Obusigo bwo obw’amagumba bukola ebitundu ebirimu ebiwuziwuzi? Ka tuyige ku bulwadde bwa myelofibrosis!

Wali owuliddeko ku bulwadde obuyitibwa myelofibrosis? Mu butuufu kika kya kookolo w’omusaayi ekitali kya bulijjo nnyo. Mu ngeri ennyangu, bwe buba ng’obusimu bw’amagumba , nga buno kye kitundu ekigonvu era ekiringa sipongi mu magumba go, bukyusiddwamu ebitundu by’enkovu ebirimu ebiwuziwuzi . Era kika kya leukemia ekimala ebbanga eddene, era kiri mu kibinja ekiyitibwa myeloproliferative disorders. Obuzibu bw’okukula kw’omusaayi (myeloproliferative disorders) bwe buba bw’obutoffaali bw’omusaayi bungi nnyo mu busimu bw’amagumba, obutoffaali bw’omusaayi gye bukolebwa.

Ddala obulwadde bwa myelofibrosis kye ki?

Kino ka tukitunuulire mu bujjuvu. Obusigo bw’amagumba go bulimu obutoffaali obusibuka mu mubiri obukola omusaayi . Oluvannyuma obutoffaali buno obusibuka mu musaayi bukula ne bufuuka obutoffaali obumyufu, obutoffaali obweru, n’obutoffaali obuyitibwa platelets. Naye mu muntu alina obulwadde bwa myelofibrosis, enkyukakyuka ebaawo mu buzaale oba DNA mu bumu ku butoffaali buno obusibuka. Kino kireetera akatoffaali okufuuka akatoffaali ka kookolo akalema. Olwo akatoffaali kano akalema ne kaawukana ne kayisa enkyukakyuka eyo mu butoffaali bwonna obupya bwe kakola.

Ekiseera bwe kigenda kiyitawo, obutoffaali buno obwa kookolo obutali bwa bulijjo buzimba mu bungi. Obumu ku butoffaali buno buleeta okuzimba mu busimu bw’amagumba. Okuzimba kuno kwe kuleetera ekitundu ky’enkovu kye nnayogeddeko emabegako okutondebwa. Kale, olw’ekitundu kino eky’enkovu n’obutoffaali bwa kookolo obuyitiridde, obusigo bw’amagumba bufiirwa obusobozi bwabwo okukola obutoffaali bw’omusaayi obulamu.

Bika ki ebikulu eby’obulwadde bwa myelofibrosis?

Waliwo ebika bibiri ebikulu eby’obulwadde bwa myelofibrosis.

• Primary myelofibrosis: Kino kye kika ekibaawo mu ngeri eyeetongodde, nga tekirina kakwate konna na bulwadde bulala bwonna.
• Secondary myelofibrosis: Kino kiva ku buzibu obulala mu musaayi. Okugeza, kiyinza okuva ku ndwadde nga primary thrombocytosis oba polycythemia vera. Ekika kino eky’okubiri kikola wakati wa 10% ne 20% ku balwadde abazuuliddwa.

Obulwadde buno obuyitibwa Myelofibrosis butera kwenkana wa?

Myelofibrosis mu butuufu mbeera etali ya bulijjo nnyo . Ng’ekyokulabirako, mu Amerika, kigambibwa nti obulwadde buno bubeera mu bantu nga 1.5 ku buli 100,000 buli mwaka. Kiyinza okukwata omuntu yenna, ka kibeere myaka ki. Tewali njawulo ya myaka, naye esinga kuzuulibwa mu bantu abasukka mu myaka 50. Singa obulwadde bwa myelofibrosis buba mu baana abato, butera okuzuulibwa nga tebannaweza myaka 3.

Obulwadde bwa myelofibrosis buyinza kukola ki ku mubiri gwo?

Obutoffaali bw’omusaayi bwe bukolebwa mu ngeri etaali ya bulijjo mu ngeri eno, ebizibu eby’enjawulo bisobola okubaawo. Ka tulabe kiki kye bali:

• Okukendeera kw’omusaayi: Eno mbeera ng’obutoffaali obumyufu tebumala. Nga bw’omanyi, obutoffaali obumyufu bwe butambuza omukka gwa oxygen mu mubiri gwonna. N’olwekyo obutoffaali obumyufu bwe butamala, ebitundu by’omubiri tebifuna mukka gwa oxygen gumala. Kino kiyinza okuleeta obubonero ng’obukoowu , obunafu, n’okussa obubi .

• Thrombocytopenia: Eno mbeera nga platelets ntono nnyo. Platelets ze ziyamba omusaayi okuzimba nga waliwo obuvune. N’olwekyo omuwendo gw’obutoffaali bw’omusaayi bwe guba mutono, omubiri gwo guyinza okuvaamu omusaayi n’okunyiga .

• Splenomegaly: Ensigo yo efuga obutoffaali bw’omusaayi n’okuggya obutoffaali obumyufu obwonooneddwa mu mubiri gwo. N’olwekyo bw’okola obutoffaali bw’omusaayi obungi obutali bwa bulijjo, ennywanto yo erina okukola ennyo. Kiyinza okugaziwa. Ensigo yo bw’egaziwa, oyinza okuwulira ng’olubuto lwo lujjudde oba nga tolina mirembe mu kitundu kya waggulu ekya kkono eky’olubuto lwo .

• Extramedullary hematopoiesis: Kino kwe kukula kw’obutoffaali obukola omusaayi ebweru w’obusimu bw’amagumba , mu bitundu by’omubiri ebirala. Obutoffaali buno busobola okutondebwa mu bifo ng’amawuggwe, mu lubuto, omugongo, obwongo oba ennywanto. Obutoffaali buno busobola okukuŋŋaana ne bukola ebizimba , ebiyinza okulumba ebitundu ebirala oba okutaataaganya emirimu gyabyo.

• Portal hypertension: Kino kweyongera kwa puleesa mu musuwa ogutwala omusaayi okuva mu nnywanto yo okutuuka mu kibumba kyo. Kino kisinga kuva ku kwonooneka kw’emisuwa olw’okukola omusaayi ogw’ebweru w’omubiri (extramedullary hematopoiesis) ogwayogeddwako emabegako.

Ekirala kiri nti ku bantu nga 12% abalina obulwadde bwa myelofibrosis obusookerwako, embeera eno esobola okukulaakulana n’efuuka ekika kya kookolo w’omusaayi ow’amaanyi ennyo ayitibwa acute myeloid leukemia .

Kiki ekivaako obulwadde bwa myelofibrosis?

Bannasayansi n’okutuusa kati tebamanyi kituufu kivaako obulwadde bwa myelofibrosis. Naye bakizudde nti kikwatagana n'enkyukakyuka mu DNA y'obuzaale obumu . Ekika kya puloteyina ekiyitibwa Janus-associated kinases (JAKs) kye kikwatibwako. Puloteeni zino eza JAKs zifuga okukola obutoffaali bw’omusaayi mu busimu bw’amagumba, ne zisindika obubonero eri obutoffaali okweyawulamu n’okukula. Singa puloteyina zino eza JAKs zifuuka ezisukkiridde okukola, obutoffaali bw’omusaayi bungi oba butono nnyo busobola okukolebwa.

Wakati wa 60% ne 65% ku bantu abalina myelofibrosis balina enkyukakyuka mu JAK2 gene . Ebitundu ebirala 5% ku 10% .Waliwo enkyukakyuka mu buzaale bwa myeloproliferative leukemia (MPL). Era, enkyukakyuka eyitibwa Calreticulin (CALR) erabibwa mu balwadde ba myelofibrosis wakati wa 20% ne 25%.

Ani asinga okubeera mu bulabe olw’ensonga eno?

Oyinza okuba mu bulabe obw’okulwala obulwadde bwa myelofibrosis olw’ensonga zino wammanga:

• Bwoba olina emyaka egisukka mu 50 .
• Bw’oba ​​olina obuzibu bw’omusaayi obuyitibwa primary thrombocytosis oba polycythemia vera.
• Bw’oba ​​obadde ofunye obusannyalazo obuyitibwa ionizing radiation oba eddagala ly’amafuta nga benzene ne toluene.

Bubonero ki obulaga obulwadde bwa myelofibrosis?

Obulwadde bwa Myelofibrosis bulwadde obugenda bugenda mpola , n’olwekyo oyinza okumala emyaka nga tolina bubonero bwonna. Abalwadde nga kimu kya kusatu tebalina bubonero bwonna mu kusooka.

Singa obubonero bubaawo, obusinga okulabika kwe kukoowa ennyo (olw’okubulwa omusaayi) n’okugaziwa kw’ennywanto . Obubonero era buyinza okuli:

• Okukola ennyo
• Omusujja
• Okusiiwa
• Olususu olumyufu
• Okugejja
• Entuuyo z’ekiro
• Obulumi mu magumba oba mu binywa
• Okukwatibwa yinfekisoni enfunda eziwera
• Ensigo oba ekibumba okugaziwa
• Okuzimba omusaayi okutannyonnyolwa
• Okuvaamu omusaayi oba ebiwundu ebitali bya bulijjo
• Okugaziwa kw’emisuwa mu lubuto n’omumwa gwa nnabaana (emisuwa gino giyinza okukutuka ne givaamu omusaayi).

Osobola otya okuzuula obulwadde buno?

Omusawo naddala omusawo wa kkansa ajja kukukebera mu mubiri, akubuuze ku byafaayo by’obujjanjabi bwo, era akubuuze ku bubonero bwo mu kiseera kino. Era bagenda kukebera oba n’ennywanto egaziye n’obubonero bw’okubulwa omusaayi.

Olwo, okukeberebwa okw’enjawulo kukolebwa okuggyawo embeera endala n’okukakasa oba olina obulwadde bwa myelofibrosis.

Okukebera omusaayi

• Complete blood count (CBC): Kino kipima omuwendo gw’obutoffaali obw’enjawulo mu musaayi gwo. Singa obutoffaali bwo obumyufu buba wansi okusinga ku bwa bulijjo, oba obutoffaali bwo obweru n’obutoffaali obukola omusaayi tebiba bya bulijjo, kiyinza okuba akabonero k’obulwadde bwa myelofibrosis.
• Peripheral blood smear (PBS): Kino kikebera obunene, enkula, n’engeri endala ez’obutoffaali bwo okulaba oba tebulina buzibu bwonna. Singa wabaawo obutoffaali obulabika ng’obutali bwa bulijjo n’obutoffaali bw’omusaayi obungi obutakuze, era kiyinza okuba akabonero k’obulwadde bwa myelofibrosis.
• Okukebera kemiko w’omusaayi:Ebigezo bino bipima emiwendo gy’ebintu eby’enjawulo ebifulumizibwa mu musaayi okuva mu bitundu byo. Kino kiyinza okukutegeeza engeri ekitundu ky’omubiri gye kikola obulungi. Ebintu nga uric acid, bilirubin, ne lactic dehydrogenase ebingi mu musaayi nakyo kiyinza okuba akabonero k’obulwadde bwa myelofibrosis.

Okukebera obusigo bw’amagumba

• Okukebera obusigo bw’amagumba: Kino kizingiramu okukwata akatundu akatono ku busimu bwo n’obukebera ng’okozesa microscope. Obutoffaali buno bwekenneenyezebwa okuzuula oba olina obulwadde bwa myelofibrosis.
• Okusiba obusigo bw’amagumba: Kino kizingiramu okuggya amazzi mu busimu bw’amagumba n’okugakebera oba temuli bubonero bwa myelofibrosis.

Kiyinza okwetaagisa okwongera okukeberebwa

Kiyinza okwetaagisa okukeberebwa okulala okukakasa nti olina obulwadde buno:

• Okwekenenya enkyukakyuka mu buzaale: Omusawo wo ajja kukebera obutoffaali bwo mu musaayi n’obusimu bw’amagumba okulaba enkyukakyuka mu buzaale ezikwatagana n’obulwadde bwa myelofibrosis, gamba nga JAK2, CALR, ne MPL. Obujjanjabi obumu butunuulira obutoffaali bwa kookolo obulina enkyukakyuka ya JAK2.
• Enkola y’okukuba ebifaananyi: Omusawo ayinza okukola ‘ultrasound’ okukebera oba ennywanto egaziye. Bayinza n’okukola MRI okukebera oba waliwo enkovu mu busimu bw’amagumba. Ekitundu kino eky’enkovu kiyinza okuba akabonero k’obulwadde bwa myelofibrosis.

Obulwadde bwa myelofibrosis bujjanjabwa butya?

Bw’oba ​​tolaga bubonero, oyinza obuteetaaga bujjanjabi. Kyokka ne bw’oba ​​teweetaaga bujjanjabi bwa mangu, omusawo ajja kusigala ng’alondoola embeera yo.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), ne Vonjo® (pacritinib) ddagala erikkirizibwa ekitongole kya U.S. Food and Drug Administration (FDA) . Zikozesebwa okujjanjaba obulwadde bwa myelofibrosis obw’akabi obw’ekigero oba obw’amaanyi. Eddagala lino lyonna essatu likola nga JAK inhibitors . Kwe kugamba, zikendeeza ku bubonero bwa Janus-associated kinase (JAK) obusukkiridde. N’ekyavaamu, eddagala lino liyamba okumalawo obubonero obukwatagana n’obulwadde bwa myelofibrosis, gamba ng’ennywanto okukula, entuuyo ekiro, okusiiwa, okugejja n’omusujja.

Bangi, ekigendererwa ekikulu eky’obujjanjabi kwe kuddukanya embeera ezikwatagana n’obulwadde bwa myelofibrosis, gamba ng’okukendeera kw’omusaayi n’okugaziwa kw’ennywanto.

Obujjanjabi bw’obulwadde bw’okukendeera kw’omusaayi

Waliwo obujjanjabi bw’okukendeera kw’omusaayi nga:

• Androgens: Okumira androgens nga danazol kiyinza okwongera okukola obutoffaali obumyufu.
• Eddagala erikyusa obusimu obuziyiza endwadde: Eddagala lino lyongera ku busobozi bw’abaserikale b’omubiri gwo okulwanyisa obutoffaali bwa kookolo, bwe kityo ne kikendeeza ku bubonero. Eddagala nga interferon, thalidomide (Thalomid®), ne lenalidomide (Revlimid®) liri mu kibinja kino. Era ziyinza okuweebwa nga zigatta wamu ne glucocorticoids nga prednisone.
• Eddagala erijjanjaba eddagala: Eddagala erimu liyinza okukendeeza ku bubonero obuva ku butoffaali bw’omusaayi okweyongera n’ennywanto okugaziwa. Hydroxyurea ne cladribine ddagala ng’eryo.
• Okuteekebwamu omusaayi: Bw’oba ​​olina okukendeeza ku musaayi ogw’amaanyi, okuteekebwamu omusaayi buli kiseera kiyinza okwongera ku bungi bw’obutoffaali obumyufu.

Obujjanjabi bw’obulwadde bwa Splenomegaly

Ebiziyiza JAK, eddagala eriweweeza ku busimu obuziyiza endwadde, n’eddagala eriweweeza ku bulwadde (chemotherapy drugs) bikozesebwa okuddukanya ennywanto egaziye. Mu mbeera enzibu, oyinza okwetaaga okulongoosebwa ennywanto yo (okuggyamu ennywanto) oba okulongoosebwa n’obujjanjabi obw’amasannyalaze okutuuka ku nnywanto.

Obujjanjabi bw’amasannyalaze era buyinza okwetaagisa okujjanjaba embeera eyitibwa extramedullary hematopoiesis, obutoffaali bw’omusaayi mwe bukula ebweru w’obusimu bw’amagumba.

Obulwadde bwa myelofibrosis busobola okuwona ddala?

Okusimbuliza obutoffaali obukola omusaayi (Allogeneic hematopoietic cell transplantation - HCT) kuyinza okuba nga kwe kuyinza okuwonya obulwadde buno. Wabula eno nkola ya bulabe era tesaanira buli muntu.

Mu nkola eno, obutoffaali bwo obw’omusaayi obutali bwa bulijjo bukyusibwamu obutoffaali obuva mu muntu omulamu obulungi. Nga tonnaba kusimbuliza, ojja kuweebwa eddagala eriweweeza ku bulwadde bwa ‘chemotherapy’ oba ‘radiation therapy’ okutta obutoffaali bwo obulwadde. Olwo, obutoffaali obupya okuva eri oyo agaba busobola okutwala emirimu gy’omubiri gwo.

Obujjanjabi bwa HCT butwala obulabe bungi obw'ebizibu . N’olwekyo, esaanira ekibinja ky’abantu abaalondeddwa bokka. Obulabe bw’ebizibu businga n’okubeera ku bantu abalina embeera endala ez’obujjanjabi. Oba osaanira kino kisinziira ku bintu bingi, gamba ng’emyaka gyo, obuzibu bw’obubonero bwo, n’emikisa gy’obujjanjabi buno okusobola okutuuka ku buwanguzi.

Obulamu bwe buba butya ng’obeera n’obulwadde buno?

Obulwadde bwa Myelofibrosis kookolo wa maanyi nnyo . Omuwendo gw’abalwadde bano abawangaala wakati gutera kuba gwa myaka nga mukaaga. Omuwendo gw’abantu abawangaala wakati kitegeeza nti abantu abamu bawangaala emyaka egitawera mukaaga, ate omuwendo gwe gumu bawangaala emyaka egisukka mu mukaaga. Kino si kye kimu eri buli muntu, kyawukana okusinziira ku muntu.

Waliwo ensonga eziwerako ezisalawo engeri obulwadde bwo gye buyinza okulabika:

• emyaka gyo.
• Obubonero bwo.
• Omuwendo gw’obutoffaali bwo mu musaayi.
• Obuzibu bw’enkovu mu busimu bw’amagumba.
• Oba waliwo enkyukakyuka mu buzaale oba nedda (JAK2, CALR, MPL).

Ekisinga obukulu kwe kwogera mu lwatu n’omusawo wo ku ngeri obulwadde bwo gye bunaakwata ku bulamu bwo.

Nze nneerabirira ntya? (Okwefaako) .

Buuza omusawo wo oba oyinza okuganyulwa mu bujjanjabi obukkakkanya obulumi . Ttiimu z’abajjanjabi abakkakkanya obulumi ziyinza okubeeramu abasawo, ba nurse, abakola ku nsonga z’abantu, n’abakugu abalala. Basobola okukuwa eby’obugagga n’obuyambi bwe weetaaga okubeera n’obulwadde buno. Ng’oggyeeko obujjanjabi bw’ofuna okuva eri omusawo wo ow’obulwadde bwa kkansa, abantu bano bayinza okukuwa obuwagizi obw’amaanyi. Abakugu mu kujjanjaba abalwadde ba kookolo basobola okuyamba okutumbula omutindo gw’obulamu bwo ng’omuntu alina kookolo.

N’ekisembayo, ebintu ebikulu by’olina okujjukira

Myelofibrosis kika kya kookolo w’omusaayi atatera kulabika ng’obusimu bwo obw’amagumba bukyusibwamu ebitundu ebirimu ebiwuziwuzi. Eno mbeera ya maanyi eyeetaaga okulondoola abasawo obutasalako ne/oba okujjanjabibwa. Okusinziira ku mbeera gy’olimu, oyinza okumala emyaka nga tolina bubonero bwonna. Mu mbeera endala, obubonero busobola okukula amangu ne kizibuwalira okukola emirimu gya buli lunaku. Wabula waliwo obujjanjabi obusobola okuyamba okufuga obubonero obutaataaganya obulamu bwo obwa bulijjo.

Mu kiseera kino, buuza omusawo wo ku bintu ebiyinza okukuyamba okugumira enkyukakyuka zino. Ebibiina by‟okulabirira okukkakkanya obulumi n‟okuwagira biyamba nnyo nga bw‟omanyiira obulamu ne kookolo.

Jjukira nti toli wekka. Abasawo, ab’omu maka gaabwe, n’emikwano bonna baliwo okukuyamba. Totya kwogera na musawo wo ku bibuuzo byonna by’oyinza okuba nabyo.

` Myelofibrosis, kookolo w’omusaayi, obusigo bw’amagumba, okukendeera kw’omusaayi, okugaziwa kw’ennywanto, JAK inhibitors, leukemia