Matendawa Amayambitsa Wodalirika M'magazi Akumagazi
Paroxysmal usiku wothandizira hemoglobinuria (PNH) ndi magazi omwe amapezedwa ndi matenda a maselo. Poyamba linkatchedwa chizindikiro cha mitsempha yamdima (hemoglobinuria) yomwe imapangidwa m'mawa. Iwo ankaganiza kuti kuwonongeka kwachilendo kwa maselo ofiira ofiira ( hemolysis ) omwe amachititsa kuti mitsempha yamdima ichitike m'ziphuphu (zozizwitsa) zomwe zinachitika usiku. Kafukufuku wasonyeza kuti hemolysis mu PNH, yomwe imayambitsa matenda a chilengedwe, imachitika tsiku lonse ndipo sizimachitika pang'onopang'ono.
Kusintha kwa mtundu wa mkodzo kumachitika nthawi iliyonse koma kumakhala kovuta kwambiri mu mkodzo wa usiku.
Kupititsa patsogolo kumvetsetsa kwa mazira a dzuwa amachititsa kuti matendawa azikhala ndi hemolysis, magazi m'mitsempha (thrombosis), komanso kusowa kwa mitundu yonse ya maselo ( hematopoiesis ). PNH imakhudza amuna ndi akazi a mafuko onse ndipo amatha kukhala ndi zaka zilizonse, koma amapezeka makamaka pakati pa achinyamata.
Zizindikiro
Anthu ambiri omwe ali ndi PNH alibe zizindikiro. Zizindikiro za PNH zimadalira ngati hemolysis, thrombosis, kapena hematopoiesis ilibe.
- Hemolysis - mkodzo wa cola (hemoglobinuria), hemolytic anemia
- Thrombosis - kumene chimapezeka chimayambitsa zizindikiro. Ngati chovalacho chili mumtumbo wa chiwindi, pangakhale chiwindi ndi chiwindi chofutukuka. Mimba, chovala chimayambitsa kupweteka m'mimba; mutu, mutu. Nsalu za khungu zimayambitsa, zopweteka, zofiira zofiira pamadera ambiri a khungu, monga kumbuyo.
- Matenda a hematopoiesis omwe sali ochepa (maselo ofiira a magazi ofiira), omwe amatha kutenga matenda (osadziwika maselo oyera a magazi), komanso kutuluka kwa magazi mopanda malire.
Kudziwa
Pali mayesero angapo a ma laboratory omwe angathandize kupezetsa matenda a puloteni omwe amachititsa kuti dzuwa lizizira. Chodziwika bwino ndi kuyesa magazi kuti azindikire CD55 ndi CD59, mapuloteni awiri.
Kupezeka kwa mapuloteni ameneĊµa pa maselo ofiira a magazi kumatsimikizira kuti matendawa ndi a PNH. Pakuyezetsa magazi, mayeso ena a magazi adzachitika kuphatikizapo kuwerengera kwathunthu kwa maselo a magazi (CBC), count reticulocyte, serum lactate dehydrogenase (LDH), bilirubin, ndi haptoglobin. Chitsanzo cha msupa (biopsy) chingatengedwe ndikuyang'aniranso pang'onopang'ono.
Chithandizo
Kuchiza kumachokera pa zizindikiro zomwe zilipo. Anthu ambiri akuluakulu amachiritsidwa ndi steroid (prednisone) tsiku ndi tsiku pamene hemolysis imapezeka ndipo amasintha kukhala masiku ena panthawi yachisoni. Iron ndi folic acid zimapereka thandizo m'malo mwa kutayika chifukwa cha hemolysis. Anthu ena akhoza kulandira mahomoni a androgenic kuti apangitse kupanga maselo ofiira a magazi. Kupha magazi kwambiri, kuika magazi kungakhale kofunikira. Magazini a magazi angagwiritsidwe ntchito kuchepetsa kukula kwa magazi. Mankhwala otchedwa hematopoiesis omwe amatha kutayika akhoza kuthandizidwa ndi antithymocyte globulin (ATG).
Kuphatikizidwa kwa mafupa (fupa la mafupa) kumalowa m'malo mwa maselo osaperewera a PNH ndi zinthu zachilendo. Pafupifupi anthu onse omwe ali ndi PNH adzafuna kubzala kuti akhale ndi moyo wautali. Ngati kuika kwachitika mochedwa panthawi ya matenda, zotsatira zake si zabwino chifukwa munthuyo akudwala kwambiri panthawiyo.
Mankhwala Oyamba a PNH Ovomerezeka
Pa March 16, 2007, US Food and Drug Administration (FDA) inavomereza Soliris (eculizumab) kuti azitha kulandira PNH. Chivomerezocho chinachokera pa zotsatira za maphunziro angapo omwe amasonyeza chithandizo ndi eculizumab zomwe zinapangitsa kuti kuchepa kwakukulu ku hemolysis, komanso masiku a hemoglobinuria athake.
Kuchokera
> Parker, Charles, Mitsuhiro Omine, Stephen Richards, et al. "Kuzindikira ndi Kulamulira kwa Paroxysmal Nocturnal Hemoglobinuria." Magazini 106 (2005): 3699 - 3709.