Ukuphazamiseka Kubangelwa Umthetheli Ezingxenyeni Zegazi
I-Paroxysmal nocturnal hemoglobinuria (i-PNH) i-blood stem cell disorder etholakalayo. Kwakubizwa ngokuthi okokuqala uphawu lwaso lomchamo omnyama (i-hemoglobinuria) olwenziwe ekuseni. Kwacatshangwa ukuthi ukwehla okungavamile kwamangqamuzana egazi abomvu (i- hemolysis ) okwenza ukuba umchamo omnyama kwenzeke ekuqhumeni (ama-paroxysms) okwenzeka ebusuku. Nokho, ucwaningo lubonise ukuthi i-hemolysis e-PNH, ebangelwa ukukhubazeka kwezinto eziphilayo, kwenzeka nsuku zonke futhi ayitholakali.
Ukushintsha kombala womchamo kungenzeka nganoma isiphi isikhathi kepha kuyamangalisa kakhulu kumchamo wokulala ebusuku.
Ukuqonda okuthuthukisiwe kwe-paroxysmal nocturnal hemoglobinuria kuye kwahlenga kabusha lesi sifo njengokuba ne-hemolysis, ama- blood clots emithanjeni (thrombosis), nokuntuleka kokukhiqizwa kwazo zonke izinhlobo zamangqamuzana egazi ( hematopoiesis ). I-PNH ithinta bobabili abesilisa nabesifazane bazo zonke izizinda ezihlukahlukene futhi kungenzeka kunanoma yisiphi isikhathi, kodwa kuvame ukutholakala kubantu abadala.
Izimpawu
Abantu abaningi abane-PNH abanayo izimpawu. Izimpawu ze-PNH zixhomeke ekutheni i-hemolysis, i-thrombosis, noma i-hematopoiesis engakwazi yini.
- I-Hemolysis - i-cola-colored urine (hemoglobinuria), i-hemolytic anemia
- I-Thrombosis - lapho i-clot kwenzeka khona iveza izimpawu. Uma i-clot isendaweni yesibindi sesibindi, kungenzeka kube ne-jaundice nesibindi esandisiwe. Esiswini, i-clot ingabangela ubuhlungu besisu; ekhanda, ikhanda. Izindwangu esikhumbeni zidala imbangela ephakanyisiwe, ebuhlungu, ebomvu phezu kwezindawo ezinkulu zesikhumba, njengemuva.
- I-hematopoiesis ehlulekayo - i-anemia (hhayi okwanele amangqamuzana egazi obomvu), ukutholakala kwezifo (hhayi okwanele amangqamuzana egazi amhlophe), nokusuka okungavamile (amapulethi anganele).
Ukuxilongwa
Kunezivivinyo eziningana ze-laboratory ezingasiza ukuxilongwa kwe-paroxysmal nocturnal hemoglobinuria. Okucacile kakhulu ukuhlola igazi ukuthola i-CD55 ne-CD59, amaprotheni amabili.
Ukungabikho kwala maprotheni ngamaseli abomvu aqinisekisa ukuxilongwa kwe-PNH. Ngesikhathi sokuxilongwa, ezinye izivivinyo zegazi zizokwenziwa kufaka phakathi inani eliphelele le-cell (CBC), isibalo se-reticulocyte, i-serum lactate dehydrogenase (LDH), i-bilirubin, ne-haptoglobin. Isampuli yamathambo (i-biopsy) singathathwa bese sihlolwe ngaphansi kwe-microscope.
Ukwelapha
Ukwelashwa kusekelwe kuzo izimpawu ezikhona. Abantu abaningi abadala baphathwa nge-steroids (prednisone) nsuku zonke lapho i-hemolysis ikhona futhi ishintshela ezinsukwini ezingezansi ngesikhathi sokuxolelwa. I-Iron ne-folic acid supplements zisiza ukufaka esikhundleni sokulahlekelwa ngenxa ye-hemolysis. Abanye abantu bangathola ama-hormone e-androgenic ukugqugquzela ukukhiqizwa kwamaseli abomvu. E-anemia enamandla, ukumpompela igazi kungadingeka. I-Blood thinners ingasetshenziselwa ukunciphisa ukuthuthukiswa kwezindwangu zegazi. I-hematopoiesis engaphelele ingaphathwa nge-antithymocyte globulin (ATG).
Ukufakelwa kwe-marrow cell (stem cell) kungashintsha amangqamuzana egazi angenasici e-PNH ngejwayelekile. Cishe bonke abantu abane-PNH ekugcineni badinga ukufakelwa ukuze baphile isikhathi eside. Uma ukufakelwa kwenziwa ngemuva kwesikhathi esifundeni lesi sifo imiphumela ayifanele ngoba umuntu ugula kakhulu ngaleso sikhathi.
Imithi yokuqala ye-PNH ivunyelwe
Ngo-Mashi 16, 2007, i-US Food and Drug Administration (FDA) yamukele iSoliris (eculizumab) yokwelashwa kwe-PNH. Ukuvunywa kwakusekelwe emiphumeleni yezifundo eziningana ezabonisa ukuthi ukwelashwa nge-eculizumab kwaveza ukunciphisa okukhulu kwe-hemolysis, futhi izinsuku ze-hemoglobinuria inyanga ngayinye yehla.
Umthombo
> Parker, Charles, Mitsuhiro Omine, uStephen Richards, et al. "Ukuxilongwa Nokuphathwa Kwe-Paroxysmal Nocturnal Hemoglobinuria." Igazi 106 (2005): 3699 - 3709.