He pilikia anei kēia protein M no kou mau puʻupaʻa? (Monoclonal Gammopathy of Renal Significance - MGRS) E kamaʻilio kākou e pili ana i kēia!

Ua ʻike kākou a pau he ʻōkana koʻikoʻi ko kākou mau puʻupaʻa e hana mālie ana i kāna hana. Eia nō naʻe, i kekahi manawa hiki i kēia mau puʻupaʻa ke maʻi mālie. I kēia lā e kamaʻilio kākou e pili ana i kahi maʻi e hoʻoulu ai i ka maʻi puʻupaʻa, kahi mea paʻakikī iki paha ke lohe ʻoe i ka inoa, akā maʻalahi ke hoʻomaopopo. Ua kapa ʻia kēia ʻo MGRS (Monoclonal Gammopathy of Renal Significance) . ʻOiai he lōʻihi iki ka inoa, mai hopohopo, e hoʻomaopopo maʻalahi kākou.

He aha ka MGRS maʻalahi?

ʻAe, e nānā kākou i ke ʻano o MGRS. I ka ʻōlelo maʻalahi, ʻo MGRS kahi hui o nā maʻi e hōʻino ana i nā puʻupaʻa. Hana ʻia kēia hōʻino ma muli o kekahi o nā cell plasma o ko mākou kino, a i ʻole nā ​​​​cell B (ʻo kēia nā ʻano o nā cell koko keʻokeʻo), hana i nā kope he nui o lākou iho, a i ʻole nā ​​​​clones . E noʻonoʻo iā ia e like me kahi mīkini kope e hana ana i nā kope he nui o ka pepa like.

ʻO nā pūnaewele plasma e hana ʻia ma kēia ʻano e hana i kahi ʻano antibody i kapa ʻia ʻo ka protein M. Hoʻopili kēia mau protein M i loko o nā ʻano kino like ʻole o ko mākou kino, ʻoi aku hoʻi nā puʻupaʻa, a hoʻomaka e hōʻino iā lākou.

ʻO ka mea nui, ʻaʻole ʻo MGRS he maʻi kanesa. Eia nō naʻe, hiki i kahi poʻe liʻiliʻi me MGRS ke loaʻa i ka maʻi kanesa, e like me ka myeloma multiple . No laila he mea nui e makaʻala i kēia.

Aia kekahi mau ʻano o MGRS?

ʻAe, aia ma mua o hoʻokahi kakini mau ʻano o nā maʻi MGRS. Ua hoʻokaʻawale ʻia kēia mau mea e like me ka ʻāpana o ka puʻupaʻa e hoʻopilikia ʻia e ka protein M. ʻEkolu mau māhele nui:

1. Nā maʻi glomerular

ʻO ke ʻano maʻamau o MGRS ʻo ia nā glomerulopathies . Hoʻopōʻino kēia i nā kīʻaha koko liʻiliʻi i loko o nā puʻupaʻa. Ua like lākou me nā ʻupena i loko o kahi kānana wai. ʻO kekahi o nā maʻi ʻē aʻe i hāʻule i loko o kēia māhele:

• ʻO ka amyloidosis Renal. Hoʻokomo pū kēia i nā ʻano i kapa ʻia ʻo AL amyloidosis, AH amyloidosis, a me AHL amyloidosis.
• ʻO ka glomerulonephritis Cryoglobulinemic (CG).
• ʻO ka glomerulopathy fibrillary (FGN).
• ʻO ka glomerulopathy Immunotactoid (ITG).
• Nā maʻi glomerular e pili ana i nā protein monoclonal. Hoʻokomo pū kēia i nā kūlana i kapa ʻia ʻo 'light chain' a me nā 'heavy chain deposition diseases'.

2. Nā maʻi Tubulointerstitial

Hoʻopilikia kēia mau maʻi i nā ʻōmole (tubules) e lawe ana i nā meaʻai a me nā wai i kānana ʻia e nā puʻupaʻa i loko o ke koko, a me nā ʻiʻo a puni lākou. ʻO kekahi mau laʻana:

• ʻO ka maʻi Fanconi kaulahao māmā (LCFS).
• ʻO ka tubulopathy proximal kaulahao māmā (LCPT) me ka ʻole o nā kristal.
• ʻO ka histiocytosis mālama kristal.

3. Nā kūlana e pili ana i nā kīʻaha koko i loko o ka puʻupaʻa (Nā ʻeha vascular intra-renal)

Hoʻopilikia kēia mau kūlana i nā kīʻaha koko i loko o nā puʻupaʻa. ʻO kahi laʻana, ʻo ia ka atypical hemolytic uremic syndrome (aHUS) . ʻO kēia ka wā e ālai ʻia ai nā kīʻaha koko liʻiliʻi i loko o nā puʻupaʻa e nā ʻūpā koko.

Pehea ka maʻamau o ka MGRS?

He maʻi laha ʻole ka MGRS. Ua manaʻo ʻia e pili ana i ka emi ma mua o 1% o ka heluna kanaka. He mea maʻamau ia i ka poʻe ma luna o 60 mau makahiki .

He aha nā hōʻailona o MGRS?

Hiki ke ʻokoʻa nā hōʻailona o MGRS ma muli o ke ʻano o MGRS āu. ʻO kekahi mau hōʻailona maʻamau:

• Manaʻo luhi loa .
• Pehu ma nā wāwae lalo, nā kuʻekuʻe wāwae, a i ʻole nā ​​​​wāwae. I kekahi manawa e pehu paha ka ʻōpū, nā lima, a i ʻole ke alo.
• Loaʻa ka maʻi nephrotic . Hōʻike ʻia kēia e ka hoʻokuʻu ʻia ʻana o ka protein i loko o ka mimi a me nā pae cholesterol kiʻekiʻe i loko o ke koko.
• Mimi huʻa a kopa paha . He hōʻailona paha kēia o ka protein i loko o ka mimi (proteinuria).
• ʻO ka mimi pinepine ʻana ka pono e mimi pinepine.
• Koko i loko o ka mimi (hematuria) a i ʻole ka mimi e lilo i poni.
• Ka luaʻi a me ka luaʻi.
• ʻO ka maʻi POEMS . He maʻi laha ʻole nō hoʻi kēia.
• Ka mae ʻana, ke kani ʻana, a i ʻole ka nāwaliwali o nā lālā (he mau hōʻailona kēia o ke neuropathy).
• Ka loli ʻana o ka ʻili (nā kiko mālamalama a ʻeleʻele paha).
• Ka paʻakikī o ka hanu ʻana (dyspnea).

He aha ke kumu o ka MGRS?

ʻO ke kumu nui o MGRS ka hōʻino ʻia o nā puʻupaʻa i hana ʻia e nā protein M i ʻōlelo ʻia ma luna. ʻO nā protein M kahi ʻano antibody i hana pono ʻole, a hana hewa ʻia. Hana ʻia lākou mai nā clones i hōʻino ʻia (nā kope) o nā cell plasma, kahi ʻano o ke koko keʻokeʻo, a i ʻole nā ​​​​cell B.

Hiki i kekahi poʻe ke loaʻa i kēia mau clones o nā cell plasma e hana ana i nā protein M me ka ʻole o ka hōʻeha ʻana i kekahi ʻōpū. Ua kapa ʻia kēia ʻano ʻo Monoclonal Gammopathy of Undetermined Significance (MGUS) . Eia nō naʻe, i ka poʻe me MGRS, hōʻiliʻili kēia mau protein M i loko o nā puʻupaʻa a hōʻino iā lākou.

He aha ke kumu nui o MGRS?

ʻAʻole maopopo loa i nā loea ke kumu o MGRS. Manaʻo ʻia nā maʻi autoimmune , nā loli genetic , nā maʻi, a me nā kumu kaiapuni e kōkua i ia.

He aha nā pilikia e hiki ke hana ma muli o MGRS?

Hiki i kēia mau clones cell plasma a me MGRS ke hana i nā pilikia koʻikoʻi ma muli o ka hōʻino ʻana o nā puʻupaʻa. ʻO kekahi o kēia mau mea:

• Maʻi puʻupaʻa mau loa (CKD) .
• Ka maʻi puʻupaʻa hope loa .
• Ka hāʻule ʻana o nā puʻupaʻa .
• Ka ulu ʻana i nā maʻi ʻē aʻe. I kekahi poʻe, piʻi nui ka nui o nā clones cell plasma a hiki ke ulu i loko o kahi ʻano maʻi kanesa koko keʻokeʻo i kapa ʻia ʻo multiple myeloma (MM) . Hiki i ka MGRS ke ulu i loko o nā maʻi kanesa ʻē aʻe, e like me Waldenström macroglobulinemia , chronic lymphocytic leukemia (CLL) , a i ʻole B-cell lymphoma .

Pehea e ʻike ʻia ai ka MGRS?

Hiki i ke kauka ke hōʻoia i ka MGRS ma ka hana ʻana i kahi biopsy puʻupaʻa . Pili kēia i ka lawe ʻana i kahi ʻāpana liʻiliʻi o ka ʻiʻo mai kou puʻupaʻa a hoʻāʻo iā ia no ka protein M. Hiki iā lākou ke hoʻāʻo i nā laʻana o kou koko, mimi, a i ʻole ka iwi iwi.

Aia kekahi lāʻau lapaʻau no MGRS?

ʻAe, aia nā lāʻau lapaʻau no MGRS. Hoʻōla nā kauka iā ia me nā lāʻau lapaʻau e like me ka chemotherapy no ke kanesa. Hoʻopau kēia mau lāʻau i nā cell pilikia a i ʻole e kāohi iā lākou mai ka hana ʻana i nā kope hou aku. Hiki i nā lāʻau lapaʻau ʻē aʻe ke kōkua i ka hōʻemi ʻana i ka pehu. ʻO ka pahuhopu nui o ka lāʻau lapaʻau ka mālama ʻana i kou mau puʻupaʻa e hana a kāohi iā lākou mai ka hōʻino ʻia.

I kekahi mau hihia o MGRS, hiki i nā kauka ke hana i kahi hoʻoili cell stem . Hoʻololi kēia i kāu mau cell i hōʻino ʻia me nā cell olakino. Inā ua hōʻino nui ʻia kou mau puʻupaʻa, pono paha ʻoe i ka dialysis a i ʻole kahi hoʻoili kidney .

Nā lāʻau lapaʻau a me nā lāʻau lapaʻau i hoʻohana ʻia no MGRS

ʻO ke ʻano o ka lāʻau lapaʻau e hāʻawi ʻia iā ʻoe e pili ana i ke ʻano o ka MGRS āu. Eia kekahi mau koho lapaʻau:

• Nā antibodies monoclonal - e.g. rituximab, daratumumab.
• Nā mea hoʻopaʻa i ka proteasome - e.g. bortezomib, carfilzomib, ixazomib.
• Nā lāʻau lapaʻau immunomodulatory - e.g. thalidomide, lenalidomide, pomalidomide.
• Nā mea hoʻohui alkylating - e.g. cyclophosphamide, bendamustine, melphalan.
• ʻO nā corticosteroids - e.g. prednisone, dexamethasone.
• Hoʻoili pūnaewele kumu autologous.

He aha nā hopena ʻaoʻao o ka lāʻau lapaʻau?

Hiki i nā lāʻau lapaʻau e like me ka chemotherapy ke hana i nā hopena ʻino he nui, i kekahi manawa hiki ke hōʻino i nā kino. ʻO ia ke kumu e koho pono ai nā kauka i nā lāʻau lapaʻau e loaʻa ai ka pōmaikaʻi nui loa mai iā lākou a hōʻemi i ka pilikia o nā hopena ʻino koʻikoʻi. ʻO kekahi o nā hopena ʻaoʻao āu e ʻike ai:

• Anemia ( ka nele o ke koko).
• Ke kahe ʻana o ke koko.
• Ka paʻa ʻana o ka ʻōpū.
• Ka maʻi pākī.
• Luhi.
• Hoʻonui ʻia ka pilikia o nā maʻi viral, bacterial a me fungal.
• ʻAʻohe ʻono o ka meaʻai.
• Ka luaʻi a me ka luaʻi.

Hiki ke pale ʻia ka MGRS?

No ka mea, ʻaʻole mākou i ʻike pono i ke kumu o MGRS, ʻaʻohe ala kikoʻī e pale aku ai.

He aha kāu e manaʻo ai inā loaʻa iā ʻoe ka MGRS?

ʻOiai he maʻi hiki ke mālama ʻia ʻo MGRS, ʻaʻole ia e nalowale loa. Hiki iā ia ke hoʻi hou ma hope o ka mālama ʻana. Inā ua hōʻino nui ʻia kou mau puʻupaʻa, pono paha ʻoe i kahi hoʻoili puʻupaʻa.

Ma kahi o 18% o ka poʻe me MGRS e loaʻa i kahi maʻi kanesa i kapa ʻia ʻo multiple myeloma. No laila, e nānā mau kāu kauka iā ʻoe me nā hāpana koko a me ka mimi, no ka mea hiki ke ʻike mua ʻia kēia inā loaʻa.

ʻEhia ka lōʻihi e hiki ai iā ʻoe ke ola me MGRS?

ʻO ka lōʻihi o kou ola ʻana me MGRS e pili ana i ke ʻano o ka MGRS āu e loaʻa ai, kona koʻikoʻi, a pehea ʻoe e pane ai i ka lāʻau lapaʻau. ʻOi aku ka maikaʻi o ka nānā ʻana o ka poʻe me ka emi ʻana o ka pōʻino o ka puʻupaʻa ma mua o ka poʻe me ka pōʻino nui. No ka laʻana, ma kekahi noiʻi, ua ola ka poʻe me ka amyloidosis e pili ana me MGRS ma ka awelika o 10 mau makahiki ma hope o ka hōʻoia ʻana. Akā, ʻoi aku ka lōʻihi o ke ola o ka poʻe me ka ʻole o ka amyloidosis. Eia kekahi, ʻo ka poʻe i pane maikaʻi i ka lāʻau lapaʻau e loaʻa nā hopena maikaʻi loa.

Pehea wau e mālama ai iaʻu iho?

E hōʻoia e hahai i nā ʻōlelo aʻoaʻo a kāu kauka e hāʻawi ai iā ʻoe no ka mālama ʻana i ke olakino o kou puʻupaʻa ma ka home. Hiki i kēia ke komo pū me ka palena ʻana i nā mea e like me ka waiʻona, a me ka kaohi ʻana i nā kumu pilikia no ka maʻi puʻupaʻa, e like me ke koko kiʻekiʻe a me ka maʻi diabetes. ʻOiai ʻaʻole hiki i kēia mau mea ke mālama pololei i ka MGRS, hiki iā lākou ke kōkua i ka hōʻemi ʻana i ka pilikia o ka hōʻino hou ʻana o ka puʻupaʻa. Hiki i kāu kauka ke kōkua iā ʻoe e hoʻokele i nā hopena ʻaoʻao o ka lāʻau lapaʻau.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe nā hōʻailona o ka pilikia o ka puʻupaʻa, e like me ka pehu ʻana, ka mimi huʻa a ʻulaʻula paha, ka mimi pinepine ʻana, a i ʻole ka mimi liʻiliʻi loa, e ʻike pono i ke kauka.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

He mea kōkua paha ke nīnau aku i kāu kauka i kēia mau nīnau:

• He aha nā lāʻau lapaʻau i loaʻa no ke ʻano o ka MGRS i loaʻa iaʻu?
• He aha kaʻu e hana ai e pale aku i ka hōʻino ʻana o ka puʻupaʻa?
• He aha nā hōʻailona hou a i ʻole e ʻoi aʻe ana ka ʻino e pono iaʻu e makaʻala?

ʻO ka mea hope loa, ʻo ka mea nui loa (Leka Lawe-Home)

He maʻi koʻikoʻi ka MGRS e hiki ai ke hōʻino i nā puʻupaʻa ma ke ʻano e hiki ai ke hoʻoweliweli i ke ola. Akā e hoʻomanaʻo, ʻaʻole e loaʻa i nā kānaka a pau me MGRS ka maʻi kanesa. E nānā mau kāu kauka iā ʻoe a e hana i nā hana e ʻike a mālama i nā pilikia hou i ka wā hikiwawe loa . Ua hiki i nā lāʻau lapaʻau hou ke hoʻomaikaʻi i kou ʻano ola a hoʻemi i kou pilikia o nā maʻi koʻikoʻi. No laila, mai makaʻu, a e hahai i ka ʻōlelo aʻo a kāu kauka.

ʻO MGRS, Monoclonal Gammopathy o ke koʻikoʻi o ka Renal, Maʻi Pūpū, Nā Protein M, Nā Plasma Cells, Biopsy Pūpū, Nephrotic Syndrome, Multiple Myeloma