Ke hana nei hoʻi kou iwi iwi i nā ʻiʻo fibrous? E aʻo kākou e pili ana i ka myelofibrosis!

Ua lohe paha ʻoe i kahi maʻi i kapa ʻia ʻo myelofibrosis? He ʻano maʻi koko kakaikahi loa ia. I ka ʻōlelo maʻalahi, ʻo ia ka wā e pani ʻia ai ka iwi iwi , ʻo ia ka ʻāpana palupalu a huʻa o kou mau iwi, e ka ʻiʻo ʻeha fibrous . He ʻano leukemia lōʻihi nō hoʻi ia, a no kahi hui i kapa ʻia ʻo nā maʻi myeloproliferative. ʻO nā maʻi myeloproliferative ka wā e hana ʻia ai nā hunaola koko he nui i loko o ka iwi iwi, kahi e hana ʻia ai nā hunaola koko.

He aha maoli ka myelofibrosis?

E nānā kākou i kēia me nā kikoʻī hou aku. Aia i loko o kou iwi iwi nā pūnaewele kumu e hoʻokumu ana i ke koko. Ma hope aku, ulu kēia mau pūnaewele kumu i nā pūnaewele koko ʻulaʻula, nā pūnaewele koko keʻokeʻo, a me nā platelets. Akā i loko o kekahi me ka myelofibrosis, loaʻa kahi mutation i loko o ka mea genetic, a i ʻole DNA, i loko o kekahi o kēia mau pūnaewele kumu. ʻO kēia ke kumu e lilo ai ke kelepona i pūnaewele maʻi kanesa kīnā. A laila māhele kēia kelepona hemahema a hoʻoili i ka mutation i nā pūnaewele hou a pau āna e hana ai.

I ka hala ʻana o ka manawa, hōʻiliʻili nui kēia mau hunaola kanesa ʻano ʻē. Hoʻoulu kekahi o kēia mau hunaola i ka mumū i loko o ka iwi iwi. ʻO kēia mumū ke kumu o ka hana ʻana o ka ʻiʻo palapū aʻu i ʻōlelo ai ma mua. No laila, ma muli o kēia ʻiʻo palapū a me nā hunaola kanesa keu, nalowale ka iwi iwi i kona hiki ke hana i nā hunaola koko olakino.

He aha nā ʻano nui o ka myelofibrosis?

ʻElua mau ʻano nui o ka myelofibrosis.

• ʻO ka myelofibrosis mua: ʻO kēia ke ʻano e kū koke mai ana, me ka ʻole o ka pilina me kekahi maʻi ʻē aʻe.
• ʻO ka myelofibrosis lua: Hoʻokumu ʻia kēia e kekahi maʻi koko ʻē aʻe. No ka laʻana, hiki ke hoʻoulu ʻia e nā maʻi e like me ka thrombocytosis mua a i ʻole ka polycythemia vera. ʻO kēia ʻano lua ka helu ma waena o 10% a me 20% o nā maʻi i ʻike ʻia.

Pehea ka maʻamau o kēia maʻi i kapa ʻia ʻo Myelofibrosis?

He maʻi laha ʻole ka Myelofibrosis. No ka laʻana, ma ʻAmelika Hui Pū ʻIa, ua hōʻike ʻia ua loaʻa kēia maʻi ma kahi o 1.5 o 100,000 mau kānaka i kēlā me kēia makahiki. Hiki iā ia ke hoʻopilikia i kekahi, me ka nānā ʻole i ka makahiki. ʻAʻohe ʻokoʻa o ka makahiki, akā ʻoi aku ka nui o ka ʻike ʻia ʻana i nā poʻe ma luna o 50 mau makahiki. Inā ulu ka myelofibrosis i nā keiki ʻōpio, ʻike pinepine ʻia ma mua o 3 mau makahiki.

He aha nā hopena o ka myelofibrosis i kou kino?

Ke hana ʻia nā hunaola koko ma ke ʻano ʻē ma kēia ʻano, hiki ke loaʻa nā pilikia like ʻole. E nānā kākou i ke ʻano o lākou:

• Anemia: He kūlana kēia kahi ʻaʻole lawa nā hunaola koko ʻulaʻula. E like me kou ʻike, ʻo nā hunaola koko ʻulaʻula nā mea e lawe ana i ka oxygen ma ke kino holoʻokoʻa. No laila, inā ʻaʻole lawa nā hunaola koko ʻulaʻula, ʻaʻole lawa ka oxygen i nā ʻiʻo o ke kino. Hiki i kēia ke hana i nā hōʻailona e like me ka luhi, ka nāwaliwali, a me ka pōkole o ka hanu .

• Thrombocytopenia: He kūlana kēia kahi i emi loa ai nā platelets. ʻO nā platelets ka mea e kōkua ai i ke kāpili ʻana o ke koko ke loaʻa kahi ʻeha. No laila, ke haʻahaʻa kāu helu platelet, hiki i kou kino ke maʻalahi i ke kahe ʻana o ke koko a me ka ʻeha .

• Splenomegaly: Hoʻomalu kou spleen i ka helu o nā cell koko a wehe i nā cell koko ʻulaʻula i hōʻino ʻia mai kou kino. No laila, ke hana ʻoe i nā cell koko ʻē he nui, pono kāu spleen e hana ikaika. Hiki ke hoʻonui ʻia. Ke hoʻonui ʻia kou spleen, hiki iā ʻoe ke manaʻo i ka piha a i ʻole ka ʻoluʻolu ʻole ma ka ʻaoʻao hema o luna o kou ʻōpū .

• ʻO ka hematopoiesis Extramedullary: ʻO kēia ka ulu ʻana o nā hunaola koko ma waho o ka iwi iwi , ma nā wahi ʻē aʻe o ke kino. Hiki i kēia mau hunaola ke hana ma nā wahi e like me nā māmā, ke ala ʻōpū, ke kaula iwi kuamoʻo, ka lolo, a i ʻole nā ​​​​kikowaena lymph. Hiki i kēia mau hunaola ke hui pū e hana i nā puʻupuʻu , hiki ke hoʻouka i nā ʻōpū ʻē aʻe a i ʻole e hoʻopilikia i kā lākou hana.

• ʻO ke koko kiʻekiʻe portal: ʻO kēia ka piʻi ʻana o ke koko i loko o ke aʻa e lawe ana i ke koko mai kou spleen i kou ake. ʻO ke kumu paha kēia o ka hōʻino ʻia ʻana o nā aʻa ma muli o ka hematopoiesis extramedullary i ʻōlelo ʻia ma mua.

ʻO kekahi mea ʻē aʻe, no kahi o 12% o ka poʻe me ka myelofibrosis mua, hiki i ke kūlana ke ulu i kahi ʻano maʻi koko koʻikoʻi loa i kapa ʻia ʻo acute myeloid leukemia .

He aha ke kumu o ka myelofibrosis?

ʻAʻole maopopo loa i nā kānaka ʻepekema ke kumu o ka myelofibrosis. Akā ua ʻike lākou ua pili ia i nā loli i loko o ka DNA o kekahi mau genes . Pili kekahi ʻano protein i kapa ʻia ʻo Janus-associated kinases (JAKs) . Hoʻomalu kēia mau protein JAKs i ka hana ʻana o nā hunaola koko i loko o ka iwi iwi, e hoʻouna ana i nā hōʻailona i nā hunaola e māhele a ulu. Inā lilo kēia mau protein JAKs i mea hana nui, hiki ke hana ʻia nā hunaola koko he nui a liʻiliʻi paha.

Ma waena o 60% a me 65% o ka poʻe me ka myelofibrosis he mutation i loko o ka gene JAK2 . ʻOi aku he 5% a 10%Aia kahi mutation i loko o ka gene myeloproliferative leukemia (MPL). Eia kekahi, ʻike ʻia kahi mutation i kapa ʻia ʻo Calreticulin (CALR) ma waena o 20% a me 25% o nā maʻi myelofibrosis.

ʻO wai ka mea i pilikia nui no kēia?

Hiki paha iā ʻoe ke hoʻonui i ka pilikia o ka hoʻomohala ʻana i ka myelofibrosis no nā kumu i lalo:

• Inā ʻoe ma luna o 50 mau makahiki .
• Inā he maʻi koko kou i kapa ʻia ʻo ka thrombocytosis mua a i ʻole polycythemia vera.
• Inā ua hōʻike ʻia ʻoe i ka radiation ionizing a i ʻole nā ​​​​​​mea kemika petro e like me benzene a me toluene.

He aha nā hōʻailona o ka myelofibrosis?

He maʻi holomua mālie ka Myelofibrosis, no laila ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona no nā makahiki. Ma kahi o ka hapakolu o nā maʻi ʻaʻohe o lākou mau hōʻailona i nā pae mua.

Inā loaʻa nā hōʻailona, ​​ʻo ka mea maʻamau ka luhi nui (ma muli o ka anemia) a me ka spleen i hoʻonui ʻia . Hiki i nā hōʻailona ke komo pū me:

• Hana paʻakikī
• kuni
• ʻĀnai
• ʻIli keʻokeʻo
• Ka emi ʻana o ke kaumaha
• Nā hou o ka pō
• ʻEha iwi a hui paha
• Nā maʻi pinepine
• Ka nui o ka spleen a i ʻole ke akepaʻa
• Nā ʻūpā koko i wehewehe ʻole ʻia
• Ke kahe koko ʻana a i ʻole ka ʻeha ʻana ma ke ʻano maʻamau
• Ka hoʻonui ʻia ʻana o nā aʻa koko i loko o ka ʻōpū a me ka esophagus (hiki i kēia mau aʻa ke pohā a kahe ke koko).

Pehea e ʻike ai i kēia maʻi?

E nānā kino ke kauka, ʻoiai he kauka maʻi kanesa, iā ʻoe, e nīnau e pili ana i kou mōʻaukala olakino, a e nīnau e pili ana i kāu mau hōʻailona o kēia manawa. E nānā pū lākou no ka spleen i hoʻonui ʻia a me nā hōʻailona o ka anemia.

A laila, hana ʻia nā hoʻokolohua like ʻole e hoʻoponopono i nā kūlana ʻē aʻe a hōʻoia inā he myelofibrosis kāu.

Nā hoʻāʻo koko

• Helu koko piha (CBC): Ke ana kēia i ka helu o nā ʻano ʻano hunaola like ʻole i loko o kou koko. Inā haʻahaʻa kou mau hunaola koko ʻulaʻula ma mua o ka maʻamau, a i ʻole he ʻano ʻē kou mau hunaola koko keʻokeʻo a me nā platelets, he hōʻailona paha ia o ka myelofibrosis.
• ʻO ka paʻi koko peripheral (PBS): Nānā kēia i ka nui, ke ʻano, a me nā ʻano ʻē aʻe o kāu mau hunaola koko no nā mea ʻē. Inā he mau hunaola ʻē aʻe a me ka nui o nā hunaola koko ʻōpiopio, hiki nō hoʻi ke lilo i hōʻailona o ka myelofibrosis.
• Nā hoʻokolohua kemika koko:Ke ana nei kēia mau hoʻokolohua i nā pae o nā mea like ʻole i hoʻokuʻu ʻia i loko o ke koko mai kou mau kino. Hiki i kēia ke haʻi iā ʻoe i ka maikaʻi o ka hana ʻana o kahi kino. ʻO nā pae kiʻekiʻe o nā mea e like me ka uric acid, bilirubin, a me ka lactic dehydrogenase i loko o ke koko hiki ke lilo i hōʻailona o ka myelofibrosis.

Nā hoʻokolohua iwi iwi

• Biopsy iwi: Pili kēia i ka lawe ʻana i kahi hāpana liʻiliʻi o kou iwi a nānā iā ia ma lalo o kahi microscope. Hoʻopaʻa ʻia nā ʻāpana e hoʻoholo ai inā he myelofibrosis kou.
• ʻO ka makemake ʻana i ka iwi iwi: Pili kēia i ka wehe ʻana i ka wai mai ka iwi iwi a hoʻāʻo iā ia no nā hōʻailona o ka myelofibrosis.

Pono paha nā hoʻokolohua hou aʻe

Pono paha nā hoʻokolohua hou aʻe e hōʻoia i kāu hōʻoia:

• Ka nānā ʻana i ka hoʻololi ʻana o ka gene: E hoʻāʻo kāu kauka i kāu mau koko a me nā ʻiwi iwi no nā hoʻololi ʻana o ka gene e pili ana me ka myelofibrosis, e like me JAK2, CALR, a me MPL. Hoʻopili kekahi mau lāʻau lapaʻau i nā ʻiwi kanesa i loaʻa ka hoʻololi ʻana o JAK2.
• Nā kaʻina hana kiʻi: Hiki i kāu kauka ke hana i kahi ultrasound e nānā i kahi spleen i hoʻonui ʻia. Hiki iā lākou ke hana i kahi MRI e nānā i ka ʻiʻo ʻeha i loko o ka iwi iwi. Hiki i kēia ʻiʻo ʻeha ke hōʻailona o ka myelofibrosis.

Pehea e mālama ʻia ai ka myelofibrosis?

Inā ʻaʻole ʻoe e hōʻike ana i nā hōʻailona, ​​ʻaʻole paha ʻoe e pono i ka lāʻau lapaʻau. Eia nō naʻe, inā ʻaʻole ʻoe e pono i ka lāʻau lapaʻau koke, e hoʻomau kāu kauka i ka nānā ʻana i kou kūlana.

ʻO Jakafi® (ruxolitinib), Inrebic® (fedratinib), a me Vonjo® (pacritinib) nā lāʻau lapaʻau i ʻāpono ʻia e ka U.S. Food and Drug Administration (FDA) . Hoʻohana ʻia lākou e mālama i ka myelofibrosis waena a kiʻekiʻe. Hana kēia mau lāʻau lapaʻau ʻekolu ma ke ʻano he mau mea hoʻopaʻa JAK . ʻO ia hoʻi, hoʻemi lākou i ka hōʻailona overactive Janus-associated kinase (JAK). ʻO ka hopena, kōkua kēia mau lāʻau lapaʻau i ka hōʻoluʻolu ʻana i nā hōʻailona e pili ana i ka myelofibrosis, e like me ka spleen i hoʻonui ʻia, ka hou ʻana i ka pō, ka maneʻo, ka pohō kaumaha, a me ke kuni.

No nā mea he nui, ʻo ka pahuhopu nui o ka mālama ʻana, ʻo ia ka mālama ʻana i nā kūlana e pili ana i ka myelofibrosis, e like me ka anemia a me ka spleen i hoʻonui ʻia.

Lapaʻau no ka Anemia

Aia kekahi mau lāʻau lapaʻau no ka anemia e like me:

• Androgens: ʻO ka lawe ʻana i nā androgens e like me danazol hiki ke hoʻonui i ka hana ʻana o nā cell koko ʻulaʻula.
• Nā Immunomodulators: Hoʻonui kēia mau lāʻau i ka hiki o kāu ʻōnaehana pale e hakakā i nā hunaola kanesa, a laila e hōʻemi ana i nā hōʻailona. No kēia papa nā lāʻau e like me ka interferon, thalidomide (Thalomid®), a me lenalidomide (Revlimid®). Hiki ke hāʻawi ʻia pū me nā glucocorticoids e like me ka prednisone.
• Nā lāʻau lapaʻau chemotherapy: Hiki i kekahi mau lāʻau chemotherapy ke hōʻemi i nā hōʻailona i hoʻokumu ʻia e ka hoʻonui ʻia o ka helu o nā cell koko a me ka spleen i hoʻonui ʻia. ʻO Hydroxyurea a me cladribine kekahi mau lāʻau.
• Nā hoʻoili koko: Inā loaʻa iā ʻoe ka anemia koʻikoʻi, hiki i nā hoʻoili koko maʻamau ke hoʻonui i kāu helu koko ʻulaʻula.

Lapaʻau Splenomegaly

Hoʻohana ʻia nā mea hoʻopaʻa JAK, nā immunomodulators, a me nā lāʻau chemotherapy e mālama i kahi spleen i hoʻonui ʻia. I nā hihia koʻikoʻi, pono paha ʻoe e wehe ʻia kou spleen ma ke ʻoki ʻana (splenectomy) a i ʻole e loaʻa ka radiation therapy i ka spleen.

Pono paha ka lāʻau lapaʻau radiation e mālama i kahi kūlana i kapa ʻia ʻo extramedullary hematopoiesis, kahi e ulu ai nā hunaola koko ma waho o ka iwi iwi.

Hiki ke ho'ōla loa ʻia ka myelofibrosis?

ʻO ke allogeneic hematopoietic cell transplantation (HCT) paha ke koho wale nō e ho'ōla ai i kēia maʻi. Eia nō naʻe, he hana pilikia kēia a ʻaʻole kūpono no nā kānaka a pau.

Ma kēia kaʻina hana, hoʻololi ʻia kāu mau hunaola koko ʻē me nā hunaola mai kahi mea hāʻawi olakino. Ma mua o ke kāpili ʻana, e hāʻawi ʻia ʻoe i ka chemotherapy a i ʻole ka radiation therapy e pepehi i kāu mau hunaola maʻi. A laila, hiki i nā hunaola hou mai ka mea hāʻawi ke lawe i nā hana o kou kino.

He kiʻekiʻe ka pilikia o nā pilikia i ka mālama ʻana i ka HCT. No laila, kūpono wale nō ia no kahi hui koho o ka poʻe. ʻOi aku ka kiʻekiʻe o ka pilikia o nā pilikia no ka poʻe me nā maʻi ʻē aʻe. Inā kūpono ʻoe no kēia, pili ia i nā kumu he nui, e like me kou makahiki, ke koʻikoʻi o kāu mau hōʻailona, ​​a me ka likelika o ka holomua o ka mālama ʻana.

Pehea ke ʻano o ke ola i ka wā e ola ai me kēia maʻi?

He maʻi ʻaʻai koʻikoʻi loa ka Myelofibrosis. ʻO ka awelika o ka ola ʻana no kēia mau maʻi ma kahi o ʻeono mau makahiki. ʻO ke ʻano o ka awelika o ka ola ʻana, ʻo ia hoʻi ke ola nei kekahi poʻe ma lalo o ʻeono mau makahiki, ʻoiai he helu like ke ola nei no nā makahiki ʻeono. ʻAʻole like kēia no kēlā me kēia kanaka, ʻokoʻa ia mai kēlā kanaka a i kēia kanaka.

Aia kekahi mau kumu e hoʻoholo ai i ka prognosis o kāu maʻi:

• kou mau makahiki.
• Kou mau hōʻailona.
• Ka helu o kou mau hunaola koko.
• Ke koʻikoʻi o ka ʻeha ma ka iwi iwi.
• Inā paha he mau hoʻololi hoʻoilina (JAK2, CALR, MPL).

ʻO ka mea nui loa, ʻo ke kamaʻilio ākea me kāu kauka e pili ana i ka hopena o kāu hōʻailona i kou ola.

Pehea e mālama ai iaʻu iho? (Mālama ponoʻī)

E nīnau i kāu kauka inā hiki iā ʻoe ke pōmaikaʻi mai ka mālama palliative . Hiki i nā hui mālama palliative ke komo pū me nā kauka, nā kahu maʻi, nā limahana hana kaiaulu, a me nā loea ʻē aʻe. Hiki iā lākou ke hāʻawi iā ʻoe i nā kumuwaiwai a me ke kākoʻo āu e pono ai e ola me kēia maʻi. Ma waho aʻe o ka lāʻau āu e loaʻa ai mai kāu oncologist, hiki i kēia poʻe ke lilo i kumu kākoʻo nui. Hiki i nā loea mālama palliative ke kōkua i ka hoʻomaikaʻi ʻana i kou ʻano ola ma ke ʻano he kanaka e noho nei me ka maʻi kanesa.

ʻO ka mea hope loa, nā mea nui loa āu e hoʻomanaʻo ai

He ʻano maʻi koko kakaikahi ka Myelofibrosis kahi e pani ʻia ai kou iwi iwi e nā ʻiʻo fibrous. He kūlana koʻikoʻi kēia e pono ai ka nānā mau ʻana a me/a i ʻole ka mālama ʻana i ke olakino. Ma muli o kou kūlana, ʻaʻole paha ʻoe e loaʻa i kekahi mau hōʻailona no nā makahiki. I nā hihia ʻē aʻe, hiki i nā hōʻailona ke holomua koke a hoʻolilo iā ia i mea paʻakikī ke hana i nā hana o kēlā me kēia lā. Eia nō naʻe, aia nā lāʻau lapaʻau e loaʻa ana e kōkua i ka kaohi ʻana i nā hōʻailona e hoʻopilikia i kou ola o kēlā me kēia lā.

I kēia manawa, e nīnau i kāu kauka e pili ana i nā kumuwaiwai e hiki ke kōkua iā ʻoe e hoʻoponopono i kēia mau loli. He mau koho kōkua nui ka mālama palliative a me nā hui kākoʻo i kou hoʻomaʻamaʻa ʻana i ke ola me ka maʻi kanesa.

E hoʻomanaʻo, ʻaʻole ʻoe hoʻokahi. Aia nā kauka, ka ʻohana, a me nā hoaaloha e kōkua iā ʻoe. Mai makaʻu e kamaʻilio me kāu kauka e pili ana i nā nīnau āu e loaʻa ai.

ʻO Myelofibrosis, maʻi ʻaʻai koko, iwi ʻiʻo, anemia, hoʻonui ʻia o ka spleen, nā mea pale JAK, leukemia