Aia kekahi pilikia me kou mau hunaola koko keʻokeʻo? E aʻo kākou e pili ana i ka Histiocytosis ma nā ʻōlelo maʻalahi!

Ua ʻike anei ʻoe i kekahi manawa e hoʻomaka ana kekahi mau ʻano o nā cell i loko o ko kākou kino e hana i kahi ʻano ʻokoʻa iki? ʻO kekahi o ia ʻano maʻi kakaikahi i kapa ʻia ʻo Histiocytosis. He mea weliweli iki paha kēia iā ʻoe, akā mai hopohopo. E kamaʻilio kākou e pili ana i ia mea ma ke ʻano maʻalahi a ma ke ʻano kūpono.

He aha ka Histiocytosis? Ma nā huaʻōlelo maʻalahi...

Loaʻa i kou kino kekahi ʻano o nā hunaola koko keʻokeʻo i kapa ʻia he histiocytes. Ma waena o kēlā mau hunaola koko keʻokeʻo, aia kekahi ʻano kūikawā o nā hunaola i kapa ʻia he histiocytes . Ua like kēia me nā kiaʻi palekana o ko kākou kino. ʻO lākou ka poʻe e hakakā nei i nā huakino e hoʻoulu ai i nā maʻi i kapa ʻia he pathogens - nā virus , bacteria , a me nā fungi - a kōkua i ka mālama ʻana i ko kākou ʻōnaehana pale kino .

Eia nō naʻe, i kekahi manawa hoʻomaka kēia mau cell histiocyte e ulu ma ke ʻano ʻē, ʻo ia hoʻi, lilo lākou i " mutant " . A laila, ulu nui kēia mau cell histiocyte maʻamau a hōʻiliʻili ma nā ʻāpana like ʻole o ko mākou kino. Ke hōʻiliʻili nui lākou, hiki i kā mākou ʻōnaehana pale ke loaʻa nā pilikia. ʻO ka mea nui , ʻo ka mumū , ʻo ia hoʻi, kahi ʻano pehu, e kū mai ana i loko o nā ʻiʻo o ke kino. Hiki i kēia mumū ke hōʻino i ko mākou mau kino i kekahi manawa.

Hiki i kēia mau pūnaewele histiocyte ʻano ʻē ke hōʻiliʻili i loko o kou mau lymph nodes (i kapa ʻia hoʻi he lymph nodes), ʻili, iwi, māmā, akepaʻa, spleen, a i ʻole ma nā wahi ʻē aʻe o kou kino. I kekahi manawa hoʻopilikia ia i hoʻokahi wale nō ʻāpana o kou kino. Kāhea mākou i kēia he kūlana 'ʻōnaehana hoʻokahi' . I kekahi manawa hiki ke hoʻopilikia i nā ʻāpana he nui o kou kino. Kāhea mākou i kēia he kūlana 'multisystem' .

ʻO ka mea nui, ʻaʻole ka histiocytosis he maʻi kanesa . Eia nō naʻe, i kekahi manawa hiki iā ia ke hana e like me ke kanesa a hōʻike i nā hōʻailona like me ke kanesa. ʻO ia ke kumu he kūlana ia e pono ai ka nānā kūikawā. No ka mea, ʻokoʻa nā hōʻailona o kēia mai ke kanaka a ke kanaka a ʻokoʻa ke koʻikoʻi o ke kūlana, hiki i kekahi manawa ke paʻakikī ke ʻike koke i ka histiocytosis.

He aha nā ʻano nui o ka histiocytosis?

I kēia manawa, ua maopopo paha iā ʻoe ke ʻano o ka histiocytosis. Aia ma mua o 100 mau ʻano o ia mea. Akā, aia ʻekolu mau ʻano nui a mākou e ʻike pinepine ai a kamaʻilio pinepine ai. E nānā kākou iā lākou.

1. ʻO ka histiocytosis o nā pūnaewele Langerhans (LCH):

• ʻO kēia ke ʻano maʻamau o ka histiocytosis.
• Hoʻopilikia pinepine kēia maʻi i nā keiki ʻōpio . Hoʻopilikia ia ma waena o 5 a me 9 o kēlā me kēia miliona mau keiki.
• ʻO ka hapa nui o ka manawa, ʻaʻole kēia he mea koʻikoʻi loa, a hiki ke maʻalahi e like me ka "asymptomatic" . Eia nō naʻe, he mea nui e hoʻomanaʻo i kekahi mau hihia, hiki ke koʻikoʻi kēia a hoʻoweliweli i ke ola.

2. Maʻi ʻo Erdheim-Chester (ECD):

• Hoʻopilikia nui kēia ʻano i nā pākeke .
• He kūlana kākaʻikahi loa kēia.
• E like me LCH, hiki i ka ECD ke piʻi mai ka maʻalahi a i ka koʻikoʻi a me ka hoʻoweliweli i ke ola.

3. Maʻi Rosai-Dorfman (RDD):

• E like me LCH, ʻike pinepine ʻia ʻo RDD i nā keiki , akā hiki ke hana pū kekahi i nā pākeke.
• He mea kākaʻikahi loa kēia. Ma kahi o hoʻokahi i loko o ʻelua haneli tausani kānaka i ʻike ʻia me kēia maʻi.
• ʻO ke ʻano nui o kēia ʻo 'classical RDD' . ʻO kēia ka wā e pehu ai kou mau lymph nodes (pehu).
• ʻO kekahi ʻano ʻē aʻe ʻo 'extranodal RDD' . Ma kēia, ua hoʻopilikia ʻia nā ʻōpū ʻē aʻe ma mua o nā lymph nodes. ʻO ia hoʻi, hiki i nā pūnaewele histiocyte keu ke hōʻiliʻili i loko o ka ʻili, nā iwi, a i ʻole nā ​​wahi ʻē aʻe.

Pehea ka laha ʻole o kēia maʻi i kapa ʻia ʻo Histiocytosis?

ʻAe, e like me kaʻu i ʻōlelo ai ma mua, he maʻi laha ʻole ka histiocytosis. ʻO ka ʻoiaʻiʻo, ʻo nā ʻano maʻi ʻoi aku ka ikaika a me ka maʻi kanesa o ia mea he emi iho ma mua o 1% o nā maʻi kanesa āpau i ʻike ʻia ma nā ʻiʻo palupalu a me nā lymph node. No laila hiki iā ʻoe ke noʻonoʻo i ka laha ʻole o kēia.

He aha nā hōʻailona o ka Histiocytosis?

I kēia manawa, e nānā kākou i nā hōʻailona i hōʻike ʻia ma kēia kūlana histiocytosis. Hiki ke ʻokoʻa kēia mau hōʻailona mai kekahi kanaka a i kekahi . ʻO ke kumu o kēia, ʻo ia nā ʻōpū a me nā ʻiʻo e hōʻiliʻili ai nā cell histiocyte keu, e like me kaʻu i ʻōlelo ai, a me ka nui o ka hōʻino ʻia i kēlā mau ʻiʻo, e hoʻopilikia i kēia.

Eia kekahi o nā hōʻailona āu e ʻike ai (a he nui aku paha):

• Nā ʻōhū ʻili : Hiki ke ʻike ʻia kēia mau mea ma nā wahi huluhulu o ke poʻo o nā pēpē ʻōpio (kapa mākou iā lākou ʻo 'cradle cap' ) a i ʻole ma kahi diaper.
• Kuni .
• Luhi, luhi .
• ʻEha poʻo.
• ʻŪhū a pōkole paha ka hanu .
• Ka pohō kaumaha i wehewehe ʻole ʻia .
• ʻEha iwi .
• Nā ʻōpuʻu lymph pehu (ʻo ia ke ʻano o ka pehu ʻana).
• Nā loli o ka ʻike a i ʻole nā ​​maka puʻupuʻu .
• Ka mimi pinepine ʻana a me ka makewai nui .
• Nā ʻōpū ʻili (hiki ke ʻike ʻia ma nā lihilihi).
• Ka paʻakikī i ka noʻonoʻo ʻana a i ʻole ka hoʻomanaʻo ʻana i nā mea .
• Nā pilikia me ke kaulike a me ka hoʻonohonoho ʻana .

Pehea e ʻike ʻia ai nā hōʻailona? Ma muli o kahi e hōʻiliʻili ai nā histiocytes!

Pili kēia mau hōʻailona i kahi o ke kino e hōʻiliʻili ai nā hunaola histiocyte. ʻOkoʻa nā wahi nui o ka hōʻiliʻili ʻana ma muli o ke ʻano o ka histiocytosis.

Nā wahi i hiki ke hoʻopilikia ʻia e ka Langerhans cell histiocytosis (LCH):

• Iwi
• ʻIli
• Nā kiko lymph (pehu ʻana)
• Ake
• ʻŌpū
• Waha
• Nā māmā
• ʻŌnaehana nerve waena (ʻo ia hoʻi, ka lolo a me ke kaula spinal)

Nā wahi e hiki ke hoʻopilikia ʻia e ka maʻi ʻo Erdheim-Chester (ECD):

• Nā iwi lōʻihi (ʻoi aku hoʻi ma nā wāwae)
• Nā maka
• ʻŌnaehana nerve waena
• Nā māmā
• Puʻuwai
• Nā kīʻaha koko
• nā puʻupaʻa
• ʻO nā ʻōpū ma ke kua o ka ʻōpū (kapa mākou iā lākou ʻo 'retroperitoneum' )

Nā wahi i hiki ke hoʻopilikia ʻia e ka maʻi Rosai-Dorfman (RDD):

• Nā kōpili Lymph (ʻoi aku ma ka ʻāʻī, akā hiki ke loaʻa ma kahi ʻē aʻe)
• ʻIli
• ʻIʻo palupalu
• ʻAla hanu kiʻekiʻe
• Iwi
• Retroperitoneum (nā ʻōpū ma hope o ka ʻōpū)
• Nā maka

He aha ke kumu o ka Histiocytosis?

ʻAʻole maopopo i ka poʻe ʻepekema ke kumu o ka histiocytosis, akā ua ʻike lākou he pili paha ia i nā hoʻololi genetic .

I ka ʻōlelo maʻalahi, loaʻa i kēlā me kēia cell i loko o ko kākou kino nā mea genetic, a i ʻole kahi hoʻonohonoho o nā kuhikuhi. Hoʻoholo kēia mau kuhikuhi i ke ʻano o ka hana ʻana o kēlā mau cell. No laila, i ka wā e loli ai kēia mau kuhikuhi, i kapa ʻia ʻo mutations, hoʻomaka nā cell e hana maʻamau ʻole. No ka laʻana, hiki i kahi mutation genetic ke hana i kahi cell e like me ka histiocyte e kope iā ia iho a hoʻomaka e laha me ka ʻole o ke kāohi ʻia.

ʻO ka ʻike ʻana i kēia mau hoʻololi genetic ua hiki i nā mea noiʻi ke hoʻomohala i nā lāʻau lapaʻau hou no ka histiocytosis. Hana kēia mau lāʻau lapaʻau ma ka pale ʻana i nā loli cellular ʻino e hiki ai i nā cell histiocyte ke ulu ma waho o ke kaohi.

Pehea e ʻike ai i ka histiocytosis?

Inā kānalua ʻoe he histiocytosis kou, e hana mua kāu kauka i kahi hoʻokolohua kino a nīnau e pili ana i kou mōʻaukala olakino. A laila, e hoʻoholo lākou i nā hoʻokolohua e hana ai ma muli o kāu mau hōʻailona a me nā ʻōpū i manaʻo ʻia ua hoʻopilikia ʻia e nā histiocytes keu.

Eia kekahi mau hoʻokolohua hiki iā ʻoe ke hana:

• Nā kaʻina hana kiʻi :
• Hiki i kāu kauka ke kauoha i kahi scan kūikawā i kapa ʻia he FDG PET/CT scan . Hiki i kēia ke ʻike i nā mea e like me nā puʻupuʻu ma ke kino holoʻokoʻa.
• Eia kekahi , pono paha nā hoʻokolohua e like me ka CT scan, MRI scan, bone scan, ultrasound scan, X-ray, a i ʻole echocardiogram (kahi scan o ka puʻuwai).

• Nā hoʻokolohua keʻena hoʻokolohua :
• E ho'āʻo kāu kauka i nā hāpana wai, e like me kou koko a mimi paha, e ʻike inā he mau hōʻailona o ka histiocytosis.
• Nānā ʻia kou mau helu koko. Ke nānā pū nei lākou i nā hormones a i ʻole nā ​​protein kikoʻī e hōʻike ana i ke ʻano o ka hana ʻana o kāu mau kino. Inā ʻaʻole hana pono kahi kino, hiki ke hōʻike he nui loa nā histiocytes i loko o kēlā kino.

• Hoʻāʻo Biopsy `(Biopsy)` :
• ʻO ka hana a kēia, lawe ke kauka i kahi hāpana ʻiʻo a nānā iā ia ma lalo o kahi microscope e ʻike inā he histiocytosis kou.
• Ke nānā nei hoʻi ia i nā hoʻololi genetic kikoʻī e pili ana me ka histiocytosis.
• I kekahi manawa, inā ʻaʻole hiki ke hana i kahi biopsy, hiki ke hoʻāʻo ʻia nā mea genetic i loko o kou koko. Ua kapa ʻia kēia he 'liquid biopsy' .

He aha nā lāʻau lapaʻau no ka Histiocytosis?

ʻO ka mālama ʻana i ka histiocytosis e pili ana i nā kumu he nui .

• Pehea ka koʻikoʻi o kou mau hōʻailona .
• Inā he hoʻokahi a he nui paha kou lesion (ʻo kēia nā wahi o nā ʻiʻo ʻē).
• Ma hea ma ke kino e loaʻa ai kēia mau "ʻeha" ?
• Inā he maʻi kanesa kēia mau puʻupuʻu a ʻaʻole paha .

Hoʻoholo nā kauka i ka lāʻau lapaʻau ma muli o kēia mau kumu. Eia kekahi mau koho lapaʻau:

• E nānā a kali : Inā ʻaʻole koʻikoʻi kāu histiocytosis a ʻaʻohe ou hōʻailona nui, hiki i kāu kauka ke haʻi aku iā ʻoe e nānā i kou kūlana.
• ʻoki kinoInā ua hōʻiliʻili nā pūnaewele histiocyte ma hoʻokahi wale nō ʻāpana o ke kino, hiki ke hana ʻia ke ʻoki kino e wehe i kēlā ʻāpana.
• Lapaʻau radiation : Pili kēia i ka hoʻohana ʻana i kahi mīkini e kuhikuhi i nā kukuna ikehu kiʻekiʻe o ka radiation i nā ʻeha. Pepehi kēia mau kukuna i nā cell maʻamau ʻole a hoʻolohi i ka ulu ʻana o nā puʻupuʻu. I kekahi manawa hiki i ka radiation therapy ke kōkua i ka hōʻemi ʻana i nā hōʻailona.
• ʻO Chemotherapy (i kapa ʻia hoʻi ʻo 'chemo') : Pili kēia i ka hoʻouna ʻana i nā kemika i loko o kou koko e luku i nā hunaola histiocyte keu i laha ma kou kino. Paipai wale nā ​​kauka i ka 'chemo' inā ua laha nā hunaola histiocyte ma kou kino.
• Corticosteroids : Hāʻule nā ​​​​lāʻau lapaʻau e like me ka prednisone i loko o kēia māhele. Hiki i kēia mau mea ke hōʻemi i ka mumū (pehu) e hele pū me ka histiocytosis. Hiki ke hāʻawi wale ʻia kēia mau lāʻau lapaʻau a i ʻole me ka hui pū ʻana me nā lāʻau lapaʻau ʻē aʻe, e like me ka chemotherapy.
• Immunotherapy : Hana kēia ma ka hoʻoikaika ʻana i kāu ʻōnaehana pale ponoʻī, e kōkua ana iā ia e ʻike a luku i nā ʻeha ʻino, e like me nā histiocytes maʻamau.
• Lapaʻau i kuhikuhi ʻia : He lāʻau lapaʻau kūikawā kēia. Hoʻohana ʻia kēia lāʻau lapaʻau no nā kūlana histiocytosis e pili ana me kekahi mau hoʻololi genetic.

Hiki ke ho'ōla loa ʻia ka Histiocytosis?

He paʻakikī ke ʻōlelo 'ʻae' a 'ʻaʻole' paha i kēia, no ka mea, pili ia i ke kūlana. Nui ka poʻe me ka histiocytosis e komo i ka mea i kapa ʻia ʻo 'durable remission.' ʻO ia hoʻi, hele lākou me ka ʻole o nā hōʻailona a i ʻole nā ​​​​hōʻailona o ka maʻi.

Hiki ke ho'ōla piha ʻia kekahi mau ʻano histiocytosis, ʻoiai inā aia nā histiocytes ma kahi hoʻokahi wale nō o ke kino, me ke ʻoki kino . E hāʻawi kāu kauka iā ʻoe i nā kipa hahai e nānā i nā hana hou.

Eia nō naʻe, inā ʻaʻole hiki ke ho'ōla loa ʻia ke ʻano o ka histiocytosis āu, e paipai kāu kauka i kahi hoʻolālā lapaʻau e hiki ke kōkua iā ʻoe e mālama i ke kūlana.

He aha ka wānana no kēia kūlana?

ʻO ka wānana o kou kūlana e pili ana i nā kumu he nui. No kekahi poʻe, e ola maikaʻi ka histiocytosis ma kāna iho me ka ʻole o ka lāʻau lapaʻau . No ka laʻana, ma kahi o 40% o ka poʻe me RDD e ola maikaʻi me ka ʻole o ka lāʻau lapaʻau. Eia kekahi, ke hoʻopilikia ka LCH i hoʻokahi wale nō ʻāpana o ke kino (kapa mākou iā ia he 'localized LCH') , e ola maikaʻi pinepine ia ma kāna iho.

Akā, i nā hihia ʻē aʻe, pono ka histiocytosis i ka mālama koʻikoʻi a me ka nānā pono ʻana, i nā keiki a me nā pākeke.I nā hihia koʻikoʻi loa, hiki i ka histiocytosis ke hōʻino i nā kino a hiki ke hoʻoweliweli i ke ola .

No laila, ʻoi aku ka maikaʻi o ka nīnau ʻana i kāu kauka e pili ana i ka wānana e pili ana i kāu hōʻailona.

Hiki ke pale ʻia ka Histiocytosis? Pehea e hiki ai ke hoʻēmi ʻia ka pilikia?

ʻOiaʻiʻo, ʻaʻohe ala e pale aku ai i ka histiocytosis, akā hiki i ka lāʻau ke kōkua iā ʻoe e mālama i kāu mau hōʻailona.

Akā, hoʻokahi mea. Hiki i ka haʻalele ʻana i ka puhipaka ke hōʻemi i kou pilikia o ka hoʻomohala ʻana i ka LCH (Langerhans cell histiocytosis), kahi maʻi kanesa māmā. Hiki i ka haʻalele ʻana i ka puhipaka a i ʻole ka noho ʻole ʻana i ka uahi ke hoʻomaikaʻi i kāu pane ʻana i ka lāʻau lapaʻau. Inā makemake ʻoe i ke kōkua no ka haʻalele ʻana i ka puhipaka, e nīnau i kāu kauka e pili ana i nā papahana kuhikuhi no ka haʻalele ʻana i ka puhipaka.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Inā ua ʻike ʻia ʻoe me ka histiocytosis, he mea maʻamau ka loaʻa ʻana o nā nīnau he nui ma kou manaʻo. He mea nui e kamaʻilio me kāu kauka e pili ana i kēia mau mea āpau a loaʻa kahi ʻike maikaʻi o kou kūlana. Eia kekahi mau nīnau hiki iā ʻoe ke nīnau:

• He aha ke ʻano o ka histiocytosis iaʻu?
• E pono anei iaʻu ka lāʻau lapaʻau?
• He aha nā koho lapaʻau āu e paipai nei?
• He aha nā hopena ʻaoʻao e hiki ke manaʻo ʻia i ka wā o ka mālama ʻana?
• He aha nā hopena e hiki ke loaʻa mai ka lāʻau lapaʻau?
• Hiki ke ho'ōla loa ʻia koʻu maʻi?

Leka Hoʻihoʻi Hope Loa

I ka ʻōlelo maʻalahi, ʻo ka histiocytosis ka inoa i hāʻawi ʻia i kahi hui o nā maʻi koko kakaikahi i hoʻokumu ʻia e ka hana nui ʻana o nā histiocytes, kahi ʻano o nā cell koko keʻokeʻo, i loko o nā ʻiʻo. Hiki i kekahi o kēia mau mea ke maʻalahi loa, me ka ʻole o nā hōʻailona, ​​​​aʻo kekahi hiki ke koʻikoʻi a hiki ke hoʻoweliweli i ke ola .

ʻO kēlā me kēia kanaka me ka histiocytosis he nui nā histiocytes i loko o ko lākou mau ʻiʻo, e hoʻoulu ai i ka mumū (pehu). Eia nō naʻe, hiki ke ʻokoʻa loa nā hōʻailona, ​​​​nā lāʻau lapaʻau, a me ka hopena o ka maʻi, ʻoiai ma waena o ka poʻe me ke ʻano like o ka histiocytosis .

ʻO kāu kauka ke kanaka maikaʻi loa e hoʻomaopopo i kāu hōʻailona a me nā kūlana kūikawā e pili ana iā ia. No laila, no ka hoʻomaopopo piha ʻana i kāu hōʻailona a me ka hoʻolālā lapaʻau, mai makaʻu e nīnau i nā nīnau. E kamaʻilio me kāu kauka e pili ana i nā mea āpau ma kou manaʻo.

ʻO Histiocytosis, Nā hunaola koko keʻokeʻo, Nā Histiocytes, ʻŌnaehana pale, LCH, ECD, RDD, Nā hōʻailona, ​​Lapaʻau