Masifunde ngemeko engaqhelekanga eyenza amajoni akho omzimba akuhlasele (iHemophagocytic Lymphohistiocytosis - HLH)

Ngaba uziva ngathi awukaze uzive ngaphambili? Ngamanye amaxesha inkqubo yethu yokuzikhusela, leyo iphilisa imizimba yethu, ingaqala ukusenza sigule. Esinye sezimo ezinzima nezingaqhelekanga yiHemophagocytic Lymphohistiocytosis, okanye (HLH) ngamafutshane. Okwenzekayo kukuba iiseli ezifana nenkqubo yokuzikhusela yomzimba wethu zanda ngokungalawulekiyo kwaye zihlasela amalungu ethu omzimba. Ukuchaneka, kufana nomlindi ongakwaziyo ukubona usapho lwakhe.

Ithetha ukuthini `(HLH)` ngokwenene? ​​Masiyiqonde ngokulula, akunjalo?

Inkqubo yomzimba wethu yokuzikhusela iyindlela emangalisayo. Ifana nomkhosi okhusela ilizwe lethu. Le nkqubo isikhusela kwiintshaba ezifana neentsholongwane, iintsholongwane, kunye neebhaktheriya ezivela ngaphandle. Iiseli ezininzi, iiproteni, amalungu omzimba, kunye nezicubu zisebenza kunye koku. Nangona kunjalo, xa umntu one-`(HLH)` efumana usulelo, le nkqubo yokuzikhusela iyavuseleleka kakhulu. Emva koko, endaweni yokulwa nosulelo, iiseli ezisandula ukwenziwa ziqala ukuhlasela imizimba yethu. Oku kusenza sigule ngakumbi. Enyanisweni, le meko ye-`(HLH)` inokuba yingozi ebomini .

Kukho iintlobo ezimbini eziphambili zezi `(HLH)`, akunjalo?

Ewe, `(HLH)` inokwahlulwahlulwa ibe ziintlobo ezimbini eziphambili:

1. I-Primary (HLH) (ebangelwa yimfuza) : Oku kubangelwa kukuguquka kwemfuza. Oku kuthetha ukuba umntu uzalwa enesifo esithile. Iimpawu zihlala ziqala ukubonakala kwiminyaka embalwa yokuqala yobomi. Amaxesha amaninzi, iimpawu zibonakala emva kokuba umntu emdala. Oku ngamanye amaxesha kubizwa ngokuba yi-"Familial HLH".

2. I-Secondary (HLH) (ebangelwa yenye imeko yezonyango) : Oku kwenzeka xa amajoni akho omzimba echaphazeleka yenye imeko yezonyango osele unayo. Umzekelo, inokubangelwa zizinto ezifana nomhlaza, usulelo (ingakumbi i-Epstein-Barr virus), okanye iimeko zokuzikhusela komzimba. I-Secondary (HLH) ixhaphake kakhulu kune-primary (HLH).

Ngubani onokuthi aphuhlise le `(HLH)`?

`(HLH)` Ingakhula nakubani na wanoma yiphi na iminyaka. Nangona kunjalo, idla ngokubonakala kubantwana abancinci nakwiintsana . Nangona kunjalo, oko akuthethi ukuba abantu abadala abanakuyikhula.

Ixhaphake kangakanani le meko?

I-HLH yimeko engaqhelekanga kakhulu . Kunzima ukutsho ngqo ukuba bangaphi abantu abanayo, kuba ngamanye amaxesha inokuxilongwa gwenxa okanye ingachongwanga ngokwaneleyo. Malunga nama-75% abantu abafunyaniswe bene-HLH ​​bane-HLH ​​yesibini. Ama-25% kuphela ane-HLH ​​yokuqala, emalunga nomntu omnye kuma-50,000 kwihlabathi liphela.

Zithini iimpawu ze-`(HLH)`?

Iimpawu ze-(HLH) zingahluka ukusuka kumntu ukuya komnye, kwaye zinokuhluka ngokuxhomekeke kubukhali besifo. Nazi ezinye zezona mpawu zixhaphakileyo:

• Umkhuhlane ongapheliyo nokuba usebenzisa amayeza okubulala iintsholongwane.
• Ukuqhambuka kwesikhumba.
• Isibindi esikhulu (i-hepatomegaly).
• Ukwanda kwespleen (i-splenomegaly).
• Amaqhuqhuva e-lymph avuvukileyo (amathumba).

Cinga ngoku: Ukuba umkhuhlane womntwana awunciphi kangangeentsuku ezininzi, kwaye ukuba awuphucuki nasemva kokusebenzisa amayeza, oko kungaba luphawu lwe `(HLH)`. Ke ngoko, kubaluleke kakhulu ukufuna ingcebiso kagqirha ukuba uyaqhubeka.

Ukongeza koku, ezinye iimpawu zingabonakala:

• I-anemia ithetha ukunqongophala kwegazi.
• Inani eliphantsi leeplatelet egazini (`Thrombocytopenia`).
• Ubuthathaka, ubuthathaka.
• Ukuqaqamba okanye isiyezi.
• Ukucaphuka rhoqo nokungazoli.
• Intloko ebuhlungu .
• Ulusu oluphaphathekileyo.
• I-Jaundice.

Nangona kunjalo, kukwakho neempawu ezinobungozi ezinokuba yingozi ebomini . Ukuba uzibona ezi, kuya kufuneka uye esibhedlele ngoko nangoko :

• Ubunzima bokuphefumla (ukuphelelwa ngamandla).
• Ukuxhuzula .
• Ukopha ngaphakathi emehlweni (`Ii-retinal hemorrhages`).
• Ukulahlekelwa yingqondo.
• I-Coma.

Eyona nto ibalulekileyo kukubona ugqirha ngaphandle kokulibazisa ukuba unezinye zezi mpawu, ingakumbi ezo zinokuba yingozi ebomini. Ukuxilongwa kwangoko kunye nonyango zezona ndlela zibalaseleyo zokufumana iziphumo ezilungileyo.

Kutheni le nto `(HLH)` isenzeka? Zithini izizathu?

Ngamafutshane, `(HLH)` ibangelwa kukuba inkqubo yomzimba yokuzikhusela isebenza kakhulu okanye ayisebenzi kakuhle. Njengoko besitshilo ngaphambili, kukho iintlobo ezimbini ze`(HLH)`, kwaye izizathu zalo nganye zahlukile. Kodwa kuzo zombini iimeko, iintlobo ezimbini zeeseli zenkqubo yomzimba yokuzikhusela, ezibizwa ngokuba yi`histiocytes` kunye ne`T cells`, ziveliswa ngokugqithisileyo kwaye endaweni yokuhlasela utshaba olufana nentsholongwane oluvela ngaphandle, zihlasela imizimba yethu. Iimpawu ze`(HLH)` zenzeka kuba ezi seli zegazi ezimhlophe azinazo imiyalelo kwi-DNA yazo yokwenza umsebenzi wazo ngokufanelekileyo.

Izizathu ze-`(HLH)` eyintloko:

I-`(HLH)` yokuqala ibangelwa kukuguquka kwezakhi zofuzo. Kukho iizakhi zofuzo ezininzi ezichaphazela oku:

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

Ezi jini zezona zixelela iiseli zethu ukuba zenze iiproteni. Ezi protein zinceda amajoni ethu omzimba ukuba atshabalalise abahlaseli bamanye amazwe njengeebhaktheriya kunye neentsholongwane kwaye zisigcine sisempilweni. Nangona kunjalo, ukuba kukho utshintsho kwenye yezi jini, iiseli azifumani miyalelo ipheleleyo yokwenza ezi proteni. Emva koko iiproteni ezenziweyo aziphelelanga, okanye azikwazi ukwenza umsebenzi wazo ngokufanelekileyo ngaphakathi emzimbeni. Ezi nguqu zejini zizuzwa kubazali bobabini ngexesha lokukhulelwa. Oku kubizwa ngokuba yi-"autosomal recessive pattern."

Izizathu zesibini `(HLH)`:

I-'Secondary `(HLH)` yimeko efunyanwayo. Oku kuthetha ukuba ayilofuzo oluvela ekuzalweni, kwaye akukho mfuneko yokuba kubekho imbali yosapho. Ibangelwa kukusabela okungaqhelekanga kwenkqubo yomzimba yokuzikhusela. Uphando lusaqhubeka ukuze kuchongwe isizathu sokuba oku kwenzeke.

Ngamanye amaxesha, "i-HLH" yesibini inokubangelwa yimeko yempilo. Ezi meko ziquka:

• Usulelo lwe-Epstein-Barr virus (EBV).
• Ezinye izifo ezibangelwa yibhaktiriya, iintsholongwane kunye nefungus.
• Ubuthathaka benkqubo yomzimba yokuzikhusela.
• Imeko yokuzikhusela komzimba.
• Isifo sokudumba okuzenzakalelayo.
• Izifo zerheumatological (umz. i-juvenile idiopathic arthritis).
• Iingxaki zemetabolism.
• Umhlaza (umz. i-non-Hodgkin lymphoma).

Oogqirha bayixilonga njani imeko `(HLH)`?

Ukuba ugqirha ukrokrela ukuba une-HLH, uya kuqala enze uvavanyo lomzimba. Emva koko uya kuyalela iimvavanyo ezininzi ukuze afunde ngakumbi ngeempawu zakho. Ugqirha uya kukhangela ezi ndlela zilandelayo zokuxilonga:

• Ifiva.
• Udaka olukhulu.
• Amanqanaba aphantsi eeseli ezibomvu zegazi, iiseli ezimhlophe zegazi, okanye iiplatelets egazini (i-cytopenia).
• Amanqanaba aphezulu ohlobo lwamafutha olubizwa ngokuba yi-triglyceride egazini (hypertriglyceridemia) okanye amanqanaba aphantsi eproteni (fibrinogen) enceda ukujiya kwegazi (hypofibrinogenemia).
• Ukonakala okanye ukutshatyalaliswa kweeseli zegazi kumongo wethambo (`hemophagocytosis`).
• Ukuncipha komsebenzi weeseli ze-T egazini.
• Amanqanaba aphezulu e-`ferritin` egazini (`ferritinemia`).
• Amanqanaba aphezulu e-'soluble interleukin-2 receptor (sCD25)` egazini.

Ukuba uneempawu ezintlanu kwezi, ugqirha wakho unokurhanela ukuba une-HLH.

Uvavanyo lokuchonga `(HLH)`:

Ezinye iimvavanyo ezinokunceda ekuxilongeni `(HLH)` zezi:

• Uvavanyo lwe-genetic (ingakumbi ukuba kukho ukrokrelo lwe-primary (HLH).
• Ubalo lwegazi olupheleleyo.
• Uvavanyo lwegazi olongezelelweyo ukujonga i-ferritin, amanqanaba e-triglycerides, iimpawu zosulelo, kunye nokuba igazi liqina kangakanani na.
• Uvavanyo lokusebenza kwesibindi.
• I-biopsy yomongo wethambo.

Ziziphi iindlela zonyango ze-`(HLH)`?

Kukho iindlela ezahlukeneyo zonyango lwe-(HLH). Ezi zinokwahluka ngokuxhomekeke kuhlobo lwesifo, ubukhali baso, kunye nemeko yesigulana:

• Amayeza okunyanga izifo (ii-antibiotics okanye ii-antivirals).
• Amayeza okunciphisa umsebenzi wenkqubo yomzimba yokuzikhusela (ii-immunosuppressants okanye ii-cytokine inhibitors).
• Ukunyanga okanye ukulawula iimeko zonyango ezifihlakeleyo (umzekelo, i-chemotherapy yomhlaza).
• Utofelo-gazi.

Kukho iyeza elitsha elibizwa ngokuba yi-`Emapalumab (Gamifant®).` Liyi-`antibody ethintela i-gamma.` Livunyiwe ukuba lisetyenziswe kwiintsana nakubantu abadala abane-`(HLH)`.

Ukuba amayeza okanye ulawulo lwesifo esisisiseko alusebenzi, oogqirha banokucinga ngokufakelwa i-allogeneic stem cell. Oku kubandakanya ukutshintsha ii-stem cells zakho ezingasebenzi kakuhle (ezivela kumongo wakho wethambo) ngee-stem cells eziphilileyo ezivela kumntu onikelayo. Oku kudla ngokulandelwa yi-chemotherapy ephezulu okanye i-radiation ukubulala ii-stem cells ezingaphili kakuhle. Le nkqubo iyingozi kakhulu, kwaye kukho iziphumo ezininzi ebezingalindelekanga . Ngoko ke ugqirha wakho uya kukuchazela yonke into ukuze ukwazi ukwenza isigqibo esinolwazi malunga nempilo yakho.

Ngaba ikho indlela yokuthintela ukwakheka kwe-`(HLH)`?

Enyanisweni, akukho nto sinokuyenza ukuthintela uphuhliso lwe-`(HLH)`, njengoko izizathu zingaphaya kwamandla ethu. Nangona kunjalo, kukho ezinye izinto esinokuzenza ukuzikhusela kwizinto ezifana nosulelo olunokubangela i-`(HLH)` yesibini:

• Yitya ukutya okunesondlo kwaye uzilolonge rhoqo.
• Hlala kude nabantu abanezifo ezibangelwa ziintsholongwane.
• Nxiba izixhobo zokuzikhusela ukuze uthintele ukwenzakala.
• Ukuba kuvela amanxeba, acoce kakuhle ukuze kuthintelwe usulelo.
• Ulawulo olufanelekileyo lweemeko zempilo ezingabonakaliyo.

Ithini i-prognosis ye-(HLH)?

Ukuba isifo sifunyenwe kwaye sinyangwa kwangethuba , iziphumo ezilungileyo okanye ezilungileyo zinokulindelwa, kuxhomekeke kubukhali beempawu. Nangona kunjalo, ukuba asinyangwa, "i-HLH" isongela ubomi . Ingakhokelela ekungasebenzini kwamalungu omzimba kwanokufa kwiinyanga ezimbalwa.

Ugqirha wakho uza kukuchazela indlela yokuxilonga kunye neendlela zonyango lweempawu zakho, kunye neengozi kunye nemiphumo emibi yonyango.

Abantu abaninzi abazibanga benale ngxaki (HLH) bafuna inkxaso kubo nakwientsapho zabo. Oku kuthetha ukuthetha nomcebisi wezempilo yengqondo , umcebisi wezemfuza, okanye unontlalontle ukuze bacebe indlela yokuphila nale meko, ukuba zithini iingozi, kunye neziphumo eziya kuba zeziphi.

Ngaba `(HLH)` inganyangeka ngokupheleleyo?

Ngonyango olufanelekileyo nokunyamekela, i-HLH inokuphela. Nangona kunjalo, ingabuya kwakhona ukuba ivuselelwe kwakhona. Abanye abantu baye bakwazi ukuthintela i-HLH ukuba ingaphindi ivele kwaye baphephe iziphumo ezisongela ubomi ngokufakelwa iiseli ze-stem. Oku kusaphandwa.

Ndifanele ndimbone nini ugqirha?

Ukuba uneempawu ze-`(HLH)`, bona ugqirha ngokukhawuleza . Musa ukulibazisa, njengoko ukuxilongwa kwangoko kunye nonyango zezona ndlela zilungileyo zokuchacha. Ukuba uneempawu ezinzima ezifana nobunzima bokuphefumla, ukulahlekelwa zingqondo, okanye ukuxhuzula, kufuneka uye kwigumbi likaxakeka ngokukhawuleza .

Ndingayibuza yiphi imibuzo ugqirha?

• Zeziphi iimvavanyo ekufuneka ndizenze?
• Loluphi unyango olucebisayo?
• Zithini iziphumo ebezingalindelekanga zonyango?
• Injani ixesha lam lokuphila?

Ukufumanisa ukuba wena okanye umntu omthandayo unesifo esingaqhelekanga nesisongela ubomi kunokuba buhlungu kakhulu. Ukuthetha nomcebisi wezempilo yengqondo , umcebisi wezemfuza, okanye ugqirha wakho kunokuba luncedo olukhulu. Banokukunceda ufunde okungakumbi malunga nendlela yokujongana nale meko, indlela yokuzinyamekela wena nosapho lwakho, kunye nonyango kunye nesimo sengqondo. Ukuba unemibuzo ngeli xesha linzima, cela uncedo kwabo bakufutshane nawe okanye kwiqela lakho lezempilo.

Eyona nto ibalulekileyo ekufuneka uyikhumbule (Umyalezo Wokuya Ekhaya)

(HLH) yimeko embi kodwa engaqhelekanga ebangelwa kukungasebenzi kakuhle kwenkqubo yethu yokuzikhusela komzimba. Kule meko, iiseli zomzimba wethu ziyasihlasela. Kukho iintlobo ezimbini eziphambili: eyokuqala (HLH) ebangelwa zizizathu zemfuza, kunye neyesibini (HLH) ebangelwa zezinye izifo.

Iimpawu ziyahluka, kodwa kufuneka ulumkele izinto ezifana nomkhuhlane ongapheliyo, amaqhakuva eluswini, kunye nokwanda kwesibindi/ipleyini. Ukuba ufumana iimpawu ezinzima ezifana nobunzima bokuphefumla okanye ukuxhuzula, funa uncedo lwezonyango ngokukhawuleza.

Ukuxilongwa kwangoko kunye nonyango kubalulekile. Iindlela zonyango ziyahluka ngokuxhomekeke kuhlobo lwesifo. Ngamanye amaxesha, kunokufuneka usebenzise izinto ezifana nokufakelwa i-stem cell.

Nangona kungekho ndlela iqinisekileyo yokuthintela oku, ukulandela imikhwa yempilo ngokubanzi kunokunciphisa umngcipheko we-`(HLH)` yesibini. Kwimeko efana nale, kubaluleke kakhulu ukuhlala uqinile engqondweni kwaye ufumane inkxaso efunekayo. Awuwedwa, kwaye ungathandabuzi ukucela uncedo.

` HLH, iHemophagocytic Lymphohistiocytosis, Inkqubo yomzimba yokuzikhusela, Izifo zofuzo, Iintsholongwane, Umkhuhlane, Iimpawu