I-Hypogammaglobulinemie

I-Hypogammaglobulinemie

I-Hypogammaglobulonemia ukwehla kwezinga lama-gamma-globulins noma ama-immunoglobulins, izinto ezinendima ebalulekile emasosheni omzimba. Lokhu kuphazamiseka kwebhayoloji kungase kube ngenxa yokuphuza izidakamizwa ezithile noma izifo ezihlukahlukene, ezinye zazo ezidinga ukuxilongwa ngokushesha. 

Incazelo ye-hypogammaglobulonemia

I-Hypogammaglobulinemia ichazwa yizinga le-gamma-globulin elingaphansi kuka-6 g/l ku-plasma protein electrophoresis (EPP). 

Ama-Gamma globulins, abizwa nangokuthi ama-immunoglobulins, ayizinto ezenziwe ngamangqamuzana egazi. Banendima ebaluleke kakhulu ekuvikelweni komzimba. I-Hypogammaglobumonemia iholela ekunciphiseni okukhulu noma okuncane kakhulu kwezivikeli mzimba. Akuvamile.

Kungani kuhlolwa i-gamma globulin?

Ukuhlola okuvumela ukunqunywa kwe-gamma-globulins, phakathi kwezinye izinto, i-electrophoresis yamaprotheni e-serum noma amaprotheni e-plasma.Kwenziwa uma kwenzeka izinsolo zezifo ezithile noma kulandela imiphumela engavamile phakathi nokuhlolwa kokuqala. 

Lokhu kuhlolwa kunqunywe uma kwenzeka izinsolo zokuthi i-humoral i-immune deficiency phambi kwezifo eziphindaphindiwe, ikakhulukazi ze-ENT kanye ne-bronchopulmonary sphere noma ukuwohloka kwesimo esijwayelekile, uma kwenzeka izinsolo ze-myeloma eminingi ( izimpawu: ubuhlungu bethambo, i-anemia, izifo ezithathelwanayo…). 

Lokhu kuhlola kungasetshenziswa futhi ngemva kwemiphumela engavamile ebonisa ukwanda noma ukuncipha kwephrotheni ye-serum, amaprotheni omchamo aphezulu, i-calcium eningi egazini, ukungavamile kwenani lamangqamuzana abomvu egazi noma amangqamuzana amhlophe egazi.

Kwenziwa kanjani ukuhlolwa kwe-gamma-globulin?

I-electrophoresis yamaprotheni e-serum ukuhlolwa okwenza kube nokwenzeka ukukala ama-gamma globulins. 

Lokhu kuhlolwa kwebhayoloji okujwayelekile (isampula legazi, ngokuvamile elisuka endololwaneni) kuvumela indlela yobuningi yezingxenye ezihlukahlukene zamaprotheni ze-serum (albumin, alpha1 kanye ne-alpha2 globulins, i-beta1 ne-beta2 globulins, i-gamma globulin). 

I-electrophoresis yamaprotheni e-serum ukuhlolwa okulula okwenza kube nokwenzeka ukuthola nokubamba iqhaza ekuqaphelweni kwama-pathologies amaningi: ama-syndromes okuvuvukala, umdlavuza othile, ukuphazamiseka ngokomzimba noma okunomsoco.

Iqondise ekuhlolweni okwengeziwe okudingekayo (i-immunofixation kanye / noma ukuhlolwa okukhethekile kwamaprotheni, ukuhlolwa kwe-hematological, ukuhlolwa kwezinso noma ukugaya).

Yimiphi imiphumela engalindelwa ekuhlolweni kwe-gamma-globulin?

Ukutholakala kwe-hypogammaglobulonemia kungase kube ngenxa yokuphuza izidakamizwa (i-oral corticosteroid therapy, immunosuppressants, anti-epileptics, tumor chemotherapy, njll.) noma izifo ezihlukahlukene. 

Ukuhlolwa okwengeziwe kuvumela ukuxilonga ukuthi kwenziwe uma imbangela yomuthi ingatholakali. 

Ukuthola izifo eziphuthumayo okuyizimo eziphuthumayo zokuxilonga (i-light chain myeloma, i-lymphoma, i-myeloid leukemia engapheli), kwenziwa izivivinyo ezintathu: ukusesha kwe-tumor syndrome (i-lymphadenopathy, i-hepato-splenomegaly), ukutholakala kwe-proteinuria kanye nenani legazi.

Uma lezi zimo eziphuthumayo sezikhishiwe ezinye izimbangela ze-hypogammaglobulonemia zibaliwe: i-nephrotic syndrome, i-exudative enteropathies. Izimbangela ze-exudative enteropathies zingaba isifo esingapheli sokuvuvukala, isifo se-celiac kanye nezimila eziqinile zokugaya ukudla noma i-lymphoid hemopathies ethile njenge-lymphoma noma i-primary amyloidosis (LA, i-light chain amyloidosis ye-immunoglobulins).

Okungavamile kakhulu, i-hypogammaglobulonemia ingase ibangelwe ukuntuleka kwe-humoral immune.

Ukungondleki kakhulu noma i-Cushing's syndrome kungase futhi kube imbangela ye-hypogammaglobulonemia.

Ukuhlolwa okwengeziwe kuvumela ukuxilonga (isithwebuli se-thoraco-abdominal-pelvic scanner, ukubalwa kwegazi, ukuvuvukala, i-albuminemia, i-proteinuria yamahora angu-24, ukunqunywa kwesisindo sama-immunoglobulins kanye ne-immunofixation yegazi)

Indlela yokwelapha i-hypogammaglobulonemia?

Ukwelashwa kuncike ekubambeni. 

Kungasethwa ukwelashwa kokuvimbela kubantu abaphethwe yi-hypogammaglobulinemia: ukugoma okulwa ne-pneumococcal kanye neminye imigomo, i-antibiotic prophylaxis, ukufaka esikhundleni se-polyvalent immunoglobulins.

shiya impendulo