Ngaba unengxaki yokubala iiseli ezimhlophe egazini lakho? Masifunde nge-Autoimmune Neutropenia (AIN)!

Ngaba wakha wazibuza ukuba kutheni inkqubo yethu yokuzikhusela, endaweni yokusikhusela, ngamanye amaxesha inokuqala ukuhlasela iiseli zethu? Leyo yimeko enzima kancinci, kodwa iyaqondakala ebizwa ngokuba yi -Autoimmune Neutropenia (AIN) . Okwenzekayo kukuba inkqubo yethu yokuzikhusela ihlasela uhlobo olubaluleke kakhulu lweeseli ezimhlophe zegazi emzimbeni wethu, ezibizwa ngokuba yi-'neutrophils.' Ngoko masithethe ngale nto ngokulula, ngendlela onokuyiqonda.

Ngoko ke, yintoni i-Autoimmune Neutropenia (AIN)?

Ngamafutshane, i-'Autoimmune Neutropenia' (AIN) yimeko enxulumene negazi. Okwenzekayo apha kukuba inkqubo yethu yomzimba yokuzikhusela, ekufanele ukuba isikhusele, ihlasela ngempazamo iiseli zethu ezimhlophe zegazi ezibizwa ngokuba yi-'neutrophils'. Khawuthelekelele oku, njengomkhosi okhusela ilizwe lethu ngempazamo uhlasela abantu bethu.

Zithini iiNeutrophils?

IiNeutrophils zizinto ezibaluleke kakhulu kwinkqubo yethu yokuzikhusela komzimba. Zifana namajoni amancinci emizimbeni yethu. Umsebenzi wazo ophambili kukufumana nokutshabalalisa izinto eziyingozi ezingena emzimbeni, ezifana neebhaktheriya kunye neefungi ezibangela izifo. Ngoko ke, kwimeko ye-'Autoimmune Neutropenia', inkqubo yethu yokuzikhusela komzimba itshabalalisa ezi seli zixabisekileyo ze-'neutrophils', ngoko ke inani le-'neutrophils' egazini lehla ngaphantsi kwamanqanaba aqhelekileyo.

Kukho iintlobo ezimbini eziphambili ze-AIN:

1. I-Primary Autoimmune Neutropenia (AIN): Oku kubonakala kakhulu kwiintsana nakubantwana abancinci . Okwenzekayo apha kukuba inani le-'neutrophils' liyehla ngaphandle kwesizathu esinye. Kodwa iindaba ezimnandi zezokuba le meko idla ngokuzilungisa kwiminyaka embalwa.

2. I-Secondary Autoimmune Neutropenia (AIN): Oku kuxhaphake kakhulu kubantwana abadala nakubantu abadala . Apha, inani eliphantsi le-neutrophil libangelwa yenye imeko yezonyango. Umzekelo, inokubangelwa lusulelo, esinye isifo segazi, okanye esinye isifo sokuzikhusela komzimba (imeko apho inkqubo yokuzikhusela yomzimba ihlasela umzimba wayo). Le meko yesibini ye-AIN idla ngokuba yengapheliyo .

Liphantsi kangakanani inani lee-neutrophils ezibizwa ngokuba yi-neutropenia?

I-Autoimmune Neutropenia lolunye uhlobo lwesifo esibanzi esibizwa ngokuba yiNeutropenia. I-Neutropenia ihlelwa njengethambileyo, ephakathi, okanye enzima, kuxhomekeke kwinani lee-neutrophils nge-microliter nganye (microL) yegazi.

• I-Neutropenia Encinci: 1,000 – 1,500 ii-neutrophils nge-microL nganye.
• I-Neutropenia ephakathi: 500 – 1,000 ii-neutrophils kwi-microL nganye.
• I-Neutropenia Eqatha: Ii-Neutrophils ezingaphantsi kwama-500 kwi-microL nganye.

Eyona nto ibalulekileyo kukuba la maxabiso aqinisekiswe ngugqirha. Ukuba unamathandabuzo malunga noku, kubaluleke kakhulu ukufuna ingcebiso kagqirha.

I-AIN iwuchaphazela njani umzimba wam?

Indlela i-AIN ekuchaphazela ngayo inokwahluka kumntu nomntu. Kuxhomekeke kwizinto ezininzi, ngoko ke kungcono ukuthetha nogqirha wakho ngale nto. Ukuba inani lee-neutrophil zakho liyehla kakhulu, unamathuba amaninzi okufumana usulelo . Ngamanye amaxesha olu sulelo lunokuba yingozi ebomini, ingakumbi ukuba awufumani nyango ngokukhawuleza.

Nangona kunjalo, kwezinye iimeko, i-autoimmune neutropenia inokubangela usulelo olungephi. Okanye inokuba neempawu ezingabonakaliyo. Yiyo loo nto kubalulekile ukusebenzisana nogqirha ukuze ufumane ukuba yintoni ebangela i-neutropenia yakho. Ngokuxhomekeke kwisizathu, ugqirha wakho unokujonga imeko yakho aze akuncede ukulawula usulelo.

Ngubani ofumana i-Autoimmune Neutropenia (AIN)?

• I-AIN eyintloko: Oku kuchaphazela kakhulu iintsana nabantwana abancinci . Idla ngokuchongwa xa sele beneminyaka eyi-8 ubudala.
• I-AIN yesibini: Oku kudla ngokuvela kwesinye isifo sokuzikhusela komzimba, esifana ne-lupus okanye i-rheumatoid arthritis. Abantwana abane-AIN yesibini badla ngokufunyaniswa bemalunga neminyaka eli-10 ubudala. Abantu abadala abane-AIN badla ngokufunyaniswa bephakathi kweminyaka engama-40 nama-60 ubudala.

Ixhaphake kangakanani i-autoimmune neutropenia?

I-AIN yokuqala yimeko engaqhelekanga kakhulu . Ichaphazela umntwana omnye kuphela kwi-100,000 yabantwana abangaphantsi kweminyaka eli-10 ubudala. Ngoko ke ungabona ukuba ayiqhelekanga kangakanani.

Ziziphi iimpawu ze-Autoimmune Neutropenia?

I-Autoimmune Neutropenia ayisoloko ibangela iimpawu ezinkulu. Nangona kunjalo, ngamanye amaxesha unokubona iimpawu zosulelo. Umzekelo:

• Usulelo lwendlebe: Olu lolunye lweempawu ezixhaphakileyo. Khawucinge ukuba umntwana wakho omncinci uhlala ehlushwa yindlebe, kwaye xa usiya kugqirha, bakuxelela ukuba lolunye usulelo lwendlebe.
• Izifo zokuphefumla: Umkhuhlane oqhelekileyo, ukukhwehlela, kunye nomkhuhlane.
• Usulelo lomlomo: Izinto ezifana nezilonda zomlomo kunye ne-gingivitis.
• Usulelo lwesikhumba.
• Ukukrala kwemiphunga.
• Isifo seMeningitis.
• Usulelo lwendlela yomchamo (UTIs).
• I-Sepsis: Olu luhlobo losulelo oluyingozi kakhulu nolusongela ubomi.

Iintsholongwane ezinxulumene ne-AIN yokuqala zihlala zincinci, kodwa izifo ezinzulu ezifana ne-pneumonia, i-meningitis, kunye ne-sepsis zixhaphake kakhulu kwi-AIN yesibini.

Yintoni ebangela i-autoimmune neutropenia?

I-AIN ihlelwa njengeyona iphambili okanye eyesibini ngokuxhomekeke kwisizathu samanqanaba aphantsi e-neutrophil.

I-Primary Autoimmune Neutropenia (i-Primary AIN)

Kwi-AIN yokuqala, ii-antibodies zijolise kwaye zitshabalalise ii-neutrophils. Ii-antibodies luhlobo lweproteni kwinkqubo yethu yokuzikhusela komzimba. Umsebenzi wazo oqhelekileyo kukutshabalalisa izinto eziyingozi, ezifana neentsholongwane kunye neebhaktheriya, ezingena emzimbeni. Kodwa okwangoku, abaphandi abasazi ukuba kutheni ii-antibodies zethu zihlasela ezi neutrophils.

I-Neutropenia yeSibini ye-Autoimmune (i-AIN yesibini)

Kwi-AIN yesibini, inani lee-neutrophils liyancitshiswa ngenxa yesinye isizathu okanye imeko. Oku kubangela ukuba inkqubo yomzimba yokuzikhusela itshabalalise ii-neutrophils.

Ezinye zezinto ezinokubangela oku zezi:

• Ezinye izifo ezibangelwa kukuzikhusela komzimba: Imizekelo ibandakanya isifo samathambo, i-lupus, kunye ne-autoimmune hemolytic anemia.
• Umhlaza: Umzekelo, `i-large granular lymphocyte leukemia`.
• Iintsholongwane: Iintsholongwane ezifana ne-'HIV', 'Parvovirus', 'hepatitis B'.
• Iithumba: Njenge-'Wilms tumor', 'isifo sikaHodgkin'.
• Izifo ze-neurological: Umzekelo, i-multiple sclerosis.
• Ukufakelwa kwezintso okanye umongo wethambo.
• Amayeza athile: Ingakumbi amanye amayeza e-chemotherapy anikezelwa kumhlaza.

Ifunyanwa njani i-Autoimmune Neutropenia? (Ukuxilongwa)

Xa unomkhuhlane okanye usulelo, ugqirha wakho unokufumanisa ukuba inani lakho le-neutrophil liphantsi ngexesha lovavanyo lwegazi ukuze kufunyanwe unobangela. Ukufumanisa i-AIN kunokuba nzima kancinci, njengoko kukho izizathu ezininzi ezinokubakho. Ugqirha wakho unokufumanisa i-AIN emva kokugqiba ezinye iimeko ezinokubangela ukuba inani lakho le-neutrophil libe phantsi.

Ukuze uxilonge okanye ubeke esweni i-`Autoimmune Neutropenia`, ugqirha wakho unokwenza ezi mvavanyo zilandelayo:

• Ubalo lwegazi olupheleleyo (CBC): I-CBC ingajonga inani lee-neutrophils egazini lakho. Ngenxa yokuba la manqanaba anokwahluka, ugqirha wakho uhlala esenza ii-CBC ezingaphezulu kwesinye ukuqinisekisa i-neutropenia kunye nokujonga amanqanaba akho e-neutrophil. Ii-CBC eziqhelekileyo zinokukunceda ukufumanisa ukuba i-AIN yakho ayiguquki na.
• I-smear yegazi engaphandle: Isampuli yegazi ihlolwa phantsi kwe-microscope ukujonga imo kunye nohlobo lwee-neutrophils. Oku kunceda nasekuxilongweni.
• Uvavanyo lweVithamin B12, i-folate, kunye ne-copper: Olu vavanyo lunokujonga ukungabikho kwezi zondlo. Umzimba uyazifuna ezi zinto ukuze wenze ii-neutrophils.
• Uvavanyo lokusebenza kwesibindi: Olu vavanyo lunokujonga ukusebenza kwesibindi kunye nokuba ingaba kukho i-hepatitis ebangelwa yintsholongwane.
• Izifundo ze-HIV kunye nezinye iintsholongwane: Usulelo lwe-HIV lunokubangela ukwehla kweeseli ezimhlophe zegazi.
• I-Flow cytometry: Olu luvavanyo lwelebhu olujonga iiseli ukuze kubonwe iimpawu zesifo. Lusetyenziselwa ukufumanisa ukuba i-neutropenia ibangelwa yimeko ye-autoimmune.
• Ukufota: Ukuba kukho ukurhaneleka kokuba i-AIN ibangelwa ngumhlaza ofana ne-lymphoma, kunokwenziwa i-MRI okanye i-CT scan.
• Ukufunxwa komongo wethambo kunye ne-biopsy: Apha, ugqirha uthatha isampuli encinci yomongo wethambo aze awuhlole ukuze abone iimpawu zesifo. Oku kunokunceda ekuqinisekiseni ukuba i-neutropenia ibangelwa ngumhlaza wegazi, njenge-leukemia.

Ugqirha wakho angayalela uvavanyo lwegazi ukujonga ii-antibodies egazini lakho ezihlasela ii-neutrophils. Ezona vavanyo zixhaphakileyo yi-I-GIFT (Indirect granulocyte immunofluorescence test) kunye ne-GAT (Granulocyte agglutination test).

Ngelishwa, ezi mvavanyo azithembekanga rhoqo ekufumaneni i-AIN. Ngokuxhomekeke kwisizathu se-neutropenia yakho kunye nexesha lovavanyo, ezi mvavanyo zisenokungazifumani ii-antibodies okanye zisenokunika iziphumo ezingezizo. Iziphumo ezingezizo ezilungileyo kuxa uvavanyo lubonisa ukuba ii-antibodies zikhona xa zingekho.

Ukuba ugqirha ukrokrela ukuba i-neutropenia ibangelwa yimeko ethile, unokuyalela iimvavanyo ezongezelelweyo.

Iphathwa njani i-Autoimmune Neutropenia?

Unyango luxhomekeke kubukhali bemeko yakho. Umzekelo, umntwana one-neutropenia engenazimpawu unokufuna kuphela ukuba ne-CBCs rhoqo kunye nenani le-neutrophil, kodwa usenokungadingi unyango olukhawulezileyo. Ukuba umntwana wakho unezifo ezixhaphakileyo, ugqirha wakho unokukunika amayeza okuthintela izifo ukuthintela izifo. Amayeza okubulala iintsholongwane adla ngokunikezelwa ukunyanga izifo, kodwa la mayeza okuthintela izifo anikwa ukuthintela izifo ukuba zingabikho.

Kumntu one-AIN yesibini, ugqirha uza kunyanga imeko ebangela amanqanaba aphantsi e-neutrophil. Banokumnika oku kulandelayo:

• I-Granulocyte colony-stimulating factor (G-CSF): Olu lolona nyango luphambili kwiimeko ezinzima ze-neutropenia. Inceda umzimba ukuba wenze ii-neutrophils ezingaphezulu. I-G-CSF inokuthintela nosulelo oluqhelekileyo oluhambelana ne-AIN yokuqala.
• IiCorticosteroids: IiCorticosteroids, ezifana ne-prednisone, zithintela impendulo yomzimba wakho kwi-immune system, zinciphisa inani lee-antibodies ezihlasela ii-neutrophils.
• Ezinye iindlela zonyango: Amanye amayeza afana ne-IVIG, i-rituximab, kunye ne-alemtuzumab nawo asetyenziswe kwizigulana ezigula kakhulu ezine-autoimmune neutropenia enzima.

Ngaba i-Autoimmune Neutropenia ingathintelwa?

Akukho nanye i-Primary AIN okanye i-Secondary AIN enokuthintelwa . Nangona kunjalo, ungazithintela iingxaki ezinxulumene ne-'Autoimmune Neutropenia', oko kukuthi, uphuhliso lwezifo. Ukuze wenze oku, kufuneka uthathe amanyathelo afunekayo ukuze uzikhusele kwiintsholongwane. Umzekelo, ukuphepha iindawo ezinabantu abaninzi, ukuhlamba izandla zakho rhoqo, kunye nokuhlala kude nabantu abagulayo.

Luhlobo luni lwekamva umntu one-Autoimmune Neutropenia anokulilindela?

I-AIN ePhambili idla ngokuzigqibela ngokwayo . Malunga nama-95% abantwana abane-AIN amanqanaba abo e-neutrophil abuyela kwisiqhelo xa beneminyaka emibini ukuya kwemithathu ubudala. Phantse bonke abantwana abane-AIN ePhambili bayachacha ngokupheleleyo xa beneminyaka eli-11 ubudala. Oku kuyathuthuzela ngokwenene, akunjalo?

Kwezinye iimeko, ezibizwa ngokuba yi-secondary AIN, ukuchacha kwakho kuxhomekeke kwizinto ezininzi, ingakumbi imeko ebangela i-neutropenia yakho. Ukunyanga loo meko nokuthintela usulelo kunokunceda ukubuyisela inani lakho le-neutrophil kwisiqhelo.

Ndingalufuna nini uncedo lwezonyango?

Ukuba une-autoimmune neutropenia enzima kwaye ubanomkhuhlane, bonana nogqirha ngoko nangoko . Ngamanye amaxesha, ugqirha wakho usenokuba umisele amayeza okubulala iintsholongwane kwangaphambili ukuba uwathathe ukuba unomkhuhlane. Kwiimeko ezininzi, kuya kufuneka unyangwe esibhedlele.

Ukuba une-autoimmune neutropenia enzima, ehlala ixesha elide, ugqirha wakho uya kukuchazela ukuba zeziphi iimpawu ekufuneka uzazi kunye nento onokuyenza ukuthintela iingxaki.

Khumbula: Ukuba wena okanye umntwana wakho nifunyaniswe ukuba nine-AIN, thetha nogqirha wakho malunga nendlela enichaphazela ngayo. Nangona iimpawu kunye nezizathu zahlukile, iindaba ezimnandi zezokuba unyango olufana ne-G-CSF lusebenza kakhulu ekwandiseni amanqanaba e-neutrophil.

Ukuba umntwana wakho une-AIN, fumana uvavanyo lwegazi rhoqo njengoko ugqirha wakho ekuyalele. Khangela iimpawu zosulelo, kwaye ufune unyango ngokukhawuleza ukuba ubona naziphi na iingxaki, ukuthintela iingxaki ezinokubakho.

Isishwankathelo (Umyalezo Wokuya Ekhaya)

Kulungile, ngoko ke ndiyathemba ukuba ngoku uyiqonda ngcono i-'Autoimmune Neutropenia' (AIN) esithethe ngayo.

• I-AIN yimeko apho amajoni ethu omzimba etshabalalisa iiseli zethu ezimhlophe zegazi ezibizwa ngokuba yi-'neutrophils'.
• Oku kunokwenzeka kubantwana abancinci (i-Primary AIN) okanye kubantu abadala (i-Secondary AIN). I-Primary AIN kubantwana abancinci idla ngokuzigqibela ngokwayo.
• Ingozi ephambili kukwanda kwezifo.
• Kukho uvavanyo lwegazi kunye nezinye iimvavanyo zokuxilonga.
• Unyango luquka ukujongwa, amayeza okulwa nosulelo, i-G-CSF, kunye ne-corticosteroids.
• Eyona nto ibalulekileyo kukungabi naxhala okanye ukoyika, kodwa funa ingcebiso kagqirha ukuba wena okanye umntwana wakho ninezi mpawu. Ukuba ufunyenwe kwangethuba kwaye ulawulwa ngokufanelekileyo, ungaphila ubomi obusempilweni nokuba une-AIN.

Ukuba unemibuzo engaphezulu ngale nto, qiniseka ukuba uthetha nogqirha wakho. Ngabo banokukunika icebiso elingcono.

I- Neutropenia, i-Autoimmune Neutropenia, iiseli ezimhlophe zegazi, inkqubo yomzimba yokuzikhusela, usulelo, izifo zegazi, ii-Pediatrics