Ðe wò ƒumeŋuɖui hã wɔa ka siwo me ka le? Mina míasrɔ̃ nu tso myelofibrosis ŋu!

Èse dɔléle aɖe si woyɔna be myelofibrosis ŋu nya kpɔa? Le nyateƒe me la, enye ʋumenugbagbeviɣidɔ ƒomevi aɖe si mebɔ kura o. Kpuie ko la, ne wotsɔ abi si me ka le ɖɔ li ƒumeŋuɖui , si nye wò ƒuwo ƒe akpa falɛfalɛ si le abe ƒumeŋuɖui ene . Enye ʋumenugbagbeviɣidɔ ƒomevi aɖe hã, eye wòle ƒuƒoƒo aɖe si woyɔna be myeloproliferative disorders me. Myeloproliferative disorders nye ne ʋumenugbagbevi geɖe le ƒumeŋuɖui me, afisi wowɔa ʋumenugbagbeviwo le.

Nuka tututue nye myelofibrosis?

Mina míalé ŋku ɖe esia ŋu tsitotsito. Lãmenugbagbevi siwo wɔa ʋu le wò ƒumeŋuɖui me . Emegbe lãmenugbagbevi siawo va zua ʋumenugbagbevi dzĩwo, ʋumenugbagbevi ɣiwo, kple ʋumenugbagbevi dzĩwo. Gake le amesi ŋu myelofibrosis le gome la, tɔtrɔ aɖe dzɔna le domenyiŋusẽfianu, alo DNA, si le lãmenugbagbevi siawo dometɔ ɖeka me. Esia wɔnɛ be lãmenugbagbevi la va zua kansa lãmenugbagbevi si gblẽ. Emegbe lãmenugbagbevi sia si gblẽ la ma eye wòtsɔa domenyiŋusẽfianu si trɔna la yia lãmenugbagbevi yeye siwo katã wòwɔ la gbɔ.

Le ɣeyiɣi aɖe megbe la, kansa lãmenugbagbevi siawo siwo mesɔ o la va ƒoa ƒu ɖe agbɔsɔsɔ gã aɖe me. Lãmenugbagbevi siawo dometɔ aɖewo nana ƒumeŋuɖui vuvu . Dzoxɔxɔ siae nana abi ƒe lãkusi si megblɔ va yi la dzɔna. Eyata le abi sia kple kansa lãmenugbagbevi siwo sɔ gbɔ ta la, ƒumeŋuɖui megate ŋu wɔa ʋumenugbagbevi siwo le lãmesẽ me o.

Nukawoe nye myelofibrosis ƒomevi veviwo?

Myelofibrosis ƒomevi vevi eve ye li.

• Primary myelofibrosis: Esia nye ƒomevi si dzɔna le eɖokui si, eye kadodo aɖeke mele eŋu kple dɔléle bubu aɖeke o.
• Secondary myelofibrosis: Ʋu ƒe dɔléle bubu aɖe gbɔe esia tsona. Le kpɔɖeŋu me, dɔlélewo abe primary thrombocytosis alo polycythemia vera ene ate ŋu atsoe. Dɔnɔ siwo ŋu wokpɔ dɔlélea le ƒe 10% va ɖo 20% tso evelia ƒomevi sia.

Aleke gbegbee dɔléle sia si woyɔna be Myelofibrosis bɔe?

Le nyateƒe me la, myelofibrosis nye dɔléle si mebɔ kura o . Le kpɔɖeŋu me, le United States la, woka nya ta be dɔléle sia dzea ame abe 1.5 ene le ame 100,000 ɖesiaɖe me ƒe sia ƒe. Ate ŋu agblẽ nu le ame sia ame ŋu, eɖanye ƒe ka kee wòɖaxɔ o. Vovototo aɖeke mele ƒe si woxɔ me o, gake anɔ eme be woakpɔe le amesiwo xɔ wu ƒe 50. Ne myelofibrosis dze ɖevi suewo dzi la, zi geɖe la, wokpɔnɛ hafi xɔ ƒe 3.

Ŋusẽ kawoe myelofibrosis ate ŋu akpɔ ɖe wò ŋutilã dzi?

Ne wowɔ ʋumenugbagbeviwo le mɔ sia nu le mɔ si mesɔ o nu la, kuxi vovovowo ate ŋu ado mo ɖa. Mina míalé ŋku ɖe nusiwo wonye ŋu:

• Ʋusɔgbɔdɔ: Esia nye nɔnɔme si me ʋumenugbagbevi dzĩwo mesɔ gbɔ o. Abe alesi nènyae ene la, ʋumenugbagbevi dzĩwoe tsɔa ɔksidzin yia ŋutilã bliboa me. Eyata ne lãmenugbagbevi dzĩwo mesɔ gbɔ o la, ŋutilãa ƒe lãkusiwo mekpɔa oxygen si sɔ o. Esia ate ŋu ahe dzesiwo abe ɖeɖiteameŋu, gbɔdzɔgbɔdzɔ, kple gbɔgbɔtsixetsixe ene vɛ .

• Thrombocytopenia: Esia nye nɔnɔme si me ʋumenugbagbevi dzĩwo mesɔ gbɔ akpa o. Ʋumenugbagbevi dzĩwoe kpena ɖe ʋu ŋu ne abi aɖe xɔ ame. Eyata ne wò ʋumenugbagbevi dzĩwo ƒe xexlẽme mede o la, ʋu ate ŋu ado le wò ŋutilã me eye wòaxɔ abi bɔbɔe wu .

• Splenomegaly: Wò dɔgbo kpɔa ʋumenugbagbeviɣiwo dzi eye wòɖea ʋumenugbagbevi dzĩ siwo gblẽ ɖa le wò ŋutilã me. Eyata ne èwɔ ʋumenugbagbevi siwo mesɔ o fũ akpa la, ele be wò dɔgbo nawɔ dɔ sesĩe wu. Ate ŋu akeke ɖe enu. Ne wò dɔgbo lolo la, àte ŋu ase le ɖokuiwò me be yeƒe ƒodo ƒe miame ƒe akpa si le etame la yɔ alo mevo o .

• Extramedullary hematopoiesis: Esia nye lãmenugbagbevi siwo wɔa ʋu ƒe tsitsi le ƒumeŋuɖui godo , le ŋutilã ƒe akpa bubuwo. Lãmenugbagbevi siawo ate ŋu adzɔ le teƒewo abe lãkusi, dɔgbo, aklã, ahɔhɔ̃, alo ʋukawo ene. Lãmenugbagbevi siawo ate ŋu aƒo ƒu awɔ dɔdzẽ , siwo ate ŋu adze ŋutinu bubuwo dzi alo ado kplamatse woƒe dɔwɔwɔ.

• Ʋusɔgbɔdɔ si le agbo nu: Esia nye ʋu ƒe sisi le ʋuka si tsɔa ʋu tso wò dɔgbo me yia wò aklã me la ƒe dzidziɖedzi. Anɔ eme be esia tsoa ʋukaawo ƒe gbegblẽ le ʋumenugbagbeviɣidɔ si míeyɔ va yi ta.

Nu bubue nye be le amesiwo ŋu myelofibrosis gbãtɔ le dometɔ siwo ade 12% gome la, nɔnɔmea ate ŋu ava zu ʋumenugbagbeviɣidɔ sesẽ aɖe ŋutɔ si woyɔna be acute myeloid leukemia .

Nukae hea myelofibrosis vɛ?

Dzɔdzɔmeŋutinunyalawo menya nusi tututu hea myelofibrosis vɛ haɖe o. Gake wokpɔe be edo ƒome kple domenyiŋusẽfianu aɖewo ƒe DNA ƒe tɔtrɔ . Protein ƒomevi aɖe si woyɔna be Janus-associated kinases (JAKs) ye kpɔa gome le eme. JAKs protein siawo kpɔa ʋumenugbagbevi siwo le ƒumeŋuɖui me dzi, eye woɖoa dzesiwo ɖe lãmenugbagbeviwo be woama ahatsi. Ne JAKs protein siawo va wɔa dɔ akpa la, woate ŋu awɔ ʋumenugbagbevi geɖe akpa alo ʋɛ akpa.

Ame siwo ŋu myelofibrosis le dometɔ 60% va ɖo 65% ƒe domenyiŋusẽfianu ƒe tɔtrɔ le JAK2 domenyiŋusẽfianu me . 5% va ɖo 10% bubu .Dzɔdzɔmenugbagbevi aɖe le ʋumenugbagbeviɣidɔ (MPL) domenyiŋusẽfianu me. Azɔ hã, wokpɔa tɔtrɔ aɖe si woyɔna be Calreticulin (CALR) le myelofibrosis dɔnɔ siwo le 20% va ɖo 25% dome.

Amekae le afɔku me wu le esia ta?

Ðewohĩ ànɔ afɔku me be nàxɔ myelofibrosis le susu siwo gbɔna ta:

• Ne èxɔ wu ƒe 50 la .
• Ne ʋumenugbagbeviɣidɔ aɖe si woyɔna be primary thrombocytosis alo polycythemia vera le ŋuwò.
• Ne èdo go keklẽŋusẽ si nye ionizing radiation alo petrochemicals abe benzene kple toluene ene kpɔ.

Nukawoe nye myelofibrosis ƒe dzesiwo?

Myelofibrosis nye dɔléle si le ŋgɔ yim vivivi , eyata ɖewohĩ dzesi aɖeke madze le ŋuwò o ƒe geɖe. Dɔnɔ siwo ade akpa etɔ̃lia mekpɔa dzesi aɖeke le woƒe gɔmedzedze o.

Ne dzesiawo dze la, esiwo bɔ wue nye ɖeɖiteameŋu sesẽ (le ʋusɔgbɔdɔ ta) kple dɔgbo si lolo . Dzesiawo ate ŋu anye:

• Dɔ sesẽ wɔwɔ
• Asrã
• Ʋuʋudedi
• Ŋutilã si ƒe ŋutigbalẽ le ɣie
• Kpekpeme dzi ɖeɖe kpɔtɔ
• Zã me fifia
• Ƒu alo ƒunukpeƒeveame
• Dɔlékuiwo xɔxɔ enuenu
• Aɖuɖɔ alo aklã si lolo
• Ʋu ƒe sisi si womeɖe o
• Ʋudodo alo abi si mebɔ o
• Ʋuka siwo le dɔgboa kple nuɖuɖutome ƒe lolome (veka siawo ate ŋu agbã eye ʋu nado).

Aleke woawɔ anya dɔléle sia?

Ðɔkta, vevietɔ kansadɔdala, ado wò kpɔ le ŋutilã me, abia wò atikewɔwɔ ŋutinya, eye abia wò dzesi siwo le ŋuwò fifia hã. Woalé ŋku ɖe aɖuɖɔtoe si lolo kple ʋusɔgbɔdɔ ƒe dzesiwo hã ŋu.

Emegbe, wowɔa dodokpɔ vovovowo tsɔ ɖea nɔnɔme bubuwo ɖa eye woɖoa kpe edzi nenye be myelofibrosis le ŋuwò.

Ʋudodokpɔwo

• Ʋu ƒe xexlẽme blibo (CBC): Esia dzidzea lãmenugbagbevi vovovo siwo le wò ʋu me ƒe xexlẽme. Ne wò ʋumenugbagbevi dzĩwo ɖiɖi wu alesi wòle, alo wò ʋumenugbagbevi ɣiwo kple ʋumenugbagbevi dzĩwo mesɔ o la, ate ŋu anye myelofibrosis ƒe dzesi.
• Peripheral blood smear (PBS): Esia léa ŋku ɖe wò ʋumenugbagbeviɣiwo ƒe lolome, nɔnɔme, kple nɔnɔme bubuwo ŋu be naneke mesɔ o hã. Ne lãmenugbagbevi siwo ƒe dzedzeme mesɔ o eye ʋumenugbagbevi gbogbo aɖewo li siwo metsi o la, ate ŋu anye myelofibrosis ƒe dzesi hã.
• Ʋu ƒe atikeŋutinunya dodokpɔwo:Dodokpɔ siawo dzidzea nu vovovo siwo dona ɖe ʋua me tso wò ŋutinuwo me ƒe agbɔsɔsɔ. Esia ate ŋu ana nànya alesi ŋutinu aɖe le dɔ wɔm nyuie. Nuwo abe uric acid, bilirubin, kple lactic dehydrogenase ƒe agbɔsɔsɔ fũ le ʋu me hã ate ŋu anye myelofibrosis ƒe dzesi.

Ƒumeŋuɖui dodokpɔwo

• Ƒumeŋuɖui ƒe lãmenugbagbeviwo ɖeɖe: Esia bia be woaɖe wò ƒumeŋuɖui sue aɖe atsɔ adoe kpɔ le nusuekpɔmɔ̃ me. Wodzroa lãmenugbagbeviawo me be woakpɔe ɖa be myelofibrosis le ŋuwò hã.
• Ƒumeŋuɖui ƒe ʋuʋu: Esia bia be woaɖe tsi si le ƒumeŋuɖui me eye woadoe kpɔ be myelofibrosis ƒe dzesiwo le eŋu hã.

Ðewohĩ ahiã be woagawɔ dodokpɔ bubuwo

Ðewohĩ ahiã be woawɔ dodokpɔ bubuwo atsɔ aɖo kpe dɔlélea dzi:

• Domenyiŋusẽfianu ƒe tɔtrɔ me dzodzro: Wò ɖɔkta ado wò ʋu kple ƒumeŋuɖui ƒe lãmenugbagbeviwo akpɔ be domenyiŋusẽfianu ƒe tɔtrɔ siwo do ƒome kple myelofibrosis, abe JAK2, CALR, kple MPL ene hã le wo me hã. Dɔdamɔnu aɖewo tɔa ŋku kansa lãmenugbagbevi siwo me JAK2 ƒe tɔtrɔ le.
• Nɔnɔmetatawo ɖeɖe ƒe mɔnuwo: Wò ɖɔkta ate ŋu awɔ ultrasound atsɔ akpɔe ɖa be dɔgboa lolo hã. Woate ŋu awɔ MRI hã atsɔ akpɔe ɖa be abi le ƒumeŋuɖui me hã. Abi sia ate ŋu anye myelofibrosis ƒe dzesi.

Aleke wodaa myelofibrosis ƒe dɔléleae?

Ne mèle dzesiwo ɖem fia o la, ɖewohĩ mahiã be woawɔ atike na wò o. Gake ne mehiã be woawɔ atike na wò enumake o hã la, wò ɖɔkta ayi edzi anɔ ŋku lém ɖe wò nɔnɔmea ŋu.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), kple Vonjo® (pacritinib) nye atike siwo dzi U.S. Nuɖuɖu Kple Atike Ŋuti Dɔwɔƒe (FDA) da asi ɖo . Wozãa wo tsɔ daa myelofibrosis si me afɔku le si sɔ gbɔ va ɖo esi lolo ŋutɔ. Atike etɔ̃ siawo katã wɔa dɔ abe JAK mɔxenuwo ene . Eyae nye be woɖea Janus-associated kinase (JAK) ƒe dzesi siwo wɔa dɔ fũu akpa dzi kpɔtɔna. Esia wɔe be atike siawo kpena ɖe ame ŋu wòɖea dzesi siwo do ƒome kple myelofibrosis, abe dɔgbo ƒe lolome, fifia le zã me, ʋuʋudedi, lolo dzi ɖeɖe kpɔtɔ, kple dzoxɔxɔ ene dzi kpɔtɔna.

Le ame geɖe gome la, atikewɔwɔ ƒe taɖodzinu vevitɔe nye be woakpɔ nɔnɔme siwo do ƒome kple myelofibrosis, abe ʋusɔgbɔdɔ kple dɔgbo ƒe lolome ene gbɔ.

Ʋusɔgbɔdɔ ƒe Atikewɔwɔ

Atike aɖewo li na ʋusɔgbɔdɔ abe:

• Androgens: Androgens abe danazol ene nono ate ŋu ana ʋumenugbagbevi dzĩwo nadzi ɖe edzi.
• Dɔlékuiwutikewo: Atike siawo dzia wò dɔlélenutsiŋutete ƒe ŋutete ɖe edzi be wòawɔ avu kple kansa lãmenugbagbeviwo, si wɔnɛ be dzesiawo dzi ɖena kpɔtɔna. Atikewo abe interferon, thalidomide (Thalomid®), kple lenalidomide (Revlimid®) le hatsotso sia me. Woate ŋu ana wo kpe ɖe glucocorticoids abe prednisone ene hã ŋu.
• Atike siwo wozãna tsɔ daa dɔlékuiwoe: Atike aɖewo ate ŋu aɖe dzesi siwo ʋumenugbagbeviɣidɔ ƒe dzidziɖedzi kple dɔgbo ƒe lolome hena vɛ dzi akpɔtɔ. Hydroxyurea kple cladribine nye atike mawo tɔgbe.
• Ʋudodo: Ne ʋusɔgbɔdɔ sesẽ le ŋuwò la, ʋudodo edziedzi ate ŋu ana wò ʋumenugbagbevi dzĩwo nadzi ɖe edzi.

Splenomegaly ƒe Dɔdamɔnu

Wozãa JAK mɔxenuwo, dɔlélenutsiŋutete ƒe dɔlékuiwutikewo, kple atike siwo wozãna tsɔ daa dɔlékuiwutikewo tsɔ kpɔa dɔgbo si lolo la gbɔ. Le nɔnɔme sesẽwo me la, ahiã be woawɔ dɔ na wò be woaɖe wò dɔgbo ɖa (woɖea dɔgboa ɖa) alo woawɔ keklẽŋusẽdɔdamɔnu na wò dɔgboa.

Ðewohĩ ahiã be woatsɔ keklẽŋusẽdɔdamɔnu hã ada dɔléle aɖe si woyɔna be extramedullary hematopoiesis, si me ʋumenugbagbeviwo dona le ƒumeŋuɖui godo.

Ðe woate ŋu ada myelofibrosis keŋkeŋa?

Allogeneic hematopoietic cell transplantation (HCT) ate ŋu anye mɔnu ɖeka kolia si dzi woato ada dɔléle sia. Gake esia nye dɔdamɔnu si me afɔku le eye mesɔ na amesiame o.

Le dɔ sia wɔwɔ me la, wotsɔa lãmenugbagbevi siwo tso ame si le lãmesẽ me gbɔ ɖɔlia wò ʋumenugbagbevi siwo mesɔ o. Hafi woado ame bubu na wò la, woana atike alo keklẽŋusẽdɔdamɔnu wò be wòawu wò lãmenugbagbevi siwo ŋu dɔléle le. Emegbe, lãmenugbagbevi yeye siwo tso nunala la gbɔ ate ŋu axɔ wò ŋutilã ƒe dɔwɔwɔwo.

HCT-dɔdamɔnua hea kuxiwo ƒe afɔku gã aɖe vɛ . Eyata ame tiatia aɖewo koe wòsɔ na. Afɔku si le kuxiwo me la lolo wu le amesiwo ŋu atikewɔwɔ me nɔnɔme bubuwo le gome. Ne èsɔ na esia hã nɔ te ɖe nu geɖe dzi, abe ƒe si nèxɔ, alesi wò dzesiawo nu sẽe, kple alesi wòanya wɔ be atikewɔwɔa nakpɔ dzidzedze ene.

Aleke ame ƒe agbenɔƒe le ne wole agbe kple dɔléle sia?

Myelofibrosis nye kansa si nu sẽ ŋutɔ . Zi geɖe la, dɔnɔ siawo ƒe agbetsitsi le mama dedie nu anɔ abe ƒe ade ene. Agbetsitsi ƒe agbɔsɔsɔme le mama dedie nu fia be ame aɖewo nɔa agbe mede ƒe ade o, evɔ xexlẽme ma tɔgbe ya nɔa agbe wu ƒe ade. Esia mesɔ na amesiame o, etoa vovo tso ame ɖeka gbɔ yi bubu gbɔ.

Nu geɖe li siwo ɖea wò dɔlélea ƒe ŋgɔdonya fiana:

• wò ƒexɔxɔ.
• Wò dzesiwo.
• Wò ʋumenugbagbeviɣiwo.
• Alesi abi le ƒumeŋuɖui me ƒe sesẽme.
• Ne domenyiŋusẽfianu ƒe tɔtrɔwo li alo meli o (JAK2, CALR, MPL).

Nu vevitɔe nye be nàƒo nu kple wò ɖɔkta gaglãa tso alesi dɔléle si wokpɔ le ŋuwò akpɔ ŋusẽ ɖe wò agbe dzii ŋu.

Aleke malé be na ɖokuinye? (Ðokuidzikpɔkpɔ) .

Bia wò ɖɔkta nenye be atikewɔwɔ si naa ame ƒe dzi dzea eme ate ŋu aɖe vi na wò hã . Ðɔktawo, dɔnɔdzikpɔlawo, hadomekpekpeɖeŋudɔwɔlawo, kple eŋutinunyala bibi bubuwo ate ŋu anɔ ƒuƒoƒo siwo kpɔa dɔlélenutsiŋutete gbɔ. Woate ŋu ana nunɔamesiwo kple kpekpeɖeŋu siwo nèhiã be nànɔ agbe kple dɔléle sia wò. Tsɔ kpe ɖe atike si nèxɔna tso wò kansadɔdala gbɔ ŋu la, ame siawo ate ŋu anye kpekpeɖeŋutsoƒe gã aɖe. Dɔlélenutsiŋutete ŋuti nunyala bibiwo ate ŋu akpe ɖe ŋuwò nàna wò agbenɔnɔ nanyo ɖe edzi abe amesi le kansa lém ene.

Mlɔeba la, nu vevitɔ kekeake siwo dzi nàɖo ŋkui

Myelofibrosis nye ʋumenugbagbeviɣidɔ ƒomevi aɖe si mebɔ o si me ka siwo me ka le ɖɔlia wò ƒumeŋuɖui. Esia nye nɔnɔme sesẽ aɖe si bia be atikewɔlawo nalé ŋku ɖe eŋu ɣesiaɣi eye/alo woawɔ atike nɛ. Le wò nɔnɔmea nu la, ɖewohĩ dzesi aɖeke manɔ ŋuwò o ƒe geɖe. Le go bubuwo me la, dzesiawo ate ŋu ayi ŋgɔ kabakaba eye wòana wòasesẽ be woawɔ gbesiagbedɔwo. Gake atikewɔmɔnu aɖewo li siwo akpe ɖe ŋuwò nàɖu dzesi siwo doa kplamatse wò gbesiagbegbenɔnɔ dzi.

Hafi ɣemaɣi naɖo la, bia wò ɖɔkta tso dɔwɔnu siwo ate ŋu akpe ɖe ŋuwò nànɔ te ɖe tɔtrɔ siawo nu ŋu. Beléle na ame ƒe dɔlélenutsiŋutete kple kpekpeɖeŋunahawo nye tiatia siwo kpena ɖe ŋuwò ŋutɔ ne èle tɔtrɔm ɖe agbenɔnɔ si ŋu kansa le ŋu.

Ðo ŋku edzi be menye wò ɖeka koe o. Ðɔktawo, ƒometɔwo, kple xɔlɔ̃wo katã le afima be yewoakpe ɖe ŋuwò. Mègavɔ̃ be yeaƒo nu kple wò ɖɔkta tso nyabiase ɖesiaɖe si anɔ asiwò ŋu o.

` Myelofibrosis, ʋumenugbagbeviɣidɔ, ƒumeŋuɖui, ʋusɔgbɔdɔ, dɔgbo ƒe lolome, JAK mɔxenuwo, ʋumenugbagbeviɣidɔ