ʻO Polcythemia vera

ʻO Polycythemia vera (PV) kahi maʻi pūpū iwi e alakaʻi i kahi piʻi maʻamau o ka helu o nā hunaola koko. Hoʻopili nui ʻia nā hunaola ʻulaʻula.

He maʻi ʻo PV o ka momona o ka iwi. Hoʻokumu nui ʻia ia i nā hunaola ʻulaʻula e hana ʻia. ʻO nā helu o nā hunaola keʻokeʻo a me nā platelet e kiʻekiʻe paha ma mua o ka maʻamau.

He maʻi maʻi laha ʻole ʻo PV e kū pinepine i nā kāne ma mua o nā wahine. ʻAʻole ʻike pinepine ʻia i ka poʻe ma lalo o ka makahiki 40. Pili pinepine ka pilikia i kahi maʻi kīnā i kapa ʻia ʻo JAK2V617F. ʻAʻole ʻike ʻia ke kumu o kēia hemahema. ʻAʻole kēia maʻi kīnā i hoʻoilina hoʻoilina.

Me ka PV, nui a hewahewa ke koko ʻulaʻula i ke kino. ʻO kēia ka hopena i ke koko mānoanoa, ʻaʻole hiki ke kahe ma o nā kīʻaha koko liʻiliʻi maʻamau, e alakaʻi i nā ʻōuli e like me:

• Hoʻopilikia i ka hanu ke moe i lalo
• ʻIli polū
• Puʻupuʻu
• Manaʻo luhi i nā manawa āpau
• Kahe nui ke koko, e like me ke kahe ʻana i ka ʻili
• ʻO ka manaʻo piha i ka ʻōpū hema hema (ma muli o ka spleen hoʻonui ʻia)
• ʻEha poʻo
• Itchiness, keu hoʻi ma hope o ka ʻauʻau mahana
• ʻO ka pena ʻulaʻula, keu hoʻi o ka helehelena
• Pokole o ka hanu
• Nā ʻōuli o ke koko clots i loko o nā uaua kokoke i ka ʻili o ka ʻili (phlebitis)
• Nā pilikia ʻike
• Ke kani nei i nā pepeiao (tinnitus)
• ʻEha hui

E hana ka mea mālama ola i kahi hoʻokolohua kino. Loaʻa iā ʻoe i nā hōʻike aʻe:

• Biopsy iwi ʻōiwi
• Helu piha ke koko me ka ʻokoʻa
• Panela metabolic piha
• Pae ʻErythropoietin
• Hōʻike genetic no ka hoʻololi JAK2V617F
• Oxygen ma saturation o ke koko
• Ka nui o ke koko ʻulaʻula
• ʻO ka pae Vitamin B12

Hiki paha i ka PV ke hoʻopili i nā hopena o nā hōʻike aʻe:

• ESR
• Lactate dehydrogenase (LDH)
• Leukocyte alkaline phosphatase
• Hōʻike hōʻuluʻulu platelet
• Serum uric acid

ʻO ka pahuhopu o ka mālama ʻana e hōʻemi i ka mānoanoa o ke koko a pale i nā pilikia kahe ʻana o ke kahe ʻana a me ke kō ʻana.

Hoʻohana ʻia kahi hana i kapa ʻia phlebotomy e hoʻemi i ka mānoanoa o ke koko. Wehe ʻia hoʻokahi anakahi koko (ma kahi o 1 pint, a i ʻole 1/2 lita) i kēlā me kēia pule a hiki i ka heleleʻi ʻana o ka nui o nā hunaola ʻulaʻula. Hoʻomau ʻia ka lapaʻau ʻana e like me ka mea e pono ai.

ʻO nā lāʻau e hiki ke hoʻohana ʻia:

• Hydroxyurea e hōʻemi i ka helu o nā hunaola ʻulaʻula i hana ʻia e ka iwi o ka iwi. Hiki ke hoʻohana ʻia kēia lāʻau lapaʻau ke kiʻekiʻe pū ka helu o nā ʻano ʻē aʻe o ke koko.
• Interferon e hoʻohaʻahaʻa i nā helu o ke koko.
• Anagrelide e hoʻohaʻahaʻa i nā helu platelet.
• ʻO Ruxolitinib (Jakafi) e hoʻemi i ka helu o nā hunaola ʻulaʻula a hoʻēmi i kahi ākea hoʻonui. Kau ʻia kēia lāʻau i ka wā i holo pono ʻole ai ka hydroxyurea a me nā lāʻau ʻē aʻe.

Lawe i ka aspirin e hōʻemi i ka makaʻu o ke kahe koko ke koho ʻia no kekahi poʻe. Akā, hoʻonui ka aspirin i ka makaʻu o ke kahe o ka ʻōpū.

Hiki i ke kukui uila ʻo Ultraviolet-B ke hōʻemi i ka makaʻu nui i ʻike ʻia e kekahi poʻe.

ʻO nā hui aʻe nā kumuwaiwai maikaʻi no ka ʻike ma polycythemia vera:

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

Hoʻolālā mālie ʻo PV. ʻAʻole loaʻa i ka hapanui o nā kānaka nā ʻōuli e pili ana i ka maʻi i ka manawa o ke kuhi. Hoʻomaopopo pinepine ʻia ke ʻano ma mua o ka hiki ʻana mai o nā hōʻailona koʻikoʻi.

Pākuʻi ʻia paha nā komplication o PV:

• Huʻi myelogenous leukemia (AML)
• Kahe mai ka ʻōpū a i ʻole nā ​​ʻāpana ʻē aʻe o ke kōpū o ka ʻōpū
• Gout (ʻehaʻeha o ka hui)
• Hewa puʻuwai
• Myelofibrosis (maʻi o ka iwi iwi kahi i kuapo ʻia ka momona e ka fibrous scar tissue)
• Thrombosis (kāpili koko, i hiki ai ke hōʻeha, hōʻeha puʻuwai, a i ʻole nā ​​hōʻino kino ʻē aʻe)

Kāhea i kāu mea hāʻawi inā ulu nā ʻōuli o PV.

ʻO polycythemia mua; Polycythemia rubra vera; Maʻi Myeloproliferative; ʻErythremia; Polycythemia Splenomegalic; Maʻi o Vaquez; Maʻi ʻo Osler; Polycythemia me ka cyanosis mau; Erythrocytosis megalosplenica; Polycythemia Cryptogenic

Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. The polycythemias. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 68.

Pūnaewele ʻo National Cancer Institute. Mālama myeloproliferative neoplasms lapaʻau (PDQ) - mana loea mana. www.cancer.gov/types/myeloproliferative/hp/cornic-treatment-pdq#link/_5. Hoʻohou i Pepeluali 1, 2019. Loaʻa ʻia ma Malaki 1, 2019.

ʻO Tefferi A. Polycythemia vera, thrombositosis pono, a me myelofibrosis kumu. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 25th hua. Piladelapia, PA: Elsevier Saunders; 2016: chap 166.