Histiocytosis

Histiocytosis ke lebitso le akaretsang la sehlopha sa mathata kapa "syndromes" se kenyelletsang keketseho e sa tloaelehang ea palo ea lisele tse tšoeu tsa mali tse bitsoang li-histiocyte.

Morao tjena, tsebo e ncha ka lelapa lena la maloetse e entse hore litsebi li hlahise sehlopha se secha. Ho hlahisitsoe mekhahlelo e mehlano:

• L sehlopha - se kenyelletsa Langerhans cell histiocytosis le lefu la Erdheim-Chester
• Sehlopha sa C - se kenyelletsa histiocytosis e seng Langerhans cell e amang letlalo
• Sehlopha sa M - se kenyelletsa histiocytosis e kotsi
• R sehlopha - se kenyelletsa lefu la Rosai-Dorfman
• H Group - e kenyelletsa hemophagocytic lymphohistiocytosis

Sengoliloeng sena se shebile feela sehlopheng sa L, se kenyeletsang Langerhans cell histiocytosis le lefu la Erdheim-Chester.

Ho bile le ngangisano mabapi le hore na lefu la Langerhans cell histiocytosis le Erdheim-Chester li na le ho ruruha, mathata a 'mele ea ho itšireletsa mafung kapa maemo a kang a mofets'e. Haufinyane, ka ts'ebeliso ea li-genomics bo-rasaense ba fumane hore mefuta ena ea histiocytosis e bonts'a liphetoho tsa liphatsa tsa lefutso (liphetoho) liseleng tse tšoeu tsa mali tsa pele. Sena se lebisa boitšoarong bo sa tloaelehang liseleng. Lisele tse sa tloaelehang li ea eketseha likarolong tse fapaneng tsa 'mele ho kenyeletsoa masapo, letlalo, matšoafo le libaka tse ling.

Langerhans cell histiocytosis ke lefu le sa tloaelehang le ka amang batho ba lilemo tsohle. Sekhahla se phahameng ka ho fetisisa ke sa bana ba lilemo li 5 ho isa ho tse 10. Mefuta e meng ea bokuli ke lefutso, ho bolelang hore ba futsitse.

Lefu la Erdheim-Chester ke mofuta o sa tloaelehang oa histiocytosis o amang haholo batho ba baholo o kenyang likarolo tse ngata tsa 'mele.

Bobeli Langerhans cell histiocytosis le lefu la Erdheim-Chester li ka ama mmele ohle (systemic disorder).

Matšoao a ka fapana lipakeng tsa bana le batho ba baholo, empa a kanna a ba le matšoao a tšoanang.Lihlahala masapong a jereng boima, joalo ka maoto kapa mokokotlo, li ka etsa hore masapo a robehe ntle le lebaka.

Matšoao ho bana a ka kenyelletsa:

• Bohloko ba mpeng
• Bohloko ba masapo
• Ho lieha ho kena bonneng kapa bosaling
• Botsoa
• Letsoai la tsebe le tsoelang pele nako e telele
• Mahlo a hlahang a kolla koana le koana
• Ho teneha
• Ho hloleha ho atleha
• Feberu
• Ho rota khafetsa
• Ho opeloa ke hlooho
• Jaundice
• Ho hlotsa
• Ho fokotseha ha kelello
• Moferefere
• Seborrheic dermatitis ea letlalo la hlooho
• Ho oa
• Seemo se sekgutshwane
• Ho ruruha litšoelesa tsa lymph
• Nyora
• Ho hlatsa
• Ho fokotsa boima ba 'mele

Tlhokomeliso: Hangata bana ba fetang lilemo tse 5 ba na le karolo feela ea masapo.

Matšoao ho batho ba baholo a ka kenyelletsa:

• Bohloko ba masapo
• Bohloko ba sefuba
• Khohlela
• Feberu
• Ho se utloise bohloko ka kakaretso, ho se phutholohe kapa ho kula
• Eketseha palo ea moroto
• Moferefere
• Phefumoloho e khutšoanyane
• Ho nyoriloe le ho noa metsi a mangata
• Ho fokotsa boima ba 'mele

Ha ho na liteko tse ikhethang tsa mali bakeng sa lefu la Langerhans cell histiocytosis kapa lefu la Erdheim-Chester. Lihlahala li hlahisa ponahalo ea "phunyeletsoeng" ho x-ray ea masapo. Liteko tse ikhethileng lia fapana, ho latela lilemo tsa motho.

Liteko tsa bana li ka kenyelletsa:

• Biopsy ea letlalo ho hlahloba lisele tsa Langerhans
• Bops marrow biopsy ho hlahloba lisele tsa Langerhans
• Palo e felletseng ea mali (CBC)
• X-ray ea masapo 'ohle' meleng ho fumana hore na ke masapo a makae a amehileng
• Teko ea phetoho ea lefutso ho BRAF V600E

Liteko tsa batho ba baholo li ka kenyelletsa:

• Biopsy ea hlahala kapa boima
• Ho nahana ka 'mele, ho kenyelletsa x-ray, CT scan, MRI, kapa PET scan
• Bronchoscopy e nang le biopsy
• Liteko tsa tšebetso ea pulmonary
• Ho hlahlojoa mali le lisele tsa liphatsa tsa lefutso ho kenyelletsa BRAF V600E. Teko ena e kanna ea hloka ho etsoa setsing se ikhethang.

Langerhans cell histiocytosis ka linako tse ling e amahanngoa le mofetše. Lisebelisoa tsa CT le biopsy li lokela ho etsoa ho thibela kankere e ka bang teng.

Batho ba nang le langerhans cell histiocytosis e amang sebaka se le seng feela (joalo ka lesapo kapa letlalo) ba ka phekoloa ka ts'ebetso ea lehae. Leha ho le joalo, li tla hloka ho lateloa haufi-ufi ho batla matšoao a hore lefu lena le nametse.

Batho ba nang le lefu la Langerhans cell histiocytosis kapa lefu la Erdheim-Chester ba hloka meriana ho fokotsa matšoao le ho laola ho ata ha lefu lena. Liphuputso tsa morao-rao li bonts'a hore hoo e ka bang batho bohle ba baholo ba nang le histiocytosis e atileng ba na le liphatsa tsa lefutso lihlahala, tse bonahalang li baka bothata. Meriana e thibelang liphetoho tsena tsa lefutso, joalo ka vemurafenib ea fumaneha hajoale. Lithethefatsi tse ling tse tšoanang le tsona lia nts'etsopele.

Langerhans cell histiocytosis le lefu la Erdheim-Chester ke mathata a sa tloaelehang haholo. Ka hona ho na le tlhaiso-leseling e fokolang ka kalafo e nepahetseng. Batho ba nang le maemo ana ba kanna ba batla ho nka karolo litekong tse tsoelang pele tsa bongaka tse etselitsoeng ho khetholla kalafo e ncha.

Meriana e meng kapa liphekolo li ka sebelisoa, ho ipapisitsoe le chebo ea maikutlo (prognosis) le karabelo ea meriana e qalang. Kalafo e joalo e ka kenyelletsa:

• Alpha ea li-interferon
• Cyclophosphamide kapa vinblastine
• Etoposide
• Methotrexate
• Vemurafenib, haeba phetoho ea BRAF V600E e ka fumanoa
• Ho kenyelletsa sethala sa stem

Mefuta e meng ea kalafo e ka kenyelletsa:

• Lithibela-mafu ho loantša tšoaetso
• Ts'ehetso ea ho hema (ka mochini o phefumolohang)
• Phekolo ea phetoho ea Hormone
• Phekolo ea 'mele
• Li-shampoo tse khethehileng bakeng sa mathata a letlalo
• Tlhokomelo ea tšehetso (eo hape e bitsoang matšeliso) ho kokobetsa matšoao

Ntle le moo, batho ba nang le maemo ana a tsubang ba khothalletsoa ho emisa kaha ho tsuba ho ka mpefatsa karabelo ea kalafo.

Mokhatlo oa Histiocytosis www.histio.org

Langerhans cell histiocytosis le lefu la Erdheim-Chester li ka ama litho tse ngata mme tsa lebisa lefung.

Hoo e ka bang halofo ea ba nang le lefu la methapo ea histiocytosis e ea ntlafala, ha ba bang ba na le tahlehelo e sa feleng ea tšebetso ea matšoafo ka nako.

Ho batho ba bacha haholo, pono e ipapisitse le histiocytosis e ikhethang le hore na e matla hakae. Bana ba bang ba ka phela bophelo bo tloaelehileng ba sa amehe haholo ka mafu, athe ba bang ba phela hampe. Bana ba banyenyane, haholo masea, ba na le menyetla ea ho ba le matšoao a mmele a isang lefung.

Mathata a ka kenyelletsa:

• Ho fokotsa li-pulmonary fibrosis (lisele tse tebileng tsa matšoafo tse chesang le ho senyeha)
• Matšoafo a oeleng ka boithaopo

Bana le bona ba ka nts'etsapele:

• Phokolo ea mali e bakoang ke ho hasana ha lihlahala mokong oa masapo
• Lefu la tsoekere insipidus
• Mathata a matšoafo a lebisang ho hloleha ha matšoafo
• Mathata ka tšoelesa ea pituitary e lebisang ho kholo ea kholo

Bitsa mofani oa tlhokomelo ea bophelo haeba uena kapa ngoana oa hau a e-na le matšoao a lefu lena. E-ea kamoreng ea tšohanyetso haeba phefumoloho e khutšoanyane kapa bohloko ba sefuba bo hlaha.

Qoba ho tsuba. Ho khaotsa ho tsuba ho ka ntlafatsa sephetho ho batho ba nang le Langerhans cell histiocytosis e amang matšoafo.

Ha ho na thibelo e tsebahalang ea lefu lena.

Langerhans cell histiocytosis; Lefu la Erdheim-Chester

• Granuloma ea eosinophilic - x-ray ea lehata
• Tsamaiso ea phefumoloho

Goyal G, JR e monyane, Koster MJ, et al. Polelo ea tumellano ea Mayo Clinic Histiocytosis bakeng sa tlhahlobo le tlhahlobo ea bakuli ba baholo ba nang le li-neoplasms tsa histiocytic: Lefu la Erdheim-Chester, Langerhans cell histiocytosis le lefu la Rosai-Dorfman. Mayo Clin Proc. 2019; 94 (10): 2054-2071 (Khatisitsoeng) PMID: 31472931 e fetotsoe.ncbi.nlm.nih.gov/31472931/.

Rollins BJ, Berliner N. Histiocytoses. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 160.