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He kiʻekiʻe loa anei ka helu o kou mau hunaola koko ʻulaʻula? E kamaʻilio kākou e pili ana i ka Polycythemia Vera (PV)!

He kiʻekiʻe loa anei ka helu o kou mau hunaola koko ʻulaʻula? E kamaʻilio kākou e pili ana i ka Polycythemia Vera (PV)!

Ke luhi pinepine nei ʻoe, i kekahi manawa he ʻano ʻūlū, a lohe wale paha i ke kani ʻana o kou mau pepeiao? ʻOiai ke ʻano maʻamau kēia mau mea, i kekahi manawa aia kekahi kumu ʻē aʻe ma hope o lākou. No laila, ʻo ka maʻi a mākou e kamaʻilio ai i kēia lā ʻo Polycythemia Vera (PV) , he ʻano ia i hoʻokumu ʻia e ka hana nui ʻana o nā hunaola koko ʻulaʻula i loko o ko mākou kino. ʻOiai he inoa lōʻihi kēia, e hoʻomaopopo maʻalahi kākou.

He aha ka Polycythemia Vera (PV)? I ka ʻōlelo maʻalahi...

I ka ʻōlelo maʻalahi, ʻo Polycythemia Vera (PV) kahi maʻi kahi e hana ai kou kino, ʻoiai kou iwi iwi (ka mea a mākou e kapa nei he "Bone Marrow"), i nā hunaola koko ʻulaʻula ma mua o ka mea e pono ai. E like me ke kaʻa ʻana, i ka wā e nui ai nā kaʻa ma ke alanui, ua ālai ʻia ke kahe ʻana o ke koko. Hoʻomānoanoa kēia i ke koko a hele mālie. Hoʻonui kēia i ka pilikia o nā ʻūpā koko. Hiki i kēia ke alakaʻi i nā maʻi koʻikoʻi e like me nā hōʻeha puʻuwai a me nā hahau .

ʻAʻole wale kēia, i kekahi manawa hiki ke loaʻa nā hōʻailona e like me ka ʻili maneʻo, ke kani ʻana o nā pepeiao (`(Tinnitus)`), ka ʻeha o ka ʻōpū, ke kahe ʻana o ke koko ihu, a me ka ʻike pōwehiwehi.

He maʻi mau loa ka Polycythemia Vera (PV) ʻaʻole hiki ke ho'ōla loa ʻia. Akā, mai hopohopo. Me ka mālama maikaʻi ʻana i ke olakino, hiki iā ʻoe ke kāohi i nā hōʻailona a hōʻemi i ka pilikia o nā hopena ʻaoʻao. Ua kapa pū kekahi poʻe iā ia he 'polycythemia mua', 'polycythemia rubra vera', 'erythema' a me 'Osler-Vaquez disease'.

He maʻi ʻaʻai anei ka Polycythemia Vera (PV)?

ʻAe, he ʻano maʻi kanesa kēia. Akā, ʻaʻole ia he ʻano koʻikoʻi a laha wikiwiki paha e like me kā mākou e manaʻo ai ke lohe mākou i ka huaʻōlelo kanesa. He maʻi kanesa koko kēia no kahi hui i kapa ʻia ʻo `myeloproliferative neoplasm (MPN) . ʻO `MPN` e pili ana i kahi hui o nā maʻi e hoʻoulu ai i ka hana nui ʻana o nā ʻano ʻano koko like ʻole.

ʻO ke kumu nui o ka Polycythemia Vera (PV) he loli (mutation) i loko o kahi gene i loko o ko kākou kino. ʻAʻole kēia he mea i hānau ʻia me kākou, akā he mea e kū mai ana i ko kākou ola no kekahi kumu i ʻike ʻole ʻia. Lohi loa ka ulu ʻana o kēia maʻi. Hoʻomaopopo pinepine ʻia ma hope o 60 mau makahiki. Kākaʻikahi kēia maʻi ʻaʻai e make pololei ai. Loaʻa ka pilikia nui mai nā pilikia i hoʻokumu ʻia e nā ʻūpā koko. Eia kekahi, ma kahi pakeneka liʻiliʻi loa, hiki iā ia ke lilo i nā ʻano maʻi koko koʻikoʻi ʻē aʻe.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia kūlana?

He maʻi laha ʻole ka Polycythemia Vera (PV). Ma kahi ʻāina e like me ʻAmelika Hui Pū ʻIa, loaʻa ia ma kahi o 50 no 100,000 poʻe. ʻOi aku ka maʻamau i ka poʻe ma luna o 60 mau makahiki.

Pehea ka hopena o kēia maʻi i ko mākou kino?

E like me kā mākou i ʻōlelo ai ma mua, ʻo ka Polycythemia Vera (PV) ke kumu o ke kino e hana i nā hunaola koko ʻulaʻula he nui loa. ʻO kēia keu o nā hunaola e hoʻonui ai i ka pilikia o ke kahe ʻana o ke koko, ka palapū, a me nā ʻūpā koko. Ma muli o ka mānoanoa o ke koko a me ka hele mālie ʻana, ʻaʻole i loaʻa i nā ʻiʻo a me nā ʻōkana o ko mākou kino ka oxygen e pono ai.

Hoʻokomo pū kēia i ka nui o ka hana ma ka spleen o ko mākou kino. ʻO kekahi o nā hana nui o ka spleen, ʻo ia ke kānana i ke koko a wehe i nā hunaola koko kahiko. Ke hoʻonui ʻia nā hunaola koko ʻulaʻula, hana nui ʻia ka spleen, hiki ke hoʻoulu i ka pehu a me ka ʻeha. Ua kapa ʻia kēia ʻo `(Splenomegaly)`. I ka hala ʻana o ka manawa, hiki i kēia ʻano ke hana i nā hōʻailona ʻoluʻolu ʻole. Eia kekahi, hiki i ka PV ke hana i nā ʻano lua ʻē aʻe.

He aha nā pilikia a me nā pilikia o ka Polycythemia Vera (PV)?

ʻOiai he maʻi holomua ka PV, aia kahi pilikia o ke koko koke ʻana ma muli o ka hana nui ʻana o nā hunaola koko. I ka hala ʻana o ka manawa, hiki i ka PV ke hana i nā kūlana lua ʻē aʻe. He mea kākaʻikahi loa, hiki ke lilo i maʻi ʻaʻai koʻikoʻi.

Nā ʻūpā koko

ʻO kēia ka pilikia nui a koke loa o ka PV. Inā hoʻokumu ʻia kahi ʻaʻa koko a hele i ka puʻuwai a i ʻole ka lolo, hiki iā ia ke hana i ka hōʻeha puʻuwai a i ʻole ka hahau . Inā hele ia i nā māmā (Pulmonary Embolism - PE), hiki iā ia ke hana i ke koko kiʻekiʻe i loko o nā māmā (pulmonary hypertension) a me ka hāʻule ʻana o ka puʻuwai. Inā paʻa kahi ʻaʻa koko i loko o kahi aʻa (Venous Thromboembolism - VTE), hiki iā ia ke make i nā ʻiʻo a i ʻole ka lawa ʻole o ka venous. Inā paʻa kahi ʻaʻa koko i loko o ke aʻa koko nui i ke akepaʻa (hepatic vein thrombosis), hiki iā ia ke hana i nā ʻaʻa koko i loko o ke akepaʻa (Budd-Chiari syndrome), jaundice a me ka hāʻule ʻana o ke akepaʻa.

Nā kūlana lua

Ke hana nui ʻia nā hunaola koko ʻulaʻula a wāwahi ʻia, piʻi ka nui o ka `uric acid` i loko o ke kino. Hiki i kēia ke hana i kekahi mau pilikia:

  • Nā pōhaku puʻupaʻa: Ke waiho ʻia ka waikawa uric i loko o nā puʻupaʻa.
  • Gout: He ʻeha ʻeha ka maʻi ʻaʻai e kū mai ana i ka wā e hōʻiliʻili ai ka uric acid i loko o nā hono.
  • ʻŌpū ʻōpū: Hoʻonui pū nā hunaola koko ʻulaʻula keu i ka nui o ka waikawa ʻōpū. Hoʻokuʻu nā hunaola koko ʻulaʻula keu i ke kino i kahi kemika i kapa ʻia ʻo histamine, kahi e hoʻoulu ai i ka ʻōpū e hana i ka waikawa hou aʻe e pale aku i ka maʻi. ʻO ka poʻe me PV he ʻehā manawa ka nui o ka loaʻa ʻana o nā ʻōpū ʻōpū (maʻi ʻōpū peptic).

Ke huli nei i ka Leukemia

He maʻi ʻaʻai o ka iwi iwi ka Polycythemia Vera, akā ʻaʻole ia he maʻi hoʻoweliweli i ke ola. Me ka mālama pono ʻana, hiki ke mālama maikaʻi ʻia no nā makahiki. Eia nō naʻe, no kekahi poʻe, hiki i ka PV, ʻoiai he kakaikahi,Hiki iā ia ke lilo i maʻi ʻaʻai koko koʻikoʻi e like me ka 'Acute Leukemia'.

Nā Pae o ka maʻi Polycythemia Vera (PV)

Hoʻomau ka maʻi PV penei:

  • Ke kahua mua ('PV Mua'): I kēia kahua, he kakaikahi a ʻaʻohe paha nā hōʻailona.
  • Ke holomua nei ka PV: Ke lilo nei ka PV i mea koʻikoʻi, e hoʻomaka paha ʻoe e ʻike i nā hōʻailona ʻoluʻolu ʻole, a i ʻole e ulu aʻe nā kūlana lua.
  • Ka pae i hoʻohana ʻia: Ua kapa ʻia kēia ʻo "ka pae i hoʻohana ʻia." I kēia manawa, hoʻonui nui ʻia nā hunaola koko ʻē aʻe e hoʻoulu ai i ka maʻi a ālai loa i ka iwi ʻōpala, ka mea e hana ai i nā hunaola koko. Ke make kēia mau hunaola ʻē, hoʻokumu ʻia nā ʻiʻo ʻeha ma ko lākou wahi. Ke piha ka hapa nui o ka iwi ʻōpala me kēia ʻiʻo ʻeha, ʻaʻole hiki iā ia ke hana i nā hunaola koko olakino. ʻO ka mea kupanaha, hoʻoulu kēia i ka anemia - ka nele o nā hunaola koko ʻulaʻula olakino. Hoʻonui pū ia i ka pilikia o ke kahe ʻana o ke koko.

ʻO nā maʻi koko ʻē aʻe e hiki ke hana ma hope o ka PV

Ke koʻikoʻi ka PV, hiki iā ia ke alakaʻi i nā maʻi koko ʻē aʻe.

  • ʻO Myelofibrosis (MF): ʻAʻohe ʻokoʻa nui ma waena o ka "split phase" o PV a me "Myelofibrosis (MF)" , ʻo ia kekahi ʻano maʻi koko ʻē aʻe. Manaʻo kekahi mau kauka he like lāua. ʻO "Myelofibrosis" ka wā e hana ai nā cell abnormal i nā ʻiʻo ʻeha i loko o ka iwi iwi a hoʻopiha iā ia. Hiki i kēia mau cell abnormal ke pālahalaha ma waho o ka iwi iwi a i loko o nā ʻāpana ʻē aʻe o ke kino. ʻO "MF" kekahi "maʻi myeloproliferative." Ma kahi o 10% o nā maʻi "MF" e loaʻa i ka "Acute Myeloid Leukemia."
  • ʻO ka Myelodysplastic Syndrome (MDS): He mea kākaʻikahi loa, hiki i ka PV ke ulu i kahi kūlana i kapa ʻia ʻo Myelodysplastic Syndrome (MDS) . He kūlana kēia kahi e oʻo pono ʻole ai nā hunaola koko, ulu ma ke ʻano ʻē, a make koke. ʻAʻole hiki i ke kino ke hana i nā hunaola olakino a oʻo, e hopena ana i ka emi ʻana o ka helu o nā ʻano hunaola koko like ʻole. ʻOi aku ka ʻino o ka MDS ma mua o PV a i ʻole MF, a he 30% ka pilikia o ka loaʻa ʻana o ka Acute Myeloid Leukemia (AML).
  • ʻO ka Acute Myeloid Leukemia (AML): Ma kahi o 3% o ka poʻe me Polycythemia Vera e loaʻa i ka Acute Myeloid Leukemia (AML) i loko o 10 mau makahiki o ka ʻike ʻia ʻana. He maʻi koko koʻikoʻi ka AML e hoʻomaka ana ma ka iwi iwi a hiki ke laha koke i ke koko a laila i nā ʻāpana ʻē aʻe o ke kino. Pono ia i ka mālama koke ʻana, akā kiʻekiʻe ka manawa e ola ai.

He aha nā hōʻailona o ka Polycythemia Vera (PV)?

ʻIke pinepine ʻia nā hōʻailona me ka lohi loa, a i kekahi manawa ʻaʻohe hōʻailona iki. ʻO ka mea mua, ʻo ia nā hōʻailona maopopo ʻole i maʻa mau i nā maʻi he nui:

  • ʻEha poʻo.
  • Pōʻaiʻī.
  • Luhi.
  • Ke koko kiʻekiʻe.
  • ʻIke pōwehiwehi a ʻike pālua paha.
  • Ke lohe ʻana i ke kani ʻana o nā pepeiao (`(Tinnitus)`).

I ka hala ʻana o ka manawa, hiki ke ʻike ʻia nā hōʻailona kikoʻī hou aʻe:

  • Ka hou ʻana, ʻoi aku hoʻi i ka pō.
  • Ka paʻakikī o ka hanu ʻana, ʻoiai i ka wā e moe ana.
  • ʻIli ʻāʻī, ʻoi aku hoʻi ma hope o ka ʻauʻau ʻana i ka wai wela. (He hōʻailona kikoʻī kēia o ka PV)
  • ʻUlaʻula, wela, mae, a i ʻole ke kuni ʻana o nā lālā.
  • Ke kahe koko nui a i ʻole ka palapū.
  • Ke kahe ʻana o ke koko mai ka ihu a me nā ʻāʻī.
  • Ka wiwi ʻana me ke kumu ʻole.

Inā lilo ka PV i mea koʻikoʻi a holomua i nā kūlana lua, hiki ke loaʻa nā hōʻailona pili. Eia kekahi laʻana:

Nā hōʻailona o ka Splenomegaly:

  • ʻO ka ʻeha e like me kekahi mea e paʻa ana ma ka ʻaoʻao hema o luna o ka ʻōpū.
  • He ʻike hoʻopiha ʻōpū.
  • ʻOiai ma hope o ka ʻai iki ʻana, ua piha wau.

Nā hōʻailona o nā ʻōpū ʻōpū ('Peptic ulcer disease'):

  • ʻEha e hele mai me ka ʻeha o ka ʻōpū.
  • ʻĀhaʻi puʻuwai.
  • ʻO ka reflux waikawa.

Nā hōʻailona o ka Gout:

  • ʻO ka pehu ʻeha o nā hono.
  • ʻOʻoleʻa o nā hono.
  • Ka pehu ʻana o ka manamana wāwae nui.

Nā hōʻailona o nā pōhaku puʻupaʻa:

  • ʻEha ma ka ʻōpū lalo a i ʻole ka ʻaoʻao.
  • ʻEha i ka wā e mimi ai.
  • Pono pinepine e mimi.

Nā hōʻailona o ka maʻi Budd-Chiari:

  • ʻAke pehu, ʻeha ma ka ʻaoʻao ʻākau o luna o ka ʻōpū, a me ka pehu ʻana.
  • Melemele o nā maka a me ka ʻili (jaundice).
  • ʻO nā Ascites a me ka Edema.

Nā hōʻailona o ka Deep Vein Thrombosis (DVT):

  • Ka pehu ʻana ma ka lima a i ʻole ka wāwae a me ka ʻeha ke hoʻopā ʻia.
  • Aia ka ʻulaʻula a i ʻole ka wela ma ia wahi.
  • Ke ʻano o nā kīʻaha koko e pehu ana ma luna o ka ʻili.

Nā hōʻailona o ke koko i loko o nā māmā (Pulmonary Embolism - PE):

  • ʻEha koke ka umauma.
  • Pōkole o ka hanu.
  • Piʻi ka helu o ka puʻuwai.

Nā hōʻailona o ka Anemia (i nā pae hope o ka maʻi PV):

  • ʻŌnionio (vertigo).
  • ʻIli keʻokeʻo.
  • Luhi.

He aha ke kumu o ka Polycythemia Vera (PV)?

Hoʻomaka ka Polycythemia Vera (PV) ma ka ʻāpana palupalu a huʻahuʻa o ko kākou mau iwi i kapa ʻia ʻo ka iwi iwi. Maanei kahi e hana ʻia ai nā hunaola koko hou. Hoʻomaka ka PV me kahi hoʻololi ʻana o ka gene hoʻokahi i loko o kahi hunaola kumu hoʻokahi i loko o ka iwi iwi. Ma mua o 90% o ka manawa, aia kēia hoʻololi ʻana i loko o kahi gene i kapa ʻia ʻo JAK2.

Ke haʻi aku nei kēia gene i hoʻololi ʻia i ke kelepona kumu e hoʻomau i ka hana hou ʻana. Ke hoʻomau nei kēlā me kēia kelepona i hana ʻia e hana hou iā ia iho. I ka hopena, hoʻopiha kēia mau kelepona ʻano ʻē i ka iwi iwi, ʻaʻohe wahi no nā kelepona maʻamau.

I ka hapanui o nā hihia, ʻaʻole kēia hoʻololi ʻana o ka gene `JAK2` he mea ia a mākou e hoʻoilina ai. He mea ia e hana ʻia i ka wā o ke ola, no nā kumu i ʻike ʻole ʻia. Eia nō naʻe, he mea kākaʻikahi loa, ua loaʻa nā hōʻike e pili ana i kekahi mau lālā o ka ʻohana e ulu ana i ka PV.

Pehea e ʻike ʻia ai ka Polycythemia Vera (PV)?

Ua hāpai ka World Health Organization (WHO) i ʻekolu mau kumu nui no ka ʻike ʻana i ka Polycythemia Vera (PV). Pono ke kauka e hōʻoia i nā kumu ʻekolu.

Paepae 1: He hoʻāʻo koko e hōʻike ana i ka helu koko ʻulaʻula kiʻekiʻe:

Hiki ke ana ʻia nā hunaola koko ʻulaʻula:

  • Nā pae hemoglobin kiʻekiʻe (kahi protein i loaʻa i loko o nā hunaola koko ʻulaʻula).
  • ʻO ke kiʻekiʻe o ka hematocrit (ka pakeneka o nā hunaola koko ʻulaʻula i loko o ke koko).
  • Ka nui o ke koko (ka nui o nā ʻulaʻula koko).

Pae 2: ʻO ka biopsy iwi e hōʻike ana i kekahi o kēia mau mea:

  • Nui loa nā hunaola koko i loko o ka iwi iwi, a i ʻole
  • ʻO ka nui o nā megakaryocytes makua (nā pūnaewele e hana i nā platelets koko).

Paepae 3: Hiki ke hoʻokō ʻia ke paepae ʻekolu ma ka hōʻike ʻana i kekahi o kēia mau mea ʻelua:

  • Ka hōʻoia ʻana o ke alo o ka hoʻololi ʻana o ka gene JAK2 ma o ka hoʻāʻo molekala, a i ʻole
  • Ua hōʻoia ka hoʻāʻo koko ua haʻahaʻa loa ka pae o ka `erythropoietin' (kahi hormone e hoʻoulu ai i ka hana ʻana o nā hunaola koko ʻulaʻula e nā puʻupaʻa) i loko o ke koko.

"E nānā ke kauka i kēia mau mea a pau e haʻi pololei iā ʻoe inā he Polycythemia Vera kou a i ʻole. No laila, inā loaʻa iā ʻoe kekahi mau hōʻailona, ​​​​ʻoi aku ka maikaʻi o ke ʻike ʻana i ke kauka a loaʻa ka ʻōlelo aʻoaʻo."

Pehea e mālama ʻia ai ka Polycythemia Vera (PV)?

I nā pae mua o ka PV, he maʻalahi ka mālama ʻana. ʻO nā lāʻau lapaʻau maʻamau:

Phlebotomy (ke kahe koko ʻana)

ʻO kēia ke ʻano lapaʻau nui a maʻamau no ka PV. Hana ʻia ia ma ke ʻano like me kāu e hāʻawi ai i ke koko. Hoʻokomo kahi mea mālama ola i kahi nila i loko o kahi aʻa koko ma kou lima a wehe i kahi koko liʻiliʻi (ma kahi o hoʻokahi paina, akā hiki ke ʻokoʻa ka nui ma muli o kou kūlana). Hoʻemi kēia i ka nui o kou koko a me ka helu o nā hunaola koko keu.

ʻAspirin haʻahaʻa

He lāʻau lapaʻau kēia hiki iā ʻoe ke kūʻai ma ka hale kūʻai lāʻau. Hoʻohana pinepine ʻia ia e hōʻemi i ka pilikia o nā ʻōpū koko. Ke lawe ʻia i kahi mahele haʻahaʻa i hoʻokahi manawa i ka lā, pale ka aspirin i nā platelets koko mai ka pipili ʻana. Kōkua nō hoʻi ia i ka hōʻemi ʻana i nā hōʻailona e like me ka pehu ʻana i nā lālā. Eia nō naʻe, he ʻino iki ia i ka ʻōpū a hoʻonui i ke kahe ʻana o ke koko. No laila, ʻaʻole kēia he manaʻo maikaʻi inā loaʻa iā ʻoe nā ʻōpū ʻōpū.

Inā koʻikoʻi kou mau hōʻailona, ​​a inā he moʻolelo kou o nā ʻōpū koko ma mua (thrombosis), hiki ke hoʻokomo ʻia ʻoe i loko o kahi hui pilikia kiʻekiʻe a hiki ke hāpai ʻia nā koho lapaʻau ʻē aʻe. ʻO kekahi o kēia mau mea:

Nā lāʻau lapaʻau e hōʻemi ai i ka ʻeha:

Ke koʻikoʻi ka PV, hiki i ke kauka ke kuhikuhi i nā lāʻau lapaʻau e like me kēia e mālama ai i ka ʻeha:

  • Nā Antihistamine: He mau lāʻau lapaʻau maʻamau kēia i hāʻawi ʻia no nā maʻi allergies.
  • Phototherapy: He lāʻau lapaʻau e hoʻohui i ka mālamalama ultraviolet (UVA) me kahi kemika i kapa ʻia ʻo psoralen (kahi hui organik e hoʻonui ai i ka ʻili i ka light therapy).
  • ʻO SSRIs (Selective Serotonin Reuptake Inhibitors): He ʻano lāʻau lapaʻau kēia i kuhikuhi pinepine ʻia no ke kaumaha. Eia nō naʻe, i ka wā e hāʻawi ʻia ai i nā mahele haʻahaʻa loa, ua ʻike ʻia hoʻi he kūpono lākou no nā hōʻailona kino mau e like me ka maneʻo.

Nā lāʻau lapaʻau e hoʻemi ana i ka helu o nā ʻāpana koko ʻulaʻula:

Hiki ke hoʻohana ʻia kēia mau mea hoʻokahi a i ʻole e hui pū kekahi me kekahi. Nā Laʻana:

  • ʻO ka Hydroxyurea
  • ʻO ka Interferon alpha
  • ʻO Ruxolitinib
  • ʻO Busulfan

Nā hoʻokolohua lapaʻau

Hāʻawi kēia i nā kānaka i ka manawa kūpono e hoʻāʻo i nā lāʻau lapaʻau hou loa a hāʻawi i ka noiʻi lapaʻau. ʻO kekahi o nā lāʻau lapaʻau i kēia manawa i ka pae noiʻi:

  • ʻO ka Peginterferon alfa-2a
  • ʻO Givinostat
  • ʻIdasanutlin

Hoʻoili iwi iwi

I kekahi mau hihia, hiki ke ʻōlelo ʻia kahi hoʻoili iwi iwi. E hoʻoholo ke kauka i kēia ma muli o kekahi mau kumu, e like me ke koʻikoʻi o kou maʻi a me ka hiki o kou kino ke ho'ōla.

Mālama kākoʻo

Inā e hoʻomau ka ʻino o kāu PV me ka nānā ʻole i ka lāʻau lapaʻau, e kālele ana kāu kauka i ka hoʻokele ʻana i kāu mau hōʻailona. I nā pae hope o ka PV, hiki iā ʻoe ke loaʻa i ka anemia a me ka spleen i hoʻonui ʻia. Hiki ke hāʻawi ʻia iā ʻoe nā lāʻau lapaʻau e like me:

  • Nā lāʻau hoʻopau ʻeha.
  • Nā hoʻoili koko.
  • Lapaʻau radiation haʻahaʻa i ka spleen.

He aha ke kūlana o ke ola o kekahi kanaka me ka Polycythemia Vera (PV)?

Hōʻike nā haʻawina hou nei ʻo ka awelika o ke ola ma hope o ka ʻike ʻia ʻana o Polycythemia Vera (PV) ma kahi o 20 mau makahiki. ʻO ka awelika o ka makahiki o ka make ma kahi o 77 mau makahiki. ʻO ke kumu nui o ka make, ʻo ia nā pilikia mai nā ʻōpū koko (ma kahi o 33%). ʻO ka lua o ke kumu nui, ʻo ia ka maʻi ʻaʻai (ma kahi o 15%).

"Mai makaʻu i kēia mau helu helu. He mau waiwai maʻamau kēia. Inā loaʻa iā ʻoe ka lāʻau kūpono a hoʻololi i kou nohona, hiki iā ʻoe ke noho olakino no ka manawa lōʻihi."

Pehea e hiki ai iaʻu ke mālama iaʻu iho a hoʻokele i kaʻu mau hōʻailona?

Inā ʻoe e noho nei me ka Polycythemia Vera (PV), he mea nui e noho mau i ka launa pū ʻana me kāu kauka. E kōkua kēia i kāu kauka e nānā i ke ʻano o ka holomua ʻana o kou kūlana. Hiki iā ʻoe ke haʻi i kāu kauka pehea ʻoe e pane nei i ka lāʻau lapaʻau a inā paha ʻoe e ʻike nei i kekahi mau hopena ʻaoʻao. Hiki iā ʻoe ke ola lōʻihi me ka ʻole o nā hōʻailona koʻikoʻi.

Ma waho aʻe o nā hoʻokolohua maʻamau a me ka mālama ʻana, e kuhikuhi pū kāu kauka i kekahi mau hoʻololi maʻalahi o ke ʻano o ka nohona e hiki ke kōkua i ka pale ʻana i nā ʻūpā koko a me nā pilikia ʻē aʻe. Eia kekahi laʻana:

  • Hoʻoikaika kino. Hiki i kahi hoʻoikaika kino liʻiliʻi ke kōkua i ka hoʻomaikaʻi ʻana i ke kahe koko.
  • E pale i ka puhipaka. Hoʻohaiki ka puhipaka i kou mau kīʻaha koko.
  • E pale i nā wahi me nā pae oxygen haʻahaʻa. Hiki i nā wahi kiʻekiʻe ke hoʻohaʻahaʻa i nā pae oxygen koko.
  • E kāohi i kou koko. ʻO ka mālama ʻana i ke kaumaha olakino e kōkua i kēia.

ʻOiai ʻo ka Polycythemia Vera (PV) he ʻano maʻi ʻaʻai, nui ka poʻe e ola maikaʻi ana no nā makahiki he ʻumi ma hope o ka ʻike ʻia ʻana o ka maʻi. Loaʻa kēia maʻi ʻaʻai ma muli o ka hana ʻia ʻana o nā pūnaewele ma waho o ke kaohi. Eia nō naʻe, lōʻihi ka manawa no ka hana ʻole ʻia ʻana o nā pūnaewele koko ʻulaʻula e hoʻomaka ai e hoʻopilikia iā ʻoe. Inā hana ʻoe, hiki iā ʻoe ke hoʻokele i ke kūlana no ka manawa lōʻihi ma ka wehe ʻana i kekahi o kou koko keu.

ʻO ka hoʻoweliweli nui loa mai ka Polycythemia Vera (PV) ʻaʻole ia ke kanesa ponoʻī, akā ʻo ka pilikia o nā ʻūpā koko. Inā ʻoe e noho nei me ka PV, e ʻike pono i kāu kauka i nā manawa a pau. A mai poina e mālama iā ʻoe iho me nā "mea liʻiliʻi" - ʻo ia hoʻi, nā ʻano nohona olakino. Kōkua kēia i ka mālama ʻana i ke olakino holoʻokoʻa o kāu ʻōnaehana cardiovascular, kahi mea nui paha ma mua o kekahi lāʻau lapaʻau no Polycythemia Vera.

He aha ka leka a mākou e makemake ai e lawe i ka home mai kēia moʻolelo?

ʻAe, ua kamaʻilio nui mākou e pili ana i ka Polycythemia Vera (PV). Eia kekahi o nā mea nui e hoʻomanaʻo:

  • He maʻi koko ka PV kahi e hana ai ke kino i nā hunaola koko ʻulaʻula he nui loa a ulu aʻe ma ke ʻano ʻē. No loko ia o kahi hui o nā maʻi ʻaʻai i kapa ʻia ʻo `myeloproliferative neoplasm (MPN)`.
  • ʻO ka pilikia nui mai ka mānoanoa o ke koko a me ke kāpili ʻana o ke koko, hiki ke alakaʻi i nā maʻi koʻikoʻi e like me ka hōʻeha puʻuwai a me nā hahau.
  • Hiki i nā hōʻailona ke komo pū me ka ʻili maneʻo (ʻoi aku hoʻi ma hope o ka ʻauʻau ʻana), ka manaʻo palaualelo, ke poʻo ʻeha, a me ka poniuniu.
  • ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia, hiki ke mālama maikaʻi ʻia me ka mālama kūpono, nā loli o ke ʻano o ka nohona, a me ka lāʻau lapaʻau. ʻO ka phlebotomy (bloodletting) a me ka aspirin haʻahaʻa ka lāʻau lapaʻau mua.
  • ʻO ka hoʻololi pinepine ʻana o ka gene `JAK2` ke kumu o kēia.
  • Inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona, ​​e ʻike pono i ke kauka no ka ʻōlelo aʻo. Hoʻonui ka ʻike mua ʻana i kou manawa kūpono e pale aku ai i nā pilikia a me ke ola ʻana i kahi ola olakino.

Mai makaʻu, akā, mai hoʻomāmā hoʻi. E mālama i kou olakino. E kamaʻilio pinepine me kāu kauka, e loaʻa i nā hoʻokolohua a me nā lāʻau lapaʻau e pono ai i ka manawa kūpono. A laila hiki iā ʻoe ke noho olakino me kēia maʻi!


ʻO Polycythemia Vera, Nā Kelepona Koko ʻUlaʻula, Nā ʻAʻa Koko, MPN, JAK2, Phlebotomy, Kanesa

⚠️ Important: The medical articles and information on Nirogi Lanka are for general awareness only, and are by no means a substitute for professional medical advice, diagnosis, or treatment. For any medical problem you have, consult a qualified physician immediately.

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ʻAʻohe manaʻo i kau ʻia. Hoʻohui i kāu manaʻo ma ʻaneʻi no ka manawa mua.

Hoʻohui i kāu manaʻo

E ʻoluʻolu e helu: 2 + 4 =
He kiʻekiʻe loa anei ka helu o kou mau hunaola koko ʻulaʻula? E kamaʻilio kākou e pili ana i ka Polycythemia Vera (PV)!

He kiʻekiʻe loa anei ka helu o kou mau hunaola koko ʻulaʻula? E kamaʻilio kākou e pili ana i ka Polycythemia Vera (PV)!

Ke luhi pinepine nei ʻoe, i kekahi manawa he ʻano ʻūlū, a lohe wale paha i ke kani ʻana o kou mau pepeiao? ʻOiai ke ʻano maʻamau kēia mau mea, i kekahi manawa aia kekahi kumu ʻē aʻe ma hope o lākou. No laila, ʻo ka maʻi a mākou e kamaʻilio ai i kēia lā ʻo Polycythemia Vera (PV) , he ʻano ia i hoʻokumu ʻia e ka hana nui ʻana o nā hunaola koko ʻulaʻula i loko o ko mākou kino. ʻOiai he inoa lōʻihi kēia, e hoʻomaopopo maʻalahi kākou.

He aha ka Polycythemia Vera (PV)? I ka ʻōlelo maʻalahi...

I ka ʻōlelo maʻalahi, ʻo Polycythemia Vera (PV) kahi maʻi kahi e hana ai kou kino, ʻoiai kou iwi iwi (ka mea a mākou e kapa nei he "Bone Marrow"), i nā hunaola koko ʻulaʻula ma mua o ka mea e pono ai. E like me ke kaʻa ʻana, i ka wā e nui ai nā kaʻa ma ke alanui, ua ālai ʻia ke kahe ʻana o ke koko. Hoʻomānoanoa kēia i ke koko a hele mālie. Hoʻonui kēia i ka pilikia o nā ʻūpā koko. Hiki i kēia ke alakaʻi i nā maʻi koʻikoʻi e like me nā hōʻeha puʻuwai a me nā hahau .

ʻAʻole wale kēia, i kekahi manawa hiki ke loaʻa nā hōʻailona e like me ka ʻili maneʻo, ke kani ʻana o nā pepeiao (`(Tinnitus)`), ka ʻeha o ka ʻōpū, ke kahe ʻana o ke koko ihu, a me ka ʻike pōwehiwehi.

He maʻi mau loa ka Polycythemia Vera (PV) ʻaʻole hiki ke ho'ōla loa ʻia. Akā, mai hopohopo. Me ka mālama maikaʻi ʻana i ke olakino, hiki iā ʻoe ke kāohi i nā hōʻailona a hōʻemi i ka pilikia o nā hopena ʻaoʻao. Ua kapa pū kekahi poʻe iā ia he 'polycythemia mua', 'polycythemia rubra vera', 'erythema' a me 'Osler-Vaquez disease'.

He maʻi ʻaʻai anei ka Polycythemia Vera (PV)?

ʻAe, he ʻano maʻi kanesa kēia. Akā, ʻaʻole ia he ʻano koʻikoʻi a laha wikiwiki paha e like me kā mākou e manaʻo ai ke lohe mākou i ka huaʻōlelo kanesa. He maʻi kanesa koko kēia no kahi hui i kapa ʻia ʻo `myeloproliferative neoplasm (MPN) . ʻO `MPN` e pili ana i kahi hui o nā maʻi e hoʻoulu ai i ka hana nui ʻana o nā ʻano ʻano koko like ʻole.

ʻO ke kumu nui o ka Polycythemia Vera (PV) he loli (mutation) i loko o kahi gene i loko o ko kākou kino. ʻAʻole kēia he mea i hānau ʻia me kākou, akā he mea e kū mai ana i ko kākou ola no kekahi kumu i ʻike ʻole ʻia. Lohi loa ka ulu ʻana o kēia maʻi. Hoʻomaopopo pinepine ʻia ma hope o 60 mau makahiki. Kākaʻikahi kēia maʻi ʻaʻai e make pololei ai. Loaʻa ka pilikia nui mai nā pilikia i hoʻokumu ʻia e nā ʻūpā koko. Eia kekahi, ma kahi pakeneka liʻiliʻi loa, hiki iā ia ke lilo i nā ʻano maʻi koko koʻikoʻi ʻē aʻe.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia kūlana?

He maʻi laha ʻole ka Polycythemia Vera (PV). Ma kahi ʻāina e like me ʻAmelika Hui Pū ʻIa, loaʻa ia ma kahi o 50 no 100,000 poʻe. ʻOi aku ka maʻamau i ka poʻe ma luna o 60 mau makahiki.

Pehea ka hopena o kēia maʻi i ko mākou kino?

E like me kā mākou i ʻōlelo ai ma mua, ʻo ka Polycythemia Vera (PV) ke kumu o ke kino e hana i nā hunaola koko ʻulaʻula he nui loa. ʻO kēia keu o nā hunaola e hoʻonui ai i ka pilikia o ke kahe ʻana o ke koko, ka palapū, a me nā ʻūpā koko. Ma muli o ka mānoanoa o ke koko a me ka hele mālie ʻana, ʻaʻole i loaʻa i nā ʻiʻo a me nā ʻōkana o ko mākou kino ka oxygen e pono ai.

Hoʻokomo pū kēia i ka nui o ka hana ma ka spleen o ko mākou kino. ʻO kekahi o nā hana nui o ka spleen, ʻo ia ke kānana i ke koko a wehe i nā hunaola koko kahiko. Ke hoʻonui ʻia nā hunaola koko ʻulaʻula, hana nui ʻia ka spleen, hiki ke hoʻoulu i ka pehu a me ka ʻeha. Ua kapa ʻia kēia ʻo `(Splenomegaly)`. I ka hala ʻana o ka manawa, hiki i kēia ʻano ke hana i nā hōʻailona ʻoluʻolu ʻole. Eia kekahi, hiki i ka PV ke hana i nā ʻano lua ʻē aʻe.

He aha nā pilikia a me nā pilikia o ka Polycythemia Vera (PV)?

ʻOiai he maʻi holomua ka PV, aia kahi pilikia o ke koko koke ʻana ma muli o ka hana nui ʻana o nā hunaola koko. I ka hala ʻana o ka manawa, hiki i ka PV ke hana i nā kūlana lua ʻē aʻe. He mea kākaʻikahi loa, hiki ke lilo i maʻi ʻaʻai koʻikoʻi.

Nā ʻūpā koko

ʻO kēia ka pilikia nui a koke loa o ka PV. Inā hoʻokumu ʻia kahi ʻaʻa koko a hele i ka puʻuwai a i ʻole ka lolo, hiki iā ia ke hana i ka hōʻeha puʻuwai a i ʻole ka hahau . Inā hele ia i nā māmā (Pulmonary Embolism - PE), hiki iā ia ke hana i ke koko kiʻekiʻe i loko o nā māmā (pulmonary hypertension) a me ka hāʻule ʻana o ka puʻuwai. Inā paʻa kahi ʻaʻa koko i loko o kahi aʻa (Venous Thromboembolism - VTE), hiki iā ia ke make i nā ʻiʻo a i ʻole ka lawa ʻole o ka venous. Inā paʻa kahi ʻaʻa koko i loko o ke aʻa koko nui i ke akepaʻa (hepatic vein thrombosis), hiki iā ia ke hana i nā ʻaʻa koko i loko o ke akepaʻa (Budd-Chiari syndrome), jaundice a me ka hāʻule ʻana o ke akepaʻa.

Nā kūlana lua

Ke hana nui ʻia nā hunaola koko ʻulaʻula a wāwahi ʻia, piʻi ka nui o ka `uric acid` i loko o ke kino. Hiki i kēia ke hana i kekahi mau pilikia:

  • Nā pōhaku puʻupaʻa: Ke waiho ʻia ka waikawa uric i loko o nā puʻupaʻa.
  • Gout: He ʻeha ʻeha ka maʻi ʻaʻai e kū mai ana i ka wā e hōʻiliʻili ai ka uric acid i loko o nā hono.
  • ʻŌpū ʻōpū: Hoʻonui pū nā hunaola koko ʻulaʻula keu i ka nui o ka waikawa ʻōpū. Hoʻokuʻu nā hunaola koko ʻulaʻula keu i ke kino i kahi kemika i kapa ʻia ʻo histamine, kahi e hoʻoulu ai i ka ʻōpū e hana i ka waikawa hou aʻe e pale aku i ka maʻi. ʻO ka poʻe me PV he ʻehā manawa ka nui o ka loaʻa ʻana o nā ʻōpū ʻōpū (maʻi ʻōpū peptic).

Ke huli nei i ka Leukemia

He maʻi ʻaʻai o ka iwi iwi ka Polycythemia Vera, akā ʻaʻole ia he maʻi hoʻoweliweli i ke ola. Me ka mālama pono ʻana, hiki ke mālama maikaʻi ʻia no nā makahiki. Eia nō naʻe, no kekahi poʻe, hiki i ka PV, ʻoiai he kakaikahi,Hiki iā ia ke lilo i maʻi ʻaʻai koko koʻikoʻi e like me ka 'Acute Leukemia'.

Nā Pae o ka maʻi Polycythemia Vera (PV)

Hoʻomau ka maʻi PV penei:

  • Ke kahua mua ('PV Mua'): I kēia kahua, he kakaikahi a ʻaʻohe paha nā hōʻailona.
  • Ke holomua nei ka PV: Ke lilo nei ka PV i mea koʻikoʻi, e hoʻomaka paha ʻoe e ʻike i nā hōʻailona ʻoluʻolu ʻole, a i ʻole e ulu aʻe nā kūlana lua.
  • Ka pae i hoʻohana ʻia: Ua kapa ʻia kēia ʻo "ka pae i hoʻohana ʻia." I kēia manawa, hoʻonui nui ʻia nā hunaola koko ʻē aʻe e hoʻoulu ai i ka maʻi a ālai loa i ka iwi ʻōpala, ka mea e hana ai i nā hunaola koko. Ke make kēia mau hunaola ʻē, hoʻokumu ʻia nā ʻiʻo ʻeha ma ko lākou wahi. Ke piha ka hapa nui o ka iwi ʻōpala me kēia ʻiʻo ʻeha, ʻaʻole hiki iā ia ke hana i nā hunaola koko olakino. ʻO ka mea kupanaha, hoʻoulu kēia i ka anemia - ka nele o nā hunaola koko ʻulaʻula olakino. Hoʻonui pū ia i ka pilikia o ke kahe ʻana o ke koko.

ʻO nā maʻi koko ʻē aʻe e hiki ke hana ma hope o ka PV

Ke koʻikoʻi ka PV, hiki iā ia ke alakaʻi i nā maʻi koko ʻē aʻe.

  • ʻO Myelofibrosis (MF): ʻAʻohe ʻokoʻa nui ma waena o ka "split phase" o PV a me "Myelofibrosis (MF)" , ʻo ia kekahi ʻano maʻi koko ʻē aʻe. Manaʻo kekahi mau kauka he like lāua. ʻO "Myelofibrosis" ka wā e hana ai nā cell abnormal i nā ʻiʻo ʻeha i loko o ka iwi iwi a hoʻopiha iā ia. Hiki i kēia mau cell abnormal ke pālahalaha ma waho o ka iwi iwi a i loko o nā ʻāpana ʻē aʻe o ke kino. ʻO "MF" kekahi "maʻi myeloproliferative." Ma kahi o 10% o nā maʻi "MF" e loaʻa i ka "Acute Myeloid Leukemia."
  • ʻO ka Myelodysplastic Syndrome (MDS): He mea kākaʻikahi loa, hiki i ka PV ke ulu i kahi kūlana i kapa ʻia ʻo Myelodysplastic Syndrome (MDS) . He kūlana kēia kahi e oʻo pono ʻole ai nā hunaola koko, ulu ma ke ʻano ʻē, a make koke. ʻAʻole hiki i ke kino ke hana i nā hunaola olakino a oʻo, e hopena ana i ka emi ʻana o ka helu o nā ʻano hunaola koko like ʻole. ʻOi aku ka ʻino o ka MDS ma mua o PV a i ʻole MF, a he 30% ka pilikia o ka loaʻa ʻana o ka Acute Myeloid Leukemia (AML).
  • ʻO ka Acute Myeloid Leukemia (AML): Ma kahi o 3% o ka poʻe me Polycythemia Vera e loaʻa i ka Acute Myeloid Leukemia (AML) i loko o 10 mau makahiki o ka ʻike ʻia ʻana. He maʻi koko koʻikoʻi ka AML e hoʻomaka ana ma ka iwi iwi a hiki ke laha koke i ke koko a laila i nā ʻāpana ʻē aʻe o ke kino. Pono ia i ka mālama koke ʻana, akā kiʻekiʻe ka manawa e ola ai.

He aha nā hōʻailona o ka Polycythemia Vera (PV)?

ʻIke pinepine ʻia nā hōʻailona me ka lohi loa, a i kekahi manawa ʻaʻohe hōʻailona iki. ʻO ka mea mua, ʻo ia nā hōʻailona maopopo ʻole i maʻa mau i nā maʻi he nui:

  • ʻEha poʻo.
  • Pōʻaiʻī.
  • Luhi.
  • Ke koko kiʻekiʻe.
  • ʻIke pōwehiwehi a ʻike pālua paha.
  • Ke lohe ʻana i ke kani ʻana o nā pepeiao (`(Tinnitus)`).

I ka hala ʻana o ka manawa, hiki ke ʻike ʻia nā hōʻailona kikoʻī hou aʻe:

  • Ka hou ʻana, ʻoi aku hoʻi i ka pō.
  • Ka paʻakikī o ka hanu ʻana, ʻoiai i ka wā e moe ana.
  • ʻIli ʻāʻī, ʻoi aku hoʻi ma hope o ka ʻauʻau ʻana i ka wai wela. (He hōʻailona kikoʻī kēia o ka PV)
  • ʻUlaʻula, wela, mae, a i ʻole ke kuni ʻana o nā lālā.
  • Ke kahe koko nui a i ʻole ka palapū.
  • Ke kahe ʻana o ke koko mai ka ihu a me nā ʻāʻī.
  • Ka wiwi ʻana me ke kumu ʻole.

Inā lilo ka PV i mea koʻikoʻi a holomua i nā kūlana lua, hiki ke loaʻa nā hōʻailona pili. Eia kekahi laʻana:

Nā hōʻailona o ka Splenomegaly:

  • ʻO ka ʻeha e like me kekahi mea e paʻa ana ma ka ʻaoʻao hema o luna o ka ʻōpū.
  • He ʻike hoʻopiha ʻōpū.
  • ʻOiai ma hope o ka ʻai iki ʻana, ua piha wau.

Nā hōʻailona o nā ʻōpū ʻōpū ('Peptic ulcer disease'):

  • ʻEha e hele mai me ka ʻeha o ka ʻōpū.
  • ʻĀhaʻi puʻuwai.
  • ʻO ka reflux waikawa.

Nā hōʻailona o ka Gout:

  • ʻO ka pehu ʻeha o nā hono.
  • ʻOʻoleʻa o nā hono.
  • Ka pehu ʻana o ka manamana wāwae nui.

Nā hōʻailona o nā pōhaku puʻupaʻa:

  • ʻEha ma ka ʻōpū lalo a i ʻole ka ʻaoʻao.
  • ʻEha i ka wā e mimi ai.
  • Pono pinepine e mimi.

Nā hōʻailona o ka maʻi Budd-Chiari:

  • ʻAke pehu, ʻeha ma ka ʻaoʻao ʻākau o luna o ka ʻōpū, a me ka pehu ʻana.
  • Melemele o nā maka a me ka ʻili (jaundice).
  • ʻO nā Ascites a me ka Edema.

Nā hōʻailona o ka Deep Vein Thrombosis (DVT):

  • Ka pehu ʻana ma ka lima a i ʻole ka wāwae a me ka ʻeha ke hoʻopā ʻia.
  • Aia ka ʻulaʻula a i ʻole ka wela ma ia wahi.
  • Ke ʻano o nā kīʻaha koko e pehu ana ma luna o ka ʻili.

Nā hōʻailona o ke koko i loko o nā māmā (Pulmonary Embolism - PE):

  • ʻEha koke ka umauma.
  • Pōkole o ka hanu.
  • Piʻi ka helu o ka puʻuwai.

Nā hōʻailona o ka Anemia (i nā pae hope o ka maʻi PV):

  • ʻŌnionio (vertigo).
  • ʻIli keʻokeʻo.
  • Luhi.

He aha ke kumu o ka Polycythemia Vera (PV)?

Hoʻomaka ka Polycythemia Vera (PV) ma ka ʻāpana palupalu a huʻahuʻa o ko kākou mau iwi i kapa ʻia ʻo ka iwi iwi. Maanei kahi e hana ʻia ai nā hunaola koko hou. Hoʻomaka ka PV me kahi hoʻololi ʻana o ka gene hoʻokahi i loko o kahi hunaola kumu hoʻokahi i loko o ka iwi iwi. Ma mua o 90% o ka manawa, aia kēia hoʻololi ʻana i loko o kahi gene i kapa ʻia ʻo JAK2.

Ke haʻi aku nei kēia gene i hoʻololi ʻia i ke kelepona kumu e hoʻomau i ka hana hou ʻana. Ke hoʻomau nei kēlā me kēia kelepona i hana ʻia e hana hou iā ia iho. I ka hopena, hoʻopiha kēia mau kelepona ʻano ʻē i ka iwi iwi, ʻaʻohe wahi no nā kelepona maʻamau.

I ka hapanui o nā hihia, ʻaʻole kēia hoʻololi ʻana o ka gene `JAK2` he mea ia a mākou e hoʻoilina ai. He mea ia e hana ʻia i ka wā o ke ola, no nā kumu i ʻike ʻole ʻia. Eia nō naʻe, he mea kākaʻikahi loa, ua loaʻa nā hōʻike e pili ana i kekahi mau lālā o ka ʻohana e ulu ana i ka PV.

Pehea e ʻike ʻia ai ka Polycythemia Vera (PV)?

Ua hāpai ka World Health Organization (WHO) i ʻekolu mau kumu nui no ka ʻike ʻana i ka Polycythemia Vera (PV). Pono ke kauka e hōʻoia i nā kumu ʻekolu.

Paepae 1: He hoʻāʻo koko e hōʻike ana i ka helu koko ʻulaʻula kiʻekiʻe:

Hiki ke ana ʻia nā hunaola koko ʻulaʻula:

  • Nā pae hemoglobin kiʻekiʻe (kahi protein i loaʻa i loko o nā hunaola koko ʻulaʻula).
  • ʻO ke kiʻekiʻe o ka hematocrit (ka pakeneka o nā hunaola koko ʻulaʻula i loko o ke koko).
  • Ka nui o ke koko (ka nui o nā ʻulaʻula koko).

Pae 2: ʻO ka biopsy iwi e hōʻike ana i kekahi o kēia mau mea:

  • Nui loa nā hunaola koko i loko o ka iwi iwi, a i ʻole
  • ʻO ka nui o nā megakaryocytes makua (nā pūnaewele e hana i nā platelets koko).

Paepae 3: Hiki ke hoʻokō ʻia ke paepae ʻekolu ma ka hōʻike ʻana i kekahi o kēia mau mea ʻelua:

  • Ka hōʻoia ʻana o ke alo o ka hoʻololi ʻana o ka gene JAK2 ma o ka hoʻāʻo molekala, a i ʻole
  • Ua hōʻoia ka hoʻāʻo koko ua haʻahaʻa loa ka pae o ka `erythropoietin' (kahi hormone e hoʻoulu ai i ka hana ʻana o nā hunaola koko ʻulaʻula e nā puʻupaʻa) i loko o ke koko.

"E nānā ke kauka i kēia mau mea a pau e haʻi pololei iā ʻoe inā he Polycythemia Vera kou a i ʻole. No laila, inā loaʻa iā ʻoe kekahi mau hōʻailona, ​​​​ʻoi aku ka maikaʻi o ke ʻike ʻana i ke kauka a loaʻa ka ʻōlelo aʻoaʻo."

Pehea e mālama ʻia ai ka Polycythemia Vera (PV)?

I nā pae mua o ka PV, he maʻalahi ka mālama ʻana. ʻO nā lāʻau lapaʻau maʻamau:

Phlebotomy (ke kahe koko ʻana)

ʻO kēia ke ʻano lapaʻau nui a maʻamau no ka PV. Hana ʻia ia ma ke ʻano like me kāu e hāʻawi ai i ke koko. Hoʻokomo kahi mea mālama ola i kahi nila i loko o kahi aʻa koko ma kou lima a wehe i kahi koko liʻiliʻi (ma kahi o hoʻokahi paina, akā hiki ke ʻokoʻa ka nui ma muli o kou kūlana). Hoʻemi kēia i ka nui o kou koko a me ka helu o nā hunaola koko keu.

ʻAspirin haʻahaʻa

He lāʻau lapaʻau kēia hiki iā ʻoe ke kūʻai ma ka hale kūʻai lāʻau. Hoʻohana pinepine ʻia ia e hōʻemi i ka pilikia o nā ʻōpū koko. Ke lawe ʻia i kahi mahele haʻahaʻa i hoʻokahi manawa i ka lā, pale ka aspirin i nā platelets koko mai ka pipili ʻana. Kōkua nō hoʻi ia i ka hōʻemi ʻana i nā hōʻailona e like me ka pehu ʻana i nā lālā. Eia nō naʻe, he ʻino iki ia i ka ʻōpū a hoʻonui i ke kahe ʻana o ke koko. No laila, ʻaʻole kēia he manaʻo maikaʻi inā loaʻa iā ʻoe nā ʻōpū ʻōpū.

Inā koʻikoʻi kou mau hōʻailona, ​​a inā he moʻolelo kou o nā ʻōpū koko ma mua (thrombosis), hiki ke hoʻokomo ʻia ʻoe i loko o kahi hui pilikia kiʻekiʻe a hiki ke hāpai ʻia nā koho lapaʻau ʻē aʻe. ʻO kekahi o kēia mau mea:

Nā lāʻau lapaʻau e hōʻemi ai i ka ʻeha:

Ke koʻikoʻi ka PV, hiki i ke kauka ke kuhikuhi i nā lāʻau lapaʻau e like me kēia e mālama ai i ka ʻeha:

  • Nā Antihistamine: He mau lāʻau lapaʻau maʻamau kēia i hāʻawi ʻia no nā maʻi allergies.
  • Phototherapy: He lāʻau lapaʻau e hoʻohui i ka mālamalama ultraviolet (UVA) me kahi kemika i kapa ʻia ʻo psoralen (kahi hui organik e hoʻonui ai i ka ʻili i ka light therapy).
  • ʻO SSRIs (Selective Serotonin Reuptake Inhibitors): He ʻano lāʻau lapaʻau kēia i kuhikuhi pinepine ʻia no ke kaumaha. Eia nō naʻe, i ka wā e hāʻawi ʻia ai i nā mahele haʻahaʻa loa, ua ʻike ʻia hoʻi he kūpono lākou no nā hōʻailona kino mau e like me ka maneʻo.

Nā lāʻau lapaʻau e hoʻemi ana i ka helu o nā ʻāpana koko ʻulaʻula:

Hiki ke hoʻohana ʻia kēia mau mea hoʻokahi a i ʻole e hui pū kekahi me kekahi. Nā Laʻana:

  • ʻO ka Hydroxyurea
  • ʻO ka Interferon alpha
  • ʻO Ruxolitinib
  • ʻO Busulfan

Nā hoʻokolohua lapaʻau

Hāʻawi kēia i nā kānaka i ka manawa kūpono e hoʻāʻo i nā lāʻau lapaʻau hou loa a hāʻawi i ka noiʻi lapaʻau. ʻO kekahi o nā lāʻau lapaʻau i kēia manawa i ka pae noiʻi:

  • ʻO ka Peginterferon alfa-2a
  • ʻO Givinostat
  • ʻIdasanutlin

Hoʻoili iwi iwi

I kekahi mau hihia, hiki ke ʻōlelo ʻia kahi hoʻoili iwi iwi. E hoʻoholo ke kauka i kēia ma muli o kekahi mau kumu, e like me ke koʻikoʻi o kou maʻi a me ka hiki o kou kino ke ho'ōla.

Mālama kākoʻo

Inā e hoʻomau ka ʻino o kāu PV me ka nānā ʻole i ka lāʻau lapaʻau, e kālele ana kāu kauka i ka hoʻokele ʻana i kāu mau hōʻailona. I nā pae hope o ka PV, hiki iā ʻoe ke loaʻa i ka anemia a me ka spleen i hoʻonui ʻia. Hiki ke hāʻawi ʻia iā ʻoe nā lāʻau lapaʻau e like me:

  • Nā lāʻau hoʻopau ʻeha.
  • Nā hoʻoili koko.
  • Lapaʻau radiation haʻahaʻa i ka spleen.

He aha ke kūlana o ke ola o kekahi kanaka me ka Polycythemia Vera (PV)?

Hōʻike nā haʻawina hou nei ʻo ka awelika o ke ola ma hope o ka ʻike ʻia ʻana o Polycythemia Vera (PV) ma kahi o 20 mau makahiki. ʻO ka awelika o ka makahiki o ka make ma kahi o 77 mau makahiki. ʻO ke kumu nui o ka make, ʻo ia nā pilikia mai nā ʻōpū koko (ma kahi o 33%). ʻO ka lua o ke kumu nui, ʻo ia ka maʻi ʻaʻai (ma kahi o 15%).

"Mai makaʻu i kēia mau helu helu. He mau waiwai maʻamau kēia. Inā loaʻa iā ʻoe ka lāʻau kūpono a hoʻololi i kou nohona, hiki iā ʻoe ke noho olakino no ka manawa lōʻihi."

Pehea e hiki ai iaʻu ke mālama iaʻu iho a hoʻokele i kaʻu mau hōʻailona?

Inā ʻoe e noho nei me ka Polycythemia Vera (PV), he mea nui e noho mau i ka launa pū ʻana me kāu kauka. E kōkua kēia i kāu kauka e nānā i ke ʻano o ka holomua ʻana o kou kūlana. Hiki iā ʻoe ke haʻi i kāu kauka pehea ʻoe e pane nei i ka lāʻau lapaʻau a inā paha ʻoe e ʻike nei i kekahi mau hopena ʻaoʻao. Hiki iā ʻoe ke ola lōʻihi me ka ʻole o nā hōʻailona koʻikoʻi.

Ma waho aʻe o nā hoʻokolohua maʻamau a me ka mālama ʻana, e kuhikuhi pū kāu kauka i kekahi mau hoʻololi maʻalahi o ke ʻano o ka nohona e hiki ke kōkua i ka pale ʻana i nā ʻūpā koko a me nā pilikia ʻē aʻe. Eia kekahi laʻana:

  • Hoʻoikaika kino. Hiki i kahi hoʻoikaika kino liʻiliʻi ke kōkua i ka hoʻomaikaʻi ʻana i ke kahe koko.
  • E pale i ka puhipaka. Hoʻohaiki ka puhipaka i kou mau kīʻaha koko.
  • E pale i nā wahi me nā pae oxygen haʻahaʻa. Hiki i nā wahi kiʻekiʻe ke hoʻohaʻahaʻa i nā pae oxygen koko.
  • E kāohi i kou koko. ʻO ka mālama ʻana i ke kaumaha olakino e kōkua i kēia.

ʻOiai ʻo ka Polycythemia Vera (PV) he ʻano maʻi ʻaʻai, nui ka poʻe e ola maikaʻi ana no nā makahiki he ʻumi ma hope o ka ʻike ʻia ʻana o ka maʻi. Loaʻa kēia maʻi ʻaʻai ma muli o ka hana ʻia ʻana o nā pūnaewele ma waho o ke kaohi. Eia nō naʻe, lōʻihi ka manawa no ka hana ʻole ʻia ʻana o nā pūnaewele koko ʻulaʻula e hoʻomaka ai e hoʻopilikia iā ʻoe. Inā hana ʻoe, hiki iā ʻoe ke hoʻokele i ke kūlana no ka manawa lōʻihi ma ka wehe ʻana i kekahi o kou koko keu.

ʻO ka hoʻoweliweli nui loa mai ka Polycythemia Vera (PV) ʻaʻole ia ke kanesa ponoʻī, akā ʻo ka pilikia o nā ʻūpā koko. Inā ʻoe e noho nei me ka PV, e ʻike pono i kāu kauka i nā manawa a pau. A mai poina e mālama iā ʻoe iho me nā "mea liʻiliʻi" - ʻo ia hoʻi, nā ʻano nohona olakino. Kōkua kēia i ka mālama ʻana i ke olakino holoʻokoʻa o kāu ʻōnaehana cardiovascular, kahi mea nui paha ma mua o kekahi lāʻau lapaʻau no Polycythemia Vera.

He aha ka leka a mākou e makemake ai e lawe i ka home mai kēia moʻolelo?

ʻAe, ua kamaʻilio nui mākou e pili ana i ka Polycythemia Vera (PV). Eia kekahi o nā mea nui e hoʻomanaʻo:

  • He maʻi koko ka PV kahi e hana ai ke kino i nā hunaola koko ʻulaʻula he nui loa a ulu aʻe ma ke ʻano ʻē. No loko ia o kahi hui o nā maʻi ʻaʻai i kapa ʻia ʻo `myeloproliferative neoplasm (MPN)`.
  • ʻO ka pilikia nui mai ka mānoanoa o ke koko a me ke kāpili ʻana o ke koko, hiki ke alakaʻi i nā maʻi koʻikoʻi e like me ka hōʻeha puʻuwai a me nā hahau.
  • Hiki i nā hōʻailona ke komo pū me ka ʻili maneʻo (ʻoi aku hoʻi ma hope o ka ʻauʻau ʻana), ka manaʻo palaualelo, ke poʻo ʻeha, a me ka poniuniu.
  • ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia, hiki ke mālama maikaʻi ʻia me ka mālama kūpono, nā loli o ke ʻano o ka nohona, a me ka lāʻau lapaʻau. ʻO ka phlebotomy (bloodletting) a me ka aspirin haʻahaʻa ka lāʻau lapaʻau mua.
  • ʻO ka hoʻololi pinepine ʻana o ka gene `JAK2` ke kumu o kēia.
  • Inā loaʻa iā ʻoe kekahi o kēia mau hōʻailona, ​​e ʻike pono i ke kauka no ka ʻōlelo aʻo. Hoʻonui ka ʻike mua ʻana i kou manawa kūpono e pale aku ai i nā pilikia a me ke ola ʻana i kahi ola olakino.

Mai makaʻu, akā, mai hoʻomāmā hoʻi. E mālama i kou olakino. E kamaʻilio pinepine me kāu kauka, e loaʻa i nā hoʻokolohua a me nā lāʻau lapaʻau e pono ai i ka manawa kūpono. A laila hiki iā ʻoe ke noho olakino me kēia maʻi!


ʻO Polycythemia Vera, Nā Kelepona Koko ʻUlaʻula, Nā ʻAʻa Koko, MPN, JAK2, Phlebotomy, Kanesa

⚠️ Important: The medical articles and information on Nirogi Lanka are for general awareness only, and are by no means a substitute for professional medical advice, diagnosis, or treatment. For any medical problem you have, consult a qualified physician immediately.

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