Ka tuyige ku mbeera etali ya bulijjo ereetera abaserikale b’omubiri bo okulumba (Hemophagocytic Lymphohistiocytosis - HLH)

Owulira nga towulirangako? Oluusi abaserikale baffe bennyini abaziyiza endwadde, eyo ewonya omubiri gwaffe, bayinza okutandika okutulwaza. Emu ku mbeera ng’ezo ez’amaanyi era ezitatera kubaawo ye Hemophagocytic Lymphohistiocytosis, oba (HLH) mu bufunze. Ekibaawo kwe kuba nti obutoffaali obulinga enkola y’omubiri gwaffe obw’okwekuuma bweyongera nga tebufugibwa ne bulumba ebitundu byaffe. Okubeera omutuufu, kiringa omukuumi atasobola kutegeera maka ge.

Mu butuufu `(HLH)` kitegeeza ki? Ka tukitegeere mu ngeri ennyangu ennyo, nedda?

Abaserikale b’omubiri gwaffe enkola eyeewuunyisa nnyo. Kiringa eggye erikuuma eggwanga lyaffe. Enkola eno etukuuma okuva ku balabe nga obuwuka, akawuka, ne bakitiriya abava ebweru. Obutoffaali bungi, puloteyina, ebitundu by’omubiri, n’ebitundu by’omubiri bikolagana ku kino. Wabula omuntu alina `(HLH)` bw'afuna yinfekisoni, abaserikale bano abaserikale basusse okusikirizibwa. Olwo, mu kifo ky’okulwanyisa obuwuka buno, obutoffaali obupya obutondeddwawo butandika okulumba omubiri gwaffe. Kino kituleetera okulwala. Mu butuufu, embeera eno eya `(HLH)` eyinza okuba ey'obulamu mu matigga .

Waliwo ebika bibiri ebikulu ebya bino `(HLH)`, nedda?

Yee, `(HLH)` esobola okwawulwamu ebika bibiri ebikulu:

1. Primary (HLH) (genetically caused) : Kino kiva ku nkyukakyuka mu buzaale. Kino kitegeeza nti omuntu azaalibwa ng’alina omuze gw’okugufuna. Obubonero butera okutandika okulabika mu myaka egisooka egy’obulamu. Tekitera nnyo, obubonero bulabika oluvannyuma lw’okukula. Kino oluusi kiyitibwa ``Familial HLH``.

2. Secondary (HLH) (ekiva ku mbeera endala ey’obujjanjabi) : Kino kibaawo ng’abaserikale b’omubiri bo bakosebwa embeera endala ey’obujjanjabi gy’olina edda. Okugeza, kiyinza okuva ku bintu nga kookolo, yinfekisoni (naddala akawuka ka Epstein-Barr), oba embeera z’abaserikale b’omubiri. Secondary (HLH) esinga kubeera primary (HLH).

Ani asinga okukulaakulanya `(HLH)` eno?

`(HLH)` Kiyinza okukula mu muntu yenna ow'emyaka gyonna. Kyokka, kisinga kulabibwa mu baana abato n’abalongo . Kyokka ekyo tekitegeeza nti abantu abakulu tebasobola kubufuna.

Embeera eno ya bulijjo etya?

HLH mbeera ya bbula nnyo . Kizibu okwogera abantu bameka ddala ababulina, kubanga oluusi buyinza okuzuulibwa obubi oba obutazuulibwa bulungi. Abantu nga 75% abazuuliddwa nga balina HLH balina HLH ey’okubiri. Ebitundu 25% bokka be balina HLH eyasooka, nga eno ebeera nga omu ku buli bantu 50,000 mu nsi yonna.

Bubonero ki obwa `(HLH)`?

Obubonero bwa (HLH) busobola okwawukana okusinziira ku muntu, era buyinza okwawukana okusinziira ku buzibu bw’obulwadde. Bino bye bimu ku bubonero obusinga okulabika:

• Omusujja ogutaggwaawo ne bwe bakozesa eddagala eritta obuwuka.
• Okusiiyibwa kw’olususu.
• Ekibumba ekigazi (hepatomegaly).
• Okugaziwa kw’ennywanto (splenomegaly).
• Ensigo ezizimba (ebizimba).

Lowooza ku kino: Singa omusujja gw’omwana tegukendeera okumala ennaku eziwera, era bwe gutatereera ne bwe gumala okumuwa eddagala, kiyinza okuba akabonero ka `(HLH)`. N’olwekyo, kikulu nnyo okunoonya amagezi g’abasawo singa kiba kinyweredde.

Ng’oggyeeko kino, obubonero obulala buyinza okulabika:

• Okukendeera kw’omusaayi kitegeeza obutaba na musaayi.
• Omuwendo gw’obutoffaali obutono mu musaayi (`Thrombocytopenia`).
• Obunafu, obunafu.
• Okuziyira oba okuziyira.
• Okunyiiga buli kiseera n’obutawummula.
• Okulumwa omutwe .
• Olususu olumyufu.
• Obulwadde bwa Jaundice.

Kyokka, waliwo n’obubonero obw’amaanyi obuyinza okutta omuntu . Bwolaba bino, olina okugenda mu ddwaaliro mu bwangu :

• Obuzibu mu kussa (dyspnea).
• Okukonziba .
• Okuvaamu omusaayi munda mu maaso (`Retinal hemorrhages`).
• Okufiirwa okutegeera.
• Coma.

Ekisinga obukulu kwe kulaba omusawo awatali kulwawo singa oba olina obubonero buno naddala obuyinza okutta obulamu. Okuzuula amangu n’okujjanjaba y’engeri esinga obulungi ey’okufunamu ebirungi.

Lwaki kino `(HLH)` kibaawo? Ensonga ze ziruwa?

Mu ngeri ennyangu, `(HLH)` kiva ku baserikale b’omubiri okukola ennyo oba obutakola bulungi. Nga bwe twayogeddeko emabegako, waliwo ebika bibiri ebya `(HLH)`, era ebivaako buli kimu bya njawulo. Naye mu mbeera zombi, ebika bibiri eby’obutoffaali bw’abaserikale b’omubiri, obuyitibwa `histiocytes` ne `T cells`, bikolebwa mu kisusse era mu kifo ky’okulumba omulabe nga akawuka okuva ebweru, bilumba omubiri gwaffe. Obubonero bwa `(HLH)` bubaawo kubanga obutoffaali buno obweru tebulina biragiro mu DNA yaabyo okukola omulimu gwabwo obulungi.

Ebivaako `(HLH)` enkulu:

Primary `(HLH)` kiva ku nkyukakyuka mu buzaale. Waliwo obuzaale obuwerako obukosa kino:

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

Obuzaale buno bwe bugamba obutoffaali bwaffe okukola puloteyina. Proteins zino ziyamba abaserikale baffe abaserikale okusaanyaawo ebirumba ebiva ebweru nga bakitiriya ne virus ne batukuuma nga tuli balamu bulungi. Kyokka, singa wabaawo enkyukakyuka mu emu ku buzaale buno, obutoffaali tebufuna biragiro bijjuvu okukola puloteyina zino. Olwo obutoffaali obukolebwa tebutuukiridde, oba tebusobola kukola bulungi mulimu gwabyo munda mu mubiri. Enkyukakyuka zino ez’obuzaale zisikira abazadde bombi nga bafunye olubuto. Kino kiyitibwa ``enkola ya autosomal recessive pattern.''

Ebivaako `(HLH)` eyokubiri:

Secondary `(HLH)` mbeera efunibwa. Kino kitegeeza nti si buzaale obubeerawo ng’azaalibwa, era tekyetaagisa kuba na byafaayo bya maka. Kiva ku kuddamu okutali kwa bulijjo kw’abaserikale b’omubiri. Okunoonyereza kukyagenda mu maaso okuzuula lwaki kino kibaawo.

Oluusi, ``HLH'' ey'okubiri esobola okuva ku mbeera y'obujjanjabi. Embeera ng’ezo mulimu:

• Obulwadde bw’akawuka ka Epstein-Barr (EBV).
• Yinfekisoni endala eziva ku bakitiriya, akawuka ne ffene.
• Okunafuwa kw’abaserikale b’omubiri.
• Embeera y’abaserikale b’omubiri (autoimmune).
• Obulwadde obuyitibwa autoinflammatory disease.
• Endwadde z’enkizi (e.g. juvenile idiopathic arthritis).
• Obuzibu mu nkyukakyuka y’emmere.
• Kookolo (e.g. obulwadde bwa non-Hodgkin lymphoma).

Abasawo bazuula batya embeera ya `(HLH)`?

Singa omusawo ateebereza nti olina HLH, bajja kusooka kukeberebwa mubiri. Olwo bajja kulagira okukeberebwa okuwerako okumanya ebisingawo ku bubonero bwo. Omusawo ajja kunoonya emitendera gino wammanga egy’okuzuula obulwadde:

• Omusujja.
• Ensigo ezigaziwa.
• Obutoffaali obumyufu, obutoffaali obweru oba platelets mu musaayi obutono (cytopenia).
• Ebika by’amasavu ebiyitibwa triglyceride ebingi mu musaayi (hypertriglyceridemia) oba obutoffaali obutono (fibrinogen) obuyamba omusaayi okuzimba (hypofibrinogenemia).
• Okwonooneka oba okusaanyaawo obutoffaali bw’omusaayi mu busimu bw’amagumba (`hemophagocytosis`).
• Okukendeera kw’emirimu gy’obutoffaali bwa T mu musaayi.
• Okweyongera kw’emiwendo gya `ferritin` mu musaayi (`ferritinemia`).
• Okwongera ku miwendo gya `soluble interleukin-2 receptor (sCD25)` mu musaayi.

Bw’oba ​​olina obubonero buno waakiri butaano , omusawo ayinza okuteebereza HLH.

Ebigezo okuzuula `(HLH)`:

Ebimu ku bigezo ebiyinza okuyamba okuzuula `(HLH)` bye bino:

• Okukebera obuzaale (naddala nga primary (HLH) eteeberezebwa).
• Okubala omusaayi mu bujjuvu.
• Okukebera omusaayi okulala okukebera oba ferritin, triglycerides, obubonero obulaga nti omuntu alina obulwadde, n’engeri omusaayi gye guzimbamu obulungi.
• Okukebera enkola y’ekibumba.
• Okukebera obusimu bw’amagumba.

Bujjanjabi ki obwa `(HLH)`?

Waliwo obujjanjabi obuwerako ku (HLH). Bino biyinza okwawukana okusinziira ku kika ky’obulwadde, obuzibu bwabwo, n’embeera y’omulwadde:

• Eddagala erijjanjaba yinfekisoni (eddagala eritta obuwuka oba eritta akawuka).
• Eddagala okukendeeza ku mirimu gy’abaserikale b’omubiri (immunosuppressants oba cytokine inhibitors).
• Okujjanjaba oba okuddukanya embeera z‟obujjanjabi ezisibukako (okugeza, eddagala eriweweeza ku kookolo).
• Okuteekebwamu omusaayi.

Waliwo eddagala eppya eriyitibwa `Emapalumab (Gamifant®).` Lino `gamma-blocking antibody.` Likkirizibwa okukozesebwa mu baana abawere n'abantu abakulu abalina `(HLH)`.

Singa eddagala oba enzirukanya y’obulwadde obusibukako tekola, abasawo bayinza okulowooza ku ky’okukyusa obutoffaali obusibuka mu mubiri (allogeneic stem cell transplant). Kino kizingiramu okukyusa obutoffaali bwo obutakola bulungi (obuva mu busimu bw’amagumba go) n’ossaamu obutoffaali obulamu obulungi okuva mu muntu agaba. Kino kitera okukulemberwa obujjanjabi obw’amaanyi obw’eddagala oba obusannyalazo okutta obutoffaali obusibuka mu mubiri obutali bulamu. Enkola eno ya bulabe nnyo, era erimu ebizibu bingi . Kale omusawo wo ajja kukunnyonnyola buli kimu osobole okusalawo mu ngeri ey’amagezi ku bulamu bwo.

Waliwo engeri y'okuziyiza okutondebwawo kwa `(HLH)`?

Mu butuufu, tewali kye tuyinza kukola kulemesa nkulaakulana ya `(HLH)`, anti ebivaako bisukka obuyinza bwaffe. Naye waliwo ebintu bye tuyinza okukola okwekuuma ebintu nga yinfekisoni eziyinza okuleeta `(HLH)` ez'okubiri:

• Lya emmere ennungi era dduyiro buli kiseera.
• Weewale abantu abalina endwadde z’akawuka.
• Yambala eby’okwekuuma obutafuna buvune.
• Singa ebiwundu bibaawo, biyonje bulungi okuziyiza obuwuka.
• Enzirukanya ennungi ey’embeera z’obujjanjabi ezisirikitu.

Enteekateeka y’okuteebereza (HLH) eri etya?

Singa obulwadde buzuulibwa era ne bujjanjabwa nga bukyali , ekivaamu ekirungi oba ekirungi ennyo kiyinza okusuubirwa, okusinziira ku buzibu bw’obubonero. Naye singa erekebwa nga tejjanjabiddwa, ``HLH`` ebeera ya bulabe eri obulamu . Kiyinza okuvaako ebitundu by’omubiri okulemererwa n’okufa mu myezi mitono.

Omusawo ajja kukunnyonnyola engeri y’okuzuulamu n’engeri y’okujjanjaba obubonero bwo, awamu n’obulabe n’ebizibu ebiva mu bujjanjabi obwo.

Abantu bangi abeeyita abalina (HLH) banoonya obuyambi eri bo bennyini n‟amaka gaabwe. Kino kitegeeza okwogera n ‟omubuulirizi w‟obulamu bw‟omutwe , omubuulirizi w‟obuzaale, oba omukozi w‟ensonga z‟abantu okuteekateeka engeri y‟okubeera n‟embeera eno, obulabe bwe buli, n‟ebinaavaamu.

`(HLH)` esobola okuwona ddala?

Bw’ofuna obujjanjabi obulungi n’okulabirira, embeera `(HLH)` esobola okugenda. Kyokka era esobola okudda singa eddamu okusindikibwa. Abantu abamu basobodde okuziyiza okuddamu kwa `(HLH)` nga bayita mu `(Stem cell transplantation)` ne beewala ebiyinza okuvaamu obulamu. Okunoonyereza okulala kugenda mu maaso ku nsonga eno.

Ddi lwe nnina okulaba omusawo?

Bwoba olina obubonero bwa `(HLH)`, laba omusawo mu bwangu . Tolwawo kubanga okuzuula amangu n’okubujjanjaba y’engeri esinga okuwona. Bw’oba ​​olina obubonero obw’amaanyi ng’okukaluubirirwa okussa, okuzirika, oba okukonziba, olina okugenda mu kisenge eky’amangu amangu ddala .

Bibuuzo ki bye nnina okubuuza omusawo?

• Bigezo ki bye nneetaaga okukola?
• Bujjanjabi ki bw’oteesa?
• Biki ebiva mu bujjanjabi buno?
• Obulamu bwange buli butya?

Okukizuula nti ggwe oba omuntu wo alina embeera etatera kulabika era eyinza okumutta obulamu kiyinza okukunyiiza ennyo era n’okukuluma. Okwogera n’omubuulirizi w’ebyobulamu bw’obwongo , omubuulirizi ku by’obuzaale, oba omusawo wo kiyinza okukuyamba ennyo. Ziyinza okukuyamba okumanya ebisingawo ku ngeri gy’oyinza okugumira embeera eno, engeri gy’oyinza okwerabirira n’ab’omu maka go, n’engeri y’okujjanjabibwamu n’engeri gy’oyinza okutunuuliramu. Bw’oba ​​olina ebibuuzo mu kiseera kino ekizibu, saba obuyambi okuva eri abo abakuli ku lusegere oba ttiimu yo ey’ebyobulamu.

Ekintu ekisinga obukulu okujjukira (Take-Home Message)

(HLH) mbeera ya maanyi naye nga tetera kubaawo ereetebwa enkola y’abaserikale b’omubiri gyaffe mu ngeri etaali ya bulijjo. Mu kino, obutoffaali bw’omubiri gwaffe bwennyini butulumba. Waliwo ebika bibiri ebikulu: primary (HLH) ekiva ku buzaale, ne secondary (HLH) ekiva ku ndwadde endala.

Obubonero bwawukana, naye olina okwegendereza singa oba olina omusujja ogutaggwaawo, ebiwundu ku lususu, oba ekibumba/ennywanto egaziye. Bw’ofuna obubonero obw’amaanyi ng’okukaluubirirwa okussa oba okukonziba, genda mangu omusawo.

Okuzuula amangu n’okujjanjabibwa kyetaagisa nnyo. Enkola z’obujjanjabi zaawukana okusinziira ku kika ky’obulwadde. Oluusi, kiyinza okwetaagisa okuddukira mu bintu ng’okusimbuliza obutoffaali obusibuka mu mubiri.

Wadde nga tewali ngeri nkakafu ey’okuziyiza kino, okugoberera emize gy’ebyobulamu egy’awamu kiyinza okukendeeza ku bulabe bw’obulwadde bwa `(HLH)` obw’okubiri. Mu mbeera nga eno, kikulu nnyo okusigala ng’oli mugumu mu birowoozo n’okufuna obuwagizi obwetaagisa. Toli wekka, era tolwawo kusaba buyambi.

` HLH, Hemophagocytic Lymphohistiocytosis, Abaserikale b’omubiri, Endwadde z’obuzaale, Yinfekisoni, Omusujja, Obubonero