Histiocytosis

Histiocytosis izita rakajairika reboka rematambudziko kana "syndromes" ayo anosanganisira kuwedzera zvisina kujairika kwenhamba yemasero eropa akachena anonzi histiocytes.

Munguva pfupi yapfuura, ruzivo rutsva nezve iyi mhuri yezvirwere rwatungamira nyanzvi kukudziridza kupatsanura kutsva. Zvikamu zvishanu zvakagadziriswa:

• L boka - rinosanganisira Langerhans cell histiocytosis uye Erdheim-Chester chirwere
• C boka - inosanganisira isiri-Langerhans cell histiocytosis inosanganisira ganda
• M boka - inosanganisira yakaipa histiocytosis
• R boka - rinosanganisira chirwere cheRosai-Dorfman
• H Boka - inosanganisira hemophagocytic lymphohistiocytosis

Ichi chinyorwa chinotarisana chete neboka reL, iro rinosanganisira Langerhans cell histiocytosis uye chirwere cheErdheim-Chester.

Pakave nekukakavadzana pamusoro pekuti Langerhans cell histiocytosis uye Erdheim-Chester chirwere chiri kupisa, kusagadzikana kwezvirwere mumuviri, kana mamiriro akaita segomarara. Munguva pfupi yapfuura, kuburikidza nekushandisa genomics masayendisiti akawana kuti aya marudzi e histiocytosis anoratidza kuchinja kwemajini (mutations) mumasero machena eropa. Izvi zvinotungamira kune zvisina kujairika maitiro mumasero. Iwo maseru asina kujairika anobva awedzera munzvimbo dzakasiyana dzemuviri kusanganisira mapfupa, ganda, mapapu, uye dzimwe nzvimbo.

Langerhans cell histiocytosis chirwere chisingawanzo chinogona kukanganisa vanhu veese mazera. Chiyero chepamusoro chiri pakati pevana vane makore mashanu kusvika pagumi. Dzimwe mhando dzechirwere idiki, izvo zvinoreva kuti vakagara nhaka.

Erdheim-Chester chirwere chisingawanzo chimiro che histiocytosis chinokanganisa kunyanya vanhu vakuru chinosanganisira zvikamu zvakawanda zvemuviri.

Ose ari maviri Langerhans cell histiocytosis uye Erdheim-Chester chirwere chinogona kukanganisa muviri wese (systemic kusagadzikana).

Zviratidzo zvinogona kusiyana pakati pevana nevakuru, asi vanogona kunge vaine zvimwe zvezviratidzo zvakafanana.Tumota mumapfupa ane huremu, senge makumbo kana musana, zvinogona kukonzera kuti mapfupa atsemuke pasina chikonzero chiri pachena.

Zviratidzo muvana zvinogona kusanganisira:

• Marwadzo emudumbu
• Kurwadziwa kwepfupa
• Kunonoka kuyaruka
• Dzungu
• Nzeve drainage inoenderera kwenguva refu
• Maziso anoita kunge anonamira kunze zvakanyanya
• Kushatirwa
• Kutadza kubudirira
• Kupindwa nechando
• Kakawanda weti
• Kutemwa nemusoro
• Jaundice
• Kukamhina
• Pfungwa dzadzikira
• Mapundu
• Seborrheic dermatitis yeganda
• Kufenda
• Pfupi chimiro
• Kuzvimba lymph glands
• Nyota
• Kusvipa
• Kuonda

Ongorora: Vana vanopfuura makore mashanu ekuberekwa vanowanzo kuve nekubatanidzwa kwemapfupa.

Zviratidzo muvakuru zvinogona kusanganisira:

• Kurwadziwa kwepfupa
• Kurwadziwa pachifuva
• Kukosora
• Kupindwa nechando
• Kazhinji kusagadzikana, kusagadzikana, kana kurwara
• Kuwedzera huwandu hweti
• Mapundu
• Kufema kwekufema
• Nyota uye kuwedzera kunwa kwemvura
• Kuonda

Iko hakuna chaiwo kuongororwa kweropa kweLangerhans cell histiocytosis kana Erdheim-Chester chirwere. Iwo mamota anoburitsa "chibhakera-kunze" kutarisa pane pfupa x-ray. Miedzo chaiyo inosiyana, zvinoenderana nezera remunhu.

Kuedzwa kwevana kunogona kusanganisira:

• Biopsy yeganda kuti utarise masero eLangerhans
• Bone marrow biopsy kutarisa masero eLangerhans
• Yakazara kuverenga ropa (CBC)
• X-ray yemapfupa ese mumuviri kuti uone kuti mangani mapfupa akabatwa
• Kwayedza yekuchinja kwemajini muBRAF V600E

Kuedzwa kwevakuru kunogona zvakare kusanganisira:

• Biopsy yechero bundu kana mashoma
• Kufananidza kwemuviri, kusanganisira x-ray, CT scan, MRI, kana PET scan
• Bronchoscopy ine biopsy
• Pulmonary basa bvunzo
• Ropa uye zvinyama kuyedzwa kwejen mutations kusanganisira BRAF V600E. Uku kuyedzwa kungangoda kuitirwa kune yakasarudzika nzvimbo.

Langerhans cell histiocytosis dzimwe nguva inosanganiswa nekenza. CT scans uye biopsy inofanirwa kuitwa kutonga kunze kunogoneka gomarara.

Vanhu vane Langerhans cell histiocytosis iyo inosanganisira chete nzvimbo imwechete (senge pfupa kana ganda) vanogona kurapwa nekuvhiyiwa kwenzvimbo. Zvisinei, ivo vachada kuteverwa zvakanyanya kuti vatarise zviratidzo kuti chirwere chapararira.

Vanhu vane chirwere chakapararira cheLangerhans cell histiocytosis kana chirwere cheErdheim-Chester vanoda mishonga yekuderedza zviratidzo uye kudzora kupararira kwechirwere. Ongororo dzichangobva kuitika dzinoratidza kuti vangangoita vese vanhu vakuru vane kwakapararira histiocytosis vane magene ekuchinja mumamota, ayo anoita kunge anokonzera kusagadzikana. Mishonga inodzivisa aya majini shanduko, senge vemurafenib aripo parizvino. Mimwe mishonga yakafanana iri kusimukira.

Langerhans cell histiocytosis uye Erdheim-Chester chirwere zvakanyanya kusagadzikana kusagadzikana. Naizvozvo pane ruzivo rwakaganhurwa nezve nzira yekurapa yakanakisa. Vanhu vane aya mamiriro vangangoda kutora chikamu mune inoenderera kiriniki miedzo yakagadzirirwa kuona mitsva mishonga.

Mimwe mishonga kana kurapwa kunogona kushandiswa, zvinoenderana nemaonero (kufungidzira) uye kupindura kumishonga yekutanga. Mishonga yakadaro inogona kusanganisira:

• Interferon alpha
• Cyclophosphamide kana vinblastine
• Etoposide
• Methotrexate
• Vemurafenib, kana iyo BRAF V600E shanduko ikawanikwa
• Stem sero kupinza

Mimwe mishonga inogona kusanganisira:

• Mishonga inorwisa mabhakitiriya kurwisa hutachiona
• Kufema rutsigiro (nemuchina wekufema)
• Hormone kutsiva kurapa
• Kurapa kwepanyama
• Shampoo dzakakosha dzematambudziko emusoro
• Kuchengetedza kwekutsigira (kunonziwo nyaradzo) kubvisa zviratidzo

Uye zvakare, vanhu vane aya mamiriro vanosvuta vanokurudzirwa kuti varege sezvo kusvuta kunogona kukuvadza mhinduro kurapa.

Histiocytosis Sangano www.histio.org

Langerhans cell histiocytosis uye Erdheim-Chester chirwere chinogona kukanganisa mitezo yakawanda uye inogona kutungamira kurufu.

Inenge hafu yeavo vane pulmonary histiocytosis inovandudza, nepo vamwe vane zvachose kurasikirwa kwemapapu basa nekufamba kwenguva.

Muvanhu vadiki kwazvo, iwo maonero anoenderana neiyo chaiyo histiocytosis uye kuti yakaoma sei. Vamwe vana vanogona kurarama hupenyu hwakajairika vasingabatanidzwe nezvirwere, asi vamwe vachiita zvisina kunaka. Vana vadiki, kunyanya vacheche, vanowanzove nezviratidzo zvemuviri zvinotungamira kurufu.

Zvinetso zvinogona kusanganisira:

• Kuparadzanisa kupindirana kwepulmonary fibrosis (yakadzika mapapu maturu ayo anopisa uye ndokukuvara)
• Kuzvarwa kwakadonha mapapu

Vana vanogona zvakare kukura:

• Anemia inokonzerwa nekupararira kwemamota kusvika kumongo wemapfupa
• Chirwere cheshuga insipidus
• Matambudziko emapapu anotungamira kukundikana kwemapapu
• Matambudziko nepituitary gland inotungamira mukukundikana kutadza

Fonera wako mupi wezvehutano kana iwe kana mwana wako aine zviratidzo zvechirwere ichi. Enda kuimba yekukurumidzira kana kupfupika kwemhepo kana kurwadziwa pachipfuva kuchikura.

Dzivisa kusvuta. Kurega kusvuta kunogona kuvandudza mhedzisiro muvanhu vane Langerhans cell histiocytosis iyo inokanganisa mapapu.

Iko hakuna kuzivikanwa kudzivirira kwechirwere ichi.

Langerhans cell histiocytosis; Erdheim-Chester chirwere

• Eosinophilic granuloma - x-ray yehenya
• Kufuridzira system

Goyal G, Mudiki JR, Koster MJ, et al. Iyo Mayo Clinic Histiocytosis Working Group chibvumirano chirevo chekuongororwa uye kuongororwa kwevakuru varwere vane histiocytic neoplasms: Erdheim-Chester chirwere, Langerhans cell histiocytosis, uye chirwere cheRosai-Dorfman. Mayo Clin Proc. 2019; 94 (10): 2054-2071. PMID: 31472931 pubmed.ncbi.nlm.nih.gov/31472931/.

Rollins BJ, Berliner N. Nhoroondo. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chits. 160.