IPolycythemia vera

IPolycythemia vera (PV) sisifo somongo wethambo esikhokelela kukonyuka okungaqhelekanga kwinani leeseli zegazi. Iiseli ezibomvu zegazi zichaphazeleka ikakhulu.

I-PV kukuphazamiseka komongo wethambo. Inokubangela ukuba iiseli ezibomvu ezininzi kakhulu ziveliswe. Inani leeseli ezimhlophe zegazi kunye neeplatelet zinokuphakama ngaphezu kwesiqhelo.

I-PV sisifo esinqabileyo esenzeka rhoqo emadodeni kunabafazi. Akuqhelekanga ukubonwa kubantu abangaphantsi kweminyaka engama-40. Ingxaki ihlala inxulunyaniswa nesiphene semfuza esibizwa ngokuba yiJAK2V617F. Unobangela wesi siphene semfuza awaziwa. Esi siphene semfuza asisiyo isifo esizuzwe njengelifa.

Nge-PV, zininzi kakhulu iiseli ezibomvu zegazi emzimbeni. Oku kubangela ukuba ligazi elingqindilili, elingakwaziyo ukuhamba kwimithambo yegazi encinci ngesiqhelo, kukhokelela kwiimpawu ezinje:

• Ingxaki yokuphefumla xa ulele phantsi
• Ulusu oluhlaza okwesibhakabhaka
• Ukuba nesiyezi
• Ndiziva ndidiniwe ngalo lonke ixesha
• Ukopha kakhulu, njengokuphuma kolusu
• Imvakalelo epheleleyo kwisisu esingasentla sasekhohlo (ngenxa yethambo elandisiweyo)
• Intloko ebuhlungu
• Ukuluma, ngakumbi emva kwebhafu efudumeleyo
• Umbala obomvu wolusu, ngakumbi ebusweni
• Ukuqhawukelwa ngumphefumlo
• Iimpawu zamahlwili egazi kwimithambo ecaleni komphezulu wolusu (phlebitis)
• Iingxaki zombono
• Ukukhala ezindlebeni (tinnitus)
• Intlungu edibeneyo

Umboneleli wezempilo uya kwenza uvavanyo lomzimba. Unako ukufumana ezi mvavanyo zilandelayo:

• Umongo wethambo
• Gcwalisa ubalo lwegazi ngokwahluka
• Iphaneli yemetabolism ebanzi
• Inqanaba le-Erythropoietin
• Uvavanyo lwe-Genetic lokutshintsha kweJAK2V617F
• Ukugcwaliswa kweoksijini yegazi
• Ubunzima beeseli ezibomvu zegazi
• Inqanaba leVitamin B12

I-PV inokuchaphazela neziphumo zolu vavanyo lulandelayo:

• I-ESR
• Lactate dehydrogenase (LDH)
• Leukocyte alkaline phosphatase
• Uvavanyo lokuhlanganiswa kweplatelet
• I-Serum uric acid

Injongo yonyango kukunciphisa ubukhulu begazi kunye nokuthintela ukopha kunye neengxaki zokunqanda.

Indlela ebizwa ngokuba yi-phlebotomy isetyenziselwa ukunciphisa ubukhulu begazi. Iyunithi yegazi (malunga ne-1 pint, okanye i-1/2 litre) isuswa nganye ngeveki de kube lihla inani leeseli ezibomvu zegazi. Unyango luyaqhubeka njengoko kufuneka.

Amayeza anokusetyenziswa abandakanya:

• I-Hydroxyurea yokunciphisa inani leeseli ezibomvu zegazi ezenziwe ngumongo wethambo. Eli chiza linokusetyenziswa xa amanani ezinye iintlobo zeeseli zegazi nazo ziphezulu.
• I-Interferon ukunciphisa ukubala kwegazi.
• I-Anagrelide ukuthoba ubalo lweplatelet.
• Ruxolitinib (Jakafi) ukunciphisa inani leeseli ezibomvu zegazi kunye nokunciphisa ipeni eyandisiweyo. Eli chiza limiselwe xa ihydroxyurea kunye nolunye unyango lusilele.

Ukuthatha i-aspirin ukunciphisa umngcipheko wamahlwili egazi kunokuba yinto enokukhetha kubantu abathile. Kodwa, i-aspirin inyusa umngcipheko wokuphuma kwesisu.

Unyango olukhanyayo lwe-Ultraviolet-B lunokunciphisa ukurhawuzelela okunzima kwabanye abantu.

Le mibutho ilandelayo zizibonelelo ezifanelekileyo zolwazi kwi-polycythemia vera:

• Umbutho weSizwe weengxaki zoRare- rarediseases.org/rare-diseases/polycythemia-vera
• IZiko loLwazi lweZifo zeMvelo zeNiH kunye neRare-rarediseases.info.nih.gov/diseases/7422/polycythemia-vera

I-PV ihlala ikhula kancinci. Uninzi lwabantu alunazo iimpawu ezinxulumene nesi sifo ngexesha lokuchongwa. Imeko ihlala ifunyaniswa ngaphambi kokuba kwenzeke iimpawu ezinzima.

Iingxaki ze-PV zinokubandakanya:

• Ileukemia eyi-myelogenous (AML)
• Ukopha esiswini okanye kwezinye iindawo zendlela yamathumbu
• Igawuthi (ukudumba okubuhlungu kwendibaniselwano)
• Ukumelwa yintliziyo
• I-Myelofibrosis (ukungahambi kakuhle komongo wethambo apho umongo uthatyathelwa indawo sisihlunu esibomvu)
• I-Thrombosis (ukujiya kwegazi, kunokubangela ukubetha, isifo sentliziyo, okanye omnye umonakalo emzimbeni)

Tsalela umnikezeli wakho ukuba iimpawu ze-PV ziyavela.

Iipolycythemia zaseprayimari; IPolycythemia rubra vera; Ingxaki ye-Myeloproliferative; Erythremia; I-polycythemia ye-Splenomegalic; Isifo sikaVaquez; Isifo sika-Osler; Polycythemia kunye cyanosis ezingapheliyo; Erythrocytosis megalosplenica; I-Cryptogenic polycythemia

UKremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R.Iipolycythemias. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 68.

Kwiwebhusayithi yeZiko loMhlaza weSizwe. Unyango lwe-myeloproliferative neoplasms engapheliyo (PDQ) -uhlobo lobungcali kwezempilo. www.cancer.gov/types/myeloproliferative/hp/tronic-treatment-pdq#link/_5. Ukuhlaziywa ngoFebruwari 1, 2019. Ifikeleleke ngo-Matshi 1, 2019.

I-Tefferi A. Polycythemia vera, i-thrombocythemia ebalulekileyo, kunye ne-myelofibrosis ephambili. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Umhla wama-25. IPhiladelphia, PA: Elsevier Saunders; I-2016: isahluko 166.