Myelofibrosis

I-Myelofibrosis kukuphazamiseka komongo wethambo apho umongo uthatyathelwa indawo sisihlunu esinefibrous scar.

Umongo wethambo sisithambo esithambileyo esinamafutha ngaphakathi kwamathambo akho. Iiseli zesinti ziiseli ezingakhuliyo kumongo wethambo okhula ube ziiseli zakho zegazi. Igazi lakho lenziwa:

• Iiseli ezibomvu zegazi (ezithwala ioksijini kwizicubu zakho)
• Amaseli egazi amhlophe (alwa nosulelo)
• Iiplatelets (ezinceda igazi lakho)

Xa umongo wethambo unqabile, awunako ukwenza iiseli zegazi ezaneleyo. I-anemia, iingxaki zokopha, kunye nomngcipheko ophezulu wosulelo.

Ngenxa yoko, isibindi kunye ne-spleen zizama ukwenza ezinye zeeseli zegazi. Oku kubangela ukuba la malungu adumbe.

Unobangela we-myelofibrosis uhlala engaziwa. Akukho bungozi baziwayo. Xa isenzeka, ihlala ikhula kancinci kubantu abangaphezulu kweminyaka engama-50. Abasetyhini kunye namadoda bayachaphazeleka ngokulinganayo. Kukho ukwanda okwenzekayo kule meko e Ashkenazi amaYuda.

Igazi kunye nomhlaza womhlaza wethambo, njenge-myelodysplastic syndrome, i-leukemia, kunye ne-lymphoma, kunokubangela ukuba umongo wethambo ubonakale. Oku kubizwa ngokuba yi-myelofibrosis yesibini.

Iimpawu zinokubandakanya nayiphi na kwezi zinto zilandelayo:

• Ukugcwala kwesisu, iintlungu, okanye ukuziva ugcwele ngaphambi kokugqiba ukutya (ngenxa yethambo elandisiweyo)
• Intlungu yethambo
• Ukopha ngokulula, ukugruzuka
• Ukudinwa
• Ukwanda kwamathuba okufumana usulelo
• Ulusu oluthuthu
• Ukuphefumla kancinci ngokwenza umthambo
• Ukuhla ukusinda
• Ukubila ebusuku
• Umkhuhlane ophantsi
• Ukwandiswa kwesibindi
• Ukukhohlela owomileyo
• Ulusu olurhawuzayo

Umboneleli wezempilo uya kwenza uvavanyo lomzimba kwaye abuze malunga neempawu.

Uvavanyo olunokwenziwa lunokubandakanya:

• Gcwalisa ubalo lwegazi (CBC) ngegazi smear ukujonga iintlobo ezahlukeneyo zeeseli zegazi
• Ukulinganisa ukonakala kwetishu (inqanaba le-enzyme ye-LDH)
• Uvavanyo lwemfuzo
• I-bone marrow biopsy ukufumanisa imeko kunye nokujonga umhlaza womongo wethambo

Umongo wethambo okanye ukufakelwa kweeseli ezinesiqu kungaphucula iimpawu, kwaye kungasinyanga isifo. Olu nyango luhlala luqwalaselwa kubantu abancinci.

Olunye unyango lunokubandakanya:

• Utofelo-gazi kunye namayeza ukulungisa i-anemia
• Imitha kunye nekhemotherapy
• Amayeza ekujoliswe kuwo
• Ukususa udakada (splenectomy) ukuba ukudumba kubangela iimpawu, okanye ukunceda ukuba neanemia

Njengokuba esi sifo siba mandundu, umongo wethambo uthi chu uyeke ukusebenza. Inani eliphantsi leplatelet likhokelela ekopha ngokulula. Ukudumba kwamathambo kunokuba kubi ngakumbi kunye ne-anemia.

Ukusinda kwabantu abane-myelofibrosis yokuqala malunga neminyaka emi-5. Kodwa abanye abantu basinda amashumi eminyaka.

Iingxaki zinokubandakanya:

• Uphuhliso lwegazi le-acute
• Usulelo
• Ukopha
• Amahlwili egazi
• Ukungaphumeleli kwesibindi

Yenza idinga nomnikezeli wakho ukuba unempawu zesi sifo. Funa ukhathalelo lwezonyango kwangoko ukopha okungalawulwayo, ukuphefumla kancinci, okanye i-jaundice (isikhumba esimthubi kunye nabamhlophe bamehlo) esiba mandundu.

Idiopathic myelofibrosis; IMetaplasia ye-Myeloid; I-Agnogenic myeloid metaplasia; I-myelofibrosis yaseprayimari; I-myelofibrosis yesibini; Umongo wethambo - i-myelofibrosis

IGotlib J. Polycythemia vera, itrombocythemia ebalulekileyo, kunye ne-myelofibrosis ephambili. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl. 157.

Ixesha elide i-NM, iKavanagh EC. Myelofibrosis. Ku: Pope TL, Bloem HL, Beltran J, Morrison WB, Wilson DJ, ii-eds. Ukujonga iMusculoskeletal. Ngomhla wesi-2. IPhiladelphia, PA: Elsevier Saunders; 2015: isahluko 76.

UMascarenhas J, uNajfeld V, uKremyanskaya M, uKeyzner A, uSalama ME, uHoffman R. wamabanga aphambili e-myelofibrosis. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 70.