I-Myelofibrosis

I-Myelofibrosis ukuphazamiseka komnkantsha wamathambo lapho umongo wawo uthathelwa indawo izicubu ezibomvu ezinemicu.

Umnkantsha wamathambo yizicubu ezithambile, ezinamafutha ngaphakathi kwamathambo akho. Ama-stem cell amangqamuzana angavuthiwe emnkantsheni wethambo akhula abe ngamangqamuzana akho egazi. Igazi lakho lenziwa:

• Amaseli abomvu egazi (ahambisa i-oxygen ezicutshini zakho)
• Amaseli egazi amhlophe (alwa nokutheleleka)
• Ama-Platelet (asiza igazi lakho)

Lapho umongo wethambo unesibazi, awukwazi ukwenza amangqamuzana egazi anele. Ukushoda kwegazi, izinkinga zokopha, kanye nengozi ephezulu yokutheleleka kungenzeka.

Ngenxa yalokho, isibindi nobende kuzama ukwenza amanye alawa maseli egazi. Lokhu kwenza ukuthi lezi zitho zivuvuke.

Imbangela ye-myelofibrosis ivame ukungaziwa. Azikho izinto eziyingozi ezaziwayo. Uma kwenzeka, kuvame ukukhula kancane kubantu abangaphezu kweminyaka engama-50. Abesifazane nabesilisa bathinteka ngokulinganayo. Kukhona okwenzekayo okwandayo kwalesi simo kuma-Ashkenazi amaJuda.

Amagciwane omdlavuza wegazi namathambo, njenge-myelodysplastic syndrome, i-leukemia, ne-lymphoma, nawo angadala isibazi somnkantsha. Lokhu kubizwa nge-myelofibrosis yesibili.

Izimpawu zingafaka noma yikuphi okulandelayo:

• Ukugcwala esiswini, ubuhlungu, noma ukuzizwa ugcwele ngaphambi kokuqeda ukudla (ngenxa yobende obukhulisiwe)
• Ubuhlungu bamathambo
• Ukopha okulula, ukulinyazwa
• Ukukhathala
• Ukwanda kwamathuba okuthola ukutheleleka
• Isikhumba esiphaphathekile
• Ukuphefumula kanzima ngokuzivocavoca umzimba
• Ukwehla kwesisindo
• Ukujuluka ebusuku
• Umkhuhlane webanga eliphansi
• Isibindi esandisiwe
• Ukukhwehlela okomile
• Isikhumba esilumayo

Umhlinzeki wezokunakekelwa kwempilo uzokwenza ukuhlolwa komzimba abuze ngezimpawu.

Ukuhlolwa okungenziwa kufaka phakathi:

• Qedela ukubalwa kwegazi (i-CBC) nge-smear yegazi ukuhlola izinhlobo ezahlukahlukene zamaseli egazi
• Ukulinganisa ukulimala kwezicubu (ileveli ye-LDH enzyme)
• Ukuhlolwa kofuzo
• I-bone marrow biopsy ukuxilonga lesi simo nokuhlola umdlavuza womnkantsha

Umnkantsha wethambo noma ukufakelwa kwe-stem cell kungathuthukisa izimpawu, futhi kungaselapha lesi sifo. Le ndlela yokwelashwa ivame ukubhekelwa abantu abasha.

Olunye ukwelashwa kungabandakanya:

• Ukumpontshelwa igazi nemithi yokulungisa ukushoda kwegazi
• Imisebe kanye nokwelashwa ngamakhemikhali
• Imithi ehlosiwe
• Ukususwa kobende (splenectomy) uma ukuvuvukala kubangela izimpawu, noma ukusiza nge-anemia

Njengoba lesi sifo siba sibi, umnkantsha wethambo uyeka kancane ukusebenza. Ukubalwa kweplatelet okuphansi kuholela ekopheni okulula. Ukuvuvukala kobende kungahle kube kubi kakhulu kanye ne-anemia.

Ukusinda kwabantu abane-myelofibrosis eyinhloko cishe iminyaka emihlanu. Kepha abanye abantu basinda amashumi eminyaka.

Izinkinga zingafaka:

• Ukuthuthukiswa kwe-acute myelogenous leukemia
• Izifo
• Ukopha
• Amahlule egazi
• Ukuhluleka kwesibindi

Yenza i-aphoyintimenti nomhlinzeki wakho uma unezimpawu zalesi sifo. Funa ukunakekelwa kwezokwelapha khona manjalo ukopha okungalawuleki, ukuphefumula okuncane, noma i-jaundice (isikhumba esiphuzi kanye namhlophe wamehlo) okuya ngokuya kuba kubi.

Idiopathic myelofibrosis; I-Myeloid metaplasia; I-Agnogenic myeloid metaplasia; I-myelofibrosis eyinhloko; I-myelofibrosis yesibili; Umnkantsha wamathambo - i-myelofibrosis

IGotlib J. Polycythemia vera, i-thrombocythemia ebalulekile, kanye ne-myelofibrosis eyinhloko. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 157.

I-NM ende, iKavanagh EC. I-Myelofibrosis. Ku: Pope TL, Bloem HL, Beltran J, Morrison WB, Wilson DJ, abahleli. Ukufanekisa Imisipha. Okwesibili. IPhiladelphia, PA: Elsevier Saunders; 2015: isahluko 76.

UMascarenhas J, Najfeld V, Kremyanskaya M, Keyzner A, Salama ME, Hoffman R. Primary myelofibrosis. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 70.