Mina míasrɔ̃ nu tso nɔnɔme aɖe si mebɔ o si nana wò ŋutɔ wò dɔlélenutsiŋutete dzea dziwò (Hemophagocytic Lymphohistiocytosis - HLH) ŋu .

Ðe nèsena le ɖokuiwò me kpɔ be yeƒe ŋutilã le gbegblẽm ɖe edzia? Ɣeaɖewoɣi la, mía ŋutɔwo ƒe dɔlélenutsiŋutete, si daa gbe le míaƒe ŋutilã ŋu, ate ŋu adze dɔ wɔwɔ gɔme na mí. Nɔnɔme sesẽ mawo dometɔ ɖeka si mebɔ o enye Hemophagocytic Lymphohistiocytosis, alo (HLH) kpuie. Nusi dzɔnae nye be lãmenugbagbevi siwo le abe míaƒe ŋutilã ƒe ametakpɔnu ene la dzina ɖe edzi le wo ɖokui si eye wodzea mía ŋutɔwo ƒe ŋutinuwo dzi. Ne míagblɔe tututu la, ele abe dzɔla si mete ŋu dea dzesi eya ŋutɔ ƒe ƒomea o ene.

Nuka tututue `(HLH)` fia? Mina míase egɔme bɔbɔe ŋutɔ, alo?

Míaƒe ŋutilã ƒe dɔlélenutsiŋutete nye mɔ̃ wɔnuku aɖe ŋutɔ. Ele abe aʋakɔ si kpɔa míaƒe dukɔa ta ene. Nuɖoanyi sia kpɔa mía ta tso futɔwo abe dɔlékuiwo, dɔlékuiwo, kple dɔlékui siwo tso egodo ene gbɔ. Lãmenugbagbevi, protein, ŋutinu, kple lãkusi geɖe wɔa dɔ ɖekae hena esia. Gake ne amesi ŋu `(HLH)` le xɔ dɔlékui aɖe la, dɔlélenutsiŋutete sia doa ŋusẽe wògbɔa eme. Emegbe le esi teƒe be lãmenugbagbevi yeyeawo nawɔ avu kple dɔlékuia la, wodzea mía ŋutɔwo míaƒe ŋutilã dzi dzedze gɔme. Esia nana míedzea dɔ wu. Le nyateƒe me la, `(HLH)` ƒe nɔnɔme sia ate ŋu ade ame ƒe agbe afɔku me .

`(HLH)` siawo ƒomevi vevi eve li, alo?

Ẽ, woate ŋu ama `(HLH)` ɖe hatsotso vevi eve me:

1. Gbãtɔ (HLH) (si tso domenyiŋusẽfianu gbɔ) : Esia tsoa domenyiŋusẽfianu ƒe tɔtrɔ gbɔ. Esia fia be wodzia ame kple didi be yeaxɔe. Zi geɖe la, dzesiawo dzea egɔme le eƒe agbenɔƒe ʋɛ gbãtɔwo me. Ƒã ŋutɔ hafi dzesiawo dzena le ame tsitsi megbe. Woyɔa esia ɣeaɖewoɣi be ``Ƒome HLH``.

2. Secondary (HLH) (si tso atikewɔwɔ ƒe nɔnɔme bubu gbɔ) : Esia dzɔna ne atikewɔwɔ ƒe nɔnɔme bubu si le ŋuwò xoxo gblẽ nu le wò dɔlélenutsiŋutete ŋu. Le kpɔɖeŋu me, ate ŋu atso nuwo abe kansa, dɔlékuiwo (vevietɔ Epstein-Barr dɔlékui), alo dɔlélenutsiŋutete ƒe nɔnɔmewo gbɔ. Evelia (HLH) bɔ wu gbãtɔ (HLH).

Amekae ate ŋu axɔ `(HLH)` sia wu?

`(HLH)` Ate ŋu adze egɔme le ame sia ame si xɔ ƒe ɖesiaɖe me. Gake ɖevi suewo kple vidzĩwo ŋue wokpɔnɛ zi geɖe wu . Ke hã, ema mefia be ame tsitsiwo mate ŋu axɔe o.

Aleke gbegbee nɔnɔme sia bɔe?

HLH nye dɔléle si mebɔ kura o . Esesẽ be woagblɔ ame agbɔsɔsɔme si tututu wòle le eŋu, elabena ɣeaɖewoɣi la, woate ŋu akpɔe le mɔ gbegblẽ nu alo womakpɔe nyuie o. Ame siwo ŋu wokpɔ HLH le la dometɔ siwo ade 75% xɔ HLH evelia. 25% koe ŋu HLH gbãtɔ le, si ade ame ɖeka le 50,000 ɖesiaɖe me le xexeame godoo.

Nukae nye `(HLH)` ƒe dzesiwo?

(HLH) ƒe dzesiwo ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ, eye woate ŋu ato vovo le alesi dɔlélea nu sẽe nu. Dzesi siwo bɔ wu dometɔ aɖewoe nye esi:

• Asrã si nu meyina ne wozã dɔlékuiwutikewo gɔ̃ hã o.
• Ŋutilã ƒe ʋuʋudedi.
• Aklã si lolo (aklã ƒe lolome).
• Aɖuɖɔtoe ƒe lolome (splenomegaly).
• Lãmetsiŋusẽ ƒe ʋuka siwo vuvu (ƒoƒo).

Bu esia ŋu kpɔ: Ne ɖevi ƒe dzoxɔxɔ meɖiɖi o ŋkeke geɖe, eye ne meva nyo ɖe edzi le atike nono vɔ megbe gɔ̃ hã o la, ate ŋu anye `(HLH)` ƒe dzesi. Eyata ele vevie ŋutɔ be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo ne eyi edzi.

Tsɔ kpe ɖe esia ŋu la, dzesi bubuwo ate ŋu adze:

• Ʋusɔgbɔdɔ fia ʋu ƒe anyimanɔmanɔ.
• Ʋumenugbagbeviɣidɔ ƒe xexlẽme si bɔbɔ (`Thrombocytopenia`).
• Gbɔdzɔgbɔdzɔ, gbɔdzɔgbɔdzɔ.
• Tagbɔdɔ alo taɖuame.
• Dzikudodo kple dzimaɖitsitsi ɣesiaɣi.
• Taɖuame .
• Ŋutilã si ƒe ŋutigbalẽ le ɣie.
• Jaundice ƒe dɔléle.

Gake dzesi sesẽ aɖewo hã li siwo ate ŋu ade ame ƒe agbe afɔku me . Ne èkpɔ esiawo la, ele be nàyi kɔdzi enumake :

• Yagbɔgbɔ sesẽ (gbɔgbɔtsixetsixe).
• Alɔ̃madɔmadɔ .
• Ʋu si le dodom le ŋkuwo me (`Retinal hemorrhages`).
• Ame ƒe susu meganɔa ame ŋu o.
• Coma.

Nu vevitɔe nye be nàyi ɖɔkta gbɔ kaba ne dzesi siawo dometɔ aɖe le ŋuwò, vevietɔ esiwo ate ŋu ade wò agbe afɔku me. Dɔlélea didi kaba kple edada nye mɔ nyuitɔ siwo dzi woato akpɔ nu nyuitɔ kekeake.

Nukatae `(HLH)` sia dzɔna? Nukawoe nye susu siwo tae?

Kpuie ko la, `(HLH)` tso dɔlélenutsiŋutete ƒe dɔwɔwɔ fũ alo dɔmawɔmawɔ nyuie gbɔ. Abe alesi míegblɔe va yi ene la, `(HLH)` ƒomevi eve li, eye nusiwo gbɔ wo dometɔ ɖesiaɖe tsona la to vovo. Gake le nɔnɔme eveawo siaa me la, wowɔa dɔlélenutsiŋutete ƒe lãmenugbagbevi ƒomevi eve, siwo woyɔna be `histiocytes` kple `T cells`, wògbɔa eme eye le esi teƒe be woadze futɔ abe dɔlékui ene dzi tso egodo la, wodzea mía ŋutɔwo míaƒe ŋutilã dzi. `(HLH)` ƒe dzesiwo dzɔna elabena mɔfiame siwo le ʋumenugbagbevi ɣi siawo me le woƒe DNA me be woawɔ woƒe dɔ nyuie o.

Nusiwo gbɔ `(HLH)` gbãtɔ tsona:

Domenyiŋusẽfianu ƒe tɔtrɔ gbɔe `(HLH)` gbãtɔ tsona. Domenyiŋusẽfianu geɖe li siwo kpɔa ŋusẽ ɖe esia dzi:

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

Domenyiŋusẽfianu siawoe gblɔna na míaƒe lãmenugbagbeviwo be woawɔ protein. Protein siawo kpena ɖe míaƒe dɔlélenutsiŋutete ŋu wòtsrɔ̃a amedzro siwo va dzea mía dzi abe dɔlékuiwo kple dɔlékuiwo ene eye wònana míenɔa lãmesẽ me. Gake ne tɔtrɔ aɖe va le domenyiŋusẽfianu siawo dometɔ ɖeka me la, lãmenugbagbeviawo mexɔa mɔfiame blibo siwo woatsɔ awɔ protein siawo o. Ekema protein siwo wowɔ la mede blibo o, alo womete ŋu wɔa woƒe dɔ nyuie le ŋutilãa me o. Domenyiŋusẽfianu ƒe tɔtrɔ siawo nyea dome tso dzila evea siaa gbɔ le fufɔɣi. Woyɔa esia be ``autosomal recessive pattern.''

Nusiwo hea evelia ƒe `(HLH)` vɛ:

Secondary `(HLH)` nye nɔnɔme si woxɔ. Esia fia be menye domenyiŋusẽfianu ƒe nɔnɔme si nɔa anyi le vidzidzi me o, eye mehiã be woaŋlɔ ƒome ŋutinya o. Dɔlélenutsiŋutete ƒe dɔwɔwɔ si mesɔ o gbɔe wòtsona. Wogale numekuku wɔm be woakpɔ nusitae esia dzɔna.

Ɣeaɖewoɣi la, ``HLH'' evelia ateŋu atso atikewɔwɔ ƒe nɔnɔme aɖe gbɔ. Nɔnɔme mawo dometɔ aɖewoe nye:

• Epstein-Barr dɔlékui (EBV) ƒe dɔléle.
• Dɔlékui, dɔlékui kple fungi ƒe dɔlékui bubuwo.
• Dɔlélenutsiŋutete ƒe gbɔdzɔgbɔdzɔ.
• Dɔlélenutsiŋutete ƒe dɔléle aɖe.
• Dɔléle si nana ame ŋutɔ ƒe dɔléle.
• Lãmetsiŋusẽdɔlélewo (e.g. ɖeviwo ƒe ƒunukpeƒetetedɔ si nye idiopathic arthritis).
• Lãmetsiŋusẽ ƒe dɔlélewo.
• Kansa (e.g. ʋumenugbagbeviɣidɔ si menye Hodgkin o).

Aleke ɖɔktawo wɔa dea dzesi nɔnɔme si nye `(HLH)`?

Ne ɖɔkta aɖe susui be HLH le ŋuwò la, woawɔ ŋutilãmedɔdada gbã. Emegbe woaɖe gbe be woawɔ dodokpɔ geɖe be woanya nu geɖe tso wò dzesiawo ŋu. Ðɔktaa adi dɔléle ŋuti dzidzenu siwo gbɔna:

• Asrã.
• Aɖuɖɔtoe si lolo.
• Ʋumenugbagbevi dzĩwo, ʋumenugbagbevi ɣiwo, alo ʋumenugbagbevi dzĩwo ƒe agbɔsɔsɔ si bɔbɔ ɖe ʋua me (cytopenia).
• Ami ƒomevi aɖe si woyɔna be triglyceride ƒe agbɔsɔsɔ fũ le ʋu me (hypertriglyceridemia) alo protein (fibrinogen) si kpena ɖe ʋu ŋu ƒe agbɔsɔsɔ si mede o (hypofibrinogenemia).
• Ʋumenugbagbevi siwo le ƒumeŋuɖui me ƒe gbegblẽ alo wo tsɔtsrɔ̃ (`hemophagocytosis`).
• T lãmenugbagbeviwo ƒe dɔwɔwɔ dzi ɖe kpɔtɔ le ʋua me.
• `Ferritin` ƒe agbɔsɔsɔ si dzi ɖe edzi le ʋu me (`ferritinemia`).
• `Soluble interleukin-2 receptor (sCD25)` ƒe agbɔsɔsɔ si dzi ɖe edzi le ʋu me.

Ne dzesi siawo dometɔ atɔ̃ ya teti le ŋuwò la, wò ɖɔkta ate ŋu asusu be HLH le ŋuwò.

Dodokpɔ siwo woawɔ atsɔ ade dzesi `(HLH)`:

Dodokpɔ aɖewo siwo ate ŋu akpe ɖe ame ŋu wòanya `(HLH)` enye:

• Domenyiŋusẽfianu dodokpɔ (vevietɔ ne wosusu be gbãtɔ (HLH)).
• Ʋu xexlẽ bliboe.
• Ʋudodokpɔ bubuwo be woatsɔ akpɔ `ferritin`, `triglycerides` ƒe agbɔsɔsɔ, dɔléle ƒe dzesiwo, kple alesi ʋua le ƒoƒom.
• Aklã ƒe dɔwɔwɔ dodokpɔ.
• Ƒumeŋuɖui ŋuti numekuku aɖe.

Nukae nye atikewɔwɔ na `(HLH)`?

Atikewɔwɔ vovovowo li na (HLH). Esiawo ate ŋu ato vovo le dɔlélea ƒomevi, eƒe sesẽme, kple dɔnɔa ƒe nɔnɔme nu:

• Atike siwo woatsɔ ada dɔlékuiwo (dɔlékuiwutikewo alo dɔlékuiwutikewo).
• Atike siwo woatsɔ aɖe dɔlélenutsiŋutete ƒe dɔwɔwɔ dzi akpɔtɔ (immunosuppressants alo cytokine inhibitors).
• Atikewɔwɔ me nɔnɔme siwo gbɔ wòtso la dada alo wo dzi kpɔkpɔ (le kpɔɖeŋu me, atikewɔmɔnu na kansa).
• Ʋudodo.

Atike yeye aɖe li si woyɔna be `Emapalumab (Gamifant®).` Enye `gamma-blocking antibody.` Woda asi ɖe edzi be woazãe le vidzĩwo kple ame tsitsi siwo ŋu `(HLH)` le me.

Ne atikewo alo dɔléle si gbɔ wòtso dzi kpɔkpɔ mewɔ dɔ o la, ɖɔktawo ate ŋu abu allogeneic stem cell dodo na ame ŋu. Esia bia be nàtsɔ lãmenugbagbevi siwo le lãmesẽ me siwo tso nunala aɖe gbɔ aɖɔli wò lãmenugbagbevi siwo mewɔa dɔ nyuie o (siwo tso wò ƒumeŋuɖui me). Zi geɖe la, wozãa atike alo keklẽŋusẽ si sɔ gbɔ do ŋgɔ na esia be woatsɔ awu lãmenugbagbevi siwo mele lãmesẽ me o la. Afɔku le dɔ sia wɔwɔ me ŋutɔ, eye eƒe nugbegblẽ le ame ŋu geɖe . Eyata wò ɖɔkta aɖe nusianu me na wò ale be nàte ŋu atso nya me le wò lãmesẽ ŋu nyuie.

Ðe mɔ aɖe li si dzi woato axe mɔ ɖe `(HLH)` ƒe wɔwɔme nua?

Le nyateƒe me la, naneke meli míate ŋu awɔ atsɔ axe mɔ ɖe `(HLH)` ƒe ŋgɔyiyi nu o, elabena nusiwo gbɔ wòtso la gbɔ míaƒe ŋusẽ ŋu. Ke hã, nu aɖewo li míate ŋu awɔ atsɔ akpɔ mía ɖokui ta tso nuwo abe dɔlékui siwo ate ŋu ahe `(HLH)` evelia vɛ me:

• Ðu nuɖuɖu si da sɔ eye nàde kame edziedzi.
• Te ɖa tso amesiwo ŋu dɔlékuiwo ƒe dɔlélewo le gbɔ.
• Do ametakpɔnu be màgaxɔ abi o.
• Ne abiwo do la, kɔ wo ŋu nyuie be dɔlékuiwo nagage ɖe wo me o.
• Atikewɔwɔ ƒe nɔnɔme siwo le ete la dzi kpɔkpɔ nyuie.

Nukae nye (HLH) ƒe ŋgɔdonya?

Ne wokpɔ dɔlélea eye wodae kaba , woate ŋu akpɔ mɔ be nu nyui alo nu nyui aɖe ado tso eme, le alesi dzesiawo nu sẽe nu. Gake ne womewɔ atike na ``HLH`` o la, edea ame ƒe agbe afɔku me . Ate ŋu ana ŋutinuwo nawɔ dɔ nyuie o eye wòaku gɔ̃ hã le ɣleti ʋɛ aɖewo ko me.

Wò ɖɔkta aɖe dɔlélea didi kple atikewɔmɔnu siwo woate ŋu azã na wò dzesiwo me na wò, kpakple afɔku kple nugbegblẽ siwo le atikewɔwɔawo me.

Ame geɖe siwo gblɔna be (HLH) le yewo si la dia kpekpeɖeŋu na yewo ɖokui kple yewoƒe ƒomewo. Esia fia be nàƒo nu kple susumedɔléle ŋuti aɖaŋuɖola , domenyiŋusẽfianu ŋuti aɖaŋuɖola, alo hadomekpekpeɖeŋudɔwɔla be nàwɔ ɖoɖo ɖe alesi woanɔ agbe ɖe nɔnɔmea nu, afɔku siwo le eme, kple nusiwo ado tso eme ŋu.

Ðe woate ŋu ada `(HLH)` keŋkeŋa?

Ne wowɔ atike nɛ nyuie eye wokpɔ egbɔ nyuie la, nɔnɔme `(HLH)` ate ŋu nu ayi. Gake ate ŋu atrɔ ava hã ne wogade dzoe ake. Ame aɖewo te ŋu xe mɔ ɖe `(HLH)` ƒe gbugbɔgadzɔ nu to `(Stem cell transplantation)` dzi eye woƒo asa na emetsonu siwo ate ŋu ade woƒe agbe afɔku me. Wole numekuku bubuwo wɔm tso esia ŋu.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne `(HLH)` ƒe dzesiwo le ŋuwò la, yi ɖɔkta enumake . Mègahe ɖe megbe o, elabena dɔlélea didi kaba kple edadae nye mɔ nyuitɔ siwo dzi nàto ahaya. Ne dzesi sesẽwo abe gbɔgbɔ sesẽ, susu ƒe dɔmawɔmawɔ nyuie, alo alɔ̃madɔmadɔ ene le ŋuwò la, ele be nàyi xɔ si me wowɔa dɔ kpata le enumake .

Nya kawoe wòle be mabia ɖɔktaa?

• Dodokpɔ kawoe wòle be mawɔ?
• Atikewɔwɔ kawoe nèkafu?
• Nukawoe nye atikewɔwɔa ƒe nugbegblẽ le ame ŋu?
• Aleke nye agbenɔƒe le?

Ne èva nya be dɔléle aɖe si mebɔ o si ate ŋu ade wò agbe afɔku me le ŋuwò alo wò ame vevi aɖe ate ŋu aɖe fu na wò ŋutɔ eye wòase veve. Nuƒoƒo kple susumedɔléle ŋuti aɖaŋuɖola , domenyiŋusẽfianu ŋuti aɖaŋuɖola, alo wò ɖɔkta ate ŋu akpe ɖe ŋuwò ŋutɔ. Woate ŋu akpe ɖe ŋuwò nàsrɔ̃ nu geɖe tso ale si nànɔ te ɖe nɔnɔmea nu, ale si nàlé be na ɖokuiwò kple wò ƒomea, kpakple atikewɔwɔ kple nukpɔsusu si anɔ asiwò ŋu. Ne nya aɖewo le asiwò le ɣeyiɣi sesẽ sia me la, bia kpekpeɖeŋu tso amesiwo te ɖe ŋuwò alo wò lãmesẽnyawo gbɔ kpɔha gbɔ.

Nu vevitɔ kekeake si dzi wòle be nàɖo ŋkui (Take-Home Message) .

(HLH) nye dɔléle sesẽ aɖe si mebɔ o si míaƒe dɔlélenutsiŋutete ƒe dɔwɔwɔ si mesɔ o hena vɛ. Le esia me la, mía ŋutɔwo míaƒe ŋutilã ƒe lãmenugbagbeviwo dzea mía dzi. Wole ƒomevi vevi eve: gbãtɔ (HLH) si tso domenyiŋusẽfianu gbɔ, kple evelia (HLH) si tso dɔléle bubuwo gbɔ.

Dzesiawo to vovo, gake ele be nàɖɔ ŋu ɖo ne dzoxɔxɔ si nu metsina o, ŋutigbalẽ ƒe abi le ŋuwò, alo aklã/aɖuɖɔtoe si lolo. Ne èkpɔ dzesi sesẽwo abe gbɔgbɔ sesẽ alo alɔ̃madɔmadɔ ene la, yi atikewɔƒe enumake.

Ele vevie be woakpɔ dɔlélea kaba ahadae. Atikewɔwɔ ƒe tiatia vovovowo le dɔlélea ƒomevi nu. Ɣeaɖewoɣi la, ate ŋu ahiã be woato nuwo abe lãmenugbagbeviwo dodo na ame ene dzi.

Togbɔ be mɔ si ŋu kakaɖedzi le meli si dzi woato axe mɔ ɖe esia nu o hã la, lãmesẽmenɔnɔ ƒe nɔnɔme siwo bɔ dzi wɔwɔ ate ŋu aɖe `(HLH)` evelia ƒe afɔkua dzi akpɔtɔ. Le nɔnɔme sia tɔgbe me la, ele vevie ŋutɔ be wò susu nasẽ eye nàxɔ kpekpeɖeŋu si hiã. Menye wò ɖeka koe o, mègahe ɖe megbe le kpekpeɖeŋu biabia me o.

` HLH, Hemophagocytic Lymphohistiocytosis, Dɔlélenutsiŋutete, Domenyiŋusẽfianu ƒe dɔlélewo, Dɔlékuiwo, Dzoxɔxɔ, Dzesiwo