Makemake pū ʻoe e ʻike e pili ana i kēia maʻi koko laha ʻole? E kamaʻilio kākou e pili ana iā Waldenström Macroglobulinemia ma nā ʻōlelo maʻalahi!

Ua lohe paha ʻoe iā Waldenström Macroglobulinemia? He inoa lōʻihi a paʻakikī ke haʻi ʻia, ʻeā? He ʻano maʻi kanesa ia e ulu mālie ana i loko o ke koko. ʻAʻole paha ʻoe i lohe i ia mea, no ka mea, he maʻi kakaikahi ia. Akā, ʻaʻohe pilikia, i kēia lā e kamaʻilio maʻalahi kākou e pili ana i ia mea, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo.

He aha ka Waldenstrom macroglobulinemia (WM)?

I ka ʻōlelo maʻalahi, ʻo Waldenstrom macroglobulinemia, a i ʻole WM no ka pōkole, he maʻi kanesa koko . He ʻano maʻi kanesa ia i kapa ʻia ʻo non-Hodgkin lymphoma, i ʻike ʻia hoʻi ʻo lymphoplasmacytic lymphoma. He mea kākaʻikahi loa ia. E noʻonoʻo wale, ʻoiai ma kahi ʻāina e like me ʻAmelika, ʻekolu a ʻehā wale nō kānaka mai ka miliona e loaʻa i kēia maʻi.

No laila, pehea e ulu ai kēia `(WM)`? Loaʻa iā mākou ka iwi iwi i loko o ko mākou kino, a ma laila i hana ʻia ai ko mākou mau hunaola koko. Hoʻomaka kēia maʻi i ka wā e lilo ai kekahi o nā hunaola i kapa ʻia ʻo `(B cells)` (he ʻano hunaola kēia i loko o kā mākou ʻōnaehana pale, ʻo ia hoʻi, nā hunaola e pale iā mākou mai nā maʻi) i loko o kēia iwi iwi i mau hunaola kanesa.

ʻO kēia mau hunaola kanesa, ma kahi o ka waiho wale ʻia ʻana, hoʻomaka lākou e hana i nā hunaola hou aʻe e like me lākou iho. E like me nā nāhelehele i loko o ka nahele. He aha ka mea e hana ʻia a laila? ʻAʻohe wahi o nā hunaola koko olakino e pono ai ko kākou kino, a hoʻomaka lākou e emi iho.

• Ke haʻahaʻa nā hunaola koko ʻulaʻula, loaʻa ka anemia. Hoʻolilo kēia iā ʻoe i ka luhi a me ke keʻokeʻo.
• Ke haʻahaʻa nā hunaola koko keʻokeʻo, e kū mai kahi kūlana i kapa ʻia ʻo "neutropenia", e hoʻolilo ana iā ʻoe i mea maʻalahi i ka maʻi.
• Ke haʻahaʻa nā platelets (nā pūnaewele e kōkua i ke kāpili ʻana o ke koko) (thrombocytopenia), ʻaʻole paha e pau maʻalahi ke kahe ʻana o ke koko.

ʻAʻole wale ia, hoʻopuka kēia mau hunaola kanesa i kahi protein ʻano ʻē i kapa ʻia ʻo `(immunoglobulin M)` a i ʻole `(IgM)`. Ke piʻi nui kēia protein `(IgM)` i loko o ke koko, lilo ko kākou koko i mānoanoa, e like me ka meli . Ua kapa ʻia kēia ʻo `(hyperviscosity syndrome).` Ke mānoanoa ke koko e like me kēia, lilo ia i mea paʻakikī i ke koko ke kahe ma o nā kīʻaha koko liʻiliʻi ma ke kino holoʻokoʻa. A laila, hiki ke ʻike ʻia kekahi mau hōʻailona koʻikoʻi.

ʻO ka mea nui, ʻaʻohe lāʻau lapaʻau no WM i kēia manawa. Akā, mai hopohopo. Aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona, ​​i kekahi manawa e hoʻopau iā lākou, a kōkua iā ʻoe e noho olakino. He maʻi lohi pinepine ka WM, no laila hiki iā ʻoe ke ola maikaʻi me ia no nā makahiki.

He aha nā hōʻailona o kēia maʻi `(WM)`?

E noʻonoʻo, ma kahi o hoʻokahi o ka ʻehā o ka poʻe me kēia maʻi ʻaʻole hōʻike i kekahi mau hōʻailona . ʻIke wale lākou e pili ana i kēia maʻi ke hele lākou e ʻike i ke kauka no kekahi kumu ʻē aʻe. Akā, i ka wā e ʻike ʻia ai nā hōʻailona, ​​​​hiki pinepine lākou i ka lohi . E nānā kākou i kēia mau hōʻailona:

• Ka nāwaliwali a me ka luhi mau:Me he mea lā ua make ka pila.
• Kuni: Wela ke kino me ke kumu ʻole.
• Anorexia: ʻAʻole makemake e ʻai.
• Hou ka pō: Hou nui ka hou ʻana i ka wā e hiamoe ana.
• Pohō kaumaha: Lilo ʻoe i wīwī no ke kumu ʻole.
• Huikau: Paʻakikī i ka noʻonoʻo ʻana, huikau iki.
• Ka pehu ʻana o ke ake, ka spleen, a i ʻole nā ​​​​​​lime lymph: ʻO ke kauka wale nō ke haʻi iā ʻoe me ka maopopo.
• Nā hōʻailona o ka neuropathy peripheral, e like me ka mae ʻana o nā manamana lima a me nā manamana wāwae : Manaʻo ia e like me ke ʻano ʻūlū, a i ʻole ka nalowale ʻana o ka ʻike.
• Nā hōʻailona o ke koko ʻana: ke kahe ʻana o ka ihu, nā ʻāʻī koko i ka wā e palaki ai i kou mau niho, ka poniuniu, ke poʻo, a me ka ʻike pohihihi.

ʻOiai inā loaʻa iā ʻoe kekahi a ʻelua paha o kēia mau hōʻailona, ​​ʻaʻole ia he manaʻo he WM kou. Akā inā hoʻomau kēia mau hōʻailona, ​​ʻoi aku ka maikaʻi o ka ʻimi ʻana i ke aʻo lapaʻau.

He aha nā kumu no ka hoʻokumu ʻia ʻana o `(WM)`?

ʻO ke kumu nui o ka macroglobulinemia o Waldenstrom, ʻo ia nā hoʻololi genetic . ʻOi aku ma mua o 90% o ka poʻe me ka maʻi, a i ʻole ʻeiwa o ka ʻumi o ka poʻe, he mutation i loko o ka gene i kapa ʻia ʻo MYD88. Ma kahi o 40% o ka poʻe he mutation pū kekahi i loko o ka gene i kapa ʻia ʻo CXCR4. Kōkua kēia mau hoʻololi genetic ʻelua i nā cell abnormal ma WM e māhele koke.

ʻO ka mea nui, ʻaʻole kēia mau loli genetic he mea hoʻoilina . ʻO ia hoʻi, ʻaʻole ia he mea āu e loaʻa ai mai kou mau mākua mai, ʻaʻole hoʻi he mea āu e hoʻoili ai i kāu mau keiki. Hana ʻia lākou i ke ola holoʻokoʻa. Eia nō naʻe, ʻaʻole maopopo i nā kānaka noiʻi ke kumu o kēia mau hoʻololi genetic ma kahi mua.

ʻO wai ka mea i ʻoi aku ka pilikia o ka loaʻa ʻana o kēia maʻi? (Nā kumu pilikia)

Aia kekahi mau kumu e hoʻonui ai i ka manawa kūpono o ka hoʻomohala ʻana i ka `(WM)`. E nānā kākou i ke ʻano o lākou:

• Makahiki: ʻIke pinepine ʻia kēia maʻi i waena o ka poʻe i piha i 65 mau makahiki a ʻoi paha.
• Lāhui: ʻOi aku ka maʻamau o kēia maʻi i waena o ka poʻe keʻokeʻo.
• Kāne: ʻOi aku ka nui o ka loaʻa ʻana o kēia maʻi i nā kāne.
• Nā kūlana olakino ʻē aʻe: Hiki iā ʻoe ke piʻi aʻe ka pilikia inā loaʻa iā ʻoe nā maʻi e like me `(Hepatitis C)`, `(AIDS)`, `(Sjögren's Syndrome)`, a i ʻole kahi kūlana i kapa ʻia ʻo `(MGUS - Monoclonal Gammopathy of Undetermined Significance)` (he mea kēia ma mua o `(WM)`). Eia nō naʻe, he mea nui e hoʻomanaʻo ʻaʻole nā ​​​​mea āpau me `(MGUS)` e loaʻa iā `(WM)`.
• Mōʻaukala ʻohana: Inā ua loaʻa i kou mau hoahānau koko ʻo WM a i ʻole nā ​​ʻano lymphoma ʻē aʻe, hiki paha iā ʻoe ke pilikia.

He aha nā pilikia e hiki mai ana o kēia maʻi?

I kekahi mau hihia koʻikoʻi, hiki iā WM ke hana i nā pilikia ʻē aʻe.

• Amyloidosis: ʻO kēia ka wā e waiho ʻia ai nā protein hewa i loko o nā ʻōpū e like me ka puʻuwai, nā māmā, a me nā puʻupaʻa.
• Cryoglobulinemia: Ma kēia kūlana, hōʻiliʻili kekahi mau protein koko e pane ana i ke anu i loko o nā lālā a hui pū. Hiki i kēia ke hana i ka ʻeha a me ka hoʻololi ʻana o ke kala polū/keʻokeʻo (cyanosis).

Pehea e ʻike ai nā kauka i kēia maʻi? (Diagnosis)

Hiki i kāu kauka ke hoʻohana i kekahi mau hoʻokolohua e ʻike ai inā he WM kou. Ke ʻimi nui nei lākou i nā hunaola kanesa a me ka protein IgM a mākou i kamaʻilio ai ma mua.

• Nā hoʻāʻo koko a me ka mimi: Lawe ʻia nā hāpana koko a me ka mimi e nānā i nā hōʻailona o WM. Ke nānā nei lākou i nā mea e like me ka helu haʻahaʻa o nā cell koko a me nā protein IgM maʻamau ʻole.
• Nā hoʻāʻo kiʻi: Hoʻohana ʻia kahi CT scan a i ʻole CT-PET scan, ʻo ia ka hui pū ʻana o ka CT a me ka PET scan, e nānā i nā hōʻailona o WM i loko o ke kino. Ke nānā nei kēia mau hoʻāʻo i nā mea e like me nā ʻōpū pehu a me nā lymph node.
• Hoʻokolokolo maka: Nānā i ke kahe ʻana o ke koko i loko o ke kua o ka maka. He hōʻailona kēia o nā ʻūpā koko.
• Biopsy iwi ʻiwi: Pili kēia i ka lawe ʻana i kahi hāpana liʻiliʻi o ka iwi ʻiwi a nānā iā ia ma lalo o kahi microscope e ʻike ai inā he mau hunaola kanesa.

Pehea e mālama ʻia ai ʻo `(WM)`?

E like me kā mākou i ʻōlelo ai ma mua, ʻaʻohe lāʻau lapaʻau no kēia maʻi i kēia manawa. No laila, ʻo ka lāʻau lapaʻau maikaʻi loa ka hōʻoluʻolu ʻana i nā hōʻailona me nā hopena ʻaoʻao liʻiliʻi . E kamaʻilio kāu kauka me ʻoe a hana i kahi hoʻolālā lapaʻau kūpono iā ʻoe. Eia kekahi o nā koho lapaʻau no `(WM)`:

• Ke kali makaʻala: Inā ʻaʻohe ou hōʻailona, ​​ʻaʻole paha e hoʻomaka kāu kauka i ka lāʻau lapaʻau. ʻAʻole pono paha kekahi poʻe me WM i ka lāʻau lapaʻau no nā makahiki.
• Plasmapheresis / hoʻololi plasma: Pili kēia i ka hoʻohana ʻana i kahi mīkini e kānana i ka protein IgM maʻamau ʻole mai ka plasma, ka ʻāpana wai o kou koko. A laila e hoʻihoʻi ʻia ka plasma i hoʻomaʻemaʻe ʻia i loko o kou koko. Hoʻohana ʻia kēia lāʻau lapaʻau e hōʻemi i nā hōʻailona o ke kāpili ʻana o ke koko.
• Immunotherapy: Hoʻohana kēia lāʻau i kāu ʻōnaehana pale ponoʻī e luku a hoʻolohi paha i ka ulu ʻana o nā pūnaewele WM. Hāʻawi pinepine ʻia ka lāʻau Rituximab (Rituxan®) me ka chemotherapy a i ʻole me ka chemotherapy.
• Chemotherapy: Hiki ke hāʻawi wale ʻia nā lāʻau pepehi cell cancer a i ʻole e hui pū ʻia me ka immunotherapy.
• Corticosteroids: Hiki ke hāʻawi ʻia kekahi ʻano steroid, e like me dexamethasone, me ka immunosuppressive therapy a me ka chemotherapy. Kōkua lākou i ka hakakā ʻana i ka maʻi kanesa me ka hoʻēmi ʻana i nā hopena ʻaoʻao o ka lāʻau lapaʻau.
• Hoʻōla i kuhikuhi ʻia:Hana kēia lāʻau lapaʻau ma ke kāohi ʻana i nā protein a nā hunaola kanesa e hoʻohana ai e ulu. ʻO Ibrutinib (Imbruvica®) a me zanubrutinib (Brukinsa®) ʻelua mau lāʻau lapaʻau i ʻāpono ʻia e ka U.S. Food and Drug Administration (FDA) e mālama iā WM.
• Hoʻoili pūnaewele kumu: Ma kēia, hoʻoili ʻia ka iwi iwi olakino e pani i ka iwi iwi i hoʻopilikia ʻia e nā pūnaewele ʻano ʻē. ʻAʻole hana pinepine ʻia kēia, a hana wale ʻia i kekahi mau maʻi i koho ʻia.

Āhea wau e ʻimi ai i ke aʻo lapaʻau?

Inā ua ʻike ʻia ʻoe me WM, e kamaʻilio koke me kāu kauka inā loaʻa iā ʻoe kekahi mau hōʻailona hou a inā paha ʻoe e hopohopo nei no kekahi mau hōʻailona e kū nei. Ke ʻike ʻia ʻoe me WM, he mea nui e nīnau i nā nīnau e ʻike ai i ka mea e manaʻo ai. Eia kekahi mau nīnau hiki iā ʻoe ke nīnau i kāu kauka:

• Pehea ke koʻikoʻi o ka `(WM)`? Pehea e pili ai kēia i koʻu ola?
• He aha kaʻu e manaʻo ai i ka wā pōkole a me ka wā lōʻihi?
• Pono anei iaʻu e hoʻomaka koke i ka lāʻau lapaʻau?
• He aha ke ʻano o ka lāʻau lapaʻau āu e paipai nei?
• He aha nā hopena ʻaoʻao e hiki ke manaʻo ʻia mai ka lāʻau lapaʻau?

He aha kaʻu e manaʻo ai inā loaʻa iaʻu kēia maʻi?

ʻAʻole like nā kānaka a pau me ka maʻi holomua mālie e like me ka macroglobulinemia o Waldenstrom. Hōʻike ka noiʻi he 66%, a ʻoi aku paha ma mua o ʻelua o ʻekolu mau kānaka, ke ola nei i 10 mau makahiki ma hope o ka hōʻoia ʻana. Eia nō naʻe, hiki ke loli nā manawa ola me ka makahiki . ʻO ka hapa nui o ka poʻe ma luna o 65 (ka hui makahiki i ʻike pinepine ʻia me ka maʻi) make mai nā kumu i pili ʻole i ka WM.

He nui nā kumu e hoʻopilikia i ka wānana o kou maʻi. Eia kekahi laʻana:

• kou mau makahiki
• Nā hopena hoʻāʻo koko
• ʻAno o ka hoʻololi ʻana o ka genetic (i kekahi manawa ʻo ka nele o ka hoʻololi ʻana o ka `(MYD88)` hiki ke hōʻike i kahi hopena maikaʻi ʻole)

Inā he mau nīnau kāu e pili ana i kou kūlana, e kamaʻilio me kāu kauka . ʻIke maikaʻi ʻo ia iā ʻoe a me nā mea e hoʻopilikia paha i kou olakino.

Aia kekahi mea hiki iaʻu ke hana e ʻoi aku ka maikaʻi?

ʻO ka noho ʻana me kahi lymphoma e like me ka macroglobulinemia a Waldenstrom hiki ke hana i kekahi mau hoʻololi nui i kou ola. He mea nui e hoʻohana i nā kumuwaiwai āpau āu e loaʻa ai. E kamaʻilio me kāu kauka e pili ana i nā meaʻai e ʻai ai a me nā hana mālama ponoʻī e hana ai .

I mea e pale aku ai i ka manaʻo mehameha, e launa pū me nā poʻe ʻē aʻe i loaʻa i kēia maʻi laha ʻole. ʻOiai inā manaʻo ʻoe pēlā i ka wā mua ke ʻike ʻoe he maʻi kou, ʻaʻole ʻoe hoʻokahi i kēia huakaʻi . E nīnau i kāu kauka a hui pū me nā hui kākoʻo.

ʻO ka mea hope loa, e hoʻomanaʻo i kēia.

ʻAʻole i loaʻa i nā kānaka noiʻi kahi lāʻau lapaʻau no Waldenstrom macroglobulinemia (WM). Eia nō naʻe, ua loaʻa iā lākou kekahi mau lāʻau lapaʻau e maʻalahi ai ka noho ʻana me ka maʻi . Noho ka poʻe he nui me ka maʻi no nā makahiki me ka ʻole o nā hōʻailona. Ke ʻae nei nā lāʻau lapaʻau hou i ka poʻe me WM e ola lōʻihi ma mua o ka wā ma mua, me ka ʻole o ka mōhai ʻana i ko lākou ʻano ola.

Inā loaʻa iā ʻoe kēia hōʻailona, ​​mai hopohopo a nīnau i kāu kauka e pili ana i ka wā pōkole a me ka wā lōʻihi. E kōkua lākou iā ʻoe e hoʻoholo i ka hoʻolālā lapaʻau maikaʻi loa nāu. E hoʻomanaʻo, me ka ʻōlelo aʻoaʻo lapaʻau kūpono a me ke kākoʻo, hiki iā ʻoe ke ola me ka holomua me kēia maʻi.

ʻO Waldenstrom macroglobulinemia, WM, maʻi ʻaʻai koko, protein IgM, hyperviscosity syndrome, iwi ʻiʻo, lymphoma