Ka whakaputahia e tō hinu wheua he kiko muka? Me ako tātou mō te myelofibrosis!

Kua rongo koe i tētahi mate e kiia nei ko te myelofibrosis? He momo matepukupuku toto tino onge tēnei. He māmā noa iho, ko te wā tēnei e whakakapia ai te hinu wheua , te wāhanga ngohengohe, he hautai o ō wheua, e te kiko kiripaka . He momo leukemia roa anō hoki tēnei, ā, nō roto i tētahi rōpū e kiia nei ko ngā mate myeloproliferative. Ko ngā mate myeloproliferative ko te wā e nui rawa ai ngā pūtau toto e hangaia ana i roto i te hinu wheua, te wāhi e hangaia ai ngā pūtau toto.

He aha tonu te myelofibrosis?

Kia tirohia tēnei kia nui ake ngā taipitopito. Kei roto i tō hinu wheua ngā pūtau take hanga toto. Ka whanake ēnei pūtau take hei pūtau toto whero, hei pūtau toto mā, hei pereti toto. Engari i roto i te tangata he myelofibrosis, ka puta he whakarerekētanga ira i roto i te rauemi ira, i te DNA rānei, i roto i tētahi o ēnei pūtau take. Mā tēnei ka huri te pūtau hei pūtau matepukupuku hapa. Kātahi ka wehea e tēnei pūtau hapa, ka tukuna te whakarerekētanga ki ngā pūtau hou katoa e hangaia ana e ia.

I roto i te wā, ka nui haere te hanga o ēnei pūtau matepukupuku rerekē. Ko ētahi o ēnei pūtau ka puta te mumura i roto i te hinu wheua. Ko tēnei mumura te mea e puta ai te kiko whara i kōrerotia e au i mua ake nei. Nō reira, nā tēnei kiko whara me te nui rawa o ngā pūtau matepukupuku, ka ngaro te kaha o te hinu wheua ki te hanga pūtau toto hauora.

He aha ngā momo matua o te myelofibrosis?

E rua ngā momo matua o te myelofibrosis.

• Myelofibrosis tuatahi: Koinei te momo ka puta noa mai, kāore he hononga ki tētahi atu mate.
• Myelofibrosis Tuarua: Ka puta mai tēnei i tētahi atu mate toto. Hei tauira, ka puta mai pea i ngā mate pēnei i te thrombocytosis tuatahi, te polycythemia vera rānei. E 10% ki te 20% o ngā tūroro kua tātaritatia tēnei momo tuarua.

He pēhea te noa o tēnei mate e kiia nei ko te Myelofibrosis?

He tino onge te mate Myelofibrosis. Hei tauira, i te Hononga o Amerika, e kīia ana ka pā tēnei mate ki te 1.5 o te 100,000 tāngata ia tau. Ka pā ki te katoa, ahakoa te pakeke. Kāore he rerekētanga o te pakeke, engari he nui ake te tūponotanga ka kitea i roto i te hunga neke atu i te 50 tau te pakeke . Mena ka puta te myelofibrosis i roto i ngā tamariki nohinohi, ka kitea i mua i te 3 tau te pakeke.

He aha ngā pānga o te myelofibrosis ki tō tinana?

Ina puta he momo raruraru i roto i te tinana ina puta he pūtau toto i roto i tēnei āhua, ka puta pea ētahi raruraru. Me titiro tātou ki ēnei raruraru:

• Te kore toto toto: He āhuatanga tēnei kāore e ranea ngā toto toto whero. E mōhio ana koe, ko ngā toto toto whero ngā mea e kawe ana i te hāora puta noa i te tinana. Nō reira, ki te kore e ranea ngā toto toto whero, kāore e ranea te hāora o ngā kiko o te tinana. Ka puta pea ngā tohu pēnei i te ngenge, te ngoikore, me te poto o te manawa .

• Thrombocytopenia: He āhuatanga tēnei e iti rawa ai ngā pereti toto. Ko ngā pereti toto ngā mea e āwhina ana ki te totoka ina whara. Nō reira, ina iti te maha o ngā pereti toto, ka ngāwari ake te toto me te maru o tō tinana.

• Splenomegaly: Ka whakahaere tō whēkau i te maha o ngā pūtau toto, ā, ka tangohia atu ngā pūtau toto whero kua pakaru i tō tinana. Nō reira, ki te nui rawa ngā pūtau toto rerekē e hangaia ana e koe, me kaha ake te mahi a tō whēkau. Ka taea te whakanui ake. Ina whakanuia tō whēkau, tera pea ka rongo koe i te kī, i te kore rānei e pai ki te taha maui o runga o tō puku .

• Te hematopoiesis i waho o te medullary: Koinei te whanaketanga o ngā pūtau hanga toto i waho o te hinu wheua , i ētahi atu wāhanga o te tinana. Ka taea e ēnei pūtau te hanga i ngā wāhi pēnei i te pukahukahu, te ara kai, te taurakira, te roro, ngā kōpū lymph rānei. Ka taea e ēnei pūtau te huihui tahi hei hanga pukupuku , ka taea te whakaeke i ētahi atu okana, te whakararuraru rānei i tā rātou mahi.

• Te toto toto tiketike o te tomokanga: He pikinga tēnei o te toto toto i roto i te uaua e kawe ana i te toto mai i tō whēkau ki tō ate. Ko te nuinga o te wā ka puta mai tēnei i te kino o ngā uaua nā te hematopoiesis extramedullary i whakahuatia ake nei.

Ko tētahi atu mea, mō te 12% o te hunga whai myelofibrosis tuatahi, ka taea e te mate te whanake hei momo mate pukupuku toto tino kino e kiia nei ko te leukemia myeloid acute .

He aha te take o te myelofibrosis?

Kāore anō kia tino mōhiotia e ngā kaipūtaiao te take o te myelofibrosis. Engari kua kitea e rātou he hononga tēnei ki ngā huringa o te DNA o ētahi ira . Kei roto ko tētahi momo pūmua e kiia nei ko Janus-associated kinases (JAKs) . Ko ēnei pūmua JAKs te kaiwhakahaere o te hanga o ngā pūtau toto i roto i te hinu wheua, e tuku tohu ana ki ngā pūtau kia wehea, kia tipu hoki. Mena ka kaha rawa te mahi a ēnei pūmua JAKs, ka nui rawa, ka iti rawa rānei ngā pūtau toto ka puta.

I waenganui i te 60% me te 65% o te hunga whai myelofibrosis he whakarerekētanga ira kei roto i te ira JAK2 . E 5% ki te 10% anō hokiHe whakarerekētanga kei roto i te ira myeloproliferative leukemia (MPL). Waihoki, ka kitea he whakarerekētanga e kiia nei ko Calreticulin (CALR) i waenga i te 20% me te 25% o ngā tūroro myelofibrosis.

Ko wai te mea tino mōrearea mō tēnei?

Tērā pea ka nui ake tō tūponotanga ki te whakawhanake i te myelofibrosis mō ēnei take e whai ake nei:

• Mena kua neke atu i te 50 tau tō pakeke .
• Mena he mate toto tōu e kiia nei ko te thrombocytosis tuatahi, ko te polycythemia vera rānei.
• Mena kua pāngia koe e te irahiko whakakoiora , e ngā matū hinu rānei pērā i te benzene me te toluene.

He aha ngā tohu o te myelofibrosis?

He mate āta haere te Myelofibrosis, nō reira, tērā pea kāore he tohu mō ngā tau. Tata ki te hautoru o ngā tūroro kāore he tohu i ngā wāhanga tōmua.

Mena ka puta ngā tohu, ko te mea tino kitea ko te ngenge nui (nā te anemia) me te whēkau nui . Tērā pea ko ēnei tohu:

• Mahi uaua
• Kirika
• Te mākūkū
• Kiri mā
• Te whakahekenga taumaha
• Te werawera o te pō
• Mamae wheua, mamae hononga rānei
• Ngā mate pāwera auau
• Te whēkau, te ate rānei kua nui haere
• Ngā toto toto kore whakamārama
• Te toto rerekē, te maru rānei
• Te whakanui ake i ngā uaua i roto i te puku me te korokoro (ka pakaru pea ēnei uaua, ka toto).

Me pēhea te tautuhi i tēnei mate?

Ka tirohia tō tinana e te tākuta, inā koa he tākuta matepukupuku, ka pātai atu mō tō hītori hauora, me ō tohumate o nāianei. Ka tirohia anō hoki e rātou mēnā he whēkau nui, me ngā tohu o te anemia.

Kātahi ka whakahaerehia ngā whakamātautau maha hei whakakore i ētahi atu mate me te whakaū mēnā he myelofibrosis tōu.

Ngā whakamātautau toto

• Te tatau toto katoa (CBC): Ka ine tēnei i te maha o ngā momo pūtau rerekē i roto i tō toto. Mena he iti iho ō pūtau toto whero i te mea noa, he rerekē rānei ō pūtau toto mā me ō pereti toto, he tohu pea tēnei nō te myelofibrosis.
• Te whakamātautau toto ā-taha (PBS): Mā tēnei ka tirohia te rahi, te āhua, me ētahi atu āhuatanga o ō pūtau toto mō ngā āhuatanga rerekē. Mena he āhua rerekē ngā pūtau, ā, he nui ngā pūtau toto kāore anō kia pakari, he tohu pea tēnei nō te myelofibrosis.
• Ngā whakamātautau matū toto:Ka inehia e ēnei whakamātautau ngā taumata o ngā matū rerekē ka tukuna ki roto i te toto mai i ō whekau. Mā tēnei ka taea e koe te whakaatu i te pai o te mahi a tētahi whekau. Ko ngā taumata tiketike o ngā mea pēnei i te waikawa uric, te bilirubin, me te lactic dehydrogenase i roto i te toto he tohu pea nō te myelofibrosis.

Ngā whakamātautau hinu wheua

• Ko te koiora wheua wheua: Ko te tango i tētahi tauira iti o tō wheua wheua ka tirotirohia i raro i te karuārai. Ka tātarihia ngā pūtau hei whakatau mēnā he myelofibrosis tōu.
• Te tango i te wai mai i te hinu wheua: Ko te tango i te wai mai i te hinu wheua me te whakamātautau mō ngā tohu o te myelofibrosis.

Me whai whakamātautau anō pea

Tērā pea ka hiahiatia ētahi atu whakamātautau hei whakaū i tō mate:

• Tātari ira whakarerekētanga: Ka whakamātauhia e tō tākuta ō pūtau toto me te hinu wheua mō ngā whakarerekētanga ira e pā ana ki te myelofibrosis, pērā i te JAK2, CALR, me te MPL. Ko ētahi maimoatanga e aro ana ki ngā pūtau matepukupuku kei a rātou te whakarerekētanga JAK2.
• Ngā tikanga whakaahua: Ka taea e tō tākuta te tirotiro i te whēkau nui me te ultrasound. Ka taea hoki e ia te tirotiro i te kiko whara i roto i te hinu wheua. He tohu pea tēnei kiko whara o te myelofibrosis.

Me pēhea te rongoā i te myelofibrosis?

Ki te kore koe e whakaatu i ngā tohu, kāore pea koe e hiahia ki te rongoā. Heoi, ahakoa kāore koe e hiahia ki te rongoā tonu, ka haere tonu te aro turuki a tō tākuta i tō āhua.

Ko Jakafi® (ruxolitinib), Inrebic® (fedratinib), me Vonjo® (pacritinib) he rongoā kua whakaaetia e te U.S. Food and Drug Administration (FDA) . E whakamahia ana ēnei hei rongoā mō te myelofibrosis mōrearea-waenga ki te mōrearea-tiketike. E mahi ana ēnei rongoā e toru hei aukati JAK . Arā, ka whakaitihia e rātou te tohu Janus-associated kinase (JAK) tino kaha. Nō reira, ka āwhina ēnei rongoā ki te whakaora i ngā tohu e pā ana ki te myelofibrosis, pērā i te whēkau nui, te werawera pō, te mākūkū, te ngaronga taumaha, me te kirika.

Mō te tini, ko te whāinga matua o te maimoatanga he whakahaere i ngā āhuatanga e pā ana ki te myelofibrosis, pērā i te anemia me te whēkau nui.

Te Maimoatanga mō te Anemia

He maimoatanga kei reira mō te anemia pēnei i:

• Androgens: Mā te tango i ngā androgens pēnei i te danazol ka taea te whakanui ake i te hanga toto whero.
• Ngā rongoā ārai mate: Ka whakanui ake ēnei rongoā i te kaha o tō pūnaha ārai mate ki te whawhai ki ngā pūtau matepukupuku, ā, ka whakaiti i ngā tohu. Kei roto i tēnei akomanga ngā rongoā pēnei i te interferon, thalidomide (Thalomid®), me te lenalidomide (Revlimid®). Ka taea hoki te hoatu me ngā glucocorticoids pēnei i te prednisone.
• Ngā rongoā chemotherapy: Ka taea e ētahi rongoā chemotherapy te whakaiti i ngā tohu e puta mai ana i te pikinga o ngā pūtau toto me te rahi o te whēkau. Ko te Hydroxyurea me te cladribine ētahi o ēnei rongoā.
• Ngā whakawhiti toto: Mena he nui tō toto toto iti, ka taea e ngā whakawhiti toto auau te whakanui ake i te maha o ngā toto toto whero.

Te Maimoatanga o te Splenomegaly

Ka whakamahia ngā aukati JAK, ngā rongoā ārai mate, me ngā rongoā chemotherapy hei whakahaere i te whēkau kua rahi. I ngā wā tino kino, me tango pea tō whēkau mā te pokanga (splenectomy) me te rongoā irahiko rānei ki te whēkau.

E hiahiatia ana hoki te rongoā irahiko hei rongoā i tētahi āhuatanga e kiia nei ko te extramedullary hematopoiesis, arā, ko te whanaketanga o ngā pūtau toto i waho o te hinu wheua.

Ka taea te whakaora katoa i te myelofibrosis?

Ko te whakawhitinga pūtau hematopoietic allogeneic (HCT) pea te huarahi anake hei whakaora i tēnei mate. Heoi, he tikanga mōrearea tēnei, ā, kāore e pai mō te katoa.

I roto i tēnei tikanga, ka whakakapia ō pūtau toto rerekē ki ngā pūtau mai i tētahi kaituku toto hauora. I mua i te whakawhitinga toto, ka tukuna he rongoā chemotherapy, he rongoā radiation rānei ki a koe hei patu i ō pūtau mate. Kātahi ka taea e ngā pūtau hou mai i te kaituku toto te tango i ngā mahi a tō tinana.

He nui te mōrearea o ngā raruraru i roto i te maimoatanga HCT. Nō reira, he pai anake mō tētahi rōpū tāngata kua tohua. He nui ake te mōrearea o ngā raruraru mō te hunga whai mate hauora kē atu. Ko te tika o tēnei mea ka whakawhirinaki ki ngā āhuatanga maha, pēnei i tō tau, te kaha o ō tohumate, me te tūponotanga o te angitu o te maimoatanga.

He aha te roanga o te oranga ina noho tahi me tēnei mate?

He mate pukupuku tino kino te Myelofibrosis. Ko te toharite o te ora mō ēnei tūroro he ono tau te nuinga. Ko te toharite o te ora ko ētahi tāngata e ora ana i raro iho i te ono tau, engari he rite tonu te maha e ora ana mō te neke atu i te ono tau. Kāore tēnei i te rite mō te katoa, he rerekē i ia tangata, i ia tangata.

He maha ngā āhuatanga e whakatau ana i te matapae o tō mate:

• tō tau.
• Ō tohumate.
• Te maha o ō pūtau toto.
• Te kaha o te whara i roto i te hinu wheua.
• Ahakoa kei reira ngā whakarerekētanga ira (JAK2, CALR, MPL).

Ko te mea nui ko te kōrero tuwhera ki tō tākuta mō te pānga o tō tātaritanga ki tō oranga.

Me pēhea taku tiaki i ahau anō? (Te tiaki i ahau anō)

Pātai atu ki tō tākuta mēnā ka whai hua koe i te tiaki mamae . Tērā pea kei roto i ngā tīma tiaki mamae ngā tākuta, ngā nēhi, ngā kaimahi hapori, me ētahi atu tohunga. Ka taea e rātou te whakarato i ngā rauemi me te tautoko e hiahiatia ana e koe hei oranga mō tēnei mate. Haunga te maimoatanga e whiwhi ana koe mai i tō tākuta mate pukupuku, ka taea e ēnei tāngata te noho hei puna tautoko nui. Ka taea e ngā tohunga tiaki mamae te āwhina ki te whakapai ake i tō kounga o te oranga hei tangata e ora ana me te mate pukupuku.

Hei whakamutunga, ko ngā mea tino nui hei maumahara māu

He momo mate pukupuku toto onge te Myelofibrosis, ka whakakapia tō hinu wheua ki te kiko muka. He mate kino tēnei e hiahia ana kia aroturukitia tonutia, kia rongoatia rānei e te rata. I runga i tō āhua, tērā pea kāore he tohu mō ngā tau. I ētahi atu wā, ka tere te horapa o ngā tohu, ā, ka uaua ki te mahi i ngā mahi o ia rā. Heoi, kei reira ngā maimoatanga hei āwhina i te whakahaere i ngā tohu e whakararuraru ana i tō oranga o ia rā.

I tēnei wā, pātai atu ki tō tākuta mō ngā rauemi hei āwhina i a koe ki te aro atu ki ēnei huringa. He tino āwhina ngā rōpū tiaki mamae me ngā rōpū tautoko i a koe e urutau ana ki te oranga me te mate pukupuku.

Kia maumahara, kāore koe i te mokemoke. Kei reira ngā tākuta, te whānau, me ngā hoa hei āwhina i a koe. Kaua e wehi ki te kōrero ki tō tākuta mō ngā pātai katoa kei a koe.

` Myelofibrosis, matepukupuku toto, hinu wheua, anemia, whakanui whēkau, aukati JAK, leukemia