Ake sifunde ngesimo esingavamile esenza amasosha omzimba wakho akuhlasele (i-Hemophagocytic Lymphohistiocytosis - HLH)

Uzizwa sengathi awukaze uzizwe ngaphambili? Ngezinye izikhathi amasosha omzimba ethu, lawo aphilisa imizimba yethu, angaqala ukusenza sigule. Esinye isimo esibi nesingavamile yi-Hemophagocytic Lymphohistiocytosis, noma (HLH) ngamafuphi. Okwenzekayo ukuthi amangqamuzana afana nohlelo lokuzivikela lomzimba wethu ayanda ngokungalawuleki futhi ahlasele izitho zethu. Uma sikhuluma iqiniso, kufana nonogada ongakwazi ukubona umndeni wakhe.

Kusho ukuthini ngempela ukuthi `(HLH)`? Masikuqonde kalula, akunjalo?

Isimiso somzimba wethu sokuzivikela siyindlela emangalisayo kakhulu. Sifana nebutho elivikela izwe lethu. Lolu hlelo lusivikela ezitheni ezifana namagciwane, amagciwane, namabhaktheriya avela ngaphandle. Amaseli amaningi, amaprotheni, izitho zomzimba, nezicubu zisebenza ndawonye kulokhu. Kodwa-ke, lapho umuntu one-`(HLH)` ethola ukutheleleka, lesi simiso somzimba sokuzivikela sikhuthazwa ngokweqile. Ngemuva kwalokho, esikhundleni sokulwa nokutheleleka, amaseli asanda kwakheka aqala ukuhlasela imizimba yethu. Lokhu kusenza sigule kakhulu. Eqinisweni, lesi simo se-`(HLH)` singaba yingozi empilweni .

Kunezinhlobo ezimbili eziyinhloko zalezi `(HLH)`, akunjalo?

Yebo, `(HLH)` ingahlukaniswa ngezinhlobo ezimbili eziyinhloko:

1. Okuyinhloko (i-HLH) (okubangelwa izakhi zofuzo) : Lokhu kubangelwa ukuguquka kwezakhi zofuzo. Lokhu kusho ukuthi umuntu uzalwa enokuthambekela kukho. Izimpawu zivame ukuqala ukuvela eminyakeni embalwa yokuqala yokuphila. Akuvamile kakhulu, izimpawu zivela ngemva kokuba umuntu esemdala. Lokhu ngezinye izikhathi kubizwa ngokuthi ``i-HLH yomndeni``.

2. Okwesibili (HLH) (okubangelwa esinye isimo sezokwelapha) : Lokhu kwenzeka lapho amasosha omzimba wakho ethintwa esinye isimo sezokwelapha osunaso kakade. Isibonelo, kungabangelwa izinto ezifana nomdlavuza, izifo (ikakhulukazi igciwane le-Epstein-Barr), noma izimo zokuzivikela komzimba. Okwesibili (HLH) kuvame kakhulu kune-primary (HLH).

Ubani onamathuba amaningi okuthuthukisa lokhu `(HLH)`?

`(HLH)` Ingakhula kunoma ubani wanoma yimuphi ubudala. Kodwa-ke, ivame ukubonakala ezinganeni ezincane nasezinsaneni . Kodwa-ke, lokho akusho ukuthi abantu abadala abakwazi ukuyikhula.

Sivame kangakanani lesi simo?

I-HLH yisimo esingavamile kakhulu . Kunzima ukusho ukuthi bangaki abantu abanaso ngempela, njengoba ngezinye izikhathi singahlolwa ngokungalungile noma singahlolwanga kahle. Cishe ama-75% abantu abatholakale bene-HLH ​​bane-HLH ​​yesibili. Ama-25% kuphela ane-HLH ​​yokuqala, cishe umuntu oyedwa kwabangu-50,000 emhlabeni jikelele.

Ziyini izimpawu ze-`(HLH)`?

Izimpawu ze-(HLH) zingahluka kumuntu nomuntu, futhi zingahluka kuye ngokuthi lesi sifo sibucayi kangakanani. Nazi ezinye zezimpawu ezivame kakhulu:

• Umkhuhlane ongapheli ngisho noma usebenzisa ama-antibiotic.
• Ukuqhuma kwesikhumba.
• Isibindi esikhulu (i-hepatomegaly).
• Ukwanda kwe-spleen (i-splenomegaly).
• Ama-lymph node avuvukele (amathumba).

Cabanga ngalokhu: Uma umkhuhlane wengane ungehli izinsuku eziningana, futhi uma ungathuthuki ngisho nangemva kokuthatha imithi, kungaba uphawu lwe-`(HLH)`. Ngakho-ke, kubaluleke kakhulu ukufuna iseluleko sezokwelapha uma uqhubeka.

Ngaphezu kwalokhu, kungase kuvele ezinye izimpawu:

• I-anemia isho ukuntuleka kwegazi.
• Inani eliphansi lamaplatelet egazini (`Thrombocytopenia`).
• Ubuthakathaka, ubuthakathaka.
• Ukungaqondi kahle noma isiyezi.
• Ukucasuka okuqhubekayo kanye nokungahlaliseki.
• Ubuhlungu bekhanda .
• Isikhumba esimpunga.
• I-Jaundice.

Kodwa-ke, kunezimpawu ezinkulu ezingasongela impilo . Uma ubona lezi, kufanele uye esibhedlela ngokushesha :

• Ubunzima bokuphefumula (ukuphelelwa umoya).
• Ukuquleka .
• Ukopha ngaphakathi kwamehlo (`Ukopha kwe-retinal`).
• Ukulahlekelwa ukwazi.
• I-Coma.

Into ebaluleke kakhulu ukubona udokotela ngokushesha uma unezinye zalezi zimpawu, ikakhulukazi lezo ezingase zibe yingozi empilweni. Ukuxilongwa kusenesikhathi kanye nokwelashwa kuyindlela engcono kakhulu yokuthola imiphumela emihle kakhulu.

Kungani lokhu `(HLH)` kwenzeka? Yiziphi izizathu?

Kalula nje, `(HLH)` ibangelwa ukuthi isimiso somzimba sokuzivikela sisebenza ngokweqile noma singasebenzi kahle. Njengoba sixoxile ngaphambili, kunezinhlobo ezimbili ze-`(HLH)`, futhi izimbangela zaso ngasinye zihlukile. Kodwa kuzo zombili izimo, izinhlobo ezimbili zamaseli omzimba, abizwa ngokuthi `histiocytes` kanye `T cells`, akhiqizwa ngokweqile futhi esikhundleni sokuhlasela isitha njengegciwane elivela ngaphandle, ahlasela imizimba yethu. Izimpawu ze-`(HLH)` zenzeka ngoba la maseli amhlophe egazi awanazo iziyalezo ku-DNA yawo zokwenza umsebenzi wawo kahle.

Izimbangela ze-`(HLH)` eyinhloko:

I-`(HLH)` eyinhloko ibangelwa ukuguquka kwezakhi zofuzo. Kunezakhi zofuzo eziningana ezithinta lokhu:

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

Lezi zakhi zofuzo yizo ezitshela amangqamuzana ethu ukuthi enze amaprotheni. Lezi zakhi zofuzo zisiza amasosha omzimba ethu ukuba abhubhise abahlaseli bakwamanye amazwe njengamabhaktheriya namagciwane futhi zisigcine siphilile. Kodwa-ke, uma kukhona ukuguquka kwenye yalezi zakhi zofuzo, amangqamuzana awatholi imiyalelo ephelele yokwenza lawa maprotheni. Khona-ke amaprotheni enziwayo awaphelele, noma awakwazi ukwenza umsebenzi wawo kahle ngaphakathi emzimbeni. Lezi zakhi zofuzo zizuzwa njengefa kubazali bobabili ngesikhathi sokukhulelwa. Lokhu kubizwa ngokuthi "iphethini yokubuyisa i-autosomal."

Izimbangela ze-`(HLH)` yesibili:

I-'(HLH)' yesibili yisimo esitholwa yisiguli. Lokhu kusho ukuthi akuyona into ebangelwa izakhi zofuzo eyenzeka lapho umuntu ezalwa, futhi asikho isidingo somlando womndeni. Ibangelwa ukusabela okungavamile kwesimiso somzimba sokuzivikela. Ucwaningo lusaqhubeka ukuze kutholakale ukuthi kungani lokhu kwenzeka.

Ngezinye izikhathi, "i-HLH" yesibili ingabangelwa yisimo sezokwelapha. Izimo ezinjalo zifaka:

• Ukutheleleka kwegciwane le-Epstein-Barr (EBV).
• Ezinye izifo ezibangelwa amagciwane, amagciwane kanye nesikhunta.
• Ubuthakathaka besimiso somzimba sokuzivikela.
• Isimo sokuzivikela komzimba.
• Isifo sokuvuvukala okuzenzakalelayo.
• Izifo ze-rheumatoid (isb. i-juvenile idiopathic arthritis).
• Izinkinga ze-Metabolic.
• Umdlavuza (isib. i-non-Hodgkin lymphoma).

Odokotela basithola kanjani lesi simo `(HLH)`?

Uma udokotela esola ukuthi une-HLH, uzoqala ngokukwenza ukuhlolwa ngokomzimba. Bazobe sebeyala ukuhlolwa okuningana ukuze bafunde kabanzi ngezimpawu zakho. Udokotela uzobheka izindlela zokuxilonga ezilandelayo:

• Imfiva.
• Ubende obukhulu.
• Amazinga aphansi amangqamuzana abomvu egazi, amangqamuzana amhlophe egazi, noma ama-platelet egazini (i-cytopenia).
• Amazinga aphezulu ohlobo lwamafutha olubizwa ngokuthi i-triglyceride egazini (hypertriglyceridemia) noma amazinga aphansi ephrotheni (fibrinogen) esiza ukujiya kwegazi (hypofibrinogenemia).
• Ukulimala noma ukubhujiswa kwamaseli egazi emnkantsheni wethambo (`hemophagocytosis`).
• Ukwehla komsebenzi wamaseli e-T egazini.
• Amazinga akhuphukile e-`ferritin` egazini (`ferritinemia`).
• Amazinga akhuphukile e-'soluble interleukin-2 receptor (sCD25)` egazini.

Uma unezimpawu okungenani ezinhlanu zalezi, udokotela wakho angase asole ukuthi une-HLH.

Ukuhlolwa kokuhlonza `(HLH)`:

Ezinye izivivinyo ezingasiza ekuxilongeni `(HLH)` yilezi:

• Ukuhlolwa kofuzo (ikakhulukazi uma kusolwa ukuthi kunesifo esiyinhloko (i-HLH).
• Ukubalwa kwegazi okuphelele.
• Ukuhlolwa kwegazi okwengeziwe ukuhlola i-ferritin, amazinga e-triglycerides, izimpawu zokutheleleka, kanye nokuthi igazi liqina kangakanani.
• Ukuhlolwa kokusebenza kwesibindi.
• Ukuhlolwa komnkantsha.

Yiziphi izindlela zokwelapha `(HLH)`?

Kunezindlela eziningana zokwelapha (i-HLH). Lokhu kungahluka kuye ngohlobo lwesifo, ubukhulu baso, kanye nesimo sesiguli:

• Imithi yokwelapha izifo (ama-antibiotic noma ama-antiviral).
• Imithi yokunciphisa umsebenzi wesimiso somzimba sokuzivikela (ama-immunosuppressants noma ama-cytokine inhibitors).
• Ukwelapha noma ukuphatha izimo zezokwelapha eziyisisekelo (isibonelo, i-chemotherapy yomdlavuza).
• Ukumpontshelwa igazi.

Kukhona umuthi omusha obizwa ngokuthi `Emapalumab (Gamifant®).` Uyi-`antibody evimba i-gamma.` Uvunyelwe ukusetshenziswa ezinsaneni nakubantu abadala abane-`(HLH)`.

Uma imithi noma ukuphathwa kwesifo esiyisisekelo kungasebenzi, odokotela bangase bacabangele ukufakelwa i-allogeneic stem cell. Lokhu kuhilela ukufaka esikhundleni amaseli akho okuqala angasebenzi kahle (kusuka emnkantsheni wakho) ngamaseli okuqala aphilile avela kumnikeli. Lokhu kuvame ukulandelwa yi-chemotherapy noma imisebe ephezulu yokubulala amaseli okuqala angaphili kahle. Le nqubo iyingozi kakhulu, futhi kunemiphumela eminingi emibi . Ngakho udokotela wakho uzokuchazela konke ukuze ukwazi ukwenza isinqumo esinolwazi mayelana nempilo yakho.

Ingabe ikhona indlela yokuvimbela ukwakheka kwe-`(HLH)`?

Eqinisweni, akukho esingakwenza ukuvimbela ukuthuthukiswa kwe-`(HLH)`, njengoba izimbangela zingaphezu kwamandla ethu. Kodwa-ke, kunezinto ezithile esingazenza ukuze sizivikele ezintweni ezifana nezifo ezingabangela i-`(HLH)` yesibili:

• Yidla ukudla okunempilo futhi uvivinye umzimba njalo.
• Hlala kude nabantu abanezifo ezibangelwa amagciwane.
• Gqoka izingubo zokuzivikela ukuze uvimbele ukulimala.
• Uma kuvela amanxeba, wahlanze kahle ukuze uvimbele ukutheleleka.
• Ukuphathwa kahle kwezimo zezokwelapha eziyisisekelo.

Iyini i-prognosis ye-(HLH)?

Uma lesi sifo sitholakala futhi selashwa kusenesikhathi , kungalindelwa umphumela omuhle noma omuhle, kuye ngokuthi izimpawu zibucayi kangakanani. Kodwa-ke, uma singelashwa, "i-HLH" isongela ukuphila . Ingaholela ekuhlulekeni kwezitho zomzimba ngisho nokufa zingakapheli izinyanga ezimbalwa.

Udokotela wakho uzochaza izindlela zokuxilongwa kanye nokwelashwa kwezimpawu zakho kuwe, kanye nezingozi nemiphumela emibi yokwelashwa.

Abantu abaningi abaziveza njengabane-(HLH) bafuna ukwesekwa bona kanye nemindeni yabo. Lokhu kusho ukukhuluma nomeluleki wezempilo yengqondo , umeluleki wezakhi zofuzo, noma isisebenzi sezenhlalakahle ukuze bahlele ukuthi bangaphila kanjani nalesi simo, ukuthi ziyini izingozi, nokuthi imiphumela izoba yini.

Ingabe `(HLH)` ingalapheka ngokuphelele?

Ngokwelashwa nokunakekelwa okufanele, isimo `(HLH)` singaphela. Kodwa-ke, singabuya futhi uma siphinde saqala. Abanye abantu bakwazile ukuvimbela ukuphinda kuvele `(HLH)` ngokufakelwa iseli le-stem kanye nokugwema imiphumela esongela impilo. Ucwaningo olwengeziwe luyenziwa ngalokhu.

Kufanele ngimbone nini udokotela?

Uma unezimpawu ze-`(HLH)`, bona udokotela ngokushesha . Ungalibali, njengoba ukuxilongwa kusenesikhathi kanye nokwelashwa kuyizindlela ezinhle kakhulu zokululama. Uma unezimpawu ezinzima njengobunzima bokuphefumula, ukulahlekelwa ukwazi, noma ukuxhuzula, kufanele uye ekamelweni eliphuthumayo ngokushesha .

Yimiphi imibuzo okufanele ngiyibuze udokotela?

• Yiziphi izivivinyo okudingeka ngizenze?
• Yikuphi ukwelashwa okuncomayo?
• Iyini imiphumela emibi yokwelashwa?
• Injani impilo yami?

Ukuthola ukuthi wena noma othandekayo wakho unesifo esingavamile nesisongela ukuphila kungaba yinto ekhathazayo futhi ebuhlungu kakhulu. Ukukhuluma nomeluleki wezempilo yengqondo , umeluleki wezakhi zofuzo, noma udokotela wakho kungaba usizo olukhulu. Bangakusiza ufunde kabanzi mayelana nendlela yokubhekana nalesi simo, indlela yokunakekela wena nomndeni wakho, kanye nokwelashwa kanye nesimo sengqondo. Uma unemibuzo phakathi nalesi sikhathi esinzima, cela usizo kulabo abaseduze nawe noma ithimba lakho lezempilo.

Into ebaluleke kakhulu okufanele uyikhumbule (Umyalezo Wokuya Ekhaya)

(HLH) yisimo esibi kodwa esingavamile esibangelwa ukusebenza okungajwayelekile kwesimiso sethu somzimba sokuzivikela. Kulokhu, amangqamuzana omzimba wethu ayasihlasela. Kunezinhlobo ezimbili eziyinhloko: eyinhloko (HLH) ebangelwa izimbangela zofuzo, kanye neyesibili (HLH) ebangelwa ezinye izifo.

Izimpawu ziyahlukahluka, kodwa kufanele uqaphele uma unomkhuhlane oqhubekayo, izilonda zesikhumba, noma isibindi/ubende okhuphukile. Uma uhlangabezana nezimpawu ezinzima njengobunzima bokuphefumula noma ukuxhuzula, funa usizo lwezokwelapha ngokushesha.

Ukuxilongwa kusenesikhathi kanye nokwelashwa kubalulekile. Izindlela zokwelapha ziyahlukahluka kuye ngohlobo lwesifo. Ngezinye izikhathi, kungadingeka ukusebenzisa izinto ezifana nokufakelwa iseli le-stem.

Nakuba kungekho ndlela eqinisekile yokuvimbela lokhu, ukulandela imikhuba yezempilo ejwayelekile kunganciphisa ingozi ye-`(HLH)` yesibili. Esimweni esinjengalesi, kubaluleke kakhulu ukuhlala uqinile engqondweni futhi uthole ukwesekwa okudingekayo. Awuwedwa, ungangabazi ukucela usizo.

` I-HLH, i-Hemophagocytic Lymphohistiocytosis, Isistimu yomzimba, Izifo zofuzo, Ukutheleleka, Umkhuhlane, Izimpawu