kwimveliso yeeseli ezingaqhelekanga ezikhokelela ekubunjweni kwamanxeba kumongo wethambo ngokuhamba kwexesha.

Ngenxa yokwanda kweeseli ezingaqhelekanga, i-myelofibrosis yinxalenye yeqela lotshintsho lwehematological olubizwa ngokuba yi-myeloproliferative neoplasia. Esi sifo sinokuvela kancinci kwaye, ke ngoko, iimpawu kunye neempawu zibonakala kuphela kumanqanaba aphezulu esi sifo, nangona kunjalo kubalulekile ukuba unyango luqaliswe kwakamsinya nje ukuba kufunyaniswe isifo ukuthintela ukuqhubela phambili kwesi sifo kunye nokuqhubela phambili umzekelo wegazi, umzekelo.

Unyango lwe-myelofibrosis luxhomekeke kubudala bomntu kunye nenqanaba le-myelofibrosis, kwaye kunokuba yimfuneko ukwenza utyando lomongo wethambo ukunyanga umntu, okanye ukusebenzisa amayeza anceda ukunciphisa iimpawu kunye nokuthintela ukuqhubela phambili kwesi sifo.

Iimpawu zeMyelofibrosis

I-Myelofibrosis sisifo esivela kancinci kwaye, ke ngoko, asikhokeleli ekubonakaleni kweempawu kunye neempawu kwinqanaba lesifo. Iimpawu zihlala zivela xa isifo siqhubele phambili, kwaye kunokubakho:

• Anemia;
• Ukudinwa kakhulu kunye nokuba buthathaka;
• Ukuphefumla okufutshane;
• Ulusu oluthuthu;
• Ukungahambi kakuhle esiswini;
• Ifiva;
• Ukubila ebusuku;
• Rhoqo usulelo;
• Ukuthoba umzimba kunye nokutya;
• Ukwandiswa kwesibindi kunye ne-spleen;
• Ubuhlungu emathanjeni nakumalungu.

Njengokuba esi sifo sinokuvela kancinci kwaye singenazimpawu, isifo sihlala senziwa xa umntu esiya kugqirha eyokuphanda ukuba kutheni beziva bediniwe kwaye, kuvavanyo olwenziwe, kunokwenzeka ukuqinisekisa isifo.

Kubalulekile ukuba isifo kunye nonyango siqaliswe kumanqanaba okuqala esi sifo ukuthintela ukuvela kwesi sifo kunye nokukhula kweengxaki, ezinje ngendaleko ye-leukemia kunye nokusilela kwamalungu.

Kutheni le nto isenzeka

I-Myelofibrosis yenzeka njengesiphumo soguquko olwenzeka kwi-DNA kwaye ekhokelela kutshintsho kwinkqubo yokukhula kweseli, ukwanda kunye nokufa.Olu tshintsho lufunyenwe, oko kukuthi, aluzuzwanga njengelifa kwaye, ke, umntwana womntu one-myelofibrosis akasayi kuba nesifo. Ngokwemvelaphi yayo, i-myelofibrosis inokuhlelwa ibe:

• I-myelofibrosis yaseprayimari, engenasizathu sithile;
• I-myelofibrosis yesibini, esisiphumo sokuvela kwezinye izifo ezinje ngomhlaza we-metastatic kunye ne-thrombocythemia ebalulekileyo.

Phantse i-50% yamatyala e-myelofibrosis alungile kuguquko kuhlobo lukaJanus Kinase (JAK 2), olubizwa ngokuba yiJAK2 V617F, ekuthi, ngenxa yokutshintsha kolu hlobo, kukho utshintsho kwinkqubo yokubonisa iseli, ngenxa kwiziphumo zelebhu zesifo. Ukongeza, kwafunyaniswa ukuba abantu abane-myelofibrosis banayo i-MPL gene mutation, ekwanxulumene notshintsho kwinkqubo yokwanda kweseli.

Uxilongo lwe-myelofibrosis

Ukuxilongwa kwe-myelofibrosis kwenziwa yi-hematologist okanye i-oncologist ngovavanyo lweempawu kunye neempawu ezivezwe ngumntu kunye nesiphumo sovavanyo oluceliweyo, ikakhulu ukubala kwegazi kunye novavanyo lweemolekyuli ukuchonga utshintsho olunxulumene nesi sifo.

Ngexesha lokuvavanywa kweempawu kunye novavanyo lomzimba, ugqirha unokujonga kwakhona i-splenomegaly enokuthambeka, ehambelana nokwandiswa kwepeni, elilungu elijongene nokutshabalalisa kunye nokuveliswa kweeseli zegazi, kunye nomongo wethambo. Nangona kunjalo, kwi-myelofibrosis umongo wethambo awukhubazeki, ukugcwala kwepleen kuyaphela, kukhokelela ekwandisweni kwayo.

Ubalo lwegazi lomntu one-myelofibrosis luneenguqu ezithile ezichaza iimpawu zomntu kwaye zibonisa iingxaki kumongo wethambo, njengokunyuka kwenani leeukocyte kunye neeplatelets, ubukho beeplatelets ezinkulu, ukwehla kwesixa iiseli ezibomvu zegazi, ukwanda kwenani lama-erythroblast, angamaseli abomvu angafakwanga, kunye nokubakho kwee-dacryocyte, eziziiseli ezibomvu zegazi ngohlobo lwethontsi kwaye oluqhele ukubonakala lujikeleza egazini xa kukho utshintsho kumongo. Funda ngakumbi malunga ne-dacryocyte.

Ukongeza kubalo lwegazi, iimvavanyo ze-myelogram kunye neemolekyuli ziyenziwa ukuqinisekisa isifo. I-myelogram ijolise ekuchongeni iimpawu ezibonisa ukuba umongo wethambo usengozini, kwimeko apho kukho iimpawu ezibonisa i-fibrosis, i-hypercellularityity, inani elikhulu leeseli ezivuthiweyo kumongo wethambo kunye nokwanda kwenani lee-megakaryocyte, eziziiseli zangaphambili Iiplatelets. I-myelogram luvavanyo olungenelelayo kwaye, ukuze lwenziwe, kuyimfuneko ukufaka i-anesthesia yendawo, njengoko inaliti eshinyeneyo ekwaziyo ukufikelela kwinxalenye yangaphakathi yethambo kunye nokuqokelela umongo wethambo. Qonda ukuba yenziwa njani i-myelogram.

Ukuxilongwa kweemolekyuli kwenzelwa ukuqinisekisa esi sifo ngokuchonga utshintsho lwe-JAK2 V617F kunye ne-MPL, ezibonisa i-myelofibrosis.

Unyango lwenziwa njani

Unyango lwe-myelofibrosis lungahluka ngokobunzima besifo kunye nobudala bomntu, kwaye kwezinye iimeko kunokucetyiswa ukusetyenziswa kweziyobisi ze-JAK inhibitor, ukuthintela ukuqhubela phambili kwesi sifo kunye nokunciphisa iimpawu.

Kwiimeko eziphakathi kunye nomngcipheko omkhulu, ukufakelwa umongo wethambo kuyacetyiswa ukuze kukhuthazwe umsebenzi ochanekileyo womongo wethambo kwaye, ke, kunokwenzeka ukukhuthaza ukuphucula. Ngaphandle kokuba luhlobo lonyango olunako ukukhuthaza unyango lwe-myelofibrosis, ukufakelwa umongo wethambo kulukhuni kwaye kunxulunyaniswa neengxaki ezininzi. Bona ngakumbi malunga nokufakwa komongo wethambo kunye neengxaki.