Ka nānā 'ana o ka Waldenstrom's Macroglobulinemia (WM)
Immunoglobulin Macroglobulinemia (IgM)
Hoʻomaka ka Macroglobulinemia o Waldenstrom i ka wā e hana nui ai nā sela koko keʻokeʻo keʻokeʻo i kapa ʻia ʻo B-cell lymphocytes. Immunogkapa ʻia ʻo lobulin Macroglobulin ('Oeka).
ʻO nā immunoglobulins kekahi ʻano protein i kapa ʻia hoʻi nā pūʻali. Loaʻa iā mākou nā ʻano immunoglobulins like ʻole, akā ʻo IgM ka mea nui loa, a ʻo ka mea mua i hana i kēlā me kēia manawa e loaʻa iā mākou kahi maʻi.
Ua like nā immunoglobulins me ka leka Y a me IgM me he ʻelima Y i hui pū ʻia. ʻO kā lākou hana ka hakakā ʻana i nā maʻi a me nā maʻi maʻi e mālama pono iā mākou. Ke loaʻa iā ʻoe ka nui o ka IgM i loko o kou koko, hiki i kou koko ke lilo i mānoanoa - a ʻeleʻele paha.
Ua kapa ʻia ke koko mānoanoa he hyperviscous a i ʻole hyperviscosity. Eia nō naʻe, hiki ke loaʻa kekahi mau pūnaʻi B maʻi ʻaʻai a me ka IgM ʻē aʻe i loko o kāu mau lymph nodes, e pehu ai lākou a hoʻokumu i kahi puʻupuʻu ma lalo o kou ʻili.
ʻO Waldenstrom ka inoa o ke Kauka nāna i ʻike mua i kēia maʻi a ʻo ka Macroglobulinemia ʻo ia ka nui o ka Macroglobulin i loko o kou koko a iwi iwi paha.
ʻO nā pūnaewele plasma
ʻO nā lymphocytes B-cell e hana i nā immunoglobulins ua kapa ʻia ʻo "Plasma cell" a ʻo lākou ke ʻano oʻo loa o ka lymphocyte B-cell. Ua kapa ʻia ka hapa nui o nā maʻi maʻi ʻaʻai ʻo Plasma i ka myeloma akā i ka WM, lilo ka B-cell i ka maʻi maʻi ma mua o kona oʻo ʻana i ka cell Plasma. No laila, loaʻa iā ia nā hiʻohiʻona o ka lymphoma a me ka myeloma, a ke kumu i ka hana ʻole ʻia o ka protein IgM.
ʻO wai ka mea i loaʻa iā Waldenstrom's Macroglobulinemia (WM)?
ʻO ka Waldenstrom's Macroglobulinemia (WM) ka mea maʻamau i nā kāne ma mua o nā wahine a hiki ke hana maʻamau ma hope o ke 65 mau makahiki.
ʻAʻole mākou ʻike i ke kumu o ka WM, akā inā loaʻa iā ʻoe kekahi mau maʻi, nā maʻi inflammatory a i ʻole nā maʻi autoimmune e like me Sjogren syndrome hiki iā ʻoe ke loaʻa i ka maʻi.
I kekahi mau hihia, inā loaʻa iā ʻoe kekahi lālā o ka ʻohana me WM e piʻi aʻe paha ʻoe i ka pilikia. Eia naʻe, ʻaʻohe ʻohana me ka hapa nui o ka poʻe me WM.
Cytogenetics ma WM
ʻElua mau kumu genetic nui e hiki ke hoʻopili ʻia ke loaʻa iā ʻoe ka WM. No ka ʻike inā loaʻa iā ʻoe nā ʻano genetic e pili ana i kāu maʻi, pono ʻoe e hana i nā hoʻokolohua cytogenetic.
Nā ho'āʻo Cytogenetic
Hana ʻia nā hoʻokolohua cytogenetics ma kou koko a me nā biopsies e ʻimi i nā loli i kāu chromosomes a i ʻole nā genes. Loaʻa iā mākou he 23 mau chromosomes, akā inā loaʻa iā ʻoe ka WM, ʻokoʻa iki ke ʻano o kāu mau chromosomes.
Chromosomes & DNA
ʻO nā pūnaewele a pau o ko mākou kino (koe naʻe nā keʻena koko ʻulaʻula) he nucleus, kahi e loaʻa ai kā mākou chromosomes. ʻO nā Chromosomes i loko o nā keena he mau kaula lōʻihi o DNA (deoxyribonucleic acid). ʻO DNA ka ʻāpana nui o ka chromosome e paʻa ana i nā ʻōlelo aʻo a ke kelepona, a ua kapa ʻia kēia ʻāpana he gene. Hoʻokumu ʻia kēlā me kēia kaula DNA i nā genes he nui.
Nā Genes
Hōʻike nā Genes i nā protein a me nā cell i loko o kou kino pehea e nānā ai a hana paha. Inā loaʻa kahi hoʻololi (ka hoʻololi ʻana a i ʻole ka hoʻololi ʻana) i kēia mau chromosomes a i ʻole nā genes, ʻaʻole e holo pono kāu mau protein a me nā cell, a hiki iā ʻoe ke hoʻomohala i nā maʻi like ʻole. Me WM hiki i kēia mau hoʻololi ke hoʻololi i ke ʻano o ka ulu ʻana a me ka ulu ʻana o kāu mau lymphocytes B-cell, e lilo ai lākou i maʻi kanesa a nui loa i ka IgM.
Kapa ʻia nā genes me kahi hui o nā leka a me nā helu. ʻO ka loaʻa ʻana o ka mutation inā loaʻa iā ʻoe ka WM:
- Gene MYD88 - Hoʻololi ʻia kēia ʻano ma kahi o 9 o kēlā me kēia 10 poʻe me WM
- Gene CXCR4 - Hoʻololi ʻia kēia gene ma kahi o 1 i kēlā me kēia 3 poʻe me WM.
He mea nui e ʻike inā loaʻa iā ʻoe kēia mau hoʻololi gene, no ka mea, ʻokoʻa ka hana ʻana o kekahi mau lapaʻau ma muli o ke ʻano o nā hoʻololi genetic i loaʻa iā ʻoe.
Nā hōʻailona o WM
Ma kahi o 1 i loko o 4 poʻe me WM ʻaʻohe hōʻailona, no laila ke haʻi ʻia iā ʻoe he haʻalulu maoli paha inā ʻoe i hoʻāʻo i ke koko a i ʻole nā scan no kekahi kumu ʻē aʻe.
Inā loaʻa iā ʻoe nā hōʻailona ma muli paha o ka hyperviscosity - E hoʻomanaʻo i ka wā e mānoanoa loa ai kou koko mai nā protein IgM ʻē aʻe i loko o kou koko.
Hiki ke komo i nā hōʻailona o ka hyperviscosity:
- headaches
- loli i kou ʻike maka a lohe paha
- luhi - ʻaʻole maikaʻi ka luhi a me ka nāwaliwali me ka hoʻomaha a i ʻole ka hiamoe
- ke koko kiʻekiʻe a i ʻole ka hoʻololi ʻana i kou puʻuwai puʻuwai
- ʻūpē mākala
- hilahila
- ka hopu
- helu koko haʻahaʻa.
Hiki ke helu ʻia ke koko haʻahaʻa inā loaʻa iā ʻoe ka hyperviscosity no ka mea ʻo ka piʻi ʻana o nā sela maʻi ʻaʻai a me IgM i loko o kou koko a me ka ʻiʻo iwi e hoʻopuʻi i kāu mau sela maikaʻi, e paʻakikī ai i kou kino ke hana i nā ʻāpana koko hou. Hiki ke hoʻopili ʻia nā ʻili koko i kāu mau ʻulaʻula, nā keʻokeʻo a me nā platelets.
Nā pūnaewele ʻulaʻula Loaʻa iā lākou kahi pūmua i kapa ʻia he hemoglobin (Hb) e lawe i ka oxygen a puni kou kino. Inā haʻahaʻa lākou hiki iā ʻoe ke loaʻa:
- nui
- pioloke
- nā hoʻololi i kāu ʻike
- luhi
- pōkole hanu.
Keʻokeʻo keʻokeʻo - loaʻa iā mākou nā ʻano like ʻole o ke koko keʻokeʻo. Hoʻopili lākou i kā mākou B-cell lymphocytes a me nā lymphocytes ʻē aʻe, akā ʻo nā mea ʻē aʻe i kapa ʻia he neutrophils, eosinophils, basophils, mast cell a me Plasma cell. ʻO kēia mau mea a pau ke kuleana no ka hakakā ʻana i ka maʻi a me ka maʻi. ʻO kekahi o nā mea nui ʻo ia nā neutrophils, a i ka wā haʻahaʻa ua kapa ʻia ʻo neutropenia. Inā he neutropenic ʻoe hiki iā ʻoe ke:
- loaʻa nā maʻi hele ʻole
- loaʻa nā maʻi e hoʻi mau ana
- pilikia ka ho'ōla inā ʻeha ʻoe iā ʻoe iho
- loaʻa ka wela kiʻekiʻe a i ʻole ke anuanu.
Ke loaʻa ʻoe i ka maʻi neutropenic hiki ke hele koke mai ka mana. He mea nui e hoʻokaʻaʻike aku i kāu kauka inā ʻike ʻoe i kēia mau hōʻailona, no ka mea pono paha ʻoe e hoʻomaka koke i nā lāʻau antibiotic.
Nā Kauliana – ʻo ia nā pūnaewele e kōkua ai i ko kākou koko e hoʻopaʻa i ka wā e ʻoki ai a ʻokiʻoki paha kākou iā kākou iho. ʻO kekahi inoa no nā platelets he thrombocytes, no laila ke haʻahaʻa loa kāu platelets ua kapa ʻia ʻo thrombocytopenia. Inā loaʻa iā ʻoe ka thrombocytopenia hiki iā ʻoe ke:
- ʻoi aku ka maʻalahi o ka ʻeha ma mua o ka maʻamau
- ʻoi aku ka maʻalahi o ke koko a i ʻole ka lōʻihi ma mua o ka maʻamau
- e hoʻomohala i kahi maʻi ʻino - ʻo ia nā wahi liʻiliʻi o ke koko e kahe ana ma lalo o ko mākou ʻili
- ke koko mai ka ihu, ka ʻāʻī, a i ʻole ke hele ʻana i ka lua.
B-hōʻailona
Hiki iā ʻoe ke loaʻa nā hōʻailona B me WM. He hōʻailona pinepine ia e pono ai ʻoe e hoʻomaka i ka mālama ʻana no ka mea hiki ke hana i ka wā e ulu ikaika ana ka WM. Hoʻokomo lākou i nā hōʻailona ʻekolu ma ke kiʻi ma lalo nei.
Kakaʻikahi loa, ma kahi o 1 o kēlā me kēia 100 poʻe me WM, kūkulu ʻia nā lymphocytes maʻi ʻaʻai a me nā pūnaewele plasma i loko o kāu ʻōnaehana nūnū waena (lolo a spinal cord). Kapa ʻia kēia 'Bing-Neel syndrome'. He mea kakaʻikahi a hiki ke hōʻeha i ke poʻo, pili - hopu, huikau a i ʻole nā neʻe ʻokoʻa.
ʻO ka hōʻailona a me ke kau ʻana o WM
Hiki ke ʻike ʻia ʻoe me WM ma hope o kāu hoʻāʻo koko. Akā, makemake kāu kauka e hana i kekahi mau ho'āʻo hou e hōʻoia i kēia a nānā i nā wahi o kou kino i hoʻopilikia ʻia. E komo pū kekahi o kēia mau hoʻokolohua:
- Hoʻokokoko hou aku
- Biopsy iwi iwi (aspirate and trephine)
- Computed tomography (CT) scan (inā wale nō ke manaʻo kāu kauka e pili ana kāu WM i kāu mau lymph nodes)
- Nā hoʻāʻo ma kou puʻuwai, ate a me nā puʻupaʻa
E aʻo e pili ana i ka hoʻokele ʻana o ka Waldenstrom's Macrobulinemia me Polofesa Judith Trotman, ʻĀpana Haematologist - Concord Hospital
Hana no ka ʻO ka Macroglobulinemia o Waldenstrom (WM)
Ke loaʻa i kāu kauka nā ʻike āpau mai kāu mau hoʻāʻo a me kāu scans, e noho pū lākou me ʻoe a kamaʻilio pū me ʻoe e pili ana i ke ala maikaʻi loa e hoʻokele ai iā WM. I nā manawa he nui, ʻōlelo paha lākou ʻaʻole pono ʻoe i kahi lapaʻau a ma kahi, e hoʻomaka iā ʻoe ma kahi hoʻolālā hoʻokele Watch & Wait. Hoʻohana ʻia ʻo Watch & Wait no nā ʻano lymphoma indolent, no ka mea i kekahi manawa hiki ke ʻoi aku ka nui o nā pilikia ma mua o nā pono o ka mālama ʻana. Hiki iā ʻoe ke aʻo hou aku e pili ana i Watch & Wait ma ke kaomi ʻana i ka loulou ma lalo nei.
Inā loaʻa iā ʻoe nā hōʻailona, a inā he 60g/dL kāu pae IgM a i ʻole, pono paha ʻoe e hoʻomaka i ka mālama ʻana. Inā pono ʻoe e hoʻomaka i ka lāʻau lapaʻau, e noʻonoʻo kāu mau kauka i nā kumu he nui e pili ana iā ʻoe a me kāu WM e hoʻoholo ai i ka lāʻau e kūpono iā ʻoe.
ʻO kekahi o ia mau mea:
- Kou pae IgM.
- ʻO nā hōʻailona āu e loaʻa nei.
- Inā pili kekahi o kāu mau kino e ka piʻi ʻana o nā pae IgM.
- ʻO kou mau makahiki a me kou olakino holoʻokoʻa.
- Inā loaʻa iā ʻoe a me nā kūlana olakino ʻē aʻe.
- ʻO kāu mau makemake ponoʻī ke loaʻa iā ʻoe ka ʻike āpau e pono ai ʻoe e hoʻoholo i ka ʻike.
Ma mua o kou hoʻomaka ʻana i ka lāʻau lapaʻau
Ma mua o kou hoʻomaka ʻana i ka mālama ʻana, e haʻi i kāu kauka inā manaʻo ʻoe e loaʻa nā keiki i ka wā e hiki mai ana. Hiki i nā lāʻau lapaʻau anti-cancer ke hoʻopilikia i kou ulu ʻana, a i ʻole e hōʻeha i nā pēpē i hānau ʻole ʻia, no laila he mea nui e kamaʻilio me kāu kauka e pili ana i kēia mau mea. I kekahi mau hihia, hiki iā lākou ke hoʻonohonoho i kekahi mau lāʻau lapaʻau hou e hoʻonui ai i kou kūlana o ka hāpai ʻana, a i ʻole ka hāpai ʻana i kekahi i ka wā e hiki mai ana.
Aia kekahi mau mea ʻē aʻe āu e kamaʻilio ai me kāu kauka, akā hiki ke paʻakikī ke ʻike i nā nīnau e nīnau ai i ka wā e ʻike mua ʻia ai ʻoe. No ke kōkua ʻana i ke alakaʻi ʻana i kāu kamaʻilio ʻana, ua hui mākou i kekahi mau nīnau āu e makemake ai e nīnau. Kaomi ma ka loulou ma lalo nei e kiʻi i kā mākou Nīnau e nīnau i kāu kauka.
Lapaʻau laina mua maʻamau
ʻO ka manawa mua ʻoe e hoʻomaka ai i ka mālama ʻana no WM ua kapa ʻia ʻo ia ka lāʻau lapaʻau mua hiki ke komo:
- DRC - komo deachamethasone (he steroid), rituximab (he antibody monoclonal) a me ka papa i kapa ʻia cyclophosphamide (kemohana).
- Loaʻa iā R-Benda (a i ʻole BR). rituximab (he antibody monoclonal) a bendamustine (kahi chemotherapy).
- ʻO Rituximab a me Zanubrutinib (Brukinsa ™) - kahi lāʻau lapaʻau i hoʻopaʻa ʻia i kapa ʻia ʻo BTK inhibitor.
- ʻO Zanubrutinib (Brukinsa™) wale nō- (inā ʻaʻole kūpono ka chemo-immunotherapy)
- ʻO ka hoʻokolohua lapaʻau.
- Plasmapheresis (i kapa ʻia hoʻi ka hoʻololi plasma), inā mānoanoa loa kou koko mai nā kiʻekiʻe IgM kiʻekiʻe.
- ʻO ka hoʻokahe koko no nā platelet haʻahaʻa a me nā keʻena koko ʻulaʻula haʻahaʻa.
- IntraVenous ImmunoGlobulin replacement therapy (IVIG) inā he haʻahaʻa loa kāu mau immunoglobulin (antibody) a i ʻole e loaʻa pinepine ana ʻoe i nā maʻi.
Nā hopena maʻamau o ka lāʻau lapaʻau
ʻO nā hopena ʻaoʻao āu e hilinaʻi ai i ke ʻano o ka lapaʻau āu e loaʻa ai. He mea nui e hōʻike i kāu kauka e pili ana i nā hopena ʻaoʻao hou a i ʻole nā hōʻailona āu e loaʻa ai i ka wā a ma hope o kāu mālama ʻana.
ʻO kekahi o nā hopena ʻaoʻao maʻamau o ka lāʻau lapaʻau hiki ke komo:
- ʻO Neutropenia - ʻo ia ka wā e haʻahaʻa loa ai kāu mau keʻokeʻo keʻokeʻo i kapa ʻia he neutrophils a hiki ke loaʻa iā ʻoe nā maʻi. Inā loaʻa iā ʻoe kahi mahana o 38° (degere Celsius) a ʻoi aʻe paha, ʻoluʻolu a haʻalulu - kapa ʻia ʻo rigors, a i ʻole ʻoe e ʻeha ʻole, pono ʻoe e kelepona koke i kāu kauka a i ʻole e hele i ke keʻena pilikia kokoke loa.
- ʻO ka Thrombocytopenia – ʻo ia ka haʻahaʻa loa o kāu mau platelets. Kōkua nā platelets i ko mākou koko e hoʻopaʻa ʻia e hoʻōki iā mākou mai ke koko a i ʻole ka ʻeha nui. Inā ʻike ʻoe ua ʻoi aku ka maʻalahi o ke kahe ʻana a i ʻole ka ʻeha ʻana ma mua o ka mea maʻamau, a i ʻole e loaʻa iā ʻoe kahi maʻi ʻulaʻula a ʻulaʻula paha, e kelepona i kāu kauka e hōʻike iā lākou.
- Anemia ʻo ia ke loaʻa iā ʻoe nā ʻāpana koko ʻulaʻula haʻahaʻa. Lawe kēia mau pūnaewele i ka oxygen a puni kou kino no laila ke haʻahaʻa kēia mau mea hiki iā ʻoe ke manaʻo i ka luhi, ke poni, nāwaliwali, nā loli i kou ʻike a i ʻole ka hanu. E hōʻike i kāu kauka inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona.
- ʻO ka naʻi a me ka luaʻi - e hōʻoia ʻoe e lawe ana i kāu mau lāʻau anti-maʻi e like me ke ʻano o kāu kauka a lāʻau lapaʻau i ʻōlelo ai iā ʻoe a e hōʻike iā lākou inā ʻaʻole ia e hana. ʻO nā mea ʻē aʻe āu e hoʻāʻo ai, ʻo ia ka meaʻai a i ʻole ka inu ʻana me ka ʻala i loko o ia mea, ka ʻai ʻana i nā meaʻai liʻiliʻi a me ka pale ʻana i nā meaʻai ʻala. I kekahi manawa hiki ke kōkua i ka hoʻohana ʻana i nā pahi plastik a i ʻole lāʻau a me nā ʻōpala ma mua o nā metala.
- Nā pilikia ʻōpū (constipation or diarrhea). He mea nui e kamaʻilio me kāu kauka e pili ana i nā loli i ka hana ʻana o kou ʻōpū. Aia kekahi mau lā'au lapa'au hiki iā 'oe ke ho'ohana no ka ma'i 'a'ai a me ka constipation akā e pili ana kēia i ke 'ano o ka lā'au e loa'a nei iā 'oe a me ke kumu o kou pilikia o ka ʻōpū.
- 'Oloke – ka luhi a i ʻole ka nele o ka ikehu ʻaʻole maikaʻi ma hope o ka hoʻomaha ʻana a i ʻole ka hiamoe. Hiki ke hana ma muli o kahi hōʻailona o kāu WM, ma ke ʻano he hopena ʻaoʻao o kāu mālama ʻana a i ʻole no nā kumu ʻē aʻe. E kamaʻilio me kāu kauka inā ʻaʻole ʻoe e hoʻomaikaʻi.
- Nā hōʻailona likeʻole - e pili ana i ke kuni, ka ʻiʻo a me ka ʻeha hui, ka luhi, ka ʻeha, a i ʻole ke ʻano maʻamau. He hopena ʻaoʻao kēia i nā lāʻau anticancer a pono ʻoe e hōʻike i kāu kauka inā loaʻa iā ʻoe kēia.
Malama hahai
Ke pau ka mālama ʻana, e hoʻomau kāu kauka i ka nānā ʻana i kāu mau pae IgM no ka nānā ʻana i ka maikaʻi o ka hana ʻana a ʻike inā a pono ʻoe i ka lāʻau lapaʻau hou aʻe. E hōʻike ana nā pae IgM i ke kauka inā he:
- Pane piha – CR a i ʻole nā hōʻailona o nā pae IgM ʻano ʻē ke koe, a i ʻole a
- ʻO ka pane hapa maikaʻi loa (VGPR) - kahi i loaʻa ai kahi pane maikaʻi loa a ua emi kāu pae IgM e 90%, a i ʻole a
- Pane hapa - ʻO PR a i ʻole ua hoʻemi ʻia nā pae IgM o 50% (hapalua) o ka mea ma mua o kou hoʻomaka ʻana i ka mālama ʻana.
ʻO nā ala ʻē aʻe e loiloi i kāu pane i ka lāʻau lapaʻau
Eia nō naʻe, he mea nui e hoʻomaopopo i kekahi manawa ʻo ka pahuhopu o ka mālama ʻana ʻo ia ke kōkua i ka hoʻomaikaʻi ʻana i kāu mau hōʻailona e like me ka luhi i hoʻokumu ʻia e ka anemia (ke koko ʻulaʻula haʻahaʻa a i ʻole ka haemoglobin), a i ʻole nā hōʻailona i hana ʻia e nā pane autoimmune ma muli o nā pae kiʻekiʻe IgM. No laila, hopohopo paha kāu kauka i kēia mau hōʻailona a pehea i hoʻomaikaʻi ai lākou, ma mua o nā pae IgM.
He mea nui kāu mau pae IgM inā ʻoe e noʻonoʻo ana e hele i kahi hoʻokolohua lapaʻau. ʻO kēia no ka mea, ʻo kāu mau pae IgM, a me nā hoʻololi ʻana iā ia me ka hoʻāʻo ʻana he mea nui ia o ka ʻikepili hoʻokolokolo. E kōkua ia i ka poʻe noiʻi e ʻike inā ʻoi aku ka maikaʻi o ka mālama ʻana i ka hoʻokolohua lapaʻau ma mua o nā lapaʻau maʻamau o kēia manawa i ka mālama ʻana i kāu WM.
Ka hahai lōʻihi
Inā maikaʻi nā mea a pau, e hana ʻia nā koho maʻamau no kēlā me kēia 3-6 mahina e nānā i kēia mau mea:
- E nānā i ka pono o ka lāʻau lapaʻau
- E nānā i nā hopena ʻaoʻao mai ka lāʻau lapaʻau
- E nānā i nā hopena hope loa mai ka mālama ʻana i ka manawa
- E nānā i nā hōʻailona o ka hoʻi hou ʻana o ka lymphoma
He mea koʻikoʻi nō hoʻi kēia mau koho i hiki i ka mea maʻi ke hāpai i kekahi hopohopo e pono ai lākou e kūkākūkā me ka hui lapaʻau. ʻO kahi hoʻokolohua kino a me nā hoʻāʻo koko he mau hoʻokolohua maʻamau no kēia mau koho. Ma waho aʻe o ka manawa ma hope o ka mālama ʻana e nānā i ka hana ʻana o ka mālama ʻana, ʻaʻole i hana pinepine ʻia nā scans ke ʻole he kumu no lākou. No kekahi poʻe maʻi hiki ke emi pinepine i ka manawa
Ka wānana no ka Waldenstrom's Macroglobulinemia (WM)
E like me ka hapa nui o nā ʻano liʻiliʻi o ka lymphoma, ʻaʻole hiki ke ho'ōla ʻia ʻo WM. Akā, inā makemake ʻoe i ka lāʻau lapaʻau ʻaʻole e hoʻōla ka pahuhopu akā e mālama i ka maʻi. ʻO ia ke ʻano o ka mālama ʻana i kāu mau pae IgM ma kahi pae ʻaʻole e hōʻino i kou mau kino, e hoʻopololei i kou koko, a i ʻole e loaʻa iā ʻoe nā hōʻailona hōʻoluʻolu.
Loaʻa ka hapa nui o ka poʻe i ka pane maikaʻi loa mai ka mālama ʻana, a hele i ke kala ʻana, akā naʻe, no ka hiki ʻole iā mākou ke ho'ōla iā WM, he mea maʻamau ka hoʻi ʻana mai (relapse) a pono ʻoe e mālama hou i kekahi manawa ma hope. No kekahi poʻe, he mau mahina paha ia, a no nā poʻe ʻē aʻe he mau makahiki ma mua o kou pono ʻana i ka lāʻau lapaʻau hou.
Kakaʻikahi, hiki iā WM ke hoʻololi (hoʻololi) i kahi ʻano lymphoma e ulu wikiwiki ana (kiʻekiʻe), maʻamau. Diffuse Nui B-Cell Lymphoma (DLBCL).
Hoʻihoʻi hou a i ʻole refractory (RR) WM
ʻO ka hapa nui o ka poʻe e mālama ana i ka WM e pane maikaʻi i ka lāʻau a hele i ke kala ʻana. Akā i kekahi poʻe e hoʻi hou mai ka lymphoma (hoʻi hou), a i ʻole i nā hihia liʻiliʻi ʻaʻole pane i ka lāʻau lapaʻau mua (palekana). Inā loaʻa iā ʻoe ka RR WM, pono paha ʻoe e hoʻomaka hou i ka mālama ʻana, a i ʻole e hoʻomaka i kahi lapaʻau hou. E kapa ʻia kēia lāʻau lapaʻau ʻelua.
Ma mua o kou hoʻomaka ʻana i ka lāʻau lapaʻau e hiki mai ana, e noʻonoʻo kāu kauka i nā mea he nui i ka wā e hoʻāʻo nei e ʻimi i ka lāʻau maikaʻi loa no ʻoe e like me:
- Pehea ka lōʻihi mai kāu lapaʻau hope ʻana a me ka maikaʻi o ka hana ʻana iā ʻoe.
- pehea ka loihi o kou kala ana.
- Nā hopena ʻaoʻao i loaʻa iā ʻoe me nā lāʻau lapaʻau mua.
- ʻO kou mau makahiki a me kou ola kino.
- ʻO kāu mau makemake ke loaʻa iā ʻoe ka ʻike kūpono a pau e hana i ka koho maikaʻi loa no ʻoe.
Ma muli o kou kūlana pilikino. Hiki ke hāʻawi ʻia iā ʻoe e like me kāu i hana ai ma mua inā ua hana maikaʻi ia, a ua kala ʻia ʻoe no kekahi mau makahiki. Hiki iā ʻoe ke hāʻawi ʻia i kekahi o nā lāʻau lapaʻau ʻē aʻe i helu ʻia ma luna. Inā pono ʻoe e hoʻomaka i ka lāʻau lapaʻau lua, he manaʻo maikaʻi e nīnau i kāu kauka e pili ana i nā hoʻokolohua lapaʻau āu e ʻae ai.
ʻO kekahi mau koho lapaʻau e hāʻawi ʻia iā ʻoe no RR WM
- Rituximab me ka chemotherapy a i ʻole
- Rituximab me zanubrutinib (Brukinsa™)
- ʻO Zanubrutinib (Brukinsa™) wale nō
- Plasmapheresis
- ʻO ka hoʻokolohua lapaʻau.
Nā ho'āʻo maʻi - nā lāʻau lapaʻau e hoʻokolokolo ʻia
Aia nā hoʻokolohua lapaʻau hou e hoʻomaka ana i nā manawa a pau a he manaʻo maikaʻi e hoʻomau i ka ʻike i nā lāʻau lapaʻau hou e hoʻāʻo ʻia nei e hoʻomaikaʻi ai i ka mālama ʻana, a i ʻole ka maikaʻi o ke ola no ka poʻe me WM. Inā makemake ʻoe i ke komo ʻana i kahi hoʻokolohua lapaʻau, hiki iā ʻoe ke nīnau i kāu kauka inā ʻoe e hoʻokō i nā pae hoʻohui no ke aʻo ʻana.
Aia ma lalo nā loulou i nā pūnaewele kahi āu e ʻimi ai i nā hoʻokolohua lapaʻau i hiki iā ʻoe ke ʻae. E ho'āʻo e ʻimi no:
- Waldenstroms Or ʻO Waldenstrom
- ʻO Waldenstrom's Macroglobulinemia
- WM
Nā pūnaewele hoʻokolohua lapaʻau
ʻO ke olakino a me ke olakino
ʻO ke ola olakino, a i ʻole kekahi mau loli ola maikaʻi ma hope o ka mālama ʻana he mea kōkua nui ia ma hope o kou hoʻopau ʻana i ka mālama ʻana. ʻO ka hana ʻana i nā hoʻololi liʻiliʻi e like me ka ʻai maikaʻi ʻana a me ka hoʻonui ʻana i kou olakino holoʻokoʻa hiki ke hoʻomaikaʻi i kou olakino a maikaʻi a kōkua i kou kino e ola hou. He nui nā hoʻolālā mālama ponoʻī hiki ke kōkua i ka wā o kou ho'ōla. Kaomi ma ka loulou no nā ʻōlelo aʻoaʻo hou aku e pili ana i ke ola maikaʻi.
hōʻuluʻulu manaʻo
- ʻO Waldenstrom's Macroglobulinemia (WM) he maʻi maʻi o kou mau keʻokeʻo keʻokeʻo i kapa ʻia ʻo B-cell lymphocytes.
- ʻO nā lymphocytes B-cell maʻi 'aʻai nui ka nui o ka protein i kapa ʻia ʻo Immunoglobulin Macroglobulin (IgM), hiki iā ia ke hana i kou koko e mānoanoa a pale i kou kino e hana i nā ʻāpana koko olakino ʻē aʻe.
- ʻAʻole loaʻa nā hōʻailona he nui i ka poʻe, akā hiki ke loaʻa nā hōʻailona i ka piʻi ʻana o ka WM a i ʻole ka piʻi ʻana o kāu mau IgM.
- E hōʻike i kāu kauka inā loaʻa iā ʻoe kekahi o nā hōʻailona i helu ʻia ma luna, e komo pū B-hōʻailona.
- He mea maʻamau ka loaʻa ʻana o ka maʻi anemia ke loaʻa iā ʻoe ka WM, he mea nui ia e hōʻoia i ka lawa ʻana o ka hao i kāu ʻai, a e nānā mau i kou pae hao.
- ʻAʻole paha ʻoe e hoʻomaʻamaʻa koke, a hiki ke hele ma luna E nana a e kali, a i ʻole e loaʻa iā ʻoe ka lāʻau lapaʻau me ka manaʻo e hele i ke kala ʻana a i ʻole ka mālama ʻana i kāu mau hōʻailona a hoʻemi i kāu mau pae IgM - ma mua o ka hoʻōla ʻana mai WM.
- ʻOiai e loaʻa iā ʻoe ka WM no ke koena o kou ola, hiki iā ʻoe ke noho maikaʻi me WM, hiki ke kōkua i nā koho ola olakino, a hiki ke kamaʻilio me kāu kauka no ka ʻōlelo aʻo.
- Inā makemake ʻoe e komo i kahi ka ho'āʻo hoʻokolohua, e kamaʻilio me kāu kauka.
