Polycythemia vera

Polycythemia vera (PV) chirwere chemafupa chinotungamira mukuwedzera kwakasarudzika kwenhamba yemasero eropa. Masero matsvuku eropa anonyanya kukanganiswa.

PV kusagadzikana kwepfupa remongo. Inonyanya kukonzera masero matsvuku eropa akawandisa kugadzirwa. Huwandu hwemasero machena eropa uye maplatelet anogona zvakare kuve akareba kupfuura zvakajairwa.

PV chirwere chisingawanzoitika chinowanzoitika mune varume kupfuura vakadzi. Hazviwanzoonekwa kune vanhu vari pasi pemakore makumi mana. Dambudziko rinowanzo sunganidzwa nehurema hwesini hunonzi JAK2V617F. Chikonzero cheiyi geni chirema hachizivikanwe. Uku kuremara kwemajini hakusi chirwere chinogarwa nhaka.

NePV, kune akawanda masero matsvuku eropa mumuviri. Izvi zvinoguma neropa rakakora kwazvo, risingakwanise kuyerera kuburikidza nemidziyo midiki yeropa zvakajairika, zvichitungamira kuzviratidzo zvakaita se:

• Kutambudzika kufema kana wakarara pasi
• Bluish ganda
• Dzungu
• Kunzwa kuneta nguva dzose
• Kuwedzera kubuda ropa, sekubuda ropa mukati meganda
• Kunzwa kuzere mudumbu repamusoro kuruboshwe (nekuda kwekukura kwepleen)
• Kutemwa nemusoro
• Itchiness, kunyanya mushure mekushambidza kwekushambidza
• Ruvara rutsvuku rweganda, kunyanya kumeso
• Kufema kwekufema
• Zviratidzo zveropa rakagwamba mutsinga padyo neganda (phlebitis)
• Matambudziko ekuona
• Kurira munzeve (tinnitus)
• Joint kurwadziwa

Anopa hutano anoita bvunzo dzepanyama. Iwe unogona zvakare kuve nemiedzo inotevera:

• Bone marora bhiopsy
• Yakazara kuverenga kweropa nemusiyano
• Yakakwana metabolic pani
• Erythropoietin chikamu
• Genetic bvunzo yeiyo JAK2V617F shanduko
• Oxygen kuzadza kweropa
• Tsvuku tsvuku yesero reropa
• Vitamin B12 chikamu

PV inogona zvakare kukanganisa mhedzisiro yeiyi inotevera bvunzo:

• ESR
• Lactate dehydrogenase (LDH)
• Leukocyte alkaline phosphatase
• Platelet kuwanda bvunzo
• Serum uric acid

Chinangwa chekurapa ndechekuderedza hukobvu hweropa uye kudzivirira kubuda kweropa nekugwamba kwematambudziko.

Imwe nzira inonzi phlebotomy inoshandiswa kudzikisa kukora kweropa. Yuniti yeropa (inenge 1 pint, kana 1/2 litre) inobviswa vhiki yega yega kudzamara huwandu hwamasero matsvuku eropa hwaderera. Kurapa kunoenderera sekudikanwa.

Mishonga inogona kushandiswa inosanganisira:

• Hydroxyurea yekudzikisa huwandu hwesero dzvuku reropa rakagadzirwa nemongo wemapfupa. Mushonga uyu unogona kushandiswa kana huwandu hwemamwe maseru eropa mhando akakwirira.
• Interferon kudzikisa kuwanda kweropa.
• Anagrelide kudzikisa platelet kuverenga.
• Ruxolitinib (Jakafi) kudzikisa huwandu hwesero dzvuku reropa uye kudzora yakawedzera spleen. Uyu mushonga unorayirwa apo hydroxyurea uye mamwe marapirwo atadza.

Kutora aspirin yekudzora njodzi yekuvhara ropa kungave kuri sarudzo kune vamwe vanhu. Asi, aspirin inowedzera njodzi yekubuda ropa mudumbu.

Ultraviolet-B mwenje kurapa kunogona kudzikisa kukatyamadza kwakakomba kunoitika kune vamwe vanhu.

Masangano anotevera zviwanikwa zvakanaka zvekuziva nezve polycythemia vera:

• National Sangano reRare Dambudziko - rarediseases.org/rare-diseases/polycythemia-vera
• NIH Genetic uye Rare Zvirwere Ruzivo Ruzivo - rarediseases.info.nih.gov/diseases/7422/polycythemia-vera

PV inowanzo kukura zvishoma nezvishoma. Vanhu vazhinji havana zviratidzo zvine chekuita nechirwere panguva yekuongororwa. Mamiriro acho anowanzoongororwa zvisati zvaitika zviratidzo zvakakomba.

Zvinetso zvePV zvinogona kusanganisira:

• Acute myelogenous leukemia (AML)
• Kubuda ropa mudumbu kana zvimwe zvikamu zvemudumbu
• Gout (kuzvimba kunorwadza kwejoini)
• Kutadza kwemoyo
• Myelofibrosis (kusagadzikana kwemongo wemapfupa umo mongo unotsiviwa ne fibrous scar tishu)
• Thrombosis (kuvhara ropa, uko kunogona kukonzera sitiroko, kurwiswa nemoyo, kana kukuvara kwemuviri)

Fonera waunopa kana zviratidzo zvePV zvichikura.

Primary polycythemia; Polycythemia rubra vera; Myeloproliferative kusagadzikana; Erythremia; Splenomegalic polycythemia; Chirwere cheVaquez; Chirwere chaOsler; Polycythemia ane chisingaperi cyanosis; Erythrocytosis megalosplenica; Cryptogenic polycythemia

Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. Iyo polycythemias. Muna: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Misimboti uye Maitiro. 7th ed. Philadelphia, PA: Elsevier; 2018: chitsuwa 68.

National Cancer Institute webhusaiti. Chisingaperi myeloproliferative neoplasms kurapwa (PDQ) - hutano nyanzvi vhezheni. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/_5. Yakagadziridzwa Kukadzi 1, 2019. Yakasvika munaKurume 1, 2019.

Tefferi A. Polycythemia vera, yakakosha thrombocythemia, uye yekutanga myelofibrosis. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: chap 166.