Polycythemia vera

Polycythemia vera (PV) ndimatenda am'mafupa omwe amatsogolera kuwonjezeka kosazolowereka kwama cell amwazi. Maselo ofiira ofiira amakhudzidwa kwambiri.

PV ndimatenda am'mafupa. Zimapangitsa kuti maselo ofiira ambiri azipangidwa. Chiwerengero cha ma cell oyera ndi ma platelets amathanso kukhala apamwamba kuposa zachilendo.

PV ndi matenda osowa omwe amapezeka nthawi zambiri mwa amuna kuposa akazi. Simawoneka mwa anthu ochepera zaka 40. Vutoli limalumikizidwa ndi vuto la jini lotchedwa JAK2V617F. Zomwe zimayambitsa vutoli sizikudziwika. Vutoli silimatengera kubadwa nako.

Ndi PV, pali maselo ofiira ambiri mthupi. Izi zimabweretsa magazi ochulukirapo, omwe samatha kudutsa mumitsempha yaying'ono nthawi zambiri, kumabweretsa zizindikilo monga:

• Kuvuta kupuma mukamagona
• Khungu labuluu
• Chizungulire
• Kumva kutopa nthawi zonse
• Kutaya magazi ochulukirapo, monga kutuluka magazi pakhungu
• Kumverera kwathunthu kumimba kumtunda chakumanzere (chifukwa cha nthenda yotakasa)
• Mutu
• Kutsekemera, makamaka pambuyo pa kusamba kotentha
• Mtundu wofiyira khungu, makamaka kumaso
• Kupuma pang'ono
• Zizindikiro zamagazi m'mitsempha pafupi ndi khungu (phlebitis)
• Mavuto masomphenya
• Kulira m'makutu (tinnitus)
• Ululu wophatikizana

Wothandizira zaumoyo adzayesa. Muthanso kukhala ndi mayeso otsatirawa:

• Kutupa kwa mafupa
• Kuwerengera kwathunthu kwa magazi ndikusiyanitsa
• Zowonjezera zamagetsi
• Mulingo wa erythropoietin
• Mayeso amtundu wa kusintha kwa JAK2V617F
• Kutsitsa magazi magazi
• Maselo ofiira ofiira
• Mulingo wa Vitamini B12

PV ingakhudzenso zotsatira za mayeso otsatirawa:

• ESR
• Chotupa cha dehydrogenase (LDH)
• Leukocyte zamchere phosphatase
• Mayeso ophatikizira ma Platelet
• Seramu uric acid

Cholinga cha chithandizo ndikuchepetsa makulidwe amwazi ndikupewa kutaya magazi komanso mavuto a magazi.

Njira yotchedwa phlebotomy imagwiritsidwa ntchito pochepetsa makulidwe amwazi. Gawo limodzi lamagazi (pafupifupi 1 pint, kapena 1/2 lita) limachotsedwa sabata iliyonse mpaka kuchuluka kwa maselo ofiira amwazi. Mankhwalawa akupitilira momwe amafunira.

Mankhwala omwe angagwiritsidwe ntchito ndi awa:

• Hydroxyurea kuchepetsa kuchuluka kwa maselo ofiira amwazi omwe amapangidwa ndi mafupa. Mankhwalawa atha kugwiritsidwa ntchito manambala amitundu yamagazi ambiri nawonso.
• Interferon kuti achepetse kuchuluka kwa magazi.
• Anagrelide kuti muchepetse kuwerengera kwa ma platelet.
• Ruxolitinib (Jakafi) kuchepetsa kuchuluka kwa maselo ofiira ndikuchepetsa nthenda yotakasa. Mankhwalawa amaperekedwa pamene hydroxyurea ndi mankhwala ena alephera.

Kutenga aspirin kuti muchepetse chiopsezo chamagazi kungakhale mwayi kwa anthu ena. Koma, aspirin imakulitsa ngozi zakutuluka m'mimba.

Mankhwala a ultraviolet-B amatha kuchepetsa kuyabwa kwambiri komwe anthu ena amakumana nako.

Mabungwe otsatirawa ndi zida zabwino zodziwitsa polycythemia vera:

• National Organisation for Rare Disways - rarediseases.org/rare-diseases/polycythemia-vera
• Center Center Information Information ya NIH Genetic and Rare Diseases - rarediseases.info.nih.gov/diseases/7422/polycythemia-vera

PV nthawi zambiri imayamba pang'onopang'ono. Anthu ambiri alibe zizindikilo zokhudzana ndi matendawa nthawi yodziwika. Matendawa amapezeka nthawi zambiri asanakwane.

Zovuta za PV zitha kuphatikizira izi:

• Khansa ya m'magazi (AML)
• Kutuluka magazi m'mimba kapena mbali zina zamatumbo
• Gout (kutupa kopweteka kwa cholumikizira)
• Mtima kulephera
• Myelofibrosis (matenda am'mafupa momwe mafupa amalowetsedwa ndi minofu yolimba)
• Thrombosis (kutseka magazi, komwe kumatha kuyambitsa stroke, matenda amtima, kapena kuwonongeka kwa thupi)

Itanani omwe akukuthandizani ngati zizindikiro za PV zikuyamba.

Pulayimale yoyamba; Polycythemia rubra vera; Matenda a Myeloproliferative; Erythremia; Splenomegalic polycythemia; Matenda a Vaquez; Matenda a Osler; Polycythemia ndi cyanosis yanthawi zonse; Erythrocytosis megalosplenica; Cryptogenic polycythemia

Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. Ma polycythemias. Mu: Hoffman R, Benz EJ, Silberstein LE, et al, olemba. Hematology: Mfundo Zoyambira ndi Zochita. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 68.

Tsamba la National Cancer Institute. Matenda a myeloproliferative neoplasms treatment (PDQ) - mtundu wa akatswiri azaumoyo. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/_5. Idasinthidwa pa February 1, 2019. Idapezeka pa Marichi 1, 2019.

Tefferi A. Polycythemia vera, thrombocythemia yofunikira, ndi myelofibrosis yoyamba. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. Wolemba 25. Philadelphia, PA: Elsevier Saunders; 2016: chap 166.