Ke ʻike nei ʻoe i ka helu haʻahaʻa o nā keʻena koko keʻokeʻo? E aʻo kākou e pili ana i ka Autoimmune Neutropenia (AIN)!

Ua noʻonoʻo paha ʻoe no ke aha i hiki ai i ko kākou ʻōnaehana pale ponoʻī, ma kahi o ka pale ʻana iā kākou, ke hoʻomaka i kekahi manawa e hoʻouka i kā kākou mau cell ponoʻī? He ʻano paʻakikī iki kēlā, akā he ʻano maopopo i kapa ʻia ʻo Autoimmune Neutropenia (AIN) . ʻO ka mea e hana ʻia ana, ʻo ia ka hoʻouka ʻana o kā kākou ʻōnaehana pale ponoʻī i kahi ʻano nui o ke koko keʻokeʻo i loko o ko kākou kino, i kapa ʻia ʻo 'neutrophils.' No laila e kamaʻilio maʻalahi kākou e pili ana i kēia, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo.

No laila, he aha ka Autoimmune Neutropenia (AIN)?

I ka ʻōlelo maʻalahi, ʻo ka 'Autoimmune Neutropenia' (AIN) he maʻi pili koko. ʻO ka mea e hana ʻia ma ʻaneʻi, ʻo kā mākou ʻōnaehana pale, ka mea i manaʻo ʻia e pale iā mākou, e hoʻouka hewa ana i kā mākou mau hunaola koko keʻokeʻo i kapa ʻia ʻo 'neutrophils'. E noʻonoʻo i kēia, e like me ka pūʻali koa e pale ana i ko mākou ʻāina e hoʻouka hewa ana i ko mākou poʻe ponoʻī.

He aha nā 'Neutrophils'?

He mau ʻāpana koʻikoʻi loa nā Neutrophils o kā mākou ʻōnaehana pale. Ua like lākou me nā koa liʻiliʻi i loko o ko mākou kino. ʻO kā lākou hana nui ka ʻimi a luku i nā mea ʻino e komo i loko o ke kino, e like me ka bacteria a me nā fungi e hoʻoulu ai i nā maʻi. No laila, i ke ʻano o ka 'Autoimmune Neutropenia', hoʻopau kā mākou ʻōnaehana pale ponoʻī i kēia mau hunaola 'neutrophils' waiwai nui, no laila e emi ana ka nui o nā 'neutrophils' i loko o ke koko ma lalo o nā pae maʻamau.

ʻElua ʻano nui o AIN:

1. ʻO ka Neutropenia Autoimmune mua (AIN): ʻIke pinepine ʻia kēia i nā pēpē a me nā kamaliʻi ʻōpio . ʻO ka mea e hana ʻia ma ʻaneʻi, ʻo ia ka emi ʻana o ka helu o nā 'neutrophils' me ke kumu ʻole. Akā ʻo ka nūhou maikaʻi, ʻo ia ka hoʻoponopono pinepine ʻana o kēia kūlana i loko o kekahi mau makahiki.

2. ʻO ka Neutropenia Autoimmune Lua (AIN): ʻOi aku ka maʻamau o kēia i nā keiki makua a me nā pākeke . Maanei, ʻo ka helu neutrophil haʻahaʻa i hoʻokumu ʻia e kekahi maʻi olakino ʻē aʻe. No ka laʻana, hiki ke hoʻokumu ʻia e kahi maʻi, kahi maʻi koko ʻē aʻe, a i ʻole kekahi maʻi autoimmune ʻē aʻe (kahi kūlana e hoʻouka ai ka ʻōnaehana pale i kona kino ponoʻī). ʻO kēia kūlana AIN lua he maʻi mau loa .

Pehea ka haʻahaʻa o ka helu o nā neutrophils i kapa ʻia ʻo neutropenia?

ʻO ka Autoimmune Neutropenia kekahi ʻano o ke kūlana ākea i kapa ʻia ʻo Neutropenia. Ua hoʻokaʻawale ʻia ʻo Neutropenia he ʻoluʻolu, waena, a koʻikoʻi paha, ma muli o ka helu o nā neutrophils i kēlā me kēia microliter (microL) o ke koko.

• Neutropenia ʻoluʻolu: 1,000 - 1,500 neutrophils no kēlā me kēia microL.
• Neutropenia Kaulike: 500 - 1,000 neutrophils no kēlā me kēia microL.
• Neutropenia Koʻikoʻi: ʻOi aku ka liʻiliʻi o nā neutrophils ma mua o 500 no ka microL.

ʻO ka mea nui loa, pono e hōʻoia ʻia kēia mau waiwai e ke kauka. Inā he kānalua kou e pili ana i kēia, he mea nui loa ia e ʻimi i ka ʻōlelo aʻoaʻo lapaʻau.

Pehea ka hopena o AIN i koʻu kino?

Hiki ke ʻokoʻa ke ʻano o ka hoʻopilikia ʻana o AIN iā ʻoe mai kēlā kanaka a kēia kanaka. Aia ia i kekahi mau kumu, no laila ʻoi aku ka maikaʻi o ke kamaʻilio ʻana me kāu kauka e pili ana i kēia. Inā emi haʻahaʻa loa kāu helu neutrophil, ʻoi aku ka nui o ka loaʻa ʻana o nā maʻi . I kekahi manawa hiki i kēia mau maʻi ke hoʻoweliweli i ke ola, ʻoiai inā ʻaʻole ʻoe e loaʻa koke i ke kōkua lapaʻau.

Eia nō naʻe, i kekahi mau hihia, hiki i ka neutropenia autoimmune ke hoʻoulu i nā maʻi liʻiliʻi wale nō. A i ʻole hiki ke asymptomatic. ʻO ia ke kumu he mea nui e hana pū me ke kauka e ʻike i ke kumu o kou neutropenia. Ma muli o ke kumu, hiki i kāu kauka ke nānā i kou kūlana a kōkua iā ʻoe e kāohi i ka maʻi.

ʻO wai ka mea e loaʻa ana i ka Autoimmune Neutropenia (AIN)?

• AIN Mua: Hoʻopilikia nui kēia i nā pēpē a me nā keiki liʻiliʻi . ʻIke pinepine ʻia ma kahi o 8 mahina o ka makahiki.
• AIN Lua: Hana pinepine ʻia kēia me kahi maʻi autoimmune ʻē aʻe, e like me ka lupus a i ʻole ka rheumatoid arthritis. ʻIke pinepine ʻia nā keiki me ka AIN lua ma kahi o 10 mau makahiki. ʻIke pinepine ʻia nā pākeke me ka AIN ma waena o 40 a me 60 mau makahiki.

Pehea ka maʻamau o ka neutropenia autoimmune?

He maʻi laha ʻole ka AIN mua. Hoʻopilikia wale ia i hoʻokahi i loko o 100,000 mau keiki ma lalo o 10 makahiki. No laila hiki iā ʻoe ke ʻike i kona laha ʻole.

He aha nā hōʻailona o ka Autoimmune Neutropenia?

ʻAʻole i hoʻoulu mau ʻia e ka Autoimmune Neutropenia nā hōʻailona nui. Eia nō naʻe, i kekahi manawa e ʻike paha ʻoe i nā hōʻailona o ka maʻi. Eia kekahi laʻana:

• Nā maʻi pepeiao: ʻO kēia kekahi o nā hōʻailona maʻamau. E noʻonoʻo e hoʻomau ana kāu keiki liʻiliʻi i ka ʻeha pepeiao, a i kou hele ʻana i ke kauka, haʻi lākou iā ʻoe he maʻi pepeiao ʻē aʻe ia.
• Nā maʻi hanu: Anu pinepine, ʻū, a me ke kuni.
• Nā maʻi waha: Nā mea e like me nā ʻeha waha a me ka gingivitis.
• Nā maʻi ʻili.
• Pneumonia.
• ʻO ka maʻi ʻōpū.
• Nā maʻi ʻōpū mimi (UTI).
• Sepsis: He maʻi koʻikoʻi loa kēia a hoʻoweliweli i ke ola.

ʻO nā maʻi e pili ana me ka AIN mua he akahai pinepine ia, akā ʻo nā maʻi koʻikoʻi e like me ka pneumonia, meningitis, a me ka sepsis he mea maʻamau ia i ka AIN lua.

He aha ke kumu o ka neutropenia autoimmune?

Ua hoʻokaʻawale ʻia ʻo AIN ma ke ʻano he mua a lua paha ma muli o ke kumu o nā pae neutrophil haʻahaʻa.

ʻO ka Neutropenia Autoimmune Mua (AIN Mua)

I loko o ka AIN mua, kuhikuhi nā antibodies a luku i nā neutrophils. ʻO nā antibodies kahi ʻano protein i loko o kā mākou ʻōnaehana pale. ʻO kā lākou hana maʻamau ka luku ʻana i nā mea ʻino, e like me nā virus a me nā bacteria, e komo i loko o ke kino. Akā i kēia manawa, ʻaʻole maopopo i nā mea noiʻi ke kumu e hoʻouka ai kā mākou mau antibodies ponoʻī i kēia mau neutrophils.

ʻO ka Neutropenia Autoimmune Lua (AIN Lua)

I ka AIN lua, ua emi ka helu o nā neutrophils ma muli o kekahi kumu a kūlana paha. ʻO kēia ke kumu o ka luku ʻana o ka ʻōnaehana pale i nā neutrophils.

ʻO kekahi mau mea e hiki ai ke kumu o kēia:

• Nā maʻi autoimmune ʻē aʻe: ʻO nā hiʻohiʻona e pili ana i ka rheumatoid arthritis, lupus, a me ka anemia hemolytic autoimmune.
• Ka maʻi ʻaʻai: No ka laʻana, ʻo ka `large granular lymphocyte leukemia`.
• Nā maʻi: Nā maʻi e like me ka HIV, Parvovirus, hepatitis B.
• Nā puʻupuʻu: E like me ka 'Wilms tumor', 'Hodgkin's disease'.
• Nā maʻi neurological: No ka laʻana, ka sclerosis maha.
• Nā hoʻoili puʻupaʻa a iwi paha.
• Kekahi mau lāʻau lapaʻau: ʻOi aku kekahi mau lāʻau chemotherapy i hāʻawi ʻia no ka maʻi kanesa.

Pehea e ʻike ʻia ai ka Autoimmune Neutropenia? (Diagnosis)

Ke loaʻa iā ʻoe ke kuni a i ʻole kahi maʻi, e ʻike paha kāu kauka ua haʻahaʻa kāu helu neutrophil i ka wā o ka hana koko e hoʻoholo ai i ke kumu. Hiki ke paʻakikī iki ka ʻike ʻana i ka AIN, no ka mea, he nui nā kumu hiki. Hiki i kāu kauka ke ʻike i ka AIN ma hope o ka hoʻoholo ʻana i nā kūlana ʻē aʻe e hiki ke kumu i ka haʻahaʻa o kāu helu neutrophil.

No ka ʻike ʻana a i ʻole ka nānā ʻana i ka 'Autoimmune Neutropenia', hiki i kāu kauka ke hana i nā hoʻokolohua aʻe:

• Helu koko piha (CBC): Hiki i kahi CBC ke nānā i ka helu o nā neutrophils i loko o kou koko. Ma muli o ka hiki ke ʻokoʻa kēia mau pae, e hana pinepine kāu kauka ma mua o hoʻokahi CBC e hōʻoia i ka neutropenia a nānā i kāu mau pae neutrophil. Hiki i nā CBC maʻamau ke kōkua i ka hoʻoholo ʻana inā he maʻi mau kāu AIN.
• ʻO ka palaki koko peripheral: Nānā ʻia kahi hāpana koko ma lalo o kahi microscope e nānā i ke ʻano a me ke ʻano o nā neutrophils. Kōkua pū kēia i ka ʻike ʻana.
• Nā hoʻāʻo Vitamin B12, folate, a me ke keleawe: Hiki i kēia mau hoʻāʻo ke nānā i nā hemahema i kēia mau meaola. Pono ke kino i kēia mau mea e hana i nā neutrophils.
• Nā hoʻāʻo hana ate: Hiki i kēia ke nānā i ka hana ate a inā he hepatitis viral.
• HIV a me nā haʻawina viral ʻē aʻe: Hiki i nā maʻi viral e like me ka HIV ke hana i ka emi ʻana o nā hunaola koko keʻokeʻo.
• ʻO ka cytometry kahe: He hoʻāʻo hoʻokolohua kēia e nānā ana i nā ʻāpana no nā hōʻailona o ka maʻi. Hoʻohana ʻia ia e hoʻoholo inā ua hoʻokumu ʻia ka neutropenia e kahi maʻi autoimmune.
• Kiʻi: Inā he kānalua ua hoʻokumu ʻia ʻo AIN e kahi maʻi kanesa e like me lymphoma, hiki ke hana ʻia kahi MRI a i ʻole CT scan.
• ʻO ka makemake ʻana i ka iwi iwi a me ka biopsy: Maanei, lawe ke kauka i kahi laʻana liʻiliʻi o ka iwi iwi a nānā iā ia no nā hōʻailona o ka maʻi. Hiki i kēia ke kōkua i ka hoʻoholo ʻana inā ua hoʻokumu ʻia ka neutropenia e kahi maʻi koko, e like me ka leukemia.

Hiki i kāu kauka ke kauoha i kahi hoʻāʻo koko e nānā i nā antibodies i loko o kou koko e hoʻouka i nā neutrophils. ʻO nā hoʻokolohua maʻamau ka I-GIFT (Indirect granulocyte immunofluorescence test) a me ka GAT (Granulocyte agglutination test).

ʻO ka mea pōʻino, ʻaʻole hilinaʻi mau kēia mau hoʻāʻo i ka ʻike ʻana iā AIN. Ma muli o ke kumu o kou neutropenia a me ka manawa o ka hoʻāʻo ʻana, ʻaʻole paha e ʻike kēia mau hoʻāʻo i nā antibodies a hāʻawi paha i nā hopena wahaheʻe. ʻO ka hopena wahaheʻe ka wā e hōʻike ai ka hoʻāʻo he loaʻa nā antibodies i ka wā ʻaʻole lākou.

Inā manaʻo ke kauka ua loaʻa ka neutropenia i kahi kūlana kūikawā, hiki iā ia ke kauoha i nā hoʻokolohua hou aʻe.

Pehea e mālama ʻia ai ka Autoimmune Neutropenia?

Aia ka lāʻau lapaʻau i ke koʻikoʻi o kou kūlana. No ka laʻana, ʻo kahi pēpē me ka asymptomatic neutropenia e pono wale nō e loaʻa nā CBC maʻamau a me nā helu neutrophil, akā ʻaʻole pono paha ka lāʻau lapaʻau koke. Inā loaʻa pinepine nā maʻi i kāu pēpē, hiki i kāu kauka ke kuhikuhi i nā antibiotics prophylactic e pale aku i nā maʻi. Hāʻawi pinepine ʻia nā antibiotics e mālama i nā maʻi, akā hāʻawi ʻia kēia mau antibiotics prophylactic e pale aku i nā maʻi mai ka hanana ʻana.

No kekahi me ka AIN lua, e mālama ke kauka i ke kūlana kumu e hoʻoulu ai i nā pae neutrophil haʻahaʻa. Hiki iā lākou ke kuhikuhi pū:

• ʻO ka Granulocyte colony-stimulating factor (G-CSF): ʻO kēia ka lāʻau lapaʻau nui no nā hihia koʻikoʻi o ka neutropenia. Kōkua ia i ke kino e hana i nā neutrophils hou aku. Hiki iā G-CSF ke pale aku i nā maʻi pinepine e pili ana me ka AIN mua.
• Corticosteroids: ʻO Corticosteroids, e like me prednisone, e kāohi i ka pane ʻōnaehana pale o kou kino, e hoʻemi ana i ka helu o nā antibodies e hoʻouka i nā neutrophils.
• Nā lāʻau lapaʻau ʻē aʻe: Ua hoʻohana ʻia nā lāʻau lapaʻau ʻē aʻe e like me IVIG, rituximab, a me alemtuzumab i nā poʻe maʻi koʻikoʻi me ka neutropenia autoimmune koʻikoʻi.

Hiki ke pale ʻia ka Autoimmune Neutropenia?

ʻAʻole hiki ke pale ʻia ka AIN mua a me ka AIN lua. Eia nō naʻe, hiki iā ʻoe ke pale i nā pilikia e pili ana me ka 'Autoimmune Neutropenia', ʻo ia hoʻi, ka ulu ʻana o nā maʻi. No ka hana ʻana i kēia, pono ʻoe e hana i nā hana e pono ai e pale iā ʻoe iho mai nā maʻi. No ka laʻana, e pale i nā wahi i piha i nā kānaka, e holoi pinepine i kou mau lima, a e noho mamao aku mai ka poʻe maʻi.

He aha ke ʻano o ka wā e hiki mai ana e hiki i kekahi me Autoimmune Neutropenia ke manaʻo?

Hoʻopau pinepine ʻia ka AIN mua ma kāna iho . Ma kahi o 95% o nā keiki me AIN e hoʻi hou ana ko lākou pae neutrophil i ka maʻamau i ka piha ʻana o ʻelua a ʻekolu mau makahiki. Hoʻōla piha ka hapa nui o nā keiki me ka AIN mua i ka piha ʻana o 11 mau makahiki. He mea hōʻoluʻolu maoli kēia, ʻeā?

I nā hihia ʻē aʻe, i kapa ʻia ʻo AIN lua, hilinaʻi kou hoʻōla ʻana i kekahi mau kumu, ʻoiai ke kūlana kumu i hoʻokumu ai i kou neutropenia. ʻO ka mālama ʻana i kēlā kūlana a me ka pale ʻana i nā maʻi e hiki ke kōkua i ka hoʻihoʻi ʻana i kāu helu neutrophil i ka maʻamau.

Āhea wau e ʻimi ai i ke aʻo lapaʻau?

Inā loaʻa iā ʻoe ka neutropenia autoimmune koʻikoʻi a loaʻa iā ʻoe ke kuni, e ʻike koke i ke kauka . I kekahi manawa, ua kuhikuhi paha kāu kauka i nā lāʻau antibiotic ma mua e lawe inā loaʻa iā ʻoe ke kuni. I ka hapanui o nā hihia, pono ʻoe e mālama ʻia ma ka haukapila.

Inā loaʻa iā ʻoe ka neutropenia autoimmune koʻikoʻi a lōʻihi, e wehewehe kāu kauka iā ʻoe i nā hōʻailona āu e makaʻala ai a me nā mea hiki iā ʻoe ke hana e pale ai i nā pilikia.

E hoʻomanaʻo: Inā ʻike ʻia ʻoe a i ʻole kāu keiki me AIN, e kamaʻilio me kāu kauka e pili ana i kona hopena iā ʻoe. ʻOiai ʻokoʻa nā hōʻailona a me nā kumu, ʻo ka nūhou maikaʻi, he kūpono loa nā lāʻau lapaʻau e like me G-CSF i ka hoʻonui ʻana i nā pae neutrophil.

Inā loaʻa i kāu keiki ka AIN, e hana i nā hoʻāʻo koko maʻamau e like me ke kuhikuhi a kāu kauka. E makaʻala i nā hōʻailona o ka maʻi, a e ʻimi i ka lāʻau lapaʻau i ka hikiwawe loa inā ʻike ʻoe i kekahi, e pale aku i nā pilikia hiki.

Hōʻuluʻulu Manaʻo (Leka Lawe-Home)

ʻAe, ke lana nei koʻu manaʻo ua ʻike maikaʻi ʻoe i ka 'Autoimmune Neutropenia' (AIN) a mākou i kamaʻilio ai.

• ʻO ka AIN kahi kūlana e hoʻopau ai ko kākou ʻōnaehana pale ponoʻī i ko kākou mau hunaola koko keʻokeʻo i kapa ʻia ʻo 'neutrophils'.
• Hiki ke hana kēia i nā keiki ʻōpio (AIN mua) a i ʻole i nā pākeke (AIN lua). Hoʻoholo pinepine ka AIN mua i nā keiki ʻōpio ma kāna iho.
• ʻO ka pilikia nui, ʻo ia ka maʻi pinepine.
• Aia nā hoʻāʻo koko a me nā hoʻokolohua ʻē aʻe e ʻike ai.
• Hoʻokomo ʻia ka mālama ʻana i ka nānā ʻana, nā lāʻau lapaʻau anti-inflammatoire, G-CSF, a me nā corticosteroids.
• ʻO ka mea nui loa, ʻaʻole e hopohopo a hopohopo paha a e ʻimi i ke kōkua lapaʻau inā loaʻa iā ʻoe a i ʻole kāu keiki kēia mau hōʻailona. Inā ʻike mua ʻia a mālama pono ʻia, hiki iā ʻoe ke ola i ke ola olakino me AIN.

Inā he mau nīnau hou aku kāu e pili ana i kēia, e kamaʻilio me kāu kauka. ʻO lākou ka poʻe hiki ke hāʻawi iā ʻoe i ka ʻōlelo aʻoaʻo maikaʻi loa.

ʻO Neutropenia, Autoimmune Neutropenia, Nā Kelepona Koko Keʻokeʻo, ʻŌnaehana Palekana, Nā Maʻi ʻAna, Nā Maʻi Koko, Nā Maʻi Pediatric