I immune system-in a beih theihna tur natna hmuh tur awm lo tak (Hemophagocytic Lymphohistiocytosis - HLH) chungchang i zir dawn a ni.

I taksa chu a chhe zual zel angin i inhriat ngai em? A châng chuan, kan taksa tidamtu, keimahni taksa chakna (immune system) ngei hian min ti na ṭan thei a ni. Chutiang natna khirh tak leh hmuh tur awm lo tak pakhat chu Hemophagocytic Lymphohistiocytosis, a tawi zawngin (HLH) hi a ni. Thil thleng chu kan taksa invenna system ang chi cell te hi thunun theih lohvin an lo pung a, kan taksa peng hrang hrangte chu an rawn bei ta a ni. A dik tak chuan mahni chhungte pawh hre thei lo vengtu ang mai a ni.

`(HLH)` tih awmzia tak chu eng nge ni? A awlsam zawngin i hrethiam ang u, kan hrethiam dawn em ni?

Kan taksa immune system hi mak tak mai a ni. Kan ram humhalhtu sipai ang mai a ni. He system hian pawn lam atanga lo chhuak hmêlma, hrik, virus, leh bacteria te lakah min venghim a ni. Chumi atan chuan cell, protein, taksa peng leh tissue tam tak chuan an thawk dun a ni. Mahse, `(HLH)` vei chuan natna a kai chuan he immune system hi a overstimulated a ni. Tichuan, natna hrik do ai chuan cell lo piang tharte hian kan taksa ngei chu an rawn bei tan ta a ni. Hei hian min ti na zual sauh a. Dik tak chuan he `(HLH)` dinhmun hi nunna atana hlauhawm tak a ni thei .

Heng `(HLH)` hi chi hnih lian tak a awm a ni lawm ni?

Ni e, `(HLH)` hi chi hnih ah then theih a ni:

1. Primary (HLH) (genetically caused) : Hei hi genetic mutation avanga lo awm a ni. Chumi awmzia chu mi chu chutiang duhna neia piang a ni tihna a ni. Natna lanchhuahna hi kum engemaw zat chhungin a lang tan fo thin. A tlem hle a, puitlin hnuah symptoms a lang thin. Hei hi ``Chhungkaw HLH`` an ti \hin.

II. Entirnan, cancer, infection (a bik takin Epstein-Barr virus), emaw autoimmune condition ang chi thil vang a ni thei. Secondary (HLH) hi primary (HLH) aiin a tam zawk.

He `(HLH)` hi tuin nge nei tam zawk?

`(HLH)` Kum eng pawhah a lo thang lian thei. Mahse, naupang tê leh nauteah a lang tam ber . Mahse, chu chu puitlingin an nei thei lo tihna erawh a ni lo.

Hetiang dinhmun hi engtiang chiahin nge a awm?

HLH hi a awm lo hle a ni . Mi engzat nge vei tak tak tih hi sawi chian a harsa a, a chang chuan hriat sual emaw, hriat loh emaw a nih theih avangin. HLH vei hmuhchhuah zinga 75% vel chuan secondary HLH an nei a ni. 25% chauhvin primary HLH an nei a, hei hi khawvel puma mi 50,000 zinga pakhat vel a ni.

`(HLH)` natna lanchhuahna chu engte nge ni?

(HLH) natna lan chhuah dan hi mi hrang hrangah a inang lo thei a, natna nasat dan azirin a danglam thei bawk. Heng a lan chhuah dan tlangpui thenkhat chu hetiang hi a ni:

• Antibiotics hmang pawhin a reh ngai lo khawsik.
• Vun a hring chhuak.
• Thin lian (hepatomegaly) a awm.
• A rilru a buai em em a, a rilru a hah em em bawk a.
• Lymph node a hring (boils) a awm.

Hei hi han ngaihtuah teh: Naupang khawsik ni eng emaw zat a tlahniam lo a, damdawi ei hnu pawha a dam loh chuan `(HLH)` chhinchhiahna a ni thei. Chuvangin a awm reng chuan damdawi lam thurawn lak a pawimawh hle.

Hei bakah hian symptom dang a lang thei bawk:

• Anemia tih awmzia chu thisen tlakchham tihna a ni.
• Thisen platelet a tlem (`Thrombocytopenia`).
• Chak lohna, chak lohna.
• Lu natna emaw, luak chhuak emaw.
• Thinrimna leh rilru hahdam lohna reng reng.
• Lu na .
• Vun dum (pale skin) a ni.
• Jaundice a awm.

Mahse, nunna atana hlauhawm thei chhinchhiahna na tak tak a awm bawk . Hengte hi i hmuh chuan damdawi inah i kal nghal tur a ni :

• Thâwk harsa (dyspnea).
• Seizures a awm thin .
• Mit chhunga thisen chhuak (`Retinal hemorrhages`).
• Thil hriatna hloh.
• Coma ah a awm.

A pawimawh ber chu heng symptoms te hi i neih chuan doctor pan hmasak phawt a ngai a, a bik takin nunna atana hlauhawm thei te hi a ni. A hmaa hriatchhuah leh enkawl hi result tha ber neih theihna tur kawng tha ber a ni.

Engvangin nge hetiang `(HLH)` hi a lo awm? A chhan engte nge ni?

A awlsam zawngin `(HLH)` hi immune system a overactive emaw malfunctioning emaw avanga lo awm a ni. A hmaa kan sawi tawh ang khan `(HLH)` hi chi hnih a awm a, a chhan hrang hrang a inang lo. Mahse heng pahnihah hian immune system cell chi hnih, `histiocytes` leh `T cells` an tih te chu a tam lutuk a siam chhuak a, pawn lam atanga virus ang chi hmelma beih ai chuan keimahni taksa ngei chu an rawn bei ta zawk a ni. `(HLH)` chhinchhiahna hi heng thisen var te hian an hnathawh tur dik taka thawk turin an DNA-ah thupek an neih loh vang a ni.

Primary `(HLH)` awm chhan: 1.1.

Primary `(HLH)` hi genetic mutation avanga lo awm a ni. Hei hi nghawng nei gene engemaw zat a awm a:

• `CD27` tih a ni
• `ILK` tih a ni
• `LYST` tih a ni
• `PRF1` tih a ni
• `RAB27` tih a ni
• `SH2D1A` tih a ni
• `STX11` tih a ni
• `STXBP2` tih a ni
• `UNC13D` tih a ni

Heng genes te hi kan cell te protein siam tura hrilhtu an ni. Heng protein te hian kan immune system te hian ramdang invader bacteria leh virus te a tichhia a, kan hriselna a vawng tha thei a ni. Mahse, heng gene zinga pakhatah hian mutation a awm chuan cell te hian heng protein siam dan tur thupek kimchang an dawng lo. Tichuan protein siam chhuah te chu a kim lo a, a nih loh leh taksa chhungah an hna mumal takin an thawk thei lo. Heng gene mutations te hi naupai lai hian nu leh pa pahnih atanga rochun a ni. Hei hi ``autosomal recessive pattern'' an ti a.

Secondary `(HLH)` awm chhan: 1.1.

Secondary `(HLH)` chu acquired condition a ni. Hei hian pian tirh atanga awm thin genetic predisposition a ni lo tihna a ni a, chhungkaw history pawh a ngai lo tihna a ni. Immune system-in a chhan dan dik lo avanga lo awm a ni. Hetiang thil a thlen chhan hriat tumin zirchianna an la kalpui mek a ni.

A châng chuan, secondary ``HLH'' hi damdawi lam dinhmun avanga lo awm thei a ni. Chutiang dinhmunte chu:

• Epstein-Barr virus (EBV) hrik kai a ni.
• Bacteria, virus leh fungal infection dangte.
• Immune system chak lohna.
• Autoimmune natna a ni.
• Autoinflammatory natna a ni.
• Rheumatological natna (e.g. naupang idiopathic arthritis) a awm thei.
• Metabolic lama harsatna (metabolic disorder) a awm.
• Cancer (e.g. Hodgkin ni lo lymphoma) a ni.

Engtin nge doctor-te hian `(HLH)` natna hi an hriat chhuah?

Doctor-in HLH i vei nia a rinhlelh chuan physical exam an ti hmasa ang. Chumi hnuah chuan i symptoms hriat belh nan test eng emaw zat an order ang. Doctor chuan a hnuaia diagnostic criteria te hi a zawng ang:

• Khawsik.
• Spleen lian tak a awm.
• Thisenah thisen sen (red blood cells), thisen var (white blood cells) emaw, platelets awm zat hniam (cytopenia).
• Thisenah thau chi khat triglyceride an tih tam (hypertriglyceridemia) emaw thisen zam tura puitu protein (fibrinogen) hniam (hypofibrinogenemia) emaw a awm bawk.
• Ruh chhunga thisen cell chhiat emaw tihchhiat emaw (`hemophagocytosis`).
• Thisenah T cell hnathawh a tlahniam.
• Thisenah `ferritin` level a sang (`ferritinemia`).
• Thisenah `soluble interleukin-2 receptor (sCD25)` level a sang chho zel.

Heng symptoms panga tal i neih chuan i doctor chuan HLH hi a ringhlel thei a ni.

`(HLH)` hriat theihna tur test:

`(HLH)` hriat theihna tura pui thei test thenkhat chu:

• Genetic testing (a bik takin primary (HLH) rinhlelh a nih chuan).
• Thisen chhiar kim vek a ni.
• Ferritin, triglycerides level, natna kai chhinchhiahna, leh thisen a inzawm tha em tih enfiah nan thisen test dang neih belh a ni.
• Liver hnathawh dan test.
• Bone marrow biopsy hmanga tih a ni.

`(HLH)` enkawlna tur engte nge ni?

(HLH) hi enkawlna hrang hrang a awm a. Chûngte chu natna chi hrang hrang, a nasat dân leh damlo dinhmun a zirin a danglam thei a ni:

• Infection enkawlna atana damdawi (antibiotics emaw antivirals emaw).
• Immune system hnathawh tihtlem nan damdawi (immunosuppressants emaw cytokine inhibitors emaw).
• Damdawi lam dinhmun (entir nan, cancer enkawlna atana chemotherapy) enkawl emaw enkawl emaw.
• Thisen pek a ni.

Damdawi thar `Emapalumab (Gamifant®) tih a awm a.` `gamma-blocking antibody` a ni.Nausen leh puitling `(HLH)` vei tan hman phal a ni.

Damdawi emaw, natna hnuaia awm enkawlna emaw a hlawhtlin loh chuan doctor-te chuan allogeneic stem cell transplant an ngaihtuah thei a ni. Hei hian i stem cells thawk tha lo (i ruhro atanga) te chu donor atanga stem cell hrisel tak tak hmanga thlak a huam a ni. Hei hi a hma hian stem cell hrisel lote tihhlum nan chemotherapy emaw radiation emaw dose sang tak pek a ni tlangpui. Hetiang tih dan hi a hlauhawm hle a, a side effect pawh a tam hle . Chuvangin i doctor chuan i hriselna chungchangah thutlukna dik tak i siam theih nan engkim a hrilhfiah ang che.

`(HLH)` lo piang tur ven dan a awm em?

A nihna takah chuan `(HLH)` lo thanglian zel tur venna tur hian engmah tih theih a awm lo, a chhan chu kan thunun theih loh a nih avangin. Mahse, secondary `(HLH)` thlen thei infection ang chi thil laka kan invenna tur thil engemaw zat a awm:

• Ei leh in inthlau tak ei la, exercise neih fo bawk ang che.
• Viral natna vei te lakah inthiarfihlim rawh.
• Hliam tuar lo turin protective gear vuah tur a ni.
• Hliam a awm chuan hri kai loh nan uluk takin silfai thin ang che.
• Damdawi lam dinhmun hnuaia awmte enkawl dan tha.

(HLH) hi eng prognosis nge ni?

Natna hi hriatchhuah leh enkawl hmasak a nih chuan , a rah chhuah tha emaw, a tha tawk emaw beisei theih a ni a, chu chu a natna lan chhuahna nasat dan azirin a ni. Mahse, enkawl loh chuan ``HLH`` hi nunna atana hlauhawm a ni . Thla engemaw zat chhungin organ failure leh thihna thlengin a thlen thei a ni.

I doctor chuan i natna lan chhuah dan tur leh enkawl dan tur a hrilhfiah ang che a, chubakah enkawlna avanga hlauhawmna leh a nghawng dan tur a hrilhfiah bawk ang che.

Mi tam tak, nei (HLH) tia inhriat te chuan anmahni leh an chhungte tanpui nan an zawng thin. Hei hian rilru lam hriselna counselor , genetic counselor, a nih loh leh social worker nena inbiakna neihpui a, chu natna nena nun dan tur, a hlauhawmna tur leh a rah chhuah tur chu eng nge ni ang tih ruahmanna siam tihna a ni.

`(HLH)` hi a dam kim thei em?

Enkawlna leh enkawl dan dik a nih chuan `(HLH)` natna hi a bo thei a ni. Mahse, a trigger leh chuan a rawn let leh thei bawk. Mi \henkhat chuan `(Stem cell transplantation)` hmangin `(HLH)` lo awm leh tur chu an veng thei a, nunna atana hlauhawm tak a rah chhuah chu an pumpelh thei bawk. Hemi chungchangah hian zirchianna dang neih belh zel a ni.

Engtikah nge Doctor ka hmuh ang?

`(HLH)` symptoms i neih chuan doctor pan nghal rawh . Tikhawtlai suh, a hmaa natna hriatchhuah leh enkawl hi damna tha ber a nih avangin. Thâwk harsa, hriatna hloh, emaw, seizure ang chi symptom na tak tak i neih chuan emergency room-ah i kal nghal tur a ni .

Doctor hnenah eng zawhna nge ka zawh ang?

• Eng test nge ka tih ngai?
• Eng enkawlna nge i rawt?
• Enkawlna hian eng side effect nge a neih?
• Ka dam rei zawng chu engtin nge a awm?

Nangmah emaw, i hmangaih tak emaw chuan natna hlauhawm tak, nunna atana hlauhawm tak i nei tih hriat chu a lungchhiatthlâk hle thei a, a hrehawm hle thei bawk. Mental health counselor , genetic counselor, a nih loh leh i doctor nena inbiakna neih chu ṭanpuitu ropui tak a ni thei. Anni chuan chu natna hmachhawn dân tûr te, nangmah leh i chhûngte enkawl dân tûr te, leh enkawl dân leh hmathlir dân chungchâng hriat belh tûrin an ṭanpui thei che a ni. He hun harsa karah hian zawhna i neih chuan i hnaih ber emaw, i healthcare team emaw hnen atangin tanpuina dil rawh.

Hriat reng tur pawimawh ber (Take-Home Message) .

(HLH) hi kan taksa hriselna (immune system) hnathawh dan dik lo avanga natna khirh tak, mahse a awm lo hle. Hemiah hian keimahni taksa cell-in min bei a. A chi hnih a awm a, chungte chu primary (HLH) a ni a, chu chu genetic cause avanga lo awm a ni a, secondary (HLH) chu natna dang avanga lo awm a ni.

Symptoms a inang lo a, mahse persistent fever, vun natna, emaw liver/spleen lian zawk i neih chuan fimkhur tur a ni. Thâwk harsa emaw, seizure emaw ang chi symptom na tak tak i neih chuan doctor pan vat rawh.

Hriatchhuah hmasak leh enkawl hi a pawimawh hle. Natna chi hrang hrang a zirin enkawl dan pawh a inang lo. A châng chuan, stem cell transplant ang chi thilte hman a ngai mai thei.

Hei hi ven dan tur chiang tak awm lo mah se, hriselna lama tih dan tlangpui zawm hian secondary `(HLH)` vei theihna a ti tlem thei a ni. Hetiang dinhmunah hian rilru lama chak taka awm reng a, a tul anga tanpuina neih a pawimawh hle. Nangmah chauh i ni lo, tanpuina dil hreh suh.

` HLH, Hemophagocytic Lymphohistiocytosis, Immune system, Genetic natna, Infection, Khawsik, Symptoms