Lɛ wi lan bɔt wan sik we nɔ kin apin so ɔltɛm we kin mek yu yon imyun sistɛm atak yu (Hemophagocytic Lymphohistiocytosis - HLH) .

Yu kin ɛva fil lɛk se yu bɔdi de wɔs? Sɔntɛnde, wi yon imyun sistɛm, di wan we de mɛn wi bɔdi, kin bigin fɔ mek wi sik. Wan pan dɛn kayn siriɔs ɛn rare kɔndishɔn ya na Hemophagocytic Lymphohistiocytosis, ɔ (HLH) fɔ shɔt tɛm. Wetin kin apin na dat di sɛl dɛn we tan lɛk wi bɔdi in difɛns sistɛm kin bɔku pasmak ɛn atak wi yon ɔgan dɛn. Fɔ tɔk di kɔrɛkt tin, i tan lɛk gad we nɔ ebul fɔ no in yon famili.

Wetin `(HLH)` rili min? Lɛ wi ɔndastand am simpul wan, nɔto so?

Wi bɔdi in imyun sistɛm na wan we we rili wɔndaful. I tan lɛk ami we de protɛkt wi kɔntri. Dis sistɛm de protɛkt wi frɔm ɛnimi dɛn lɛk jem, vayrɔs, ɛn baktri dɛm we de kɔmɔt na do. Bɔku sɛl dɛn, prɔtin dɛn, ɔgan dɛn, ɛn tisu dɛn kin wok togɛda fɔ dis. כltu, we pכsin we gεt `(HLH)` gεt infεkshכn, dis imyun sistεm de כva stimulate. Dɔn, instead fɔ fɛt di infɛkshɔn, di nyu sɛl dɛn we dɔn mek kin bigin fɔ atak wi yon bɔdi. Dis kin mek wi sik mɔ ɛn mɔ. infakt, dis `(HLH)` kכndyushכn kin mek yu layf de pan denja .

Tu men kayn dɛn de fɔ dɛn `(HLH)`, nɔto so?

Yes, `(HLH)` kin sheb to tu men kayn:

1. Praymari (HLH) (dεn jεnεtik kכz) : Dis kin kכz fכ wan jεnεtik mכtεshכn. Dis min se dɛn bɔn pɔsin wit prɛdispɔzishɔn fɔ am. Bɔrku tɛm, de sik kin bigin fɔ sho insay di fɔs sɔm ia wae pɔrsin de liv. Na smɔl tɛm nɔmɔ, di sayn dɛn kin apin afta pɔsin dɔn big. Sɔntɛnde dɛn kin kɔl dis ``Familial HLH``.

2. Sɛkɔndari (HLH) (we kin kam wit ɔda mɛrɛsin) : Dis kin apin we yu imyun sistɛm afɛkt ɔda mɛrɛsin we yu dɔn gɛt. Fɔ ɛgzampul, i kin kam wit tin dɛm lɛk kansa, infɛkshɔn (espɛshali di Epstein-Barr vayrɔs), ɔr ɔtoimyun kɔndishɔn. Sεkɔndari (HLH) kin kכmכn pas praymari (HLH).

Udat kin gɛt dis `(HLH)` mɔ?

`(HLH)` I kin divɛlɔp pan ɛnibɔdi we gɛt ɛni ej. Bɔt dɛn kin si am mɔ pan yɔŋ pikin dɛn ɛn bebi dɛn . Bɔt dat nɔ min se big pipul dɛn nɔ go ebul fɔ gɛt am.

Aw kɔmɔn tin dis kin apin?

HLH na wan sik we nɔ kin apin so ɔltɛm . I nɔ kin izi fɔ tɔk ɔmɔs pipul dɛn rili gɛt am, bikɔs sɔntɛnde dɛn kin nɔ no am di rayt we ɔ dɛn nɔ kin no am fayn. Na lɛk 75% pan di pipul dɛn we dɛn no se gɛt HLH gɛt sɛkɔndari HLH. Na 25% nɔmɔ gɛt praymari HLH, we na lɛk wan pan ɛvri 50,000 pipul dɛn ɔlsay na di wɔl.

Wetin na di sayn dɛm fɔ `(HLH)`?

De sayn dɛm fɔ (HLH) kin difrɛn frɔm wan pɔrsin to ɔda pɔrsin, ɛn dɛn kin difrɛn difrɛn wan bay aw di sik tranga. Na sɔm pan de sayn dɛm wae kin kam pan pɔrsin:

• Fiva we nɔ de go ivin wit antibayɔtik.
• Rash na di skin.
• Liva we dɔn big (hepatomegaly).
• di splin de big (splenomegaly).
• Di limf no dɛm we dɔn swɛla (bɔyl).

Tink bɔt dis: If pikin in fiva nɔ go dɔŋ fɔ sɔm dez, ɛn if i nɔ impɔtant ivin afta i dɔn tek mɛrɛsin, i kin bi sayn fɔ `(HLH)`. So, i rili impɔtant fɔ go to dɔktɔ if i kɔntinyu fɔ du am.

Apat frɔm dis, ɔda sayn dɛn kin sho:

• Anemia min se i nɔ gɛt blɔd.
• lכw bכdi pletlet kכnt (`Thrombocytopenia`).
• Wiknɛs, wikɛdnɛs.
• Layt ed ɔ diziz.
• I kin vɛks ɔltɛm ɛn nɔ kin rɛst.
• Ɛd we de at .
• Pale skin.
• Jandis we gɛt di sik.

Bɔt, siriɔs sayn dɛn de bak we kin mek pɔsin in layf de pan denja . If yu si dɛn tin ya, yu fɔ go na ɔspitul wantɛm wantɛm :

• I nɔ izi fɔ blo (dyspnea).
• Di sik dɛn we kin mek pɔsin sik .
• bכdi de bכn insay di yay dεm (`Rεtina εmoraji`).
• Nɔbɔdi nɔ kin no natin.
• Kɔma.

Di tin we impɔtant pas ɔl na fɔ go to dɔktɔ we yu nɔ de te if yu gɛt ɛni wan pan dɛn sik ya, mɔ di wan dɛn we kin mek yu layf de pan denja. Fɔ no di sik kwik kwik wan ɛn fɔ trit am na di bɛst we fɔ gɛt di bɛst rizɔlt.

Wetin mek dis `(HLH)` kin apin? Wetin na di rizin dɛn?

fכ simpul wan, `(HLH)` de kכz fכ di imyun sistεm we de ova aktv כ mכlfכnshכn. As wi bin dɔn tɔk, tu kayn `(HLH)` de, ɛn di tin dɛn we kin mek dɛn gɛt ɛni wan pan dɛn difrɛn. bכt insay di tu kes dεm, tu tכp imyun sistεm sεl dεm, we dεn kכl `histiocytes` εn `T cells`, dεn de prodyuz pasmak εn insted fכ atak εnimi lεk vayrus frכm כdasay, dεn de atak wi כwn bכdi. di simptom dεm fכ `(HLH)` kin apin biכs dεn wayt bכdi sεl dεm ya nכ gεt di instrכkshכn dεm na dεn DNA fכ du dεn wok fayn fayn wan.

Di tin dɛn we kin mek di praymari `(HLH)`:

praymari `(HLH)` na wan jεnεtik mכtεshכn de kכz am. Bɔku jin dɛn de we kin afɛkt dis:

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

Dɛn jin ya na di wan dɛn we de tɛl wi sɛl dɛn fɔ mek prɔtin. dis protin dεm de εp wi imyun sistεm fכ pwεl fכrin invayda dεm lεk baktri dεm εn vayrus dεm εn kip wi hεlth. Bɔt if chenj de na wan pan dɛn jin ya, di sɛl dɛn nɔ kin gɛt di kɔmplit instrɔkshɔn fɔ mek dɛn prɔtin ya. Dɔn di prɔtin dɛn we dɛn mek nɔ kin kɔmplit, ɔ dɛn nɔ kin ebul fɔ du dɛn wok fayn fayn wan insay di bɔdi. dis jin mכtεshכn dεm de kכmכt frכm dεn tu mama εn papa we dεn bεlε. dis dεn kכl am ``autosomal rεsεsiv patεn.''

Di tin dɛn we kin mek sɛkɔndari `(HLH)`:

Sɛkɔndari `(HLH)` na wan kɔndishɔn we dɛn dɔn gɛt. Dis min se nɔto jɛnɛtik prɛdispɔzishɔn we de we dɛn bɔn am, ɛn nid nɔ de fɔ gɛt famili histri. I kin kam bikɔs di imyun sistɛm nɔ de du fayn. Risach stil de go bifo fɔ no wetin mek dis kin apin.

sכmtεm, sεkכnd ``HLH'' kin trigεr bay wan mεdikal kכndyushכn. Dɛn kayn kɔndishɔn dɛn ya na:

• Epstein-Barr vayrɔs (EBV) infεkshɔn.
• Ɔda baktriyal, vayral ɛn fɛns infɛkshɔn dɛn.
• We di imyun sistɛm wik.
• Wan ɔtoimyun kɔndishɔn.
• Wan sik we de mek pɔsin in bɔdi kin wam.
• Rimatɔlɔjik sik dɛm (e.g. juvenail idiopatik atraytis).
• Disɔda dɛn we de mek pɔsin in bɔdi de chenj.
• Kansa (e.g. nɔ-Hɔdgkin limfoma).

Aw dɔktɔ dɛn kin no di sik `(HLH)`?

If dɔktɔ sɔspɛkt se yu gɛt HLH, dɛn go fɔs du yu bɔdi ɛgzam. Dɔn dɛn go ɔda fɔ du sɔm tɛst fɔ no mɔ bɔt yu sik dɛn. Di dɔktɔ go luk fɔ dɛn tin ya we dɛn kin yuz fɔ no di sik:

• Fiva.
• Spleen we dɔn big.
• di rεd bכdi sεl dεm, wayt blכd sεl dεm, כ di pletlεt dεm na di bכdi (cytopenia).
• di ay levul fכ wan kayn fεt we dεn kכl triglisεrayd na di bכdi (hypertriglyceridemia) כ di lכw lεvεl fכ wan protin (fibrinogen) we de εp di bכdi kכlכt (hypofibrinogenemia).
• di damej כ dεstrukshכn fכ di bכdi sεl dεm na di bon mכro (`hεmophagocytosis`).
• di T sel aktiviti dεm we de dכn na di bכdi.
• di inkrεs lεvεl dεm fכ `fεritin` na di bכdi (`fεritinemia`).
• inkrεs lεvεl dεm fכ `sכlubul intalyukin-2 rεsεptכr (sCD25)` na di bכdi.

If yu gɛt at le fayv pan dɛn sik ya, yu dɔktɔ kin sɔprayz se yu gɛt HLH.

Test fɔ no `(HLH)`:

Sɔm tɛst dɛm wae kin ɛp fɔ no `(HLH)` na:

• Jεnεtik tεst (especially if dεn sכspεkt praymar (HLH)).
• Wan kɔmplit blɔd kɔnt.
• כda bכdi tεst fכ chεk fכ `fεritin`, `triglycerides` lεvεl, sayn dεm fכ infεkshכn, εn aw di bכdi de kכlכt.
• Test fɔ di wok we di liva de du.
• Wan bon mɛro bayɔpsi.

Wetin na di tritmɛnt dɛm fɔ `(HLH)`?

Bɔku tritmɛnt dɛn de fɔ (HLH). Dɛn tin ya kin difrɛn fɔ di kayn sik, aw i kin tranga, ɛn aw di pɔsin de sik:

• Mɛrɛsin fɔ trit infɛkshɔn (antibaytik ɔ antivayral).
• mεdisin dεm fכ ridyus di aktiviti fכ di imyun sistεm (immunosuppressants כ cytokine inhibitors).
• Fɔ trit ɔr de manej di ɔndalayn mɛdikal kɔndishɔn dɛm (fɔ ɛgzampul, kemotɛrapi fɔ kansa).
• Blɔd transfyushɔn.

wan nyu mɛrɛsin de we dɛn kɔl `Emapalumab (Gamifant®).` Na `gamma-blocking antibody.` Dɛn dɔn gri fɔ yuz am pan bebi ɛn big pipul dɛn we gɛt `(HLH)`.

If di mɛrɛsin ɔ di we aw dɛn de manej di sik we de ɔnda di sik nɔ wok, dɔktɔ dɛn kin tink bɔt fɔ transplant allogeneic stem cell. dis involv fכ riples yu stεm sεl dεm we nכ de wok fayn (frכm yu bon mכro) wit hεlty stεm sεl dεm frכm wan dכna. Bɔku tɛm, dɛn kin gi bɔku kemotɛrapi ɔ raytin bifo dis fɔ kil di stem sɛl dɛn we nɔ fayn. Dis we aw dɛn kin du am kin rili bad, ɛn bɔku bad bad tin dɛn de we kin apin to am . So yu dɔktɔ go ɛksplen ɔltin to yu so dat yu go disayd fɔ du gud tin bɔt yu wɛlbɔdi biznɛs.

We de fɔ mek `(HLH)` nɔ fɔm?

In rialiti, natin nɔ de we wi kin du fɔ mek di `(HLH)` nɔ divɛlɔp, bikɔs di tin dɛn we de mek wi nɔ ebul fɔ kɔntrol am. Bɔt sɔm tin dɛn de we wi kin du fɔ protɛkt wisɛf frɔm tin dɛn lɛk infɛkshɔn we kin mek pɔsin gɛt sɛkɔndari `(HLH)`:

• It fayn it ɛn ɛksesaiz ɔltɛm.
• Stay away frɔm pipul dɛm wae gɛt vayral sik.
• Wear tin fɔ protɛkt yusɛf fɔ mek yu nɔ wund.
• If wund de, klin am fayn fayn wan fɔ mek yu nɔ gɛt di sik.
• Gud manejmɛnt fɔ di ɔndalayn mɛdikal kɔndishɔn dɛn.

Wetin na di prɔgnosis fɔ (HLH)?

If dɛn no di sik ɛn trit am kwik , dɛn kin op fɔ gɛt gud ɔ fayn fayn tin, i kin dipen pan aw di sayn dɛn kin tranga. Bɔt if dɛn nɔ trit am, ``HLH`` kin mek pɔsin in layf de pan denja . I kin mek di ɔgan dɛn nɔ wok fayn ɛn ivin day insay sɔm mɔnt.

Yu dɔktɔ go ɛksplen to yu aw fɔ no di sik ɛn aw fɔ trit yu sik, ɛn bak di prɔblɛm dɛn ɛn di sayd ɛfɛkt dɛn we di tritmɛnt dɛn kin gɛt.

Bɔrku pipul dɛm wae kin no se dɛn gɛt (HLH) kin luk fɔ sɔpɔt fɔ dɛnsɛf ɛn dɛn famili. Dis min se yu fɔ tɔk to pɔsin we de gi advays bɔt mɛntɛl hεlth , pɔsin we de gi advays bɔt jɛnɛtiks, ɔr soshal wokman fɔ plan aw fɔ liv wit de sik, wetin de pan denja, ɛn wetin go apin to am.

Yu tink se `(HLH)` kin wɛl ɔltogɛda?

Wit di rayt tritmɛnt ɛn kia, di kɔndishɔn `(HLH)` kin go. Bɔt i kin kam bak bak if dɛn trig am bak. sכm pipul dεn bin ebul fכ mek di `(HLH)` nכ kam bak tru `(Stem sεl transplant)` εn avכyd di kכnsekvεns dεm we de mek dεn layf de pan denja. Dɛn de du mɔ risach bɔt dis.

Ustɛm a fɔ go to dɔktɔ?

If yu gɛt sayn fɔ `(HLH)`, go to dɔktɔ wantɛm wantɛm . Nɔ delay, bikɔs fɔ no di sik kwik kwik wan ɛn fɔ trit yu na di bɛst we fɔ mek yu wɛl. If yu gɛt siriɔs sayn lɛk fɔ blo, yu nɔ de no natin, ɔ yu fɔ gɛt sik, yu fɔ go na imejensi rum wantɛm wantɛm .

Us kwɛstyɔn dɛn a fɔ aks di dɔktɔ?

• Us tɛst dɛn a nid fɔ du?
• Us tritmɛnt dɛn yu kin advays?
• Wetin na di sayd ɛfɛkt dɛm we di tritmɛnt kin gɛt?
• Aw di tɛm we a de liv mi layf de?

We yu no se yu ɔ pɔsin we yu lɛk gɛt wan sik we nɔ kin apin so ɔltɛm ɛn we kin mek yu layf de pan denja, dat kin mek yu fil bad ɛn mek yu fil pen. If yu tɔk to pɔsin we de advays yu bɔt mental wɛlbɔdi biznɛs , pɔsin we de advays yu bɔt yu jɛnɛtiks, ɔ yu dɔktɔ, dat kin rili ɛp yu. Dɛn kin ɛp yu fɔ no mɔ bɔt aw fɔ bia wit dis sik, aw fɔ kia fɔ yusɛf ɛn yu famili, ɛn bɔt aw fɔ trit yu ɛn aw fɔ si tin. If yu gɛt kwɛstyɔn dɛn insay dis tranga tɛm, aks fɔ ɛp frɔm di wan dɛn we de nia yu ɔ yu wɛlbɔdi tim.

Di tin we impɔtant pas ɔl fɔ mɛmba (Take-Home Message) .

(HLH) na siriɔs bɔt nɔ kin apin so ɔltɛm we wi imyun sistɛm nɔ de wok fayn. Insay dis, wi yon bɔdi in sɛl dɛn kin atak wi. tu men kayn dεm de: praymari (HLH) we de kכz fכ jεnεtik kכz, εn sεkכnd (HLH) we de kכz fכ כda sik dεm.

Di sayn dɛm kin difrɛn, bɔt yu fɔ tek tɛm if yu gɛt fiva we nɔ de dɔn, yu gɛt lɛsin na yu skin, ɔ yu liva/splin dɔn big. If yu gɛt siriɔs sayn lɛk fɔ mek yu nɔ ebul fɔ blo ɔ fɔ mek yu gɛt sik, go to dɔktɔ wantɛm wantɛm.

I impɔtant fɔ no di sik kwik kwik wan ɛn fɔ trit am. Di tritmɛnt we dɛn kin pik kin difrɛn fɔ di kayn sik. Sɔntɛnde, i kin nid fɔ yuz tin dɛn lɛk fɔ transplant stem sɛl.

Pan ɔl we no shɔ we nɔ de fɔ mek dis nɔ apin, fɔ fala di jenɛral wɛlbɔdi abit kin ridyus di risk fɔ sɛkɔndari `(HLH)`. Insay dis kayn tin, i rili impɔtant fɔ mek yu gɛt strɔng maynd ɛn gɛt di sɔpɔt we yu nid. Nɔto yu wan, nɔ shem fɔ aks fɔ ɛp.

` HLH, Hemophagocytic Lymphohistiocytosis, Imyun sistεm, Jεnεtik sik dεm, Infεkshכn, Fiva, Simptom dεm