Makemake pū ʻoe e ʻike e pili ana i ka Pelger-Huet Anomaly? E kamaʻilio kākou e pili ana!

Ua haʻi aku anei kekahi kauka iā ʻoe e pili ana i kahi loli liʻiliʻi i loko o kou mau keʻokeʻo koko ke loaʻa iā ʻoe kahi hoʻāʻo koko? Hiki paha iā ia ke ʻano Pelger-Huet Anomaly. He mea nui paha ka inoa, akā mai hopohopo , ʻaʻole ia he mea koʻikoʻi loa. E kamaʻilio kākou e pili ana i kēia me nā kikoʻī hou aku, ma ke ʻano maʻalahi.

He aha ka Anomaly Pelger-Huet?

I ka ʻōlelo maʻalahi, ʻo Pelger-Huet Anomaly (PHA) kahi maʻi genetic e hoʻopilikia ana i nā neutrophils, kahi ʻano koko keʻokeʻo i loko o ko mākou kino. Ua like nā Neutrophils me nā koa liʻiliʻi i loko o ko mākou kino e kōkua ana i ko mākou kino e hakakā i nā germs.

Hoʻokumu ʻia kēia kūlana e kahi ʻokoʻa o kā mākou DNA, ʻoi aku hoʻi i loko o kahi gene i kapa ʻia ʻo Lamin B Receptor (gene LBR). ʻO kēia gene LBR ka mea e kōkua i nā cell neutrophil e ulu a ulu pono.

E nānā i kēia manawa, aia i kēlā me kēia cell neutrophil kahi kikowaena hoʻomalu, kahi a mākou e kapa nei ʻo ka nucleus . Aia i loko o kēia nucleus nā ʻike DNA āpau e pono ai ke cell e hana a ulu. ʻO ka maʻamau, ua māhele ʻia ka nucleus o kahi neutrophil olakino i ʻekolu a ʻoi aku paha mau ʻāpana. Eia nō naʻe, ʻelua ʻāpana o ka nucleus o kahi neutrophil i loko o kekahi me Pelger-Huette. Ua like ia me ka dumbbell, me ka momona ma nā ʻaoʻao ʻelua a me kahi waena lahilahi. He mea kākaʻikahi loa, i kekahi poʻe, hiki i kēia nucleus neutrophil ke ʻano oval, ʻo ia hoʻi, ke ʻano e like me ka hua manu.

Akā, ʻo ka mea nui ma ʻaneʻi, ʻoiai inā ua hānau ʻia ʻoe me ka anomaly Pelger-Huette, i ka hapa nui o ka manawa e hana maʻamau kāu mau hunaola neutrophil. ʻO ke ʻano kēia, ʻaʻole lākou i hoʻopilikia nui ʻia i ka pale ʻana iā ʻoe mai ka maʻi. No laila, ʻaʻole pinepine ia e hoʻoulu i nā pilikia olakino nui.

Aia kekahi maʻi ʻē aʻe i kapa ʻia ʻo Pseudo Pelger-Huet Anomaly (PPHA) . Hiki i kēia ke hana i kekahi poʻe ma hope o ke ola. Hiki i ka PPHA ke hana ma ke ʻano he hopena ʻaoʻao o kekahi mau lāʻau lapaʻau a i ʻole he hōʻailona o kekahi maʻi ʻē aʻe, e like me kahi maʻi.

He aha nā hōʻailona o ka anomaly Pelger-Huette?

Maanei pono mākou e kālele i nā ʻano ʻelua a mākou i kamaʻilio ai ma mua.

Anomaly Pelger-Huet Hoʻoilina (HPA)

Inā loaʻa iā ʻoe ka Pelger-Huette anomaly (HPA), kahi mea i hoʻoilina ʻia, ʻaʻohe ou hōʻailona. ʻAʻole paha ʻoe e ʻike he maʻi kou. ʻIke pinepine ʻia ia i ka wā e hana ʻia ai kahi hoʻāʻo koko no kekahi kumu ʻē aʻe.

Anomaly Pseudo Pelger-Huet (PPHA)

Eia nō naʻe, inā loaʻa iā ʻoe ka pseudo-Pelger-Huette anomaly (PPHA), ʻo ia hoʻi ua ulu aʻe ma hope, hiki iā ʻoe ke ʻike i nā hōʻailona e pili ana i ke kūlana kumu i hoʻokumu ai.Eia kekahi laʻana, inā loaʻa ka PPHA e kahi maʻi, aia paha nā hōʻailona e like me ke kuni a me nā ʻeha kino e pili ana i kēlā maʻi.

He aha ke kumu o ka anomali Pelger-Huette?

ʻOkoʻa nō hoʻi ke kumu o kēia ma muli o nā ʻano ʻelua a mākou i kūkākūkā ai ma mua.

ʻO ke ʻano Pelger-Huette hoʻoilina (HPA)

Hoʻokumu ʻia kēia e kahi mutation i loko o ka gene Lamin B Receptor (LBR), e like me kā mākou i ʻōlelo ai ma mua. Ke kuhikuhi nei kēia gene LBR i ko mākou kino e hana i nā protein e pono ai e pale i nā cell neutrophil a hāʻawi iā lākou i ke ʻano kūpono. Ma ka anomaly Pelger-Huytt, hoʻopilikia kēia mutation genetic i kēlā kaʻina hana, e hoʻoulu ai i nā nuclei o neutrophils e lawe i kēlā ʻano dumbbell. He kūlana kēia e hiki ke hoʻoilina mai nā mākua a i nā keiki.

ʻAnomaly Pseudo Pelger-Huette (PPHA)

ʻAʻole kēia he maʻi hoʻoilina. Aia kekahi mau kumu e hiki ai ke kumu i kēia maʻi:

• Nā maʻi iwi ʻiwi: ʻO nā hiʻohiʻona e pili ana i nā kūlana leukemia e like me ka myelodysplastic syndrome a me ka acute myeloid leukemia (AML).
• ʻO kekahi mau maʻi: ʻoi aku hoʻi nā maʻi i lawe ʻia e ka ʻuku.
• Nā Immunosuppressants: ʻO kēia mau lāʻau lapaʻau, i hāʻawi ʻia no kekahi mau maʻi, ʻo ia paha ke kumu.
• Ka ʻike ʻia ʻana o ka radiation: No ka laʻana, i ka wā o ka mālama ʻana i ka maʻi kanesa.

Inā haʻi aku ke kauka iā ʻoe he 'PHA' a i ʻole 'PPHA' kou, he mea nui loa ia e hoʻomaopopo i ke kauka e pili ana i nā lāʻau lapaʻau āpau āu e lawe nei, ʻoiai nā huaora.

He aha nā kumu pilikia no kēia maʻi?

ʻO ke kumu nui o ka pilikia no ka Pelger-Huette anomaly (PHA) ʻo ia ka loaʻa ʻana o kahi makua hānau me ke ʻano. ʻO ke ʻano kēia, inā loaʻa i kou makuahine a makua paha ka PHA, he 50% kou manawa e hoʻoilina ai. Ua like ia me ka hoʻolei ʻana i kahi ʻāpana kālā, ʻaʻole hiki iā ʻoe ke ʻōlelo maopopo, akā hiki ke hana ʻia.

No ke kūlana `PPHA`, ʻo ka ʻike ʻana i nā kūlana olakino a i ʻole nā ​​​​lāʻau lapaʻau i ʻōlelo ʻia ma mua he mau mea pilikia.

Aia kekahi mau pilikia me kēia maʻi?

ʻO ka maʻamau, ʻaʻole ia e hoʻoulu i nā pilikia nui i ka hoʻoilina Pelger-Huette anomaly (PHA). No ka mea, ʻoiai ke hoʻololi nei ia i ke ʻano o kāu mau cell neutrophil, ʻaʻole ia e hoʻololi nui i kā lākou hana. Ke hoʻomau nei lākou i ke kaua ʻana i nā germs.

Eia nō naʻe, inā loaʻa iā ʻoe ka pseudopelgar-huwat anomalous (PPHA), hiki i ke kūlana olakino i hoʻokumu ʻia (e like me ka leukemia) ke hana i nā pilikia. No laila, he mea nui e kamaʻilio me kāu kauka e pili ana i kēia a ʻike i ka mea e manaʻo ai.

Pehea e ʻike ai nā kauka i ka maʻi ʻano Pelger-Huette?

Hoʻohana nui nā kauka i nā hoʻāʻo koko e ʻike ai i kēia kūlana.Hana ʻia kahi hoʻāʻo i kapa ʻia he peripheral blood smear. ʻO kēia hoʻāʻo e pili ana i ka lawe ʻana i kahi kulu o kāu hāpana koko, e hohola lahilahi ana ma luna o kahi slide aniani, a nānā iā ia ma lalo o kahi microscope.

Hoʻokolokolo ʻia kēia e kekahi pathologist a hematologist paha.

Inā loaʻa iā ʻoe ka anomaly Pelger-Huette, e hōʻike maopopo kēia hoʻāʻo i ke ʻano ʻē o nā nuclei o kāu mau cell neutrophil (e like me ka dumbbell).

Aia kekahi lāʻau lapaʻau no ka anomaly Pelger-Huette?

Eia ka nūhou maikaʻi! ʻO ka maʻamau, ʻaʻole hōʻike ka ʻano Pelger-Huette Hereditary (PHA) i nā hōʻailona ʻole, no laila ʻaʻole pono ia i kekahi lāʻau kūikawā. ʻOiai inā loaʻa iā ʻoe kēia maʻi, hiki iā ʻoe ke ola i kahi ola maʻamau.

Eia nō naʻe, i ke ʻano o ka `PPHA`, he mea nui e mālama i ka maʻi kumu i hoʻokumu ai. Ke ho'ōla ʻia kēlā maʻi, hiki ke nalowale ke ʻano `PPHA`.

Āhea wau e ʻike ai i ke kauka?

Inā ʻike ʻoe he Pelger-Huette anomaly kou, e hōʻoia e haʻi i kāu kauka inā loaʻa iā ʻoe kekahi mau hōʻailona hou (e like me ke kuni, ka luhi nui, nā maʻi pinepine). A laila hiki i kāu kauka ke hoʻoholo inā pili nā hōʻailona i ka 'PHA', 'PPHA', a i ʻole kekahi mea ʻē aʻe.

Ke hele ʻoe e ʻike i ke kauka, he mea maikaʻi nō hoʻi e nīnau i kēia mau nīnau:

• Loaʻa iaʻu ka maʻi hoʻoilina ʻo `(HPA)` a i ʻole ka maʻi ʻano like ʻole ʻo Pelger-Huette ʻo `(PPHA)`?
• Hiki paha i kaʻu mau keiki ke hoʻoilina i kēia maʻi? (Inā HPA)
• ʻEhia pinepine e pono ai iaʻu ke ʻike i ke kauka?
• He aha nā hōʻailona e pono iaʻu e makaʻala nui?

He aha kaʻu e manaʻo ai me kēia kūlana?

ʻAʻole ʻo Pelger-Huette anomaly (PHA) e hōʻeha iā ʻoe a ʻaʻole hoʻi e hana i kahi ʻokoʻa nui i kou ola. ʻAʻole ia e hoʻopilikia i kāu mau hana o kēlā me kēia lā.

Eia nō naʻe, inā loaʻa iā ʻoe kēia maʻi (ʻoi aku hoʻi ke ʻano 'PPHA'), hiki iā ia ke lilo i hōʻailona o kekahi maʻi olakino ʻē aʻe. ʻO ia ke kumu e hopohopo nei nā kauka no ia mea. Inā kānalua kāu kauka he 'PHA' a i ʻole 'PPHA' kou, e hana lākou i nā hoʻokolohua e pono ai e hōʻoia iā ia, a e hana pū i nā hana e kāpae i nā maʻi olakino ʻē aʻe.

Loaʻa paha iā ʻoe ka Pelger-Huette anomaly (PHA) a ʻaʻole ʻoe i ʻike no ka mea ʻaʻohe ou hōʻailona. Manaʻo paha ʻoe, "Inā ʻaʻole ia e hoʻopilikia iaʻu, no ke aha wau e noʻonoʻo nei i ia mea?" He ʻoiaʻiʻo ʻaʻole paha ʻoe e maʻi ma muli o kēia hoʻololi ʻana o nā cell. Akā he mea nui e hoʻomaopopo i kāu kauka e pili ana i ia mea. Aia kāu kauka ma laila e kōkua iā ʻoe e noho olakino. A he mea kōkua ia e ʻike e pili ana i kēia mau mea liʻiliʻi i loko o kou kino. ʻOiai he Pelger-Huette anomaly kou, he mea ʻē aʻe ia e hoʻolilo iā ʻoe i "ʻoe." ʻAʻohe mea e hopohopo ai.

Hōʻuluʻulu Manaʻo (Leka Lawe-Home)

ʻAe, e hōʻuluʻulu kākou i kekahi o nā mea a mākou i kamaʻilio ai:

• ʻO ka Pelger-Huet Anomaly (PHA) kahi maʻi genetic e hoʻoulu ai i kahi ʻano ʻē o ka nucleus o nā neutrophils, kahi ʻano keʻokeʻo koko.
• ʻAʻole kēia he mea weliweli a ʻaʻole ia e hōʻike i nā hōʻailona. Hana maʻamau nā pūnaewele neutrophil.
• ʻElua ʻano o kēia: ʻo 'HPA', he mea hoʻoilina, a me 'PPHA', i hoʻoulu ʻia ma hope e nā maʻi ʻē aʻe a i ʻole nā ​​​​lāʻau lapaʻau ʻē aʻe.
• ʻAʻole pono ka lāʻau lapaʻau no ka HPA. I ke ʻano o ka PPHA, mālama ʻia ke kumu kumu.
• Hoʻomaopopo ʻia kēia kūlana ma o kahi hoʻāʻo koko (peripheral blood smear).
• Inā ʻike ʻoe ua loaʻa iā ʻoe kēia maʻi, mai hopohopo. Eia nō naʻe, e kamaʻilio me kāu kauka e pili ana i ia mea a loaʻa i ka ʻōlelo aʻoaʻo e pono ai. E hoʻomaopopo i kāu kauka inā ʻike ʻia nā hōʻailona hou.

Manaʻolana wau ua pane kēia ʻatikala i ka hapa nui o kāu mau nīnau e pili ana i ka anomaly Pelger-Huette. E hoʻomanaʻo, me kekahi pilikia olakino, ʻoi aku ka maikaʻi o ke kamaʻilio ākea me kāu kauka.

ʻAnomaly Pelger -Huet, Neutrophils, Nā Kelepona Koko Keʻokeʻo, Nā Maʻi Hoʻoilina, Nā Hoʻāʻo Koko, PPHA, HPA