Ke ʻānai mau nei ʻoe i kou kino? Ke ʻulaʻula nei? E aʻo kākou e pili ana i kēia maʻi laha ʻole (Systemic Mastocytosis)

Ke ʻānai nei hoʻi ʻoe i kou kino a puni i kēlā me kēia manawa? Ke ʻulaʻula koke nei kou maka a me kou ʻāʻī a manaʻo e like me ʻoe e wela ana? Ke ʻeha nei kou ʻōpū, ka poniuniu, a me ka puʻuwai wikiwiki? ʻOiai inā manaʻo ʻoe he mau maʻi āpau kēia mau mea, i kekahi manawa aia paha kekahi ʻano maʻi ʻē aʻe ma hope o kēia, kahi mea a mākou i lohe ʻole ai. I kēia lā ke kamaʻilio nei mākou e pili ana i kekahi maʻi laha ʻole, ʻo Systemic Mastocytosis. Mai hopohopo, e hoʻomaopopo maʻalahi kākou i kēia.

He aha ka mastocytosis ʻōnaehana?

I ka ʻōlelo maʻalahi, he maʻi kēia i hoʻokumu ʻia e ka hoʻonui ʻia ʻana o kahi ʻano cell i kapa ʻia ʻo 'mast cells' i loko o ko mākou kino. ʻO nā cell mast kahi ʻano o ke koko keʻokeʻo. E noʻonoʻo i kēia mau cell mast e like me nā koa i loko o kā mākou ʻōnaehana pale. ʻO kā lākou hana nui ka pale ʻana iā mākou mai nā maʻi a kōkua iā mākou e ho'ōla i ka wā e ʻeha ai mākou. Akā i loko o kēia maʻi, hōʻiliʻili nui kēia mau cell mast a ma ke ʻano ʻē i nā ʻano kino like ʻole e like me ko mākou ʻili, ka iwi iwi , nā iwi, a me nā ʻōpū. He aha ka mea e hana ai? Ma muli o kekahi kumu, lilo koke kēia mau cell mast keu i mea hana a hoʻokuʻu i ka nui o nā kemika e like me 'histamine' i loko o ke kino. ʻO ia ka manawa e ʻike ʻia ai nā hōʻailona aʻu i ʻōlelo ai ma mua, e like me ka flushing, ka maneʻo, a me ka eczema .

He aha ke kumu o kēia maʻi?

He nui ka poʻe e noʻonoʻo nei inā he maʻi hoʻoilina kēia. Akā, ʻaʻole kēia he maʻi hoʻoilina maʻamau. ʻO ka hapa nui o ka manawa, hana ia i loko o kahi gene i kapa ʻia ʻo KIT, kahi i loaʻa i loko o kā mākou mau cell mast.Hoʻokumu ʻia ia e kahi loli maʻamau (mutation). Kōkua kēia gene i ka hana ʻana i kahi protein e kāohi ana i ka ulu ʻana o nā cell. No laila, ke loli kēia, hoʻomaka nā cell mast e ulu me ka hiki ʻole ke kāohi ʻia. ʻOiai hiki ke ʻike ʻia kēia mau hōʻailona i kēlā me kēia makahiki, ʻoi aku ka maʻamau i ka poʻe ʻelemakule, ʻoi aku hoʻi ma waena o 40 a me 50 mau makahiki .

Nā mea hoʻonāukiuki e hoʻonui ai i nā hōʻailona

ʻAʻole ʻike nā kānaka a pau me kēia maʻi i nā hōʻailona like. Eia kekahi, hiki ke ʻokoʻa nā mea hoʻoulu e hoʻoulu ai i nā hōʻailona mai kēlā kanaka a kēia kanaka. Eia nō naʻe, ua helu ʻia kekahi o nā mea hoʻoulu maʻamau ma ka papa ma lalo nei. He mea nui e makaʻala i kēia mau mea. He mea nui hoʻi e hoʻomanaʻo i kekahi manawa hiki ke ʻike ʻia nā hōʻailona me ke kumu ʻole.

He aha nā ʻano nui a me nā hōʻailona o kēia maʻi?

ʻElima mau ʻano nui o ka Systemic Mastocytosis. E hoʻāʻo kāu kauka iā ʻoe a haʻi iā ʻoe i ke ʻano āu i loaʻa ai.

• ʻO ka Indolent Systemic Mastocytosis (ISM): ʻO kēia ke ʻano maʻamau a ʻaʻole i ʻoi aku ka koʻikoʻi. Holo mālie ka maʻi a ʻaʻole ia e hōʻino nui i nā kino.
• ʻO ka Smoldering Systemic Mastocytosis (SSM): ʻOi aku ka ʻino ma mua o ka ISM. ʻOi aku ka ʻino o nā hōʻailona a hiki ke hoʻopilikia i nā ʻōpū i ka hala ʻana o ka manawa.
• ʻO ka Mastocytosis ʻōnaehana me kahi Neoplasm Hematological Pili (SM-AHN): I kēia hihia, hele pū ka mastocytosis me kahi maʻi pili koko ʻē aʻe (e.g. leukemia ).
• ʻO ka Mastocytosis ʻōnaehana Aggressive (ASM): Holo wikiwiki kēia ʻano a hiki ke hōʻino i nā ʻōpū e like me ke ake, ka spleen, a me nā iwi.
• Mast Cell Leukemia (MCL): ʻO kēia ke ʻano kakaikahi loa a koʻikoʻi loa. Nui ka nui o nā mast cell i loko o ka iwi iwi a me ke koko.

ʻO nā hōʻailona āu e ʻike ai ma muli o ke ʻano o ka maʻi āu e loaʻa ai a me kahi o kou kino e nui ai nā pūnaewele mast ʻano ʻē. Eia kekahi mau hōʻailona maʻamau:

• ʻĀnai, ʻulaʻula , ʻulaʻula
• ʻEha ʻōpū, pehu, maʻi kahe
• Ka luaʻi a i ʻole ka luaʻi
• ʻEha poʻo a me ka luhi
• Puʻuwai wikiwiki
• Ke koko haʻahaʻa, ka poniuniu, ka nalowale o ka ʻike
• ʻO ka pehu ʻana o ke akepaʻa, ka spleen, a me nā lymph nodes
• Anemia a me nā kūlana koko
• Ka nāwaliwali o nā iwi (Osteoporosis)
• Ma ke ʻano noʻonoʻo, nā kūlana e like me ka paʻakikī o ka noʻonoʻo ʻana, ka hopohopo pinepine, a i ʻole ke kaumaha.

Pehea e ʻike ai i ka maʻi? (Diagnosis)

E nīnau mua kāu kauka iā ʻoe e pili ana i kāu mau hōʻailona a hana i kahi hoʻokolokolo kino. A laila, hiki iā lākou ke paipai i kekahi mau hoʻokolohua e hōʻoia i ka hōʻailona:

• Biopsy ʻili: Lawe ʻia kahi ʻāpana ʻili liʻiliʻi mai kahi ʻāpana i hoʻopilikia ʻia a nānā ʻia ma lalo o kahi microscope e ʻike inā he nui nā cell mast.
• Biopsy iwi iwi: Hoʻohana ka hematologist i kahi nila lahilahi e lawe i kahi ʻāpana liʻiliʻi o kou iwi iwi a nānā iā ia.
• Nā hoʻāʻo koko a me ka mimi:Hiki ke hoʻohana ʻia ka helu koko piha (CBC) e nānā i nā kūlana ʻē aʻe, e like me ka anemia.
• Hoʻāʻo Hoʻoilina: Hoʻāʻo ʻia kahi hāpana koko a ʻiʻo paha e ʻike inā he mau loli i ka gene KIT i ʻōlelo ʻia ma mua.
• Nā hoʻokolohua kiʻi: Hiki i nā kukuna X, nā ultrasounds, a me nā scan CT ke kōkua i ka ʻike ʻana i ka nāwaliwali o ka iwi a me ka pehu ʻana o nā ʻōpū e like me ke ake a me ka spleen.
• Endoscopy a Colonoscopy paha: Hoʻohana ʻia kahi ʻōmole lahilahi me kahi kāmela i hoʻopili ʻia i mea e nānā ai i loko o ka ʻōpū a i ʻole nā ​​​​​​naʻau e nānā i nā mea ʻino, e like me nā ʻōpū.

He aha nā lāʻau lapaʻau?

E hoʻoholo ke kauka i ka hoʻolālā lapaʻau e pili ana i kāu mau hōʻailona, ​​​​nā ʻōpū i hoʻopilikia ʻia, a me ke ʻano o ka maʻi.

• Nā Antihistamine: Kōkua kēia mau lāʻau i ka kaohi ʻana i nā mea e like me ka ʻili ʻana, ka ʻulaʻula, a me ka ʻeha o ka ʻōpū.
• Epinephrine: He hormone i hoʻohana ʻia i ka hanana o kahi hopena maʻi koʻikoʻi (anaphylaxis).
• Nā mea pale o ka pamu proton: Kāohi i ka waikawa o ka ʻōpū a hōʻemi i nā mea e like me ka mumū o ka ʻōpū.
• Nā Mea Hoʻopaʻa Kelepona Mast: Nā lāʻau lapaʻau e hoʻōki ai i ka hoʻokuʻu ʻia ʻana o ka histamine mai nā kelepona mast.
• Nā Steroid: Hiki ke kōkua i nā hōʻailona ʻili a me ka hanu.
• Lapaʻau i Hoʻolālā ʻia: Nā lāʻau lapaʻau e hoʻolālā ana i kahi gene a i ʻole protein kikoʻī e pili ana i ka maʻi. Pono paha kēia mau mea no ka poʻe me nā hōʻailona koʻikoʻi.
• Chemotherapy: Pono paha kēia lāʻau lapaʻau i nā hihia kahi e pālahalaha wikiwiki ai ka maʻi a hōʻino nui i nā kino.
• Hoʻoili Kelepona Kumu: He koho lapaʻau i manaʻo ʻia i nā hihia kakaikahi loa.

ʻO ka Mastocytosis ʻōnaehana, nā pūnaewele mast, ka ʻulaʻula ʻili, anaphylaxis, anaphylaxis, gene KIT, histamine