E aʻo kākou e pili ana i kahi leukemia laha ʻole e ulu ana i loko o ke koko? (Large Granular Lymphocytic Leukemia - LGL)

Ua lohe paha ʻoe i kahi ʻano leukemia ʻē, akā kakaʻikahi loa e ulu ana i loko o ke koko? Ua lohe paha ʻoe i kahi hoaaloha nona ia mea. A i ʻole ua lohe wale paha ʻoe e pili ana iā ia a ua noʻonoʻo e pili ana iā ia. I kēia lā e kamaʻilio mākou e pili ana i kekahi leukemia kakaʻikahi akā koʻikoʻi loa. Ua kapa ʻia ʻo ia ʻo Large Granular Lymphocytic Leukemia (LGL). He lōʻihi iki paha ka inoa, akā e mālama kākou i ka maʻalahi.

He aha ka manaʻo maoli o LGL?

I ka ʻōlelo maʻalahi, ʻo LGL kahi ʻano leukemia e ulu mālie ana i loko o ke kino, he maʻi mau (ʻo ia hoʻi, he lōʻihi kona lōʻihi). Hoʻopilikia ia i kahi ʻano kūikawā o ke koko keʻokeʻo i loko o ko kākou koko. Kāhea mākou i kēia mau pūnaewele he lymphocytes . Ua ʻike anei ʻoe he mau ʻāpana koʻikoʻi loa nā lymphocytes o kā mākou ʻōnaehana pale. Ua like lākou me nā koa i loko o kā mākou pūʻali koa. ʻO lākou ka poʻe e hakakā nei i nā germs e like me nā virus e komo i loko o ko mākou kino, a ʻo lākou ka poʻe e hana i nā antibodies e pale iā mākou mai nā maʻi.

No laila, ma LGL, ʻo ka mea e hana ʻia nei, ʻo ia kēia mau mea i kapa ʻia he mau cell lymphocyte, ʻoiai nā ʻano ʻelua o nā cell i kapa ʻia ʻo Cytotoxic T cells a i ʻole Natural Killer cells (NK cells), e loli ma ke ʻano ʻē no kekahi kumu a hoʻomaka e māhele a hoʻonui me ka ʻole o ke kāohi. Ua kapa ʻia kēia mau cell maʻamau ʻole he mau cell LGL.

ʻO kēia maʻi, i kapa ʻia ʻo LGL, ulu lohi loa ia. ʻO ia ke kumu i kapa ʻia ai ʻo ka leukemia mau loa. ʻO ia ka mea maʻamau i ka poʻe ma luna o 60 mau makahiki. Hiki i nā kauka ke mālama iā LGL. Akā i kekahi manawa hiki ke hoʻi hou mai ke ʻano, a hiki ke lilo i pilikia olakino lōʻihi.

ʻElua mau ʻano nui o LGL:

Ua ʻike ʻia ʻelua ʻano nui o kēia kūlana LGL. ʻO lākou kēia:

1. Leukemia lymphocytic granular nui o nā cell T (T-LGL)

2. Ka maʻi lymphoproliferative mau loa o nā pūnaewele NK (CLPD-NK)

Ma nā hihia ʻelua, e like me ka mea i ʻōlelo ʻia ma mua, ʻo ka mea e hana ʻia, ʻo ia ka lilo ʻana o nā pūnaewele T a i ʻole nā ​​pūnaewele NK i mea ʻē.

Pehea ka hopena o kēia kūlana i ko mākou kino?

Ke noʻonoʻo nei paha ʻoe i ke ʻano o ka hopena o kēia maʻi i kapa ʻia ʻo LGL i ko kākou kino. ʻĀ, ʻoiai he ʻano kēia o ka Chronic Lymphocytic Leukemia, hele kēlā mau pūnaewele LGL maʻamau ʻole i ka iwi iwi a hoʻopilikia i ka hana ʻana o nā hunaola koko maʻamau ma laila. Ua like ia me kahi mea kanu maikaʻi ʻole e hele mai ana a lawe i kahi mea kanu maikaʻi.

Hiki i kēia ke hana i ʻelua mau pilikia nui:

• Neutropenia : ʻO kēia ka emi ʻana o ka helu o nā granulocytes i loko o ko mākou kino.ʻO ka emi ʻana o ka helu o nā hunaola koko keʻokeʻo (granulocytes) (ke ʻano maʻamau o nā hunaola koko keʻokeʻo). Ke emi kēia mau hunaola, emi iho ka hiki o ko mākou kino ke kaua i nā maʻi, a i ʻole ka pale ʻana. Hoʻonui kēia i ka pilikia o nā maʻi pinepine.
• Anemia : Ua lohe paha ʻoe i kēia. ʻO ka anemia ka wā e nalowale ai ke kino i ka nui o nā hunaola koko ʻulaʻula olakino e pono ai. Hiki ke loaʻa kēia ʻano ma muli o ka hoʻopilikia ʻana o nā hunaola LGL i ka hana ʻana o nā hunaola koko ʻulaʻula. Ke ulu ka anemia, manaʻo ʻoe ua luhi a luhi loa.

ʻO wai ka mea i loaʻa pinepine i kēia maʻi?

He maʻi laha ʻole ʻo LGL. Inā ʻoe e nānā a puni ka honua, ua hōʻike ʻia kēia ʻano ma kahi o hoʻokahi i loko o hoʻokahi miliona mau kānaka. ʻO ia hoʻi, ma kahi ʻāina e like me Sri Lanka, he kakaikahi paha kēia mau maʻi. ʻO ka hapa nui o ka manawa, i ka wā e ʻike ʻia ai kēia maʻi, aia nā maʻi i ko lākou mau makahiki 60.

He aha nā hōʻailona? Pehea mākou e maopopo ai i kēia?

Eia ka mea kupanaha iki. Hiki i kekahi poʻe me LGL ke hele no nā makahiki me ka ʻole o nā hōʻailona . Ua ʻike ʻia kekahi noiʻi ʻaʻohe hōʻailona o ka hapakolu o ka poʻe me LGL i ka wā i ʻike ʻia ai lākou. Ua ʻike wale lākou ua loaʻa iā lākou i ka wā i hōʻike ai kahi hoʻāʻo koko no kekahi kumu ʻē aʻe i kahi helu haʻahaʻa o nā hunaola koko ʻulaʻula a i ʻole kahi helu haʻahaʻa o nā hunaola koko keʻokeʻo i kapa ʻia he neutrophils.

Eia nō naʻe, aia kekahi mau hōʻailona maʻamau e ʻike ai ka poʻe e loaʻa ana nā hōʻailona:

• Luhi nui a me ka luhi : ʻO kēia ka hōʻailona maʻamau o LGL. ʻO ka hapa pinepine, ʻo kēia ma muli o ka anemia i ʻōlelo ʻia ma luna.
• Nā kuni pinepine a me nā maʻi e hoʻi hou ana : Hiki i nā maʻi bacteria ke hoʻoulu i ke kuni. ʻO kēia ma muli o ka nāwaliwali o ka ʻōnaehana pale.
• Splenomegaly: He hoʻonui ʻia kēia o ka spleen, kahi ʻōkana i loko o ko kākou kino. Hiki ke hana ʻia kēia e nā maʻi a me kekahi ʻano anemia.

E hoʻomanaʻo, ʻo ka loaʻa ʻana o kēia mau hōʻailona ʻaʻole ia he manaʻo he LGL kou. Hiki iā lākou ke lilo i mau hōʻailona o nā maʻi ʻē aʻe he nui. No laila, inā ʻoe e ʻike nei i kahi mea e like me kēia, ʻoi aku ka maikaʻi o ke ʻike ʻana i ke kauka no ka ʻōlelo aʻo.

Aia kekahi mau kūlana ʻē aʻe e pili ana me LGL?

ʻAe, ua ʻike ʻia he nui ka poʻe me LGL he mau maʻi autoimmune ʻē aʻe. ʻO ia hoʻi, nā kūlana kahi e hoʻouka ai ka ʻōnaehana pale ponoʻī o ko mākou kino i kā mākou mau cell olakino ponoʻī. I waena o kēia mau mea, ʻo ka rheumatoid arthritis ka mea maʻamau.

Eia kekahi, hiki ke pili kekahi mau kūlana ʻē aʻe me LGL:

• Anemia : Ua kamaʻilio mākou e pili ana i kēia ma mua. Lilo kekahi poʻe i ka maʻi anemia nui a pono i nā hoʻoili koko e mālama i ko lākou pae koko ʻulaʻula. ʻO kekahi poʻe me LGLHiki ke ulu aʻe kekahi ʻano anemia i kapa ʻia ʻo ka anemia hemolytic. I kēia hihia, ma kahi o ka hōʻemi ʻana i ka hana ʻana o nā cell koko ʻulaʻula, ua luku ʻia nā cell koko ʻulaʻula e kū nei.
• Lymphocytosis : ʻO kēia ka hoʻonui ʻia ʻana o ka helu o nā hunaola koko keʻokeʻo i kapa ʻia he lymphocytes i loko o ke koko. ʻIke pinepine ʻia kēia hoʻonui ʻana o nā lymphocytes i nā poʻe me nā leukemia lymphocytic, lymphomas, a i ʻole nā ​​maʻi viral.

He aha ke kumu maoli o LGL?

He mea kēia a nā kauka i maopopo ʻole ai. ʻAʻole i ʻike ʻia ke kumu kikoʻī o LGL. Eia nō naʻe, manaʻoʻiʻo lākou aia kahi pilina ma waena o kēia maʻi leukemia a me ka hana o kā mākou ʻōnaehana pale, nā maʻi autoimmune, a me nā ʻano maʻi ʻē aʻe.

• Ma kahi o 30% o ka poʻe me LGL he mau maʻi autoimmune ʻē aʻe, e like me ka rheumatoid arthritis.
• ʻO kekahi 25% a 30% paha e loaʻa i kekahi ʻano lymphoma a i ʻole kekahi ʻano maʻi kanesa ʻē aʻe.
• Eia kekahi, he nui ka poʻe me LGL i loaʻa nā loli i ko lākou mau genes. ʻOi aku hoʻi, aia nā mutations i loko o ʻelua mau genes i kapa ʻia ʻo STAT3 a me STAT5B . He kuleana koʻikoʻi kēia mau genes ʻelua i ka pale ʻana o ke kelepona a me ke ʻano o ka māhele ʻana a me ka hoʻonui ʻana o nā cell.

Pehea e ʻike ai nā kauka i kēia maʻi?

Hoʻohana nui nā kauka i nā hoʻokolohua koko a me nā loiloi genetic e ʻike ai iā LGL. Eia kekahi o nā hoʻokolohua i hana pinepine ʻia:

• Helu koko piha (CBC) me ka ʻokoʻa : Helu kēia i nā ʻano āpau o nā cell i loko o kou koko, ʻoiai ka nui o kēlā me kēia ʻano o nā cell koko keʻokeʻo āu.
• ʻO ka palaki koko peripheral : Pili kēia i ka nānā ʻana i kahi hāpana koko ma lalo o kahi microscope a me ka helu ʻana i ka helu o nā cell LGL i loko o ke koko.
• ʻO ka cytometry kahe: He hoʻāʻo hoʻokolohua kēia i hoʻohana ʻia e kālailai i nā ʻano o nā cell. Hoʻohana pinepine ʻia kēia hoʻāʻo e ʻike a hoʻokaʻawale i nā ʻano leukemia.
• Immunophenotyping : Pili kēia i ka lawe ʻana i nā laʻana koko a i ʻole nā ​​​​ʻiʻo a nānā iā lākou no nā māka kikoʻī ma ka ʻili o nā cell. Hiki ke hoʻohana ʻia kēia mau māka e ʻike i kekahi mau ʻano maʻi.
• ʻO ka loiloi hoʻonohonoho hou ʻana o ka gene receptor T-cell (TCR) : Lawe kēia i kahi hāpana o ke koko a i ʻole ka iwi iwi a nānā i nā pilikia me nā genes e kaohi ana i ka hana ʻana o nā T-cell.
• Ka hoʻāʻo ʻana i ka hoʻoilina : Hiki iā ʻoe ke hoʻāʻo no nā mutations i nā genes STAT3 a me STAT5B i ʻōlelo mua ʻia.

Ma waho aʻe o kēia mau hoʻokolohua, ʻo ka immunodeficiencyHiki ke hana ʻia nā hoʻokolohua ʻē aʻe, e like me ka hoʻokolokolo ʻana o ka iwi iwi, e kāpae i nā kūlana ʻē aʻe e like me ka immunodeficiency, rheumatoid arthritis, myelodysplasia , a me nā myeloid mutations . Hiki ke nānā ʻia nā pae immunoglobulin a me nā pae protein monoclonal .

Pehea e mālama ʻia ai ʻo LGL?

E ʻōlelo kākou he T-LGL a i ʻole CLPD-NK leukemia kou, akā ʻaʻohe ou hōʻailona . I kēlā hihia, hiki i kāu kauka ke kuhikuhi i kahi hoʻolālā i kapa ʻia ʻo "kiaʻi kali." Pili kēia i ka nānā mau ʻana i kou olakino. ʻO ka maʻamau, e loaʻa iā ʻoe nā hoʻāʻo koko i kēlā me kēia mau mahina e ʻike ai inā ulu nā hōʻailona.

No ka poʻe me nā hōʻailona, ​​hiki ke hāʻawi ʻia ka lāʻau lapaʻau immunosuppressive a me nā steroids . Hiki i nā kauka ke hoʻomaka i hoʻokahi lāʻau ma hope o kekahi, a i ʻole e hoʻomaka paha lākou i kahi lāʻau lapaʻau haʻahaʻa. No ka mea he maʻi laha ʻole ʻo LGL, ʻimi ka hapa nui o ka poʻe i ke kauka loea i kēia maʻi.

ʻAʻole anei hiki iā kākou ke hōʻemi i ka nui o kēia maʻi?

ʻO ka mea pōʻino, ʻaʻole, ʻaʻole hiki iā mākou. ʻAʻole maopopo i nā kauka ke kumu o ia mea. No laila, he paʻakikī ke ʻōlelo pehea e pale aku ai. Eia nō naʻe, ua ʻike ʻia he kiʻekiʻe ka pilikia o ka poʻe me nā maʻi autoimmune i ka loaʻa ʻana o kēia maʻi. No laila, inā loaʻa iā ʻoe kekahi o kēia mau maʻi autoimmune, he manaʻo maikaʻi e nīnau i kāu kauka inā pono ʻoe e hopohopo e pili ana iā LGL.

He maʻi make anei ʻo LGL?

I ka hapanui o nā hihia, he maʻi mau ka LGL, ʻaʻole he make koke . Ma kahi o 75% o ka poʻe me ka leukemia T-LGL a me ka leukemia CLPD-LGL e ola ana no ʻelima mau makahiki ma hope o ka ʻike ʻia ʻana. Eia nō naʻe, ma kahi o 10% o ka poʻe me kēia ʻano leukemia e make ana mai nā maʻi koʻikoʻi e hiki ke hana ma ke ʻano he pilikia o ka leukemia. No laila, he mea nui loa ia e pale iā ʻoe iho mai nā maʻi.

Pehea wau e mālama ai iaʻu iho? Pehea wau e ola ai me kēia maʻi?

Inā loaʻa iā ʻoe ʻo LGL, ʻaʻohe ou hōʻailona. Eia nō naʻe, he mea nui e mālama i kou olakino holoʻokoʻa, me ka loaʻa ʻana o nā nānā maʻamau . Inā loaʻa iā ʻoe nā hōʻailona a ʻaʻole paha, hiki i kēia mau manaʻo ke kōkua:

• E ʻai i ka meaʻai olakino : E ʻai i nā ʻiʻo momona, iʻa, nā huaʻai, nā mea kanu, a me nā palaoa holoʻokoʻa.
• E hoʻoikaika kino pinepine : E koho i kahi hoʻoikaika kino e maikaʻi ai a kūpono iā ʻoe.
• Hiamoe maikaʻi : He mea nui loa ka hoʻomaha kūpono.
• E hoʻokele i ke kaumaha.E hana i nā mea e hauʻoli ai ʻoe, e noʻonoʻo pono, e hana i ka yoga.
• E pale i kou ʻōnaehana pale : E nīnau i kāu kauka e pili ana i nā lāʻau pale a me nā mea ʻē aʻe hiki iā ʻoe ke hana e pale aku i nā maʻi. ʻOiai nā mea maʻalahi e like me ka holoi ʻana i kou mau lima me ke kopa a me ka noho ʻana ma kahi ʻē aʻe e hiki ke kōkua.

Hiki ke ola i kahi ola maʻamau me kēia maʻi?

Ma keʻano laulā, ʻo ka poʻe i mālama ʻia no LGL e ola pinepine i nā ola maʻamau . Hiki iā lākou ke ola e like me ka lōʻihi o kekahi me ka ʻole o ke ʻano. Eia nō naʻe, he mea nui e hoʻomanaʻo he hiki i kekahi poʻe me LGL ke loaʻa mua i nā maʻi koko koʻikoʻi e hoʻopilikia i ko lākou ola maikaʻi.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe nā hōʻailona e hōʻike ana e ʻoi aku ka ʻino o kou kūlana, pono ʻoe e ʻike i kāu kauka. Inā ke mālama ʻia nei ʻoe no nā hōʻailona, ​​​​pono ʻoe e haʻi i kāu kauka e pili ana i nā loli āu e ʻike ai i kou kino (no ka laʻana, e ʻoi aku ka ʻino o nā hōʻailona).

He aha nā nīnau koʻikoʻi e nīnau aku ai i ke kauka?

ʻOiai he maʻi laha ʻole ʻo LGL, he mea maʻamau ka loaʻa ʻana o nā nīnau he nui e pili ana i ia mea. Eia kekahi mau nīnau a mākou e manaʻolana nei e kōkua iā ʻoe e aʻo hou e pili ana iā LGL:

• He aha ke ʻano o LGL iaʻu?
• He aha nā lāʻau lapaʻau no kēia?
• E pono anei iaʻu ma mua o hoʻokahi lāʻau lapaʻau?
• Ua maikaʻi loa koʻu manaʻo i kēia manawa. E loaʻa anei iaʻu nā hōʻailona?
• He mau maʻi ʻē aʻe koʻu. E hoʻonui anei ʻo LGL iā lākou i ka ʻino?

Mai makaʻu e nīnau i nā nīnau e like me kēia. Aia kāu kauka ma laila e kōkua iā ʻoe. Ua kūpaʻa lākou i ka wehewehe ʻana i nā mea āpau ma ke ʻano e hiki ai iā ʻoe ke hoʻomaopopo.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

ʻO ka leukemia lymphocytic granular nui (LGL) kahi maʻi koko laha ʻole e hoʻopilikia i kou mau hunaola koko keʻokeʻo. ʻOiai inā loaʻa iā ʻoe kēia maʻi, ʻaʻole paha ʻoe e ʻike i kekahi loli i kou kino e hiki ke lilo i mau hōʻailona o LGL. I kekahi manawa, ʻaʻole paha e ʻike ʻia nā hōʻailona no nā makahiki.

ʻAʻole hiki i nā kauka ke ho'ōla loa i kēia maʻi. Eia nō naʻe, aia nā lāʻau lapaʻau e hiki ke kōkua i ka kaohi ʻana i kona mau hōʻailona. He mea maʻamau ke manaʻo i ka haʻalulu a me ka makaʻu ke lohe ʻoe he maʻi hiki ʻole ke hoʻōla ʻia kāu. E hoʻomaopopo kāu mau kauka i ke kumu o kou manaʻo pēlā.

E hauʻoli lākou e wehewehe iā ʻoe i ka mea hiki iā ʻoe ke hana e mālama i kou olakino i kēia manawa, a me ka mea e manaʻo ai inā kū mai nā hōʻailona. ʻO ka ʻike i ka mea e manaʻo ai i ka wā e hiki mai ana e hāʻawi iā ʻoe i ka hilinaʻi nui a me ka ikaika e noho me LGL. No laila, e kamaʻilio me kāu kauka e pili ana i nā nīnau āpau āu. E loaʻa ka ʻike. ʻO ia ka mea maikaʻi loa hiki iā ʻoe ke hana i ka manawa e like me kēia.

`Leukemia LGL, maʻi ʻaʻai koko, nā hunaola koko keʻokeʻo, nā lymphocytes, neutropenia, anemia, ʻōnaehana pale