I-Polycythemia vera

I-Polycythemia vera (PV) isifo somnkantsha esiholela ekwandeni okungavamile kwenani lamaseli egazi. Amaseli abomvu egazi athinteka kakhulu.

I-PV ukuphazamiseka komnkantsha wethambo. Ngokuyinhloko kubangela ukuthi kukhiqizwe amangqamuzana egazi abomvu amaningi kakhulu. Izinombolo zamaseli amhlophe egazi nama-platelets nazo zingaphakama kunokujwayelekile.

I-PV yisifo esingajwayelekile esenzeka kaningi emadodeni kunabesifazane. Akuvamisile ukubonwa kubantu abangaphansi kweminyaka engama-40. Inkinga ivame ukuxhunyaniswa nesici sofuzo esibizwa nge-JAK2V617F. Ayaziwa imbangela yalesi sifo. Lokhu kukhubazeka kofuzo akusona isifo esizuzwe njengefa.

Nge-PV, kunamaseli abomvu amaningi kakhulu emzimbeni. Lokhu kubangela igazi elijiyile kakhulu, elingakwazi ukugeleza ngemithambo yegazi emincane ngokujwayelekile, okuholela ezimpawu ezifana ne:

• Inkinga yokuphefumula lapho ulele phansi
• Isikhumba esiluhlaza okwesibhakabhaka
• Isiyezi
• Ukuzizwa ngikhathele ngaso sonke isikhathi
• Ukopha ngokweqile, njengokuphuma kwesikhumba
• Ukuzizwa okugcwele esiswini esingaphezulu kwesokunxele (ngenxa yobende obukhulisiwe)
• Ubuhlungu bekhanda
• Ukuluma, ikakhulukazi ngemuva kokugeza okufudumele
• Umbala wesikhumba obomvu, ikakhulukazi ebusweni
• Ukuphelelwa umoya
• Izimpawu zamahlule egazini emithanjeni eduze kwesikhumba (phlebitis)
• Izinkinga zombono
• Ukukhala ezindlebeni (tinnitus)
• Ubuhlungu obuhlangene

Umhlinzeki wezokunakekelwa kwempilo uzokwenza ukuhlolwa komzimba. Ungase futhi ube nokuhlolwa okulandelayo:

• I-bone marrow biopsy
• Gcwalisa inani legazi ngokuhluka
• Iphaneli yemethodi ephelele
• Izinga le-Erythropoietin
• Ukuhlolwa kwe-Genetic kokuguquka kwe-JAK2V617F
• Ukugcwaliswa komoya-mpilo kwegazi
• Isisindo samaseli abomvu
• Izinga le-Vitamin B12

I-PV ingathinta nemiphumela yokuhlolwa okulandelayo:

• ESR
• I-Lactate dehydrogenase (LDH)
• I-leukocyte alkaline phosphatase
• Ukuhlolwa kokuhlanganiswa kweplatelet
• I-Serum uric acid

Inhloso yokwelashwa ukunciphisa ubukhulu begazi nokuvikela ukopha nezinkinga zokujiya.

Indlela ebizwa nge-phlebotomy isetshenziselwa ukunciphisa ukushuba kwegazi. Iyunithi eyodwa yegazi (cishe ipayinti elilodwa, noma u-1/2 litre) isuswa isonto ngalinye kuze kwehle inani lamaseli abomvu egazi. Ukwelashwa kuyaqhubeka njengoba kudingeka.

Imithi engasetshenziswa ifaka phakathi:

• I-Hydroxyurea ukunciphisa inani lamaseli abomvu egazi elenziwe ngomnkantsha wethambo. Lesi sidakamizwa singasetshenziswa lapho izinombolo zezinye izinhlobo zamaseli egazi nazo ziphakeme.
• I-Interferon yokwehlisa ukubalwa kwegazi.
• I-Anagrelide yokwehlisa ukubalwa kweplatelet.
• Ruxolitinib (Jakafi) ukwehlisa inani lamaseli abomvu wehlise ubende obukhulisiwe. Lesi sidakamizwa sinqunywa lapho i-hydroxyurea nezinye izindlela zokwelapha zehlulekile.

Ukuthatha i-aspirin ukunciphisa ubungozi bamahlule egazi kungaba yindlela kwabanye abantu. Kodwa, i-aspirin yandisa ingozi yokuphuma kwesisu.

Ukwelashwa okulula kwe-Ultraviolet-B kunganciphisa ukulunywa okunzima abanye abantu ababhekana nakho.

Lezi zinhlangano ezilandelayo ziyizinsizakusebenza ezinhle zokwaziswa nge-polycythemia vera:

• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile - rarediseases.org/rare-diseases/polycythemia-vera
• Isikhungo Sokwaziswa se-NIH Genetic and Rare Diseases - rarediseases.info.nih.gov/diseases/7422/polycythemia-vera

I-PV ivame ukukhula kancane. Iningi labantu alinazo izimpawu ezihlobene nalesi sifo ngesikhathi sokuxilongwa. Isimo sivame ukutholakala ngaphambi kokuba kuvele izimpawu ezinzima.

Izinkinga ze-PV zingafaka:

• I-leukemia enamandla kakhulu (AML)
• Ukopha esiswini noma ezinye izingxenye zomgudu wamathumbu
• I-Gout (ukuvuvukala okubuhlungu kwejoyini)
• Ukwehluleka kwenhliziyo
• I-Myelofibrosis (ukuphazamiseka komnkantsha wamathambo lapho umongo uthathelwa indawo izicubu ezibomvu)
• I-Thrombosis (ukujiya kwegazi, okungadala isifo sohlangothi, isifo senhliziyo, noma omunye umonakalo emzimbeni)

Shayela umhlinzeki wakho uma kuvela izimpawu ze-PV.

I-polycythemia eyinhloko; IPolycythemia rubra vera; Isifo se-Myeloproliferative; I-Erythremia; I-polycythemia ye-Splenomegalic; Isifo sikaVaquez; Isifo sika-Osler; I-Polycythemia ene-cyanosis engapheli; I-erythrocytosis megalosplenica; I-polycythemia ye-Cryptogenic

UKremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R.I-polycythemias. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 68.

Iwebhusayithi yeNational Cancer Institute. Ukwelashwa okungapheli kwe-myeloproliferative neoplasms (PDQ) - inguqulo yezempilo. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/_5. Kubuyekezwe uFebhuwari 1, 2019. Kufinyelelwe kuMashi 1, 2019.

I-Tefferi A. Polycythemia vera, i-thrombocythemia ebalulekile, ne-myelofibrosis eyinhloko. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-25. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 166.