Tarihin tarihi

Histiocytosis suna ne na gaba ɗaya don ƙungiyar rikice-rikice ko "ɓarkewar cuta" waɗanda ke ƙunshe da ƙarancin haɗari cikin adadin ƙwayoyin farin jini na musamman waɗanda ake kira histiocytes.

Kwanan nan, sabon ilimin game da wannan dangin cututtukan ya sa masana suka haɓaka sabon tsari. An gabatar da rukuni biyar:

• L rukuni - ya haɗa da tarihin Langerhans cell histiocytosis da cutar Erdheim-Chester
• Cungiyar C - sun haɗa da tarihin ba-Langerhans cell histiocytosis wanda ya shafi fata
• Groupungiyar M - ya haɗa da mummunan tarihin tarihi
• R rukuni - ya haɗa da cutar Rosai-Dorfman
• H Rukuni - ya hada da hemophagocytic lymphohistiocytosis

Wannan labarin yana mai da hankali ne kawai ga ƙungiyar L, wanda ya haɗa da tarihin Langerhans cell histiocytosis da cutar Erdheim-Chester.

An yi ta muhawara game da ko tarihin Langerhans cell histiocytosis da cutar Erdheim-Chester suna da kumburi, rikicewar rigakafi, ko yanayin kama-da-kankara. Kwanan nan, ta hanyar amfani da ilimin kimiyyar kwayoyin halitta sun gano cewa waɗannan nau'ikan histiocytosis suna nuna canjin kwayoyi (maye gurbi) a cikin fararen ƙwayoyin jini na farko. Wannan yana haifar da halayyar al'ada a cikin ƙwayoyin. Kwayoyin da ba na al'ada ba suna ƙaruwa a sassa daban-daban na jiki waɗanda suka haɗa da ƙasusuwa, fata, huhu, da sauran yankuna.

Langerhans cell histiocytosis cuta ce mai saurin gaske wacce ke iya shafar mutane na kowane zamani. Matsakaicin mafi girma shine tsakanin yara masu shekaru 5 zuwa 10. Wasu nau'ikan cutar suna haifar da kwayar halitta, wanda ke nufin an gaji su.

Cutar Erdheim-Chester nau'ikan ilimin tarihi ne wanda ke shafar manya musamman waɗanda suka shafi ɓangarorin jiki da yawa.

Dukkanin kwayar cutar Langerhans cell histiocytosis da cutar Erdheim-Chester na iya shafar dukkan jiki (rashin tsari).

Kwayar cutar na iya bambanta tsakanin yara da manya, amma suna iya samun wasu alamun alamun iri ɗaya.Tumurai a cikin ƙasusuwa masu ɗaukar nauyi, kamar ƙafa ko ƙashi, na iya haifar da kasusuwa ba tare da wani dalili bayyananne ba.

Kwayar cututtuka a cikin yara na iya haɗawa da:

• Ciwon ciki
• Ciwon ƙashi
• Balaga da aka jinkirta
• Dizziness
• Magudanar kunne wanda ke ci gaba na dogon lokaci
• Idanuwan da suka bayyana suna ƙara fitowa sosai
• Rashin fushi
• Rashin cin nasara
• Zazzaɓi
• Yin fitsari akai-akai
• Ciwon kai
• Jaundice
• Gyarawa
• Rashin hankali
• Rash
• Seborrheic dermatitis na fatar kan mutum
• Kamawa
• Girman jiki
• Kumburin lymph gland
• Ishirwa
• Amai
• Rage nauyi

Lura: Yara sama da shekaru 5 galibi suna samun sa hannun kashi ne kawai.

Kwayar cututtuka a cikin manya na iya haɗawa da:

• Ciwon ƙashi
• Ciwon kirji
• Tari
• Zazzaɓi
• Babban rashin jin daɗi, rashin kwanciyar hankali, ko rashin lafiya
• Yawan fitsari
• Rash
• Rashin numfashi
• Kishirwa da karin shan ruwaye
• Rage nauyi

Babu takamaiman gwajin jini don Langerhans cell histiocytosis ko cutar Erdheim-Chester. Ciwan ya ba da hoton "naushi" a kan x-ray. Takamaiman gwaje-gwaje sun bambanta, ya danganta da shekarun mutumin.

Gwajin ga yara na iya haɗawa da:

• Biopsy na fata don bincika ƙwayoyin Langerhans
• Bincikar kasusuwa don bincika ƙwayoyin Langerhans
• Kammala ƙididdigar jini (CBC)
• X-ray na dukkan kasusuwan dake jikin mutum dan gano yadda kashin yake da tasiri
• Gwaji don maye gurbi a cikin BRAF V600E

Gwaje-gwaje ga manya na iya haɗawa da:

• Biopsy na kowane ƙari ko taro
• Hoto na jiki, gami da x-ray, CT scan, MRI, ko PET scan
• Bronchoscopy tare da biopsy
• Gwajin aikin huhu
• Gwajin jini da nama don maye gurbi ciki har da BRAF V600E. Wannan gwajin na iya buƙatar yin shi a wata cibiya ta musamman.

Langerhans cell histiocytosis wani lokaci yana da alaƙa da cutar kansa. CT scans da biopsy ya kamata a yi don kawar da yiwuwar ciwon daji.

Mutanen da ke da kwayar cutar Langerhans cell histiocytosis wanda ya ƙunshi yanki ɗaya kawai (kamar ƙashi ko fata) za a iya bi da su tare da tiyatar cikin gida. Duk da haka, za a buƙaci a bi su a hankali don neman alamun da ke nuna cewa cutar ta bazu.

Mutanen da ke da kwayar cutar Langerhans cell histiocytosis ko cutar Erdheim-Chester suna buƙatar magunguna don rage alamun da kuma kula da yaduwar cutar. Karatun da aka yi kwanan nan ya nuna cewa kusan dukkanin balagaggen da ke da tarihin tarihi suna da maye gurbi a cikin ciwace-ciwacen daji, wanda yake haifar da cutar. Akwai magunguna a yanzu wadanda ke hana wadannan maye gurbi, kamar su vemurafenib. Sauran kwayoyi irin wannan suma suna cikin cigaba.

Langerhans cell histiocytosis da cutar Erdheim-Chester cuta ce da ba a cika samun ta ba. Sabili da haka akwai iyakantaccen bayani game da mafi kyawun hanyar magani. Mutanen da ke da waɗannan sharuɗɗan na iya so su shiga cikin gwaji na asibiti mai gudana wanda aka tsara don gano sababbin jiyya.

Sauran magunguna ko jiyya na iya amfani dasu, gwargwadon hangen nesa (hangen nesa) da kuma martani ga magungunan farawa. Irin waɗannan jiyya na iya haɗawa da:

• Interferon alpha
• Cyclophosphamide ko vinblastine
• Etoposide
• Samun bayanai
• Vemurafenib, idan an sami maye gurbin BRAF V600E
• Dasa dasa kara

Sauran jiyya na iya haɗawa da:

• Magungunan rigakafi don yaƙi da cututtuka
• Tallafin numfashi (tare da injin numfashi)
• Maganin maye gurbin Hormone
• Jiki na jiki
• Shampoos na musamman don matsalolin fatar kai
• Taimako na tallafi (wanda ake kira kulawa ta'aziyya) don taimakawa bayyanar cututtuka

Bugu da ƙari, ana ƙarfafa mutanen da ke da waɗannan yanayin waɗanda ke shan sigari su daina tunda shan sigari na iya tsananta amsar magani.

Histungiyar Tarihin Tarihi www.histio.org

Langerhans cell histiocytosis da cutar Erdheim-Chester na iya shafar gabobi da yawa kuma zai iya haifar da mutuwa.

Kimanin rabin rabin waɗanda ke fama da cutar tarihin huhu na inganta, yayin da wasu ke da asarar huhu na dindindin a kan lokaci.

A cikin samari da yawa, hangen nesa ya dogara da takamaiman tarihin da kuma yadda yake da tsanani. Wasu yara na iya rayuwa ta yau da kullun tare da ƙarancin sa hannun cuta, yayin da wasu ke talaucewa. Childrenananan yara, musamman ma jarirai, suna da alamun kamuwa da dukkanin jiki wanda ke haifar da mutuwa.

Matsaloli na iya haɗawa da:

• Yada Fibrosis na huhu daga ciki (zurfin huhun huhu wanda ya zama mai kumburi sannan ya lalace)
• Bazato ba tsammani ya fadi huhu

Hakanan yara na iya haɓaka:

• Anaemia ta haifar da yaduwar ciwace-ciwacen zuwa ƙashi
• Ciwon sukari insipidus
• Matsalar huhu da ke haifar da gazawar huhu
• Matsaloli tare da gland shine yake haifar da gazawar ci gaba

Kira mai ba da sabis na kiwon lafiya idan ku ko yaranku suna da alamun wannan matsalar. Jeka dakin gaggawa idan numfashin ka ko kuma ciwon kirji ya taso.

Guji shan taba. Dakatar da shan sigari na iya inganta sakamako ga mutanen da ke da kwayar cutar Langerhans cell histiocytosis wacce ke shafar huhu.

Babu sanannun rigakafin wannan cuta.

Langerhans cell histiocytosis; Cutar Erdheim-Chester

• Eosinophilic granuloma - x-ray na kwanyar
• Tsarin numfashi

Goyal G, Young JR, Koster MJ, et al. Bayanin yarjejeniya na Mayo Clinic Histiocytosis Working Group game da ganewar asali da kimantawa ga marasa lafiyar manya da cututtukan tarihi: Erdheim-Chester cuta, Langerhans cell histiocytosis, da cutar Rosai-Dorfman. Mayo Clin Proc. 2019; 94 (10): 2054-2071. PMID: 31472931 pubmed.ncbi.nlm.nih.gov/31472931/.

Rollins BJ, Berliner N. Tarihin tarihi. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 26th ed. Philadelphia, PA: Elsevier; 2020: babi na 160.