Histiocytosis

ʻO Histiocytosis kahi inoa maʻamau no kahi hui o nā maʻi a i ʻole "syndrome" e pili ana i ka piʻi maʻamau o ka helu o nā hunaola keʻokeʻo kūikawā i kapa ʻia ʻo histiocytes.

I kēia mau lā, ua alakaʻi ka ʻike hou e pili ana i kēia ʻohana o nā maʻi i nā loea e hoʻomohala i kahi ʻano hou. Ua hāʻawi ʻia ʻelima mau ʻano:

• L pūʻulu - e pili pū ana me ka histangercytosis pūnaewele ʻo Langerhans a me ka maʻi ʻo Erdheim-Chester
• Pūʻulu C - pili pū me nā non-Langerhans cell histiocytosis e pili ana i ka ʻili
• M pūʻulu - e pili pū me ka histiocytosis malignant
• Pūʻulu R - e komo pū me ka maʻi ʻo Rosai-Dorfman
• Pūʻulu H - e pili pū ana me ka lymphohistiocytosis hemophagocytic

Kuhi wale kēia ʻatikala i ka hui L, kahi e pili ana iā Langerhans cell histiocytosis a me ka maʻi ʻo Erdheim-Chester.

Ua hoʻopaʻapaʻa ʻia inā ʻo Langerhans cell histiocytosis a me ka maʻi ʻo Erdheim-Chester he mumū, nā maʻi pale, a me nā ʻano like ʻole me ka maʻi ʻaʻai. I kēia mau lā, ma o ka hoʻohana ʻia ʻana o nā genomics ua ʻike nā ʻepekema i kēia mau ʻano o ka histiocytosis e hōʻike ai i nā loli (ke hoʻololi) ʻana o nā hunaola keʻokeʻo mua. ʻO kēia ke alakaʻi i ka hana maʻamau ʻole i loko o nā hunaola. Hoʻonui ʻia nā hunaola ʻino i nā ʻāpana like ʻole o ke kino e like me nā iwi, ka ʻili, nā akemāmā, a me nā wahi ʻē aʻe.

He maʻi maʻi laha ʻole ʻo Langerhans cell histiocytosis i hiki ke hoʻopili i ka poʻe o nā makahiki āpau. Aia ke kiʻekiʻena kiʻekiʻe ma waena o nā keiki o 5 a 10. ʻO kekahi ʻano o ka maʻi he genetical, ʻo ia hoʻi ua hoʻoilina lākou.

ʻO ka maʻi ʻo Erdheim-Chester kahi ʻano laha ʻole o ka histiocytosis e hoʻopili ana i nā mākua e pili ana i nā ʻāpana he nui o ke kino.

Hiki i nā maʻi Langerhans cell histiocytosis a me ka maʻi Erdheim-Chester ke hoʻopili i ke kino holoʻokoʻa (maʻi ʻōnaehana).

Hiki ke hoʻololi i nā ʻōuli ma waena o nā keiki a me nā mākua, akā loaʻa paha iā lākou kekahi o nā ʻōuli like.ʻO nā tumors i loko o nā iwi kaumaha, e like me nā wāwae a i ʻole ka iwi kuamoʻo, e hoʻonā i nā iwi me ka ʻole o ke kumu maopopo.

Pākuʻi ʻia paha nā ʻōuli i nā keiki:

• Abdominal eha
• ʻEha iwi
• Hoopaneeia ka manawa makua
• Puʻupuʻu
• Kahe ka pepeiao e hoʻomau i ka wā lōʻihi
• ʻO nā maka i ʻike ʻia e pipili hou aku
• Huhū
• Ka holomua ʻole ʻana
• Fiva
• Urination pinepine
• ʻEha poʻo
• ʻO Jaundice
• Limping
• Hoʻoiho noʻonoʻo
• Rash
• Dermatitis Seborrheic o ka ʻili
• Ka hopu ʻana i nā maʻi
• Kūlana pōkole
• Liʻiliʻi kelepaʻi
• Ka makewai
• ʻO ka luaʻi
• Kaumaha emi

Kahakaha: ʻO nā keiki ma mua o 5 mau makahiki i ka hoʻopili pinepine ʻana i ka iwi.

Pākuʻi ʻia paha nā ʻōuli i nā mākua:

• ʻEha iwi
• ʻEha ʻeha
• Cough
• Fiva
• General discomfort, uneasiness, a i ʻole maʻi manaʻo
• Hoʻonui i ka nui o ka mimi
• Rash
• Pokole o ka hanu
• Ka make wai a hoʻonui i ka inu ʻana i nā wai
• Kaumaha emi

ʻAʻohe o nā hoʻokolohua koko kikoʻī no ka Langerhans cell histiocytosis a i ʻole ka maʻi Erdheim-Chester. Hoʻokumu nā tumors i kahi nānā "kuʻi ʻia" ma kahi iwi x-iwi. Hoʻololi nā hoʻokolohua kikoʻī, i ka makahiki o ke kanaka.

Hoʻokomo ʻia paha nā hoʻāʻo no nā keiki:

• ʻO Biopsy o ka ʻili e nānā ai i nā pūnaewele Langerhans
• ʻO ka biopsy o ka iwi iwi e nānā ai i nā keena ʻo Langerhans
• Helu piha koko (CBC)
• ʻO nā kukuna X o nā iwi āpau i ke kino e ʻike i ka nui o nā iwi i hoʻopili ʻia
• Hōʻike no kahi hoʻololi kūʻē i BRAF V600E

Hoʻokomo pū ʻia nā hoʻāʻo no nā mākua:

• ʻO Biopsy o kekahi tumo a nui paha
• Ke kiʻi ʻana o ke kino, e laʻa me x-ray, CT scan, MRI, a i ʻole scan PET
• ʻO Bronchoscopy me ka biopsy
• Nā hoʻāʻo hana pulmonary
• ʻO ka hoʻāʻo ʻana i ke koko a me nā mea no nā hoʻololi kūʻē e like me BRAF V600E. Pono paha e hana i kēia hoʻokolohua ma kahi kikowaena kūikawā.

Hoʻohui ʻia ʻo Langerhans cell histiocytosis i kekahi manawa me ka maʻi ʻaʻai. Pono e nānā i ka CT scans a me ka biopsy e kāpae i ka maʻi ʻaʻai.

ʻO nā kānaka me ka Langerhans cell histiocytosis e hoʻopili wale ana i hoʻokahi wahi (e like me ka iwi a me ka ʻili) hiki ke mālama ʻia me ke kaila o ka wahi. Eia nō naʻe, pono lākou e ukali pono ʻia e nānā i nā hōʻailona hōʻailona ua hoʻolaha ʻia ka maʻi.

ʻO ka poʻe me ka langerhans cell histiocytosis a i ʻole Erdheim-Chester maʻi e koi i nā lāʻau e hōʻemi i nā ʻōuli a kaohi i ka hoʻolaha ʻana o ka maʻi. Hōʻike nā noiʻi hou e kokoke ana i nā mākua āpau me ka histiocytosis ākea i loaʻa i nā mutation gen i loko o nā tumors, kahi e kū nei i kēia maʻi. Loaʻa nā lāʻau e kāohi i kēia mau hoʻololi gen, e like me vemurafenib i kēia manawa. ʻO nā lāʻau ʻē aʻe e like me ka hoʻomohala.

ʻO nā maʻi maʻi ʻo Langerhans cell histiocytosis a me ka maʻi ʻo Erdheim-Chester. No laila aia ka palena o ka ʻike e pili ana i ke ʻano ʻoi loa o ka lāʻau. Makemake paha ka poʻe me kēia ʻano e komo i loko o nā hoʻokolohua hoʻokolohua hoʻomau ʻia i hoʻolālā ʻia e ʻike i nā ʻano lāʻau hou.

Hiki ke hoʻohana ʻia i nā lāʻau ʻē aʻe a i ʻole nā ​​lāʻau lapaʻau, ke kaukaʻi ʻia i ka nānā (prognosis) a me ka pane i nā lāʻau hoʻomaka. Hiki ke hoʻopili ʻia kēlā ʻano mālama ʻana:

• Interferon alpha
• Cyclophosphamide a i ʻole vinblastine
• ʻEtoposide
• Methotrexate
• Vemurafenib, inā loaʻa ka mutation BRAF V600E
• ʻO ke kanu ʻana o ka paʻa o ke aʻa

Hiki i nā mālama ʻē aʻe ke:

• Nā Antibiotics e hakakā i nā maʻi
• Kākoʻo hanu (me ka mīkini hanu)
• Hormone replacement therapy
• Hoʻoikaika kino
• Nā shampo kūikawā no nā pilikia o ke poʻo
• Mālama kōkua (i kapa ʻia hoʻi ka mālama hōʻoluʻolu) e hōʻoluʻolu i nā ʻōuli

Hoʻohui ʻia, paipai ʻia ka poʻe me kēia ʻano e puhi i ka puhihi ma muli o ka puhi ʻana paha o ka puhaka ʻana i ka pane i ka lāʻau.

ʻAhahui Histiocytosis www.histio.org

Hiki i ka langerhans cell histiocytosis a me ka maʻi ʻo Erdheim-Chester ke hoʻopili i nā kino he nui a hiki i ka make.

Ma kahi o ka hapalua o ka poʻe me ka pulmonary histiocytosis e hoʻomaikaʻi ai, ʻoiai e lilo mau ana ka lilo o ka hana māmā i ka manawa.

I ka poʻe ʻōpio loa, hilinaʻi ka maka i ka histiocytosis kikoʻī a me kona koʻikoʻi. Hiki i kekahi mau keiki ke ola i kahi ola maʻamau me ka hoʻopili liʻiliʻi ʻana o ka maʻi, ʻo ka maikaʻi ʻole o kekahi. ʻO nā keiki ʻōpio, ʻo nā keiki ʻē aʻe, he mau ʻōuli ākea ke kino i hiki i ka make.

Komo pū nā pilikia:

• Diffuse interstitial pulmonary fibrosis (hohonu nāʻaʻaʻa o ke aʻa e lilo i wela a laila ʻino)
• Pahu hāhā kūlohelohe

E ulu paha nā keiki:

• Hoʻokumu ʻia ka anemia e ka hoʻolaha ʻana o nā tumors i ka iwi iwi
• Insipidus diabetes
• Nā pilikia o ka lepa e hiki ai i ka maikaʻi ʻole o ka maʻi
• Nā pilikia me ka pituitary gland e alakaʻi i ka ulu ʻole ʻana

Kāhea i kāu kahu mālama ola inā ʻoe a ʻo kāu keiki paha nā ʻōuli o kēia maʻi. E hele i ka lumi pilikia inā ulu ka hanu a i ʻole ka ʻeha o ka umauma.

Pale i ka puhipaka. Hiki i ka haʻalele ʻana i ka puhipaka ke hoʻomaikaʻi i ka hopena i ka poʻe me ka Langerhans cell histiocytosis e hoʻopili ana i nā māmā.

ʻAʻole ʻike ʻia ka pale ʻana i kēia maʻi.

Langerhans cell histiocytosis; ʻO ka maʻi ʻo Erdheim-Chester

• Eosinophilic granuloma - x-ray o ka iwi poʻo
• ʻŌnaehana hanu

ʻO Goyal G, ʻo Young JR, ʻo Koster MJ, et al. ʻO ka manaʻo kūkākūkā a ka Mayo Clinic Histiocytosis Working Group no ke kuhi a me ka loiloi ʻana i nā poʻe maʻi me nā neoplasms histiocytic: ka maʻi ʻo Erdheim-Chester, ka histiocytosis cell Langerhans, a me ka maʻi ʻo Rosai-Dorfman. ʻO Mayo Clin Proc. 2019; 94 (10): 2054-2071. PMID: 31472931 pubmed.ncbi.nlm.nih.gov/31472931/.

ʻO Rollins BJ, Berliner N. Histiocytoses. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 160.