Reka twige ku ndwara idakunze kugaragara ituma ubudahangarwa bw'umubiri wawe bukwibasira (Hemophagocytic Lymphohistiocytosis - HLH)

Ese wumva utarigera wumva mbere? Hari igihe ubudahangarwa bwacu bw'umubiri, ari na bwo bukiza umubiri wacu, bushobora gutangira kudutera indwara. Imwe muri izo ndwara zikomeye kandi zidasanzwe ni Hemophagocytic Lymphohistiocytosis, cyangwa (HLH) mu magambo ahinnye. Ikibaho ni uko uturemangingo tumeze nk'ubwirinzi bw'umubiri wacu twiyongera mu buryo butunguranye kandi tukibasira ingingo zacu. Mu by'ukuri, ni nk'umurinzi udashobora kumenya umuryango we.

Mu by'ukuri `(HLH)` bisobanura iki? Reka tubisobanukirwe mu buryo bworoshye, si byo se?

Ubudahangarwa bw'umubiri wacu ni uburyo butangaje cyane. Ni nk'ingabo zirinda igihugu cyacu. Ubu buryo buturinda abanzi nka mikorobe, virusi, na bagiteri zituruka hanze. Uturemangingo twinshi, poroteyine, ingingo, n'uturemangingo birakorana kuri ibi. Ariko, iyo umuntu ufite `(HLH)` arwaye indwara, ubu buryo bw'ubudahangarwa burarushaho gukururwa. Hanyuma, aho kurwanya ubwandu, uturemangingo dushya twavutse dutangira kwibasira umubiri wacu. Ibi bidutera indwara. Mu by'ukuri, ubu buryo `(HLH)` bushobora gushyira ubuzima mu kaga .

Hari ubwoko bubiri bw'ingenzi bw'ibi `(HLH)`, sibyo?

Yego, `(HLH)` ishobora kugabanywamo ubwoko bubiri bw'ingenzi:

1. Iy'ibanze (HLH) (iterwa n'imiterere y'uturemangingo) : Ibi biterwa n'impinduka mu miterere y'uturemangingo. Ibi bivuze ko umuntu avukana imiterere nk'iyo. Ibimenyetso bikunze kugaragara mu myaka mike ya mbere y'ubuzima bwe. Ni gake cyane, ibimenyetso bigaragara nyuma yo gukura. Ibi rimwe na rimwe byitwa "HLH yo mu muryango".

2. Indwara ya kabiri (HLH) (iterwa n'indi ndwara) : Ibi bibaho iyo ubudahangarwa bw'umubiri wawe bugizweho ingaruka n'indi ndwara usanzwe ufite. Urugero, ishobora guterwa n'ibintu nka kanseri, indwara zandurira mu maraso (cyane cyane virusi ya Epstein-Barr), cyangwa indwara ziterwa n'ubwiyongere bw'umubiri. Indwara ya kabiri (HLH) ikunze kugaragara kurusha iy'ibanze (HLH).

Ni nde ufite amahirwe menshi yo kugira iyi `(HLH)`?

`(HLH)` Ishobora kugaragara ku muntu uwo ari we wese w’imyaka iyo ari yo yose. Ariko, ikunze kugaragara ku bana bato n’impinja . Ariko, ibyo ntibivuze ko abantu bakuru badashobora kuyikuramo.

Iki kibazo gikunze kugaragara gute?

HLH ni indwara ikunze kugaragara cyane . Biragoye kuvuga neza umubare w'abantu bayirwaye, kuko rimwe na rimwe ishobora kwirengagizwa nabi cyangwa ikaba idasuzumwa neza. Abantu bagera kuri 75% banduye HLH bafite HLH ya kabiri. 25% gusa ni bo bafite HLH y'ibanze, ni ukuvuga umuntu umwe mu bantu 50.000 ku isi.

Ni ibihe bimenyetso bya `(HLH)`?

Ibimenyetso bya (HLH) bishobora gutandukana bitewe n'umuntu ku wundi, kandi bishobora gutandukana bitewe n'uburemere bw'indwara. Dore bimwe mu bimenyetso bikunze kugaragara:

• Umuriro udashira nubwo waba ukoresheje imiti yica udukoko.
• Uduheri ku ruhu.
• Ubwinshi bw'umwijima (hepatomegaly).
• Kwaguka k'agasabo k'inkari (splenomegaly).
• Utunyangingo tw’imitsi (lymph nodes) twabyimbye.

Tekereza kuri ibi: Iyo umuriro w'umwana utagabanutse iminsi myinshi, kandi ntugakire ndetse na nyuma yo guhabwa imiti, bishobora kuba ikimenyetso cya `(HLH)`. Kubwibyo, ni ngombwa cyane gushaka inama kwa muganga niba ukomeje.

Uretse ibi, hari n'ibindi bimenyetso bishobora kugaragara:

• Kubura amaraso bivuze kubura amaraso.
• Umubare muto wa platelets mu maraso (`Thrombocytopenia`).
• Intege nke, intege nke.
• Kubabara umutwe cyangwa isereri.
• Kurakara cyane no kubura amahoro buri gihe.
• Kubabara umutwe .
• Uruhu rwerurutse.
• Indwara ya Jaundice.

Ariko kandi, hari ibimenyetso bikomeye bishobora gushyira ubuzima mu kaga . Nubibona, ugomba guhita ujya kwa muganga :

• Kugorwa no guhumeka (dyspnea).
• Indwara zo mu mutwe .
• Kuva amaraso mu maso (`Kuva amaraso mu mitsi yo mu nda`).
• Gutakaza ubwenge.
• Koma.

Igikuru ni ukubonana na muganga vuba niba ufite kimwe muri ibi bimenyetso, cyane cyane ibishobora gushyira ubuzima mu kaga. Gusuzuma no kuvura hakiri kare ni bwo buryo bwiza bwo kugera ku musaruro mwiza.

Kuki ibi `(HLH)` bibaho? Impamvu ni izihe?

Mu magambo make, `(HLH)` iterwa n'uko ubudahangarwa bw'umubiri bukora cyane cyangwa bugacika intege. Nkuko twabiganiriyeho mbere, hari ubwoko bubiri bwa `(HLH)`, kandi impamvu za buri bumwe ziratandukanye. Ariko muri ubwo buryo bwombi, ubwoko bubiri bw'uturemangingo tw'umubiri, twitwa `histiocytes` na `T cells`, turemwa cyane kandi aho kwibasira umwanzi nka virusi iturutse hanze, twibasira umubiri wacu. Ibimenyetso bya `(HLH)` bibaho kuko utwo turemangingo tw'amaraso twera tudafite amabwiriza muri ADN yatwo yo gukora akazi katwo neza.

Impamvu z'ibanze za `(HLH)`:

`` (HLH)` y'ibanze iterwa n'impinduka mu ngirabuzimafatizo. Hari ngirabuzimafatizo nyinshi zigira ingaruka kuri ibi:

• `CD27`
• `ILK`
• `LYST`
• `PRF1`
• `RAB27`
• `SH2D1A`
• `STX11`
• `STXBP2`
• `UNC13D`

Izi gene ni zo zibwira uturemangingo dukora poroteyine. Izi poroteyine zifasha ubudahangarwa bwacu mu kurimbura abatera indwara z’amahanga nka bagiteri na virusi kandi zigatuma tugumana ubuzima bwiza. Ariko, iyo habayeho impinduka muri imwe muri izi gene, uturemangingo ntitwakira amabwiriza yuzuye yo gukora izi poroteyine. Hanyuma poroteyine zikorwa ziba zituzuye, cyangwa ntizishobora gukora akazi kazo neza mu mubiri. Izi mpinduka za gene ziragwa n’ababyeyi bombi igihe cyo gusama. Ibi byitwa "ishusho ya autosomal recessive."

Impamvu za `(HLH)` za kabiri:

Indwara ya kabiri (HLH) ni indwara ivuka. Ibi bivuze ko atari indwara iterwa n’uturemangingo (genetic) ibaho igihe umuntu avuka, kandi nta mpamvu yo kuba afite amateka y’umuryango. Iterwa n’uko ubudahangarwa bw’umubiri bukora nabi. Ubushakashatsi buracyakomeje kugira ngo hamenyekane impamvu ibi bibaho.

Hari igihe, "HLH" ya kabiri ishobora guterwa n'indwara. Izo ndwara zirimo:

• Ubwandu bwa virusi ya Epstein-Barr (EBV).
• Izindi ndwara zandurira mu mikorobe, virusi n'ibihumyo.
• Gucika intege k'ubudahangarwa bw'umubiri.
• Indwara y'ubwiyongere bw'umubiri.
• Indwara yo kubyimbirwa mu buryo bwikora.
• Indwara za rubagimpande (urugero: rubagimpande y’abana).
• Indwara za metabolike.
• Kanseri (urugero: lymphoma itari iya Hodgkin).

Ni gute abaganga basuzuma indwara `(HLH)`?

Iyo umuganga akeka ko ufite HLH, abanza gukora isuzuma ry’umubiri. Hanyuma azagutegeka ibizamini byinshi kugira ngo amenye byinshi ku bimenyetso byawe. Umuganga azashaka ibi bikurikira byo gusuzuma:

• Umuriro.
• Urwagashya rwagutse.
• Ingano nto y'uturemangingo dutukura tw'amaraso, uturemangingo tw'umweru, cyangwa uduce duto tw'amaraso mu maraso (cytopenia).
• Urugero rwinshi rw'ibinure byitwa triglyceride mu maraso (hypertriglyceridemia) cyangwa urugero ruto rwa poroteyine (fibrinogen) ifasha amaraso kubumba (hypofibrinogenemia).
• Kwangirika cyangwa gusenyuka kw'uturemangingo tw'amaraso mu musokoro w'amagufwa (`hemophagocytosis`).
• Kugabanuka k'imikorere y'uturemangingo twa T mu maraso.
• Ubwiyongere bw'urugero rwa 'ferritin' mu maraso (`ferritinemia`).
• Ubwiyongere bw'urugero rwa `soluble interleukin-2 receptor (sCD25)` mu maraso.

Niba ufite nibura ibimenyetso bitanu muri ibi, muganga wawe ashobora gukeka ko afite HLH.

Ibizamini byo kumenya `(HLH)`:

Bimwe mu bipimo bishobora gufasha gusuzuma `(HLH)` ni ibi bikurikira:

• Gupima imiterere y’uturemangingo (cyane cyane niba bikekwa ko ari ubwa mbere (HLH).
• Umubare wuzuye w'amaraso.
• Isuzuma ry'inyongera ry'amaraso kugira ngo harebwe niba ferritin, urugero rwa triglycerides, ibimenyetso by'ubwandu, n'uko amaraso arimo kuvura neza.
• Ikizamini cy'imikorere y'umwijima.
• Isuzuma ry'umusemburo w'amagufwa.

Ni ubuhe buryo bwo kuvura `(HLH)`?

Hari uburyo butandukanye bwo kuvura `(HLH)`. Ubu buryo bushobora gutandukana bitewe n'ubwoko bw'indwara, ubukana bwayo, n'uko umurwayi amerewe:

• Imiti yo kuvura indwara zandura (imiti igabanya ubukana cyangwa imiti igabanya ubukana bw'indwara).
• Imiti igabanya imikorere y'ubudahangarwa bw'umubiri (imiti igabanya ubukana bw'umubiri cyangwa imiti igabanya ubukana bw'umubiri).
• Kuvura cyangwa kuvura indwara zisanzwe (urugero, imiti ivura kanseri).
• Guterwa amaraso.

Hari umuti mushya witwa `Emapalumab (Gamifant®).` Ni `antibodies ibuza gamma`. Yemejwe gukoreshwa ku bana bato n'abantu bakuru bafite `(HLH)`.

Iyo imiti cyangwa uburyo bwo kuvura indwara idakora, abaganga bashobora gutekereza gutera uturemangingo tw’umubiri two mu bwoko bwa allogeneic. Ibi bikubiyemo gusimbuza uturemangingo tw’umubiri twangiritse (dukomoka mu musokoro w’amagufwa yawe) uturemangingo tw’umubiri twangiritse duturutse ku muntu utanga uturemangingo. Ibi bikunze kubanzirizwa no guhabwa imiti myinshi cyangwa imirasire kugira ngo bice uturemangingo tw’umubiri twangiritse. Ubu buryo ni bubi cyane, kandi hari ingaruka nyinshi mbi . Bityo muganga wawe azagusobanurira byose kugira ngo ufate icyemezo cy’ubuzima bwawe.

Ese hari uburyo bwo gukumira ikorwa rya `(HLH)`?

Mu by’ukuri, nta kintu na kimwe twakora ngo tuburize iterambere rya `(HLH)`, kuko impamvu zirenze ubushobozi bwacu. Ariko, hari ibintu bimwe na bimwe twakora kugira ngo twirinde ibintu nk'ubwandu bushobora gutera `(HLH)` ya kabiri:

• Kurya indyo yuzuye kandi ugire imyitozo ngororamubiri buri gihe.
• Irinde abantu barwaye indwara za virusi.
• Ambara ibikoresho byo kwirinda kugira ngo wirinde imvune.
• Iyo habayeho ibikomere, bisukure neza kugira ngo hirindwe kwandura.
• Gucunga neza indwara zisanzwe.

Ni iki gipimo cy’indwara ya (HLH)?

Iyo indwara igaragaye kandi igavurwa hakiri kare , umuntu ashobora kwitega umusaruro mwiza cyangwa mwiza, bitewe n'uburemere bw'ibimenyetso. Ariko, iyo itavuwe, "HLH" ishyira ubuzima mu kaga . Ishobora gutera ingingo zinanirwa ndetse n'urupfu mu mezi make.

Muganga wawe azagusobanurira uburyo bwo gusuzuma no kuvura ibimenyetso byawe, ndetse n'ingaruka n'ingaruka z'ubuvuzi.

Abantu benshi biyita ko bafite (HLH) bashaka ubufasha bwabo n'imiryango yabo. Ibi bivuze kuvugana n'umujyanama mu by'ubuzima bwo mu mutwe , umujyanama mu by'uturemangingo, cyangwa umukozi ushinzwe imibereho myiza kugira ngo bateganye uburyo bwo kubaho muri iki kibazo, ingaruka zacyo, n'ingaruka zacyo.

Ese `(HLH)` ishobora gukira burundu?

Iyo ubuvuzi bukwiye kandi bukitabwaho, indwara ya `(HLH)` ishobora kurangira. Ariko, ishobora no kugaruka iyo yongeye gukururwa. Hari abantu bashoboye gukumira kongera kwandura kwa `(HLH)` binyuze mu gutera uturemangingo tw’ingirabuzimafatizo no kwirinda ingaruka zishobora gushyira ubuzima mu kaga. Ubushakashatsi bukomeje gukorwa kuri ibi.

Ni ryari ngomba kujya kwa muganga?

Niba ufite ibimenyetso bya `(HLH)`, hita ubonana na muganga . Ntugatinde, kuko gusuzuma indwara hakiri kare no kuyivura ari bwo buryo bwiza bwo koroherwa. Niba ufite ibimenyetso bikomeye nko kugorwa no guhumeka, guta ubwenge, cyangwa gukubita ibisebe, ugomba guhita ujya kwa muganga wihutirwa .

Ni ibihe bibazo ngomba kubaza muganga?

• Ni ibihe bizamini ngomba gukora?
• Ni ubuhe buryo bwo kuvura usaba?
• Ingaruka mbi z'ubuvuzi ni izihe?
• Imyaka yanjye yo kubaho imeze ite?

Kumenya ko wowe cyangwa uwo ukunda afite indwara idakunze kugaragara kandi ishobora gushyira ubuzima mu kaga bishobora kugutera agahinda no kubabara cyane. Kuganira n'umujyanama w'ubuzima bwo mu mutwe , umujyanama w'uturemangingo, cyangwa muganga wawe bishobora kugufasha cyane. Bashobora kugufasha kumenya byinshi ku bijyanye n'uburyo bwo guhangana n'iyi ndwara, uburyo bwo kwita ku buzima bwawe n'umuryango wawe, ndetse n'ubuvuzi n'uko ubuzima buhagaze. Niba ufite ibibazo muri iki gihe kigoye, saba ubufasha ku bantu ba hafi yawe cyangwa itsinda ry'abaganga bawe.

Ikintu cy'ingenzi cyo kwibuka (Ubutumwa bwo kujyana mu rugo)

(HLH) ni indwara ikomeye ariko idakunze kugaragara iterwa n'imikorere idasanzwe y'ubudahangarwa bw'umubiri wacu. Muri ibi, uturemangingo tw'umubiri wacu turatwibasira. Hari ubwoko bubiri bw'ingenzi: ubw'ibanze (HLH) buterwa n'impamvu za genetiki, n'ubwa kabiri (HLH) buterwa n'izindi ndwara.

Ibimenyetso biratandukanye, ariko ugomba kwitonda niba ufite umuriro udashira, udusebe tw’uruhu, cyangwa umwijima/impiri byaguwe. Niba ugize ibimenyetso bikomeye nko kugorwa no guhumeka cyangwa gucika intege, shaka ubufasha bwa muganga vuba.

Gusuzuma no kuvura hakiri kare ni ingenzi. Uburyo bwo kuvura buratandukanye bitewe n'ubwoko bw'indwara. Hari igihe biba ngombwa gukoresha ibintu nko gutera uturemangingo tw'umubiri.

Nubwo nta buryo bwizewe bwo kwirinda ibi, gukurikiza imyitwarire y’ubuzima muri rusange bishobora kugabanya ibyago byo kurwara indwara ya `(HLH)`. Mu bihe nk'ibi, ni ngombwa cyane gukomeza kugira ubwenge bukomeye no kubona ubufasha bukenewe. Ntabwo uri wenyine, ntutindiganye gusaba ubufasha.

` HLH, Hemophagocytic Lymphohistiocytosis, Ubudahangarwa bw'umubiri, Indwara za genetiki, Kwandura, Umuriro, Ibimenyetso