Kodi mafuta a m'mafupa anu amatulutsa minofu ya ulusi? Tiyeni tiphunzire za myelofibrosis!

Kodi mudamvapo za matenda otchedwa myelofibrosis? Ndi mtundu wosowa kwambiri wa khansa ya m'magazi. Mwachidule, ndi pamene mafupa , omwe ndi gawo lofewa, lokhala ngati siponji la mafupa anu, amalowedwa m'malo ndi minofu ya zipsera za ulusi . Ndi mtundu wa khansa ya m'magazi yomwe imatenga nthawi yayitali, ndipo ili m'gulu lotchedwa matenda a myeloproliferative. Matenda a myeloproliferative ndi pamene maselo ambiri amagazi amapangidwa m'mafupa, komwe maselo amagazi amapangidwa.

Kodi myelofibrosis kwenikweni ndi chiyani?

Tiyeni tiwone izi mwatsatanetsatane. Mafupa anu ali ndi maselo oyambira omwe amapanga magazi. Maselo oyambira awa pambuyo pake amasanduka maselo ofiira a magazi, maselo oyera a magazi, ndi ma platelet. Koma mwa munthu amene ali ndi myelofibrosis, kusintha kumachitika mu majini, kapena DNA, mu imodzi mwa maselo oyambira awa. Izi zimapangitsa kuti selo likhale selo lopanda khansa. Selo lopanda khansali limagawikana ndikugawa kusinthaku ku maselo atsopano onse omwe limapanga.

Pakapita nthawi, maselo a khansa osazolowerekawa amachulukana kwambiri. Ena mwa maselo amenewa amayambitsa kutupa m'mafupa. Kutupa kumeneku ndiko kumayambitsa minofu ya chilonda yomwe ndatchula kale. Chifukwa chake, chifukwa cha minofu ya chilonda iyi ndi maselo a khansa ochulukirapo, mafuta a m'mafupa amataya mphamvu zake zopangira maselo amagazi athanzi.

Kodi mitundu ikuluikulu ya myelofibrosis ndi iti?

Pali mitundu iwiri ikuluikulu ya myelofibrosis.

• Myelofibrosis yoyamba: Iyi ndi mtundu womwe umachitika mwangozi, popanda kugwirizana ndi matenda ena aliwonse.
• Myelofibrosis yachiwiri: Izi zimachitika chifukwa cha matenda ena a magazi. Mwachitsanzo, zimatha kuchitika chifukwa cha matenda monga thrombocytosis yoyamba kapena polycythemia vera. Mtundu wachiwiri uwu umakhala pakati pa 10% ndi 20% ya odwala omwe apezeka ndi matendawa.

Kodi matenda otchedwa Myelofibrosis ndi ofala bwanji?

Myelofibrosis kwenikweni ndi vuto losowa kwambiri . Mwachitsanzo, ku United States, akuti matendawa amapezeka mwa anthu pafupifupi 1.5 mwa anthu 100,000 pachaka. Amatha kukhudza aliyense, mosasamala kanthu za msinkhu wawo. Palibe kusiyana kwa zaka, koma nthawi zambiri amapezeka mwa anthu azaka zopitirira 50. Ngati myelofibrosis imayamba mwa ana aang'ono, nthawi zambiri imapezeka asanakwanitse zaka zitatu.

Kodi zotsatira za myelofibrosis zingakhudze bwanji thupi lanu?

Maselo a m'magazi akapangidwa modabwitsa motere, mavuto osiyanasiyana amatha kuchitika. Tiyeni tiwone zomwe zili:

• Kuchepa kwa magazi m'thupi: Iyi ndi vuto lomwe maselo ofiira a m'magazi sakwanira. Monga mukudziwa, maselo ofiira a m'magazi ndi omwe amanyamula mpweya m'thupi lonse. Choncho maselo ofiira akakhala ochepa, minofu ya thupi silandira mpweya wokwanira. Izi zingayambitse zizindikiro monga kutopa, kufooka, komanso kupuma movutikira .

• Thrombocytopenia: Iyi ndi vuto lomwe ma platelet ndi ochepa kwambiri. Ma platelet ndi omwe amathandiza magazi kuundana pamene pali kuvulala. Choncho ngati kuchuluka kwa ma platelet anu kuli kochepa, thupi lanu limatha kutuluka magazi ndi kuvulala mosavuta.

• Splenomegaly: Splenomegaly yanu imalamulira kuchuluka kwa maselo a magazi ndikuchotsa maselo ofiira owonongeka m'thupi lanu. Chifukwa chake mukapanga maselo ambiri osazolowereka amagazi, splenome yanu iyenera kugwira ntchito molimbika. Ikhoza kukula. Splenomegaly yanu ikakula, mungamve kukhuta kapena kusasangalala kumtunda kumanzere kwa mimba yanu .

• Kutuluka kwa magazi m'thupi (Extramedullary hematopoiesis): Uku ndi kukula kwa maselo opanga magazi kunja kwa mafupa , m'magawo ena a thupi. Maselo amenewa amatha kupangika m'malo monga mapapo, m'mimba, msana, muubongo, kapena m'ma lymph nodes. Maselo amenewa amatha kusonkhana pamodzi kuti apange zotupa , zomwe zimatha kulowa m'ziwalo zina kapena kusokoneza ntchito yawo.

• Kuthamanga kwa magazi m'thupi: Uku ndi kuwonjezeka kwa kuthamanga kwa magazi m'mitsempha yomwe imanyamula magazi kuchokera ku ndulu kupita ku chiwindi. Izi mwina zimachitika chifukwa cha kuwonongeka kwa mitsempha chifukwa cha kutuluka kwa magazi m'thupi komwe kwatchulidwa kale.

Chinthu china n’chakuti kwa anthu pafupifupi 12% omwe ali ndi myelofibrosis yoyamba, vutoli limatha kukhala khansa ya m’magazi yoopsa kwambiri yotchedwa acute myeloid leukemia .

Kodi n’chiyani chimayambitsa myelofibrosis?

Asayansi sakudziwabe chomwe chimayambitsa myelofibrosis. Koma apeza kuti ikugwirizana ndi kusintha kwa DNA ya majini ena . Mtundu wa puloteni wotchedwa Janus-associated kinases (JAKs) umakhudzidwa. Mapuloteni a JAK awa amawongolera kupanga maselo amagazi mu fupa la mafupa, kutumiza zizindikiro ku maselo kuti agawikane ndikukula. Ngati mapuloteni a JAK awa agwira ntchito mopitirira muyeso, maselo ambiri kapena ochepa kwambiri amagazi amatha kupangidwa.

Pakati pa 60% ndi 65% ya anthu omwe ali ndi myelofibrosis ali ndi kusintha kwa majini a JAK2 . Ena 5% mpaka 10%Pali kusintha kwa majini a myeloproliferative leukemia (MPL). Komanso, kusintha kotchedwa Calreticulin (CALR) kumapezeka pakati pa 20% ndi 25% ya odwala myelofibrosis.

Ndani amene ali pachiwopsezo chachikulu cha izi?

Mungakhale pachiwopsezo chachikulu chotenga myelofibrosis pazifukwa zotsatirazi:

• Ngati muli ndi zaka zoposa 50 .
• Ngati muli ndi vuto la magazi lotchedwa primary thrombocytosis kapena polycythemia vera.
• Ngati mwakhalapo ndi ma radiation a ayoni kapena mankhwala a petrochemical monga benzene ndi toluene.

Kodi zizindikiro za myelofibrosis ndi ziti?

Matenda a Myelofibrosis ndi matenda omwe amakula pang'onopang'ono , kotero simungakhale ndi zizindikiro zilizonse kwa zaka zambiri. Pafupifupi gawo limodzi mwa magawo atatu a odwala alibe zizindikiro zilizonse kumayambiriro.

Ngati zizindikiro zichitika, zomwe zimafala kwambiri ndi kutopa kwambiri (chifukwa cha kuchepa kwa magazi m'thupi) komanso kutupa kwa ndulu . Zizindikiro zake zingaphatikizeponso:

• Ntchito yolimba
• Malungo
• Kuyabwa
• Khungu lotumbululuka
• Kuchepetsa thupi
• Thukuta la usiku
• Kupweteka kwa mafupa kapena mafupa
• Matenda opatsirana pafupipafupi
• Nkhumba yokulirapo kapena chiwindi
• Magazi oundana osamveka bwino
• Kutuluka magazi kosazolowereka kapena kuvulala
• Kukulira kwa mitsempha m'mimba ndi m'mero ​​(mitsempha iyi imatha kuphulika ndikutuluka magazi).

Kodi mungazindikire bwanji matendawa?

Dokotala, makamaka katswiri wa khansa, adzakuyang'anirani mwakuthupi, adzakufunsani za mbiri yanu yachipatala, ndikufunsani za zizindikiro zomwe muli nazo panopa. Adzafufuzanso ngati pali ndulu yotupa komanso zizindikiro za kuchepa kwa magazi m'thupi.

Kenako, mayeso osiyanasiyana amachitidwa kuti atsimikizire ngati muli ndi matenda ena a myelofibrosis.

Kuyezetsa magazi

• Kuchuluka kwa magazi (CBC): Izi zimayesa kuchuluka kwa mitundu yosiyanasiyana ya maselo m'magazi mwanu. Ngati maselo ofiira anu ali otsika kuposa abwinobwino, kapena maselo oyera amagazi ndi ma platelet anu ndi osazolowereka, izi zitha kukhala chizindikiro cha myelofibrosis.
• Kuyeza magazi m'magazi (PBS): Izi zimafufuza kukula, mawonekedwe, ndi makhalidwe ena a maselo anu a m'magazi kuti aone ngati pali matenda enaake. Ngati pali maselo ooneka ngati osazolowereka komanso maselo ambiri osakhwima m'magazi, izi zitha kukhalanso chizindikiro cha myelofibrosis.
• Mayeso a magazi:Mayesowa amayesa kuchuluka kwa zinthu zosiyanasiyana zomwe zimatulutsidwa m'magazi kuchokera m'ziwalo zanu. Izi zingakuuzeni momwe chiwalo chikugwira ntchito bwino. Kuchuluka kwa zinthu monga uric acid, bilirubin, ndi lactic dehydrogenase m'magazi kungakhalenso chizindikiro cha myelofibrosis.

Mayeso a mafupa

• Kufufuza za mafupa: Izi zimaphatikizapo kutenga chitsanzo cha mafupa anu ndikuwayang'ana pogwiritsa ntchito maikulosikopu. Maselo amawunikidwa kuti adziwe ngati muli ndi myelofibrosis.
• Kutulutsa madzi m'mafupa: Izi zimaphatikizapo kuchotsa madzi m'mafupa ndikuyesa ngati pali zizindikiro za myelofibrosis.

Mayeso ena angafunike

Mayeso ena angafunike kuti mutsimikizire kuti muli ndi matendawa:

• Kusanthula kusintha kwa majini: Dokotala wanu adzayesa maselo anu a m'magazi ndi m'mafupa kuti awone ngati pali kusintha kwa majini komwe kumakhudzana ndi myelofibrosis, monga JAK2, CALR, ndi MPL. Mankhwala ena amakhudza maselo a khansa omwe ali ndi kusintha kwa JAK2.
• Njira zojambulira: Dokotala wanu angachite ultrasound kuti aone ngati ndulu yakula. Angachitenso MRI kuti aone ngati pali zipsera mu fupa la m'mafupa. Zipsera izi zitha kukhala chizindikiro cha myelofibrosis.

Kodi myelofibrosis imachiritsidwa bwanji?

Ngati simukuwonetsa zizindikiro, simungafunike chithandizo. Komabe, ngakhale simukufunika chithandizo mwamsanga, dokotala wanu apitiriza kuyang'anira vuto lanu.

Jakafi® (ruxolitinib), Inrebic® (fedratinib), ndi Vonjo® (pacritinib) ndi mankhwala ovomerezeka ndi U.S. Food and Drug Administration (FDA) . Amagwiritsidwa ntchito pochiza myelofibrosis yapakati mpaka yapamwamba. Mankhwala onse atatuwa amagwira ntchito ngati zoletsa za JAK . Izi zikutanthauza kuti, amachepetsa Janus-associated kinase (JAK) signaling yogwira ntchito kwambiri. Zotsatira zake, mankhwalawa amathandiza kuchepetsa zizindikiro zokhudzana ndi myelofibrosis, monga ndulu yokulira, thukuta la usiku, kuyabwa, kuchepa thupi, ndi malungo.

Kwa ambiri, cholinga chachikulu cha chithandizo ndi kuthana ndi matenda okhudzana ndi myelofibrosis, monga kuchepa kwa magazi m'thupi ndi ndulu yotupa.

Chithandizo cha Kuchepa kwa Magazi

Pali njira zochizira kuchepa kwa magazi m'thupi monga:

• Androgens: Kutenga androgens monga danazol kungapangitse kuti maselo ofiira a m'magazi apangidwe kwambiri.
• Mankhwala Oletsa Kutupa kwa Thupi: Mankhwalawa amawonjezera mphamvu ya chitetezo cha mthupi lanu yolimbana ndi maselo a khansa, motero amachepetsa zizindikiro. Mankhwala monga interferon, thalidomide (Thalomid®), ndi lenalidomide (Revlimid®) ali m'gulu ili. Angaperekedwenso pamodzi ndi glucocorticoids monga prednisone.
• Mankhwala a chemotherapy: Mankhwala ena a chemotherapy amatha kuchepetsa zizindikiro zomwe zimachitika chifukwa cha kuchuluka kwa maselo a m'magazi ndi ndulu yotupa. Hydroxyurea ndi cladribine ndi mankhwala otere.
• Kuikidwa magazi: Ngati muli ndi vuto la kuchepa kwa magazi m'thupi, kuikidwa magazi nthawi zonse kungapangitse kuti maselo ofiira a m'magazi anu achuluke.

Chithandizo cha Splenomegaly

Mankhwala oletsa JAK, ma immunomodulators, ndi mankhwala a chemotherapy amagwiritsidwa ntchito pochiza ndulu yokulira. Pa milandu yoopsa, mungafunike kuchitidwa opaleshoni yochotsa ndulu yanu (splenectomy) kapena kupatsidwa chithandizo cha radiation ku ndulu.

Chithandizo cha radiation chingakhalenso chofunikira pochiza matenda otchedwa extramedullary hematopoiesis, momwe maselo amagazi amakulira kunja kwa mafupa.

Kodi myelofibrosis ingachiritsidwe kwathunthu?

Kuika maselo a magazi otchedwa allogeneic hematopoietic cell transplantation (HCT) kungakhale njira yokhayo yochiritsira matendawa. Komabe, iyi ndi njira yowopsa ndipo si yoyenera aliyense.

Mu njira imeneyi, maselo anu osazolowereka a magazi amalowedwa m'malo ndi maselo ochokera kwa wopereka wathanzi. Musanaikidwe, mudzapatsidwa mankhwala a chemotherapy kapena radiation kuti muphe maselo anu odwala. Kenako, maselo atsopano ochokera kwa woperekayo amatha kutenga ntchito za thupi lanu.

Chithandizo cha HCT chili ndi chiopsezo chachikulu cha mavuto . Chifukwa chake, ndi choyenera gulu losankhidwa la anthu okha. Chiwopsezo cha mavutowo n'chokwera kwambiri kwa anthu omwe ali ndi matenda ena. Ngati ndinu woyenera izi zimadalira zinthu zambiri, monga msinkhu wanu, kuopsa kwa zizindikiro zanu, komanso kuthekera kwa chithandizocho kupambana.

Kodi moyo umakhala bwanji munthu akakhala ndi matendawa?

Myelofibrosis ndi khansa yoopsa kwambiri . Chiŵerengero chapakati cha kupulumuka kwa odwalawa nthawi zambiri chimakhala pafupifupi zaka zisanu ndi chimodzi. Chiŵerengero chapakati cha kupulumuka chimatanthauza kuti anthu ena amakhala ndi moyo wosakwana zaka zisanu ndi chimodzi, pomwe chiwerengero chofananacho chimakhala ndi moyo zaka zoposa zisanu ndi chimodzi. Izi sizili zofanana kwa aliyense, zimasiyana malinga ndi munthu.

Pali zinthu zingapo zomwe zimatsimikizira kuti matenda anu adzayamba liti:

• zaka zanu.
• Zizindikiro zanu.
• Kuchuluka kwa maselo a magazi anu.
• Kuopsa kwa zipsera m'mafupa.
• Kaya pali kusintha kwa majini (JAK2, CALR, MPL) kapena ayi.

Chofunika kwambiri ndikulankhulana momasuka ndi dokotala wanu za momwe matenda anu angakhudzire moyo wanu.

Kodi ndimadzisamalira bwanji? (Kudzisamalira)

Funsani dokotala wanu ngati mungapindule ndi chithandizo chamankhwala ochepetsa ululu . Magulu osamalira odwala ochepetsa ululu angaphatikizepo madokotala, anamwino, ogwira ntchito zachitukuko, ndi akatswiri ena. Angakupatseni zinthu zofunika komanso chithandizo chomwe mukufunikira kuti mukhale ndi matendawa. Kuwonjezera pa chithandizo chomwe mumalandira kuchokera kwa dokotala wanu wa khansa, anthuwa akhoza kukhala gwero labwino kwambiri la chithandizo. Akatswiri osamalira odwala ochepetsa ululu angakuthandizeni kukonza moyo wanu monga munthu amene ali ndi khansa.

Pomaliza, zinthu zofunika kwambiri zomwe muyenera kukumbukira

Myelofibrosis ndi khansa ya m'magazi yomwe imapezeka kawirikawiri pomwe mafupa anu amasinthidwa ndi minofu ya ulusi. Iyi ndi matenda oopsa omwe amafunika kuyang'aniridwa ndi dokotala nthawi zonse komanso/kapena chithandizo. Kutengera ndi matenda anu, simungakhale ndi zizindikiro kwa zaka zambiri. Nthawi zina, zizindikiro zimatha kupita patsogolo mwachangu ndikupangitsa kuti zikhale zovuta kuchita zinthu za tsiku ndi tsiku. Komabe, pali mankhwala omwe alipo kuti athandize kuchepetsa zizindikiro zomwe zimasokoneza moyo wanu watsiku ndi tsiku.

Pakadali pano, funsani dokotala wanu za zinthu zomwe zingakuthandizeni kuthana ndi kusintha kumeneku. Chisamaliro chothandiza odwala ndi magulu othandizira ndi njira zothandiza kwambiri pamene mukuzolowera moyo wanu ndi khansa.

Kumbukirani, simuli nokha. Madokotala, achibale, ndi abwenzi onse alipo kuti akuthandizeni. Musaope kulankhula ndi dokotala wanu za mafunso aliwonse omwe mungakhale nawo.

Myelofibrosis , khansa ya m'magazi, mafuta am'mafupa, kuchepa kwa magazi m'thupi, kukulitsa ndulu, zoletsa za JAK, khansa ya m'magazi