He aha ka POEMS Syndrome? Makemake ʻoe e ʻike e pili ana i kēia?

Ua lohe paha ʻoe i ka POEMS Syndrome? He maʻi laha ʻole kēia i lohe pinepine ʻole ʻia. Eia nō naʻe, he mea nui e ʻike iki e pili ana i ia mea no ka mea hiki iā ia ke hoʻopilikia i nā ʻōnaehana he nui i loko o ko mākou kino i ka manawa hoʻokahi. I ka ʻōlelo maʻalahi, he maʻi paʻakikī ia e pili ana i ko mākou koko. I kēia lā, e kamaʻilio mākou e pili ana i ke ʻano o ka POEMS Syndrome, he aha kona mau hōʻailona, ​​pehea e ʻike ʻia ai, a inā he lāʻau lapaʻau.

He aha maoli ka POEMS Syndrome?

ʻAe, e nānā kākou i ke ʻano o ka POEMS Syndrome. He maʻi koko laha ʻole kēia. Loaʻa iā kākou kahi ʻano koko i kapa ʻia he mau cell plasma i loko o ko kākou kino. Ke hiki mai ka POEMS syndrome, māhelehele kēia mau cell plasma a hoʻonui me ke ʻano ʻē, me ke kāohi ʻole ʻia. Hoʻokuʻu kēia mau cell plasma ʻē i kahi protein kūikawā i loko o ko kākou koko. Ua kapa ʻia ʻo ia he monoclonal protein, a i ʻole M-protein no ka pōkole. I kēia manawa hiki iā ʻoe ke noʻonoʻo, ʻeā? Ke hoʻonui nui ʻia kēia mau cell plasma ʻē, ʻo M-protein, i loko o ke koko, hiki iā ia ke hōʻino i ko kākou mau aʻalolo a me nā ʻōpū o loko.

Ua loaʻa ka inoa ʻo POEMS mai nā leka mua o nā hōʻailona nui ʻelima o kēia maʻi:

• P - Polyneuropathy: Pili kēia i ka hōʻino ʻana i nā aʻalolo ma ke kino holoʻokoʻa. Ma POEMS, hiki i kēia hōʻino ke hōʻeha a i ʻole nā ​​​​manaʻo ʻoluʻolu ʻole ʻē aʻe (e like me ke kani ʻana, ka wela, a me nā mea ʻē aʻe) ma kou mau wāwae, lima, kapuwaʻi, a me nā lima.
• O - Organomegaly: ʻO ke ʻano kēia, lilo nā ʻōpū o loko i mea nui loa. Hiki i ke kanaka me POEMS ke loaʻa nā ʻōpū i hoʻonui ʻia e like me ke akepaʻa, ka spleen, a me nā lymph nodes.
• E - Endocrinopathy: I ka ʻōlelo maʻalahi, he pilikia hormonal kēia. I loko o POEMS, hiki ke ʻano ʻē ka nui o nā hormones i hoʻokuʻu ʻia e kā mākou mau kelepa endocrine. Hiki ke hūnā ʻia kekahi mau hormones ma ke keu a i ʻole ma nā ʻāpana liʻiliʻi.
• M - Monoclonal protein (M-protein): ʻO kēia ka protein kūikawā e hōʻiliʻili i loko o ke koko ma POEMS. Ke hana kahi cell plasma hoʻokahi i nā kope he nui (clones) o ia iho, hoʻohui kēlā me kēia o kēlā mau cell i kēia M-protein i ke koko. I ka hopena, piʻi nui ka nui o ka M-protein i loko o ke koko.
• S - Nā loli ʻili: Hiki i ka poʻe me POEMS ke ʻike i nā loli ʻili like ʻole, e like me ka ʻeleʻele ʻana o ka ʻili a me ka mānoanoa o ka ʻili.

Ua ʻike ʻia hoʻi ka maʻi POEMS ʻo osteosclerotic myeloma , Crow-Fukase syndrome , PEP syndrome , a me Takatsuki syndrome .

ʻO wai ka mea e loaʻa pinepine ai ka maʻi POEMS?

He maʻi laha ʻole ka maʻi POEMS. He kakaikahi wale nō ka poʻe e hoʻopilikia nei i ka honua holoʻokoʻa. ʻIke pinepine ʻia ia i nā kānaka ma waena o 50 a me 60 mau makahiki. Hiki iā ia ke hoʻopilikia i nā kāne ʻelua, akā ʻoi aku ka maʻamau i nā kāne.

He aha nā hōʻailona o ka maʻi POEMS?

E like me kā mākou i ʻōlelo ai ma mua, ʻo nā leka ma ka inoa ʻo POEMS e hōʻike ana i kekahi o nā hōʻailona nui. ʻO ia hoʻi, ʻo Polyneuropathy, Organomegaly, Endocrinopathy, M-protein elevation, a me nā loli ʻili nā hōʻailona nui. ʻO ka hapa nui o nā kānaka me POEMS he polyneuropathy a me M-protein elevation. Hiki ke ʻokoʻa nā hōʻailona ʻē aʻe mai kēlā kanaka a kēia kanaka.

Nā hōʻailona i hoʻokumu ʻia e ka polyneuropathy

ʻO nā hōʻailona i hoʻokumu ʻia e kēia hōʻino aʻalolo ka mea maʻamau i nā POEMS. ʻO ka hapa pinepine, ʻike nui ʻia ka ʻeha ma nā wāwae a me nā wāwae. Akā i kekahi manawa hiki ke hoʻopilikia i nā lima a me nā lima. Eia kou manaʻo ke loaʻa iā ʻoe kēia maʻi:

• Nāwaliwali
• ʻUʻumi
• Māʻule
• Manaʻo ʻūlū / ʻōniʻoniʻo
• Manaʻo wela

Organomegaly - ka hoʻonui ʻia ʻana o nā ʻōpū o loko

Hiki i nā kaʻina hana kiʻi i hana ʻia e nā kauka ke ʻike inā ua hoʻonui ʻia kēia mau ʻōkana:

• ʻAke (Ake - `(Hepatomegaly)`)
• ʻO ka ʻōpū liʻiliʻi (Splenomegaly)
• Nā kikowaena Lymph (Nā kikowaena Lymph - `(Lymphadenopathy)`)

Endocrinopathy - nā pilikia hormonal

ʻO ka pilikia hormonal nui e hele mai me POEMS ka emi ʻana o ka pae o nā hormones wahine. Ua kapa ʻia kēia mau hormones wahine ʻo "Testosterone" i nā kāne a me "Estrogen" i nā wahine. Eia kekahi, e ʻike paha ʻoe i nā hōʻailona e like me:

• Nā pilikia e kaohi ana i nā pae kō koko (e like me ka maʻi diabetes)
• Nā pilikia thyroid (e.g. Hypothyroidism)
• ʻO ka maʻi o Addison (ka lawa ʻole o ka adrenal - `(ka maʻi o Addison)`)

Hiki i nā kāne ke ʻike i ka emi ʻana o ka hānau keiki a me ka hoʻonui ʻana o ka umauma (Gynecomastia).

No nā wāhine , hiki ke pau ka menstruation (`(Amenorrhea)`), hiki ke hoʻonui ʻia nā umauma. I ka wā hāpai, hiki ke hana nui ʻia ka hormone `(Prolactin)`, ka mea e kōkua ai i ka hana ʻana o ka waiū. No laila, hiki i ka waiū ke kahe mai nā nipples ʻoiai inā ʻaʻole ʻoe hāpai.

Hoʻonui ʻia ka M-Protein

ʻO kahi hiʻohiʻona koʻikoʻi o POEMS ka pae kiʻekiʻe o ka protein M i loko o ke koko. Hiki i kēia keu o ka protein M ke alakaʻi i ka hoʻokumu ʻia ʻana o nā puʻupuʻu i loko o ke koko. Eia kekahi, ma muli o ke kiʻekiʻe o ka protein M, ʻo kāuHiki i nā iwi ke lilo i mānoanoa a paʻakikī (osteosclerosis) .

Nā loli ʻili

Hiki ke loaʻa nā loli ʻili like ʻole, e like me:

• ʻIli ʻeleʻele
• ʻIli mānoanoa
• Nā kui lima keʻokeʻo
• Hoʻonui ʻia ka ulu ʻana o ka lauoho ma kou maka a me kou mau wāwae
• Ka ulu ʻana o nā kīʻaha koko e like me nā cherries liʻiliʻi ma ka ʻili o ka umauma

Nā hōʻailona ʻē aʻe

Ma waho aʻe o kēia, hiki ke ʻike ʻia nā hōʻailona ʻē aʻe:

• Ka pehu ʻana o nā lima a me nā wāwae
• ʻO ka pohō kaumaha me ke kumu ʻole
• ʻEha i nā iwi a me nā hono
• Nā hemahema o ka ʻike maka
• Paʻakikī ka hanu ʻana
• Luhi
• kuni

Hiki i kekahi poʻe me ka maʻi POEMS ke loaʻa i kahi maʻi i kapa ʻia ʻo Castleman Disease (CD), ka mea e hoʻopilikia ai i nā lymph nodes. Inā loaʻa iā ʻoe nā hōʻailona o ka maʻi Castleman, hiki i kāu kauka ke kānalua i ka POEMS.

He aha ke kumu o ka maʻi POEMS?

Ke loaʻa iā ʻoe nā POEMS, ʻike ʻoe i nā ʻano hōʻailona a me nā hopena like ʻole ma muli o kahi maʻi cell plasma monoclonal . I ka ʻōlelo maʻalahi, hana kahi cell plasma maʻamau i nā kope he nui o ia iho. ʻO ka nui o kēia mau cell, ʻoi aku ka nui o ka hōʻino hiki iā lākou ke hana i nā ʻiʻo. Hoʻohui kēia mau cell i ka M-protein i ke koko, e hoʻonui ana i ka nui o ka M-protein i loko o ke koko. ʻO kēia mau cell keu a me ka M-protein e hōʻino i nā ʻōnaehana like ʻole i loko o ke kino.

Eia nō naʻe, ʻaʻole maopopo i nā kānaka ʻepekema ke kumu e hoʻomaka ai kēia mau cell plasma e ulu like ʻole ma kahi mua.

Ua ʻike nā kānaka noiʻi he protein i kapa ʻia ʻo Vascular Endothelial Growth Factor (VEGF) paha ke pili. ʻO ka hapa nui o ka poʻe me ka maʻi POEMS he kiʻekiʻe ko lākou pae o VEGF i loko o ko lākou koko. Ke hoʻomau nei ka noiʻi ʻana i ka pilina ma waena o VEGF a me ka maʻi POEMS.

Pehea e ʻike ʻia ai ka maʻi POEMS? (Diagnosis)

E nīnau mua kāu kauka iā ʻoe e pili ana i kou mōʻaukala olakino a me nā hōʻailona o kēia manawa. A laila, e hana lākou i kahi hoʻokolokolo kino e nānā i nā hōʻailona o ka maʻi POEMS.

Eia kekahi, hiki i ke kauka ke hana i kekahi mau hoʻokolohua, e like me:

• Nā hoʻāʻo koko a me ka mimi: Hiki i kēia mau hoʻāʻo ke kōkua i ka nānā ʻana i nā pae kiʻekiʻe o ka M-protein a i ʻole VEGF i loko o kou koko a i ʻole mimi. Hiki iā lākou ke nānā i nā helu koko ʻē aʻe a i ʻole nā ​​​​​​kiko koko ʻē aʻe. Hiki i kēia ke lilo i mau hōʻailona o POEMS.
• Nā hoʻāʻo kiʻi: Hiki i nā hoʻāʻo e like me nā X-ray a me nā scan CT ke hōʻike inā mānoanoa a ʻoʻoleʻa paha kou mau iwi. Hiki ke hana ʻia kēia mau loli ke kiʻekiʻe nā pae M-protein.
• ʻO ka biopsy iwi: Hiki i kēia ke ʻike inā he mau cell plasma ʻano ʻē kou i loko o kou iwi, a inā paha he kiʻekiʻe loa ka helu o nā cell plasma.
• Electromyogram (EMG): He hoʻāʻo ka EMG e ana i ka hana aʻalolo. Hiki iā ia ke kōkua i kāu kauka e ʻike i ka polyneuropathy.

Ma muli o kāu mau hōʻailona, ​​​​hiki i kāu kauka ke hana i nā hoʻokolohua hou aʻe, e like me nā hoʻāʻo hanu, echocardiograms, a i ʻole nā ​​​​hoʻokolohua endocrine.

Inā ua ʻike ʻia ʻoe me kahi maʻi i kapa ʻia ʻo Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) , hiki i kāu kauka ke hoʻāʻo iā ʻoe e ʻike inā he POEMS syndrome kāu.

Pehea e mālama ʻia ai a i ʻole e mālama ʻia ai ka maʻi POEMS?

ʻAʻohe lāʻau lapaʻau no ka maʻi POEMS. Eia nō naʻe, hiki i nā lāʻau lapaʻau ke kōkua i ka kaohi a me ka mālama ʻana i nā hōʻailona. ʻO nā koho lapaʻau e komo pū me:

• Lapaʻau Radiation: Pili kēia i ke kuhikuhi ʻana i nā pae kiʻekiʻe o ka radiation ma nā cell plasma maʻamau e luku iā lākou.
• Chemotherapy: Hoʻohana ʻia nā lāʻau lapaʻau anticancer, i ʻike ʻia hoʻi ʻo "Chemotherapy," e pepehi a hoʻolohi paha i ka ulu ʻana o nā cell plasma ʻē. Ua like ka Chemotherapy no POEMS me ka mālama ʻana no ka myeloma maha. He kūpono loa kēia mau lāʻau lapaʻau a ʻaʻole pinepine lākou e hoʻoulu i nā hopena ʻaoʻao koʻikoʻi.
• Hoʻoili ʻana i nā cell kumu autologous: Hoʻololi kēia kaʻina hana i kāu mau cell plasma ʻano ʻē me nā cell kumu olakino. ʻO nā cell kumu nā cell e ulu aʻe i loko o nā cell makua e like me nā cell plasma. Ma kēia hoʻoili ʻana, wehe mua kāu kauka i kekahi mau cell kumu olakino mai kou kino. A laila, hoʻohana lākou i ka chemotherapy a i ʻole ka radiation therapy e luku i nā cell plasma ʻano ʻē. Ma hope o ka mālama ʻana, hoʻihoʻi ʻia nā cell kumu i lawe ʻia i loko o kou kino. A laila ulu lākou i loko o nā cell plasma olakino.
• Hoʻōla kino: Inā pilikia ʻoe i ka hele ʻana ma muli o ka polyneuropathy, hiki i kēia lāʻau ke kōkua.

Eia kekahi, hiki i ke kauka ke kuhikuhi i nā lāʻau lapaʻau e like me nā steroids e hōʻoluʻolu i nā hōʻailona a me nā diuretics (nā pila wai) e hōʻemi i ka pehu.

He aha nā pilikia e pili ana me ka maʻi POEMS?

Me ka maʻi POEMS, hiki ke hoʻonui ʻia ka pōʻino o ke aʻalolo a me ka nāwaliwali i ka hala ʻana o ka manawa. Hiki iā ia ke paʻakikī i ka neʻe ʻana a puni. Inā nui ka wai e hōʻiliʻili kokoke i nā māmā a i loko paha o ka lua o ka umauma, hiki iā ia ke hōʻeha i ka umauma a me ka paʻakikī o ka hanu ʻana.

Akā, e hoʻomanaʻo,Hiki i ka lāʻau lapaʻau holomua ke hoʻōki a hoʻolohi paha i ka hōʻino ʻana o ke aʻalolo. Hiki iā ia ke kōkua i ka hōʻoluʻolu ʻana i nā hōʻailona ʻē aʻe. Hiki i ka hōʻino ʻana o ke aʻalolo ke ho'ōla mālie i ka hala ʻana o ka manawa.

Aia kekahi mau mea pilikia no ka maʻi POEMS?

ʻAʻohe mea pilikia i kēia manawa no ka hoʻomohala ʻana i ka maʻi POEMS.

Hiki ke pale ʻia ka maʻi POEMS?

ʻO ka mea pōʻino, ʻaʻole hiki ke pale ʻia ka maʻi POEMS.

He aha ka wānana no ka poʻe me ka maʻi POEMS?

Aia kou olakino i nā kumu he nui, me nā ʻāpana o kou kino i hoʻopilikia ʻia, pehea ka wikiwiki o ka ʻike ʻia ʻana o ka maʻi a hoʻomaka ka mālama ʻana, a pehea ʻoe e pane ai i ka mālama ʻana.

He mea nui e loaʻa ka lāʻau no ka POEMS. Hiki i ka maʻi POEMS ke hoʻoweliweli i ke ola inā waiho ʻole ʻia. Ma ka ʻaoʻao ʻē aʻe, hiki i nā lāʻau lapaʻau holomua e hoʻopau i nā cell plasma maʻamau ke kōkua iā ʻoe e ola lōʻihi. Eia kekahi, hiki i nā lāʻau lapaʻau ke hōʻoluʻolu i nā hōʻailona a hoʻomaikaʻi i kou ʻano ola.

ʻO ka awelika o ke ola ʻana no ka hapa nui o ka poʻe me POEMS ma kahi o 14 mau makahiki. ʻO ia hoʻi, ma kahi o ka hapalua o ka poʻe e ola ana ma lalo o 14 mau makahiki, ʻoiai ʻo ka hapalua ʻē aʻe e ola ana i ka lōʻihi. Aia kou manaʻolana ola i kāu ʻike maʻi a me ka mālama ʻana.

E nīnau i kāu kauka e pili ana i kou wā e hiki mai ana ma ke ʻano he kanaka e noho nei me ka maʻi POEMS.

Āhea ʻoe e ʻike ai i ke kauka e pili ana i ka maʻi POEMS?

Inā ʻike ʻoe i kekahi mau hōʻailona e pili ana i ka maʻi POEMS, ʻoiai ka nāwaliwali ma nā lima a me nā wāwae , e ʻike koke i ke kauka.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka e pili ana i ka maʻi POEMS?

Hiki iā ʻoe ke nīnau i nā nīnau e like me kēia:

• Pehea ke koʻikoʻi o koʻu kūlana?
• He aha nā koho lapaʻau e loaʻa iaʻu?
• He aha kaʻu e hana ai e hōʻemi a mālama paha i nā hopena ʻaoʻao?
• He aha nā lawelawe kākoʻo i loaʻa iaʻu?
• Pono anei au e ʻike i ke kauka loea i nā maʻi cell plasma e alakaʻi i kaʻu lāʻau lapaʻau?

Āhea e hiki ai iaʻu ke hoʻi i kaʻu mau hana maʻamau inā loaʻa iaʻu ka maʻi POEMS?

Hiki i ka maʻi POEMS ke hoʻopilikia i kou hiki ke hoʻokō i kāu mau hana o kēlā me kēia lā. E wehewehe kāu kauka iā ʻoe pehea e hoʻopilikia ai kēia ʻano i kou ola o kēlā me kēia lā.

He maʻi autoimmune anei ka maʻi POEMS?

ʻO ka POEMS kahi maʻi paraneoplastic.ʻAʻole ia he maʻi autoimmune. ʻO ka paraneoplastic syndrome kahi hōʻiliʻili o nā hōʻailona i hoʻokumu ʻia e kahi kūlana olakino. I loko o POEMS, ʻo ka pilikia nui, ʻo ia ka hana ʻana o nā cell plasma maʻamau ʻole i ka nui o ka M-protein i loko o ke koko (kahi maʻi cell plasma monoclonal). Hoʻokumu ʻia nā hōʻailona o POEMS e nā cell plasma maʻamau ʻole a me ka M-protein e hōʻino ana i nā ʻiʻo o ke kino.

Ua like anei ka maʻi POEMS me ka myeloma maha?

He maʻi ʻaʻai ka myeloma lehulehu e hoʻopilikia ana i nā cell plasma. ʻO ka maʻi POEMS kahi hōʻiliʻili o nā hōʻailona i hoʻokumu ʻia e nā cell plasma ʻano ʻole a me ka hoʻonui ʻia o ka M-protein. ʻOi aku ka maikaʻi o ka helu ola o ka maʻi POEMS ma mua o ka manaʻolana ola o ka myeloma lehulehu.

Eia nō naʻe, like pinepine nā lāʻau lapaʻau chemotherapy no POEMS a me ka myeloma multiple. Hiki ke like kekahi o nā hōʻailona.

He maʻi paʻakikī ka maʻi POEMS. Hiki ke lōʻihi ka manawa o kāu kauka e hoʻoholo ai ua hana ʻia kāu mau hōʻailona e ka maʻi POEMS a ʻaʻole e kekahi maʻi ʻē aʻe. Ke loaʻa iā ʻoe ka maʻi, e hana pū me kāu hui lapaʻau e hoʻoholo i nā lāʻau lapaʻau e hāʻawi i ka hōʻoluʻolu nui loa mai kāu mau hōʻailona. E nīnau i kāu kauka e pili ana i nā hui a i ʻole nā ​​​​hui kākoʻo e kōkua ana i nā kānaka a me nā ʻohana e hana ana me nā maʻi e like me ka maʻi POEMS.

Leka Lawe-Home

ʻAe, ua kamaʻilio nui mākou e pili ana i ka POEMS Syndrome i kēia lā. ʻO ka mea nui e hoʻomaopopo he maʻi kakaikahi kēia, akā he maʻi koʻikoʻi . Hiki iā ia ke hoʻopilikia i nā ʻōnaehana he nui i loko o ko mākou kino.

• Ua loaʻa ka inoa ʻo POEMS mai nā leka mua o nā inoa Pelekane o nā hōʻailona nui ʻelima (nā maʻi nerve, ka hoʻonui ʻia ʻana o nā ʻōpū o loko, nā pilikia hormonal, ka hoʻonui ʻia o ka M-protein, a me nā loli o ka ʻili).
• ʻO ke kumu nui o kēia nā pūnaewele plasma kūpono ʻole a me ka protein M-a lākou e hana ai.
• ʻOiai ʻaʻohe lāʻau lapaʻau piha, aia nā lāʻau lapaʻau kūpono e kaohi i nā hōʻailona a hoʻomaikaʻi i ka maikaʻi o ke ola.
• He mea nui loa ka ʻike ʻana i ka maʻi a hoʻomaka i ka lāʻau lapaʻau i ka wā mua .

Inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona, ​​e ʻoluʻolu e ʻimi i ke kōkua lapaʻau. Mai hopohopo, me ka ʻike kūpono a me ke kākoʻo lapaʻau, hiki iā ʻoe ke hoʻoponopono i kēia kūlana.

ʻO ka maʻi POEMS, nā maʻi koko, ka hōʻino ʻana o ke aʻalolo, nā pilikia hormonal, M-protein, nā loli ʻili, nā cell plasma