Polycythemia vera

Polycythemia vera (PV) waa cudur ku dhaca dhuuxa lafta taasoo keenta koror aan caadi aheyn tirada unugyada dhiiga. Unugyada dhiigga cas ayaa inta badan saameeya.

PV waa cilad ku dhacda dhuuxa lafta. Waxay badanaa keentaa unugyada dhiigga cas ee aad u tiro badan in la soo saaro. Lambarada unugyada dhiigga cad iyo platelet sidoo kale way ka badnaan karaan sida caadiga ah.

PV waa cillad naadir ah oo ragga ku dhacda inta badan dumarka. Badanaa laguma arko dadka ka yar da'da 40. Dhibaatada badanaa waxay la xiriirtaa cillad hiddo-wadaha oo loo yaqaan JAK2V617F. Sababta cilladan hidde-wadaha lama yaqaan. Cilladaan hiddo-wadaha ah ma ahan cillad la iska dhaxlo.

Marka la qaato PV, waxaa jirka ku jira unugyo dhiig cas oo aad u tiro badan. Tani waxay keentaa dhiig aad u cufan, oo aan ku dhex mari karin marinnada dhiigga ee yar yar sida caadiga ah, taasoo keenta astaamaha sida:

• Neefsashada oo dhib ku timaadda markaad jiifto
• Maqaarka buluuga ah
• Dawakhaad
• Dareen dareemid markasta
• Dhiigbax xad-dhaaf ah, sida dhiig-baxa maqaarka
• Dareen buuxa oo ku jira caloosha bidix ee sare (sababtuna tahay beeryarada oo ballaadhatay)
• Madax xanuun
• Cuncun, gaar ahaan qubays diiran ka dib
• Midab cas cas, gaar ahaan wajiga
• Neefta oo kugu yaraata
• Calaamadaha xinjirowga dhiigga ee xididdada ku dhow maqaarka dusha sare (phlebitis)
• Dhibaatooyinka aragga
• Dhawaaqa dhegaha (tinnitus)
• Xanuun wadajir ah

Bixiyaha xanaanada caafimaadka ayaa sameyn doona baaritaan jireed. Waxa kale oo laga yaabaa inaad tijaabooyinka soo socda:

• Cad ka-qaadista lafaha dhuuxa
• Tirinta dhiigga oo dhammaystiran oo kaladuwan
• Guddi dheef-shiid kiimikaad dhammaystiran
• Heerka Erythropoietin
• Tijaabada hidda-sidaha ee isbeddelka JAK2V617F
• Ogsijiin ka dheregidda dhiigga
• Cudurka unugyada dhiigga cas
• Faytamiin B12

PV sidoo kale wuxuu saameyn ku yeelan karaa natiijooyinka tijaabooyinka soo socda:

• ESR
• Lactate dehydrogenase (LDH)
• Leukocyte fosfatase alkali
• Tijaabada isu geynta platelet
• Serum uric acid

Ujeeddada daaweyntu waa in la yareeyo dhumucda dhiigga oo laga hortago dhiigbaxa iyo xinjirowga dhibaatooyinka.

Habka loo yaqaan 'phlebotomy' waxaa loo isticmaalaa in lagu yareeyo dhumucda dhiigga. Hal unug oo dhiig ah (ilaa 1 pint, ama 1/2 litir) ayaa la saaraa toddobaad kasta illaa tirada unugyada dhiigga cas ay hoos u dhacdo. Daaweynta ayaa loo sii wadaa sidii loogu baahdo.

Daawooyinka la isticmaali karo waxaa ka mid ah:

• Hydroxyurea si loo yareeyo tirada unugyada dhiigga cas ee ay dhuuxa dhuuxa u sameeyaan. Daawadani waa la isticmaali karaa marka ay sidoo kale sarreeyaan nambarka noocyada kale ee unugyada dhiigga.
• Interferon si loo yareeyo tirada dhiigga.
• Anagrelide si loo yareeyo tirada platelet.
• Ruxolitinib (Jakafi) si loo yareeyo tirada unugyada dhiiga guduudan loona yareeyo beeryarada weynaata. Daawadani waxaa loo qoraa markii hydroxyurea iyo daaweyn kale ay fashilmaan.

Qaadashada asbiriin si loo yareeyo halista xinjirowga dhiigga ayaa laga yaabaa inay ikhtiyaar u tahay dadka qaarkood. Laakiin, asbiriintu waxay kordhisaa halista dhiig-baxa caloosha.

Daaweynta iftiinka Ultraviolet-B waxay yareyn kartaa cuncunka daran ee dadka qaarkii ay la kulmaan.

Ururada soo socdaa waa ilo wanaagsan oo loogu talagalay macluumaadka ku saabsan polycythemia vera:

• Ururka Qaranka ee Dhibaatooyinka Nadir - rarediseases.org/rare-diseases/polycythemia-vera
• NIH Xarunta Macluumaadka Cudurrada Hidda - wadaha - rarediseases.info.nih.gov/diseases/7422/polycythemia-vera

PV caadi ahaan si tartiib ah ayuu u kobcayaa. Dadka badankood ma laha calaamado la xiriira cudurka waqtiga cudurka. Xaaladda badanaa waa la ogaadaa kahor intaanay astaamo daran soo bixin.

Dhibaatooyinka PV waxaa ka mid noqon kara:

• Cudurka 'Myelogenous leukemia' (AML)
• Dhiig ka yimaada caloosha ama qaybaha kale ee marinka mindhicirka
• Gout (barar xanuun badan oo wadajir ah)
• Wadne istaag
• Myelofibrosis (cillad ku dhacda dhuuxa lafta taas oo dhuuxa ku bedelay unug nuug ah nabar)
• Thrombosis (xinjirowga dhiigga, oo sababi kara istaroog, wadno qabad, ama waxyeelo kale oo jirka ah)

Wac daryeel caafimaad haddii astaamaha PV ay soo baxaan.

Polycythemia aasaasiga ah; Polycythemia rubra vera; Cilladda myeloproliferative; Erythremia; Splenomegalic polycythemia; Cudurka Vaquez; Cudurka Osler; Polycythemia oo leh cyanosis joogto ah; Erythrocytosis megalosplenica; Cudurka loo yaqaan 'Cryptogenic polycythemia'

Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. Polycythemias. Gudaha: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Mabaadi'da Aasaasiga ah iyo Tababarka. 7aad ed. Philadelphia, PA: Elsevier; 2018: cutubka 68.

Websaydhka Machadka Kansarka Qaranka. Daaweynta neoplasms ee myeloproliferative neoplasms (PDQ) - nooca xirfadlaha caafimaadka. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/_5. La cusbooneysiiyay Febraayo 1, 2019. La helay Maarso 1, 2019.

Tefferi A. Polycythemia vera, thrombocythemia muhiim ah, iyo myelofibrosis aasaasi ah. Gudaha: Goldman L, Schafer AI, eds. Daawada Goldman-Cecil. 25aad. Philadelphia, PA: Elsevier Saunders; 2016: cutubka 166.