Langerhans cell histiocytosis: Sanadin kuma Jiyya

Langerhans Kwayoyin - farin jini Kwayoyin cewa wani bangare ne na rigakafi da kuma tsarin a al'ada yanayi taka muhimmiyar rawa a kare jiki da ƙwayoyin cuta, kwayoyin cuta da sauran cutarwa kwayoyin. An same su a cikin fata, Lymph nodes, saifa, bargo da kuma huhu.

Mene ne Langerhans cell histiocytosis?

A wannan cuta, da aka fi sani da histiocytosis X, Langerhan Kwayoyin fara radiyo a pathological sikelin. Maimakon kare jiki, manyan yawa na wadannan Kwayoyin shafi mutuncin tsokoki da kuma ko da hallaka su. Mafi sau da yawa sha daga kasũsuwa, ashe, huhu da kuma hanta. Ko da yake wannan cell yaduwa yana da yawa a cikin na kowa da ciwon daji, mafi masu bincike ba su yi ĩmãni histiocytosis X nau'i na cutar daji. Babban bambance alama a wannan mahallin, gane da al'ada tsarin m Kwayoyin da kawai matsala ne su kara girma. A halin yanzu, Langerhans cell histiocytosis (a cikin yara, shi ya kamata a lura, shi ne fiye da na kowa, fiye da manya) da aka gane cuta na rigakafi da tsarin, inda rigakafi Kwayoyin ninka ma da sauri da kuma haifar da ciwon kumburi, da lalacewar da kewaye nama.

Wannan cuta ne su iya ci gaba a cikin wani sashi na jiki da kuma a cikin wani sashin jiki. Yana iya ma faruwa a dama daban-daban yankunan lokaci guda. A mafi yawan lokuta, duk da haka, Pathology rinjayar mahara tsarin na jiki kawai a yara a karkashin shekaru biyu da haihuwa. Kadaitaccen raunuka da ake samu a da yara da manya.

dalilai

Shi ne har yanzu ba a sani ba me ya sa akwai irin wannan Pathology. Duk da haka, masana kimiyya sun mayar da hankali a kan tabbaci da wadannan jarrabawa: mafi m wani irin talakawa al'amarin a cikin yanayi, yiwu a kwayar cutar cewa ba impetus zuwa cin gaban cuta haddasa ɓarna, m dauki ta hanyar tsarin na rigakafi. Duk da haka, da kai tsaye tushen da cutar a wannan lokacin batun ganewa. Daya sani kallo ne ban sha'awa: kusan dukan marasa lafiya tare da sarrafa cuta a cikin huhu ne aiki ko tsohon taba smokers. Duk da haka, ko da idan da taro rarraba shan taba a cikin al'umma ne sosai rare Langerhans histiocytosis. A dalilai sun hada da yin amfani da wutar taba sigari a cikin jerin hadarin dalilai kamar ba, domin shi ne sau da yawa wani rashin lafiya da kuma ba ya shafar cikin huhu. A mafi m jarrabawa ne cewa illar da shan taba gane yaduwa na Langerhans Kwayoyin a marasa lafiya tare da wani rare genetically m predisposition zuwa gare shi. A cewar wani ka'idar, da tushen cutar da yake a cikin m rigakafi da tsarin cuta.

baza

Dauke da wani ciwo ne rare da ya auku a game da wanda idan na 250 dubu yara da kuma daya hali da miliyan adult. Ko da yake bayyanar cututtuka na iya faruwa a kowane zamani, shi mafi sau da yawa bincikar lafiya a yara da kuma matasa, da kuma 70% na lokuta faruwa a gano histiocytosis shekaru 17 zuwa shekaru. A ƙarami shekaru rukuni na marasa lafiya, statistics tashi a shekaru daga daya zuwa shekara uku.

nau'i na cutar

Langerhans cell histiocytosis a yara da kuma manya shafi 3 siffofin baya dauke m pathologies. Yana eosinophilic granuloma, Henda cuta - Shyullera - Kirista cutar da kuma Letterer - Siwa.

cututtuka

Cutar na iya bayyana kanta ta da wadannan siffofin:

• m zafi da kumburi a cikin kasusuwa, musamman a damtsenka ko kafa.
• a crack a cikin kashi, musamman idan aka ba wadda ta gabãta daga wani sirri rauni ko wadda ta gabãta daga wani haske, kadan duka.
• Manyan gibba tsakanin hakora a lokuta inda Langerhans cell histiocytosis a yara ya watsu, ƙashi na daga cikin muƙamuƙi.
• kunne kamuwa da cuta, ko kunne sallama, idan cutar ya shafa, ƙashi na daga cikin kwanyar kusa da kunne.
• fata rash preferentially sarrafa a gindi ko fatar kan mutum;
• lymphadenopathy.
• hanta kara girma, wanda a wasu lokuta na iya a tare da ãyõyin Gabar tabarbarewa (yellowing na fata da kuma idanu (jaundice) ko pathological jari na ruwa a cikin ciki - ascites).
• exophthalmos - hijirar na cikin eyeball gaba saboda histiocytosis da sarrafawa da idanu.
• shortness na numfashi da kuma tari, huhu da raunuka.
• jinkirin girma da kuma wuce kima urination saboda hormonal rashin daidaituwa.

Kadan na kowa bayyanar cututtuka da cutar ne:

• zazzabi.
• nauyi asara.
• irritability.
• rashin iyawa rike da nauyi da kuma ci gaba da jiki a siffar.

bincikowa da

Tun Langerhans cell histiocytosis (Photo manifestations da wannan cuta ba a kyawawan gani) ne quite rare da aka bayyana ta bayyanar cututtuka da suke sauƙi kuskure ga cututtuka da dama sauran cututtuka, ganewar asali sau da yawa ne hadaddun da daukan lokaci. Wannan shi ne dalilin da ya sa wani likita zai iya tambaye ku tambayoyi da suka shafi sauran cututtuka zuwa more yadda ya kamata bambance da nufin histiocytosis.

A primary bincike bincike ne mai al'ada jiki jarrabawa, a cikin abin da likita assesses da sauran kiwon lafiya da kuma biya musamman da hankali ga wadanda wuraren da bayyanar cututtuka na cuta ya bayyana. Idan haƙuri abubuwan da zafi a cikin kasusuwa, ko tanã kai ƙãra na busa a cikin kashi, likita zai iya rubũta wani misali X-haskoki da kashi scintigraphy. X-haskoki bayyanãwa karaya rabo kira lytic raunuka, da kuma scintigraphy zai ƙayyade yanki na rauni inda kashi da kansa yayi kokarin warke.

Hanyar lissafta Tomography (CT) amfani a cikin binciken na mayar da kafafuwa a lokuta inda likita ake zargi da cewa shi ne, akwai iya zama Aljihuna na hallakaswa canje-canje. Lokacin da cututtuka nuni da hanta tabarbarewa, za bukatar jini gwaje-gwaje. Idan akwai ãyõyi na huhu lalacewa, ko a lokuta inda wani gwani a kan tushen farko likita jarrabawa ake zargi da cewa Langerhans cell histiocytosis iya nuna shi a cikin huhu, rubũta X-haskoki ko lissafta tomography daga cikin kirji. CT scan na shugaban yi tantance jihar da kwakwalwa. A sabon visualizing fasahar, so (positron watsi tomography) kuma iya zama da amfani.

Idan ka zargin wani Langerhans cell histiocytosis a yara (ganin hotuna. A farkon labarin) biopsy na iya zama wajibi, kamar yadda wannan shine kadai hanyar tabbatar da presumptive ganewar asali. A wannan hanya, likita ta kawar da kananan samfurin na nama ko kashi da za a yi karatu a cikin dakin gwaje-gwaje. A mafi yawan lokuta, wani samfuri na nazarin halittu abu ne cirewa daga yankunan inda kai tsaye sarrafa Langerhans cell histiocytosis: kasũsuwa, ashe, fata da kuma Lymph nodes. Jini gwaje-gwaje iya taimaka ƙayyade ko da bargo da aka shafa.

duration

Yana da wuya a hango ko hasashen yadda sauri da cutar da kuma yadda shi zai bayyana kanta. A wasu lokuta, da Pathology vuya da kanta da kuma ba ya bukatar likita shiga tsakani. Wannan na faruwa a lokacin da take hakkin da aka sarrafa a daya yankin (a cikin kashi ko fata), ko a lokacin da wani smoker tare da huhu lalacewar ceases zuwa cinye taba. Duk da haka, shi ma ya faru da cewa cutar ne m. mutuwa za a iya annabta a cikin waɗannan lokuta idan jari na abnormally proliferating Kwayoyin suna kai hare hare a lokaci guda dama daban-daban gabobin ko sassan jiki. A jihar kiwon lafiya na smoker fara inganta ne kawai a lokacin da quitting.

rigakafin

Tun cikin hanyar keta da ya rage wani asiri, don haka yanzu babu wani tasiri rigakafin matakan. Duk da haka, ba abin da huhu shafa kawai a magoya hayaki taba sigari, bada har wani buri za a iya gani a matsayin m histiocytosis sarrafa a cikin huhu.

magani

Idan haƙuri yana da wani tabbatar ganewar asali "histiocytosis na Langerhans Kwayoyin", magani zai dogara ne a kan mai tsanani da kuma har na saba.

Idan abnormality aka gano a cikin daya kawai tsarin na jiki, likita zai rubũta corticosteroids, musamman "Prednisolone". Bugu da ƙari, za ka iya yi sha a hanya na jiyyar cutar sankara. Bugu da kari, ya shafa yankin na kashi cire by curettage - scraping. Idan pathologically accrescent Kwayoyin suna mayar da hankali a daya yankin na fata, da cutar za a iya warkar da rufe da abin ya shafa yankin nitrogen mustard - jiyyar cutar sankara wakili. Haka kuma an yi amfani da "Methotrexate". Radiotherapy aka gudanar a lokuta inda kashi cuta (msl, a cikin sama da kafa ko a cikin kashin baya) ya kai ga weakening na dukan kwarangwal gutsure. Musculo-roba tiyata a kashin baya - Fusion - An bada shawarar lokacin da kashi rashin zaman lafiya a cikin mahaifa yankin.

Jimlace (na tsari) Langerhans cell histiocytosis - mai yawa fiye da tsanani yanayin. Domin lura da jiyyar cutar sankara kuma za a iya amfani, duk da haka, ingancinta ne da yawa rage idan aka kwatanta da far da guda rauni. Ko da yake masana nazari da tasiri na da yawa da kwayoyi, mafi kyau duka magani ya ba tukuna aka samu. Duk da haka, domin a saka idanu da ci gaban da cuta your likita iya rubũta daya daga cikin wadannan hanyoyi:

• "Vinblastine".
• "Etoposide".
• "Mercaptopurine".
• "Cladribine".
• "Cytarabine".
• "Methotrexate".

A wasu lokuta, likitoci bayar da shawarar hanta dasawa, bargo, ko kuma huhu, amma tsakanin masu bincike yarjejeniya a kan mafi kyau duka Gabar dasawa a marasa lafiya tare da histiocytosis ya ba tukuna aka kai.

Lokacin da ganin likita?

Tabbata a tuntube ka farko kula pediatrician idan ka zargin Langerhans cell histiocytosis a yara. Ocular bayyanar cututtuka, zafi ko busa a cikin kashi, kullum fata rashes ne bayyanar cututtuka na wannan rare cuta. Idan wani daga cikin wadannan cututtuka ko Pathology a manya kamata shawarci likitan.