Polycythemia vera

Polycythemia vera (PV) cuta ce ta ɓarna da ƙashi wanda ke haifar da rashin daidaituwar adadin ƙwayoyin jini. Kwayoyin jinin ja sun fi shafa.

PV cuta ce ta kasusuwa. Yana haifar da jan ƙwayoyin jini da yawa don samarwa. Lambobin fararen ƙwayoyin jini da platelets na iya zama ma mafi girma fiye da yadda suke.

PV cuta ce mai saurin gaske wacce ke faruwa sau da yawa a cikin maza fiye da mata. Ba kasafai ake ganin hakan a cikin mutanen da shekarunsu ba su kai 40 ba. Matsalar galibi ana alakanta ta da nakasar kwayar halitta da ake kira JAK2V617F. Ba a san dalilin wannan lahani ba. Wannan nakasar haihuwa ba cuta ba ce ta gado.

Tare da PV, akwai ƙwayoyin jan jini da yawa a jiki. Wannan yana haifar da jini mai kauri sosai, wanda ba zai iya gudana ta ƙananan jijiyoyin jini ba koyaushe, wanda ke haifar da bayyanar cututtuka kamar:

• Matsalar numfashi lokacin kwanciya
• Fata ta Bluish
• Dizziness
• Jin kasala a kowane lokaci
• Zub da jini mai yawa, kamar zubar jini cikin fata
• Cikakken ji a cikin hagu na sama na hagu (saboda kara girman ciki)
• Ciwon kai
• Ciwo, musamman bayan wanka mai dumi
• Jan launin launin fata, musamman na fuska
• Rashin numfashi
• Kwayar cututtukan jini a jijiyoyin kusa da fuskar fata (phlebitis)
• Matsalar hangen nesa
• Ingara a kunnuwa (tinnitus)
• Hadin gwiwa

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki. Hakanan kuna iya samun gwaje-gwaje masu zuwa:

• Gwajin kasusuwa
• Kammala lissafin jini tare da banbanci
• M rayuwa panel
• Matsayin Erythropoietin
• Gwajin kwayar halitta don maye gurbin JAK2V617F
• Oxygen jikewa na jini
• Yawan jinin jini
• Vitamin B12 matakin

PV na iya shafar sakamakon sakamakon gwaje-gwaje masu zuwa:

• ESR
• Lactate dehydrogenase (LDH)
• Leukocyte alkaline phosphatase
• Gwajin tarin platelet
• Sinadarin uric acid

Manufar magani ita ce a rage kaurin jini da hana zubar jini da matsalar daskarewa.

Ana amfani da hanyar da ake kira phlebotomy don rage kaurin jini. Unitaya daga cikin jini (kimanin pint 1, ko lita 1/2) ana cirewa kowane mako har sai adadin jajayen ƙwayoyin jini ya saukad da. An ci gaba da jiyya kamar yadda ake buƙata.

Magungunan da za'a iya amfani dasu sun haɗa da:

• Hydroxyurea don rage adadin jajayen ƙwayoyin jini waɗanda ƙashin ƙashi yake yi. Ana iya amfani da wannan maganin lokacin da adadin wasu nau'in ƙwayoyin jini suma suka yi yawa.
• Interferon don rage ƙidayar jini.
• Anagrelide don ƙidaya ƙarancin platelet.
• Ruxolitinib (Jakafi) don rage yawan jajayen jinin jini da rage fadada saifa. An ba da wannan maganin lokacin da hydroxyurea da sauran jiyya suka kasa.

Shan aspirin don rage haɗarin daskarewar jini na iya zama zaɓi ga wasu mutane. Amma, asfirin yana kara haɗarin zubar jini a cikin ciki.

Jinyar haske ta Ultraviolet-B na iya rage tsananin itching da wasu mutane ke fuskanta.

Organizationsungiyoyi masu zuwa albarkatu ne masu kyau don bayani game da polycythemia vera:

• Nationalungiyar forasa don Rare Rashin Lafiya - rarediseases.org/rare-diseases/polycythemia-vera
• NIH Cibiyar Bayanai na Halittu da Rare Cutar - rarediseases.info.nih.gov/diseases/7422/polycythemia-vera

PV yawanci yana haɓaka a hankali. Yawancin mutane ba su da alamun da ke da alaƙa da cutar a lokacin ganowar cutar. Yawancin lokaci ana gano yanayin kafin mummunan alamun bayyanar ya faru.

Matsalolin PV na iya haɗawa da:

• Ciwon ƙwayar cutar sankarar bargo (AML)
• Zub da jini daga ciki ko wasu sassan ɓangaren hanji
• Gout (kumburi mai zafi na haɗin gwiwa)
• Ajiyar zuciya
• Myelofibrosis (cuta na ɓargo da kashi wanda aka maye gurbin da bargon da ƙyallen nama)
• Thrombosis (daskarewar jini, wanda na iya haifar da bugun jini, bugun zuciya, ko wata illa ta jiki)

Kira mai ba ku sabis idan alamun cutar PV sun ci gaba.

Primary polycythemia; Polycythemia rubra vera; Rashin lafiyar Myeloproliferative; Erythremia; Splenomegalic polycythemia; Cutar Vaquez; Cutar Osler; Polycythemia tare da kullum cyanosis; Erythrocytosis megalosplenica; Polycythemia na Cryptogenic

Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. The polycythemias. A cikin: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Ka'idoji da Aiki. 7th ed. Philadelphia, PA: Elsevier; 2018: babi na 68.

Yanar gizo Cibiyar Cancer ta Kasa. Magungunan neoplasms na yau da kullum (PDQ) - fasalin ƙwararrun kiwon lafiya. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/_5. An sabunta Fabrairu 1, 2019. An shiga Maris 1, 2019.

Tefferi A. Polycythemia vera, thrombocythemia mai mahimmanci, da myelofibrosis na farko. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: babi na 166.