He tino mōrearea te nui o ngā toto whero i roto i tō tinana? Me kōrero tātou mō te Polycythemia Vera (PV)!

He maha ngā wā ka ngenge koe, ka pāngia koe e te ngau kino i ētahi wā, ka rangona rānei he tangi i roto i ō taringa? Ahakoa he āhua noa iho ēnei, i ētahi wā he take anō kei muri i ēnei. Nō reira, ko te mate ka kōrerohia e tātou i tēnei rā ko te Polycythemia Vera (PV) , he mate e puta mai ana i te nui rawa o ngā toto toto whero i roto i tō tātou tinana. Ahakoa he ingoa roa tēnei, me mārama noa iho.

He aha te Polycythemia Vera (PV)? He māmā noa iho...

Ki te whakarapopoto, ko te Polycythemia Vera (PV) he mate e nui ake ai ngā toto whero e whakaputaina ana e tō tinana, inā koa tō hinu wheua (e kiia nei e tātou ko te "Hine wheua"), i te mea e tika ana. Pērā i ngā waka e noho ana i te huarahi, ina nui rawa ngā waka i runga i te rori, ka āraihia te rere o te toto. Ka matotoru te toto, ka pōturi ake te haere. Ka piki ake te tūponotanga o te toto e pupuhi ana. Ka taea e tēnei te arahi ki ngā mate kino pēnei i te mate manawa me te whiu .

Ehara i te mea ko tēnei anake, i ētahi wā ka puta anō hoki ngā tohu pēnei i te kiri mākūkū, te tangi o ngā taringa (`(Tinnitus)`), te mamae o te puku, te toto o te ihu, me te tirohanga pōuri.

He mate mau tonu te Polycythemia Vera (PV) e kore e taea te whakaora katoa. Kaua e manukanuka. Mā te maimoatanga hauora pai, ka taea e koe te whakahaere i ngā tohu me te whakaiti i te tūponotanga o ngā pānga taha. Ka kiia hoki e ētahi ko te 'primary polycythemia', 'polycythemia rubra vera', 'erythema' me te 'Osler-Vaquez disease'.

He mate pukupuku te Polycythemia Vera (PV)?

Ae, he momo mate pukupuku tēnei. Engari kāore i te tino kino, i te tere rānei te horapa pērā i tā tātou e whakaaro ana ina rongo tātou i te kupu mate pukupuku. He mate pukupuku toto tēnei nō tētahi rōpū e kiia nei ko te `myeloproliferative neoplasm (MPN) . Ko te `MPN` e pā ana ki tētahi rōpū mate e hua ai te whakaputa nui o ngā momo pūtau toto.

Ko te tino take o te Polycythemia Vera (PV) he panoni (whakarerekētanga) i roto i tētahi ira i roto i tō tātou tinana. Ehara tēnei i te mea i whānau mai tātou me ia, engari he mea e puta ana i roto i ō tātou oranga mō tētahi take kāore i te mōhiotia. He puhoi rawa te whanaketanga o tēnei mate. He maha ngā wā ka kitea i muri i te 60 tau te pakeke. He onge te mate o tēnei mate pukupuku. Ko te mōrearea matua ka puta mai i ngā raruraru i puta mai i ngā toto totoka. Waihoki, i roto i te ōrau iti rawa, ka taea te huri hei momo mate pukupuku toto kino kē atu.

Ko wai te mea tino pāngia e tēnei āhuatanga?

He mate tino onge te Polycythemia Vera (PV). I roto i tētahi whenua pērā i te Hononga o Amerika, e 50 pea i roto i te 100,000 tāngata ka pāngia. Ko te nuinga o te wā ka pāngia te hunga neke atu i te 60 tau te pakeke.

He aha te pānga o tēnei mate ki ō tātou tinana?

E ai ki tā mātou i kōrero ai i mua ake nei, ka nui rawa ngā toto whero e whakaputaina ana e te tinana nā te Polycythemia Vera (PV). Mā tēnei nui rawa o ngā pūtau ka nui ake te mōrearea o te toto, te maru, me te toto e pupuhi. Nā te mea ka matotoru te toto, ka puhoi ake te haere, kāore e whiwhi ngā kiko me ngā whekau o te tinana i te hāora e hiahiatia ana e rātou.

He nui hoki te mahi a tēnei ki te whēkau o tō tātou tinana. Ko tētahi o ngā mahi matua a te whēkau ko te tātari i te toto me te tango i ngā toto tawhito. Ina nui haere ngā toto whero, ka nui rawa te mahi a te whēkau, ka pupuhi, ka mamae hoki. Ka kiia tēnei ko te `(Splenomegaly)`. I roto i te wā, ka puta mai i tēnei āhuatanga ngā tohu whakararuraru. Waihoki, ka puta mai i te PV ētahi atu āhuatanga tuarua.

He aha ngā mōrearea me ngā raruraru o te Polycythemia Vera (PV)?

Ahakoa he mate haere tonu te PV, tera pea ka puta he toto totoka ohorere nā te nui rawa o ngā pūtau toto. I roto i te wā, ka taea hoki e te PV te whakaputa i ētahi atu mate tuarua. He tino onge, ka taea te whanake hei mate pukupuku kino.

Ngā toto totoka

Koinei te mōrearea matua, te mōrearea tino wawe hoki o te PV. Mēnā ka puta he toto totoka, ka haere ki te ngākau, ki te roro rānei, ka pā mai he mate manawa, he whiu rānei . Mēnā ka haere ki ngā pukahukahu (Pulmonary Embolism - PE), ka pā mai he toto toto tiketike i roto i ngā pukahukahu (pulmonary hypertension) me te ngoikore o te ngākau. Mēnā ka mau te toto totoka ki roto i te uaua (Venous Thromboembolism - VTE), ka mate te kiko, ka ngoikore rānei te uaua mau tonu. Mēnā ka mau te toto totoka ki roto i te uaua toto matua ki te ate (hepatic vein thrombosis), ka pā mai he toto totoka ki te ate (Budd-Chiari syndrome), he jaundice me te ngoikore o te ate.

Ngā āhuatanga tuarua

Ina nui rawa te hanga o ngā pūtau toto whero, ā, ina pakaruhia, ka piki ake te taumata o te 'waikawa uric' i roto i te tinana. Ka pā mai pea ētahi raruraru:

• Ngā kōhatu whatukuhu: Ina whakatakotoria te waikawa uric ki roto i ngā whatukuhu.
• Kauti: He mate rumatiki mamae e puta ana ina nui haere te waikawa uric i roto i ngā hononga.
• Ngā mate puku: Ka whakanuia hoki e ngā toto whero tāpiri te nui o te waikawa puku. Ka tukuna e ngā toto whero tāpiri te matū e kiia nei ko te histamine e te tinana, ā, ka nui ake te waikawa o te puku hei whawhai i te mate. E whā ngā wā ka nui ake te tūponotanga o te hunga whai PV ki te pāngia e ngā mate puku (mate puku peptic).

Te tahuritanga ki te mate pukupuku toto

He mate pukupuku o te hinu wheua te Polycythemia Vera, engari ehara i te mea he mate morearea ki te ora. Mēnā he maimoatanga tika, ka taea te whakahaere pai mō ngā tau. Heoi, mō ētahi tāngata, ahakoa he onge, ka taea e te PV teKa taea e ia te huri hei mate pukupuku toto kino pērā i te 'Acute Leukemia'.

Ngā Wāhanga o te Mate Polycythemia Vera (PV)

Ka ahu whakamua te mate PV penei:

• Wāhanga tōmua ('PV tōmua'): I tēnei wāhanga, he iti noa iho, he kore rānei he tohu.
• Te Haerenga o te PV: I te kaha haere o te PV, ka tīmata pea koe ki te pāngia e ngā tohumate kāore i te pai, ka puta rānei ētahi atu mate.
• Wāhanga Pau: Ka kiia tēnei ko te "wāhanga pau." I tēnei wā, ka nui haere ngā pūtau toto rerekē e puta ai te mate, ā, ka ārai katoa i te hinu wheua, e hanga ana i ngā pūtau toto. Ina mate ēnei pūtau rerekē, ka puta he kiko whara i tō rātou wāhi. Ina kī tonu te nuinga o te hinu wheua i tēnei kiko whara, kāore e taea te hanga i ngā pūtau toto hauora. He mea whakamīharo, ka puta te anemia – he korenga o ngā pūtau toto whero hauora. Ka piki ake hoki te mōrearea o te toto.

Ētahi atu mate toto ka puta i muri i te PV

Ina kaha rawa te PV, ka arahi pea ki ētahi atu mate toto.

• Myelofibrosis (MF): Kāore he rerekētanga nui i waenga i te "wāhanga pau" o te PV me te "Myelofibrosis (MF)" , tētahi atu momo matepukupuku toto. E whakaaro ana ētahi tākuta he rite tonu. Ko te "Myelofibrosis" he wā e hangaia ai e ngā pūtau rerekē he kiko whara i roto i te hinu wheua ka whakakīia. Ka horapa ēnei pūtau rerekē ki waho o te hinu wheua ka uru atu ki ētahi atu wāhanga o te tinana. Ko te "MF" he "mate myeloproliferative". Tata ki te 10% o ngā tūroro "MF" ka pāngia e te "Acute Myeloid Leukemia."
• Te Mate Myelodysplastic (MDS): He tino onge te whanaketanga o te PV hei mate e kiia nei ko te Mate Myelodysplastic (MDS) . He mate tēnei kāore e pakari tika ngā pūtau toto, e tipu rerekē ana, ā, ka tere te mate. Kāore e taea e te tinana te hanga pūtau hauora, pakari, ka heke te maha o ngā momo pūtau toto rerekē. He kino ake te MDS i te PV, i te MF rānei, ā, e 30% te mōrearea ki te whakawhanake i te Acute Myeloid Leukemia (AML).
• Te Mate Pūkahukahu Myeloid Akut (AML): Tata ki te 3% o te hunga whai Polycythemia Vera ka pāngia e te Mate Pūkahukahu Myeloid Akut (AML) i roto i ngā tau 10 o te tātaritanga. He mate pukupuku toto kino te AML e tīmata ana i roto i te hinu wheua, ā, ka tere te horapa ki te toto, kātahi ki ētahi atu wāhanga o te tinana. Me tere te maimoatanga, engari he nui te tūponotanga ka ora.

He aha ngā tohu o te Polycythemia Vera (PV)?

He puhoi rawa te puta mai o ngā tohu, ā, i ētahi wā, kāore pea he tohu. Ko te tuatahi ko ngā tohu koretake e kitea ana i roto i te maha o ngā mate:

• Te mamae o te upoko.
• Pōuriuri.
• Te ngenge.
• Te toto toto tiketike.
• Te tirohanga pōuri, te tirohanga takirua rānei.
• Te rongonga i te tangi o ngā taringa (`(Tinnitus)`).

I roto i te wā, ka puta ake pea ngā tohu motuhake ake:

• Te werawera, ina koa i te pō.
• Te uaua ki te manawa, ina koa i te takoto.
• Kiri mākūkū, inā koa i muri i te kaukau i te wai wera. (He tohu motuhake tēnei o te PV)
• Te whero, te wera, te ngoikore, te wera rānei o ngā peka.
• Te toto nui rawa, te maru rānei.
• Te toto e puta mai ana i te ihu me ngā kapia.
• Te hiroki noa iho, kāore he take.

Mena ka kino haere te PV, ā, ka huri hei āhuatanga tuarua, ka puta pea ngā tohu e pā ana. Hei tauira:

Ngā tohu o te Splenomegaly:

• He mamae e mau ana me te mea kei te taha maui o runga o te puku.
• He wheako whakakī puku.
• Ahakoa i muri i te kai iti, kua kī tonu ahau.

Ngā tohu o te mate puku ('Mate puku peptic'):

• Te mamae e haere tahi ana me te mamae o te puku.
• Te mamae o te ngākau.
• Te rerenga waikawa.

Ngā tohu o te Kauti:

• Te pupuhi mamae o ngā hononga.
• Te pakari o ngā hononga.
• Te pupuhi o te māhunga nui.

Ngā tohu o ngā kohatu whatukuhu:

• Te mamae i te puku o raro, i te taha rānei.
• Te mamae i te wā e mimi ana.
• Te hiahia tonu ki te mimi.

Ngā tohu o te mate Budd-Chiari:

• Te ate pupuhi, te mamae i te taha matau o runga o te puku, me te pupuhi.
• Te kōwhai o ngā kanohi me te kiri (te jaundice).
• Ngā Ascites me te Edema.

Ngā tohu o te Thrombosis Utauta Hōhonu (DVT):

• Te pupuhi o te ringa, o te waewae rānei, me te mamae ina pāngia.
• He whero, he wera rānei kei taua wāhi.
• Te putanga mai o ngā oko toto e pupuhi ana i runga i te mata o te kiri.

Ngā tohu o te toto totoka i roto i ngā pukahukahu (Pulmonary Embolism - PE):

• Te mamae ohorere o te uma.
• Te poto o te manawa.
• Ka piki ake te tere o te manawa.

Ngā tohu o te mate toto iti (i ngā wāhanga whakamutunga o te mate PV):

• Pōuriuri (vertigo).
• Kiri mā.
• Te ngenge.

He aha te take o te Polycythemia Vera (PV)?

Ka tīmata te Polycythemia Vera (PV) i te wāhanga ngohengohe, hautai o ō tātou wheua e kiia nei ko te hinu wheua. Koinei te wāhi e hangaia ai ngā pūtau toto hou. Ka tīmata te PV me te whakarerekētanga ira kotahi i roto i te pūtau take kotahi i roto i te hinu wheua. Neke atu i te 90% o te wā, kei roto i te ira e kiia nei ko JAK2 tēnei whakarerekētanga.

Ka kī atu tēnei ira kua whakarerekētia ki te pūtau take kia whakaputa uri tonu. Ka whakaputa uri tonu ia pūtau e puta mai ana. Ā tōna wā, ka whakakīia te hinu wheua e ēnei pūtau rerekē, ā, kāore he wāhi mō ngā pūtau noa.

I te nuinga o te wā, ehara tēnei whakarerekētanga ira `JAK2` i te mea tuku iho i a tātou. He mea e puta ana i te wā e ora ana, mō ngā take kāore i te mōhiotia. Heoi, he tino onge, kua puta he pūrongo mō ētahi mema o te whānau e pāngia ana e te PV.

Me pēhea te tautuhi i te Polycythemia Vera (PV)?

E toru ngā paearu matua kua whakaritea e te Whakahaere Hauora o te Ao (WHO) mō te tautuhi i te Polycythemia Vera (PV). Me whakaū e te tākuta ngā paearu e toru.

Paearu 1: He whakamātautau toto e whakaatu ana i te nui o ngā toto toto whero:

Ka taea te ine i ngā pūtau toto whero:

• Te nui o te hemoglobin (he pūmua kei roto i ngā toto whero).
• He taumata hematocrit teitei (te ōrau o ngā toto whero i roto i te toto).
• Te nui o te toto (te nui o ngā pūtau toto whero).

Paearu 2: Ko te koiora o te hinu wheua e whakaatu ana i tētahi o ēnei e whai ake nei:

• He nui rawa ngā pūtau toto i roto i te hinu wheua,
• Te nui rawa atu o ngā megakaryocytes kua pakeke (ngā pūtau e hanga ana i ngā pereti toto).

Paearu 3: Ka taea te whakatutuki i te paearu tuatoru mā te whakaatu i tētahi o ēnei e rua:

• Te whakaū i te aroaro o te whakarerekētanga ira JAK2 mā te whakamātautau irahiko, mā te whakamātautau rānei
• Mā te whakamātautau toto ka whakaū he tino iti te taumata o te `erythropoietin' (he homoni e whakaoho ana i te hanga o ngā toto whero e ngā whatukuhu) i roto i te toto.

"Ka tirohia ēnei katoa e te tākuta kia mōhio ai koe mēnā kei a koe te Polycythemia Vera. Nō reira, ki te pā mai ētahi tohu, he pai ake te toro atu ki te tākuta me te rapu tohutohu."

Me pēhea te rongoā i te Polycythemia Vera (PV)?

I ngā wāhanga tōmua o te PV, he tino māmā noa te maimoatanga. Ko ngā maimoatanga noa ko:

Te tango toto (phlebotomy)

Koinei te maimoatanga matua, te maimoatanga tino noa hoki mō te PV. Ka rite te mahi ki tāu e tuku toto ai. Ka werohia e te kaiwhakarato hauora he ngira ki roto i te uaua i roto i tō ringa, ā, ka tangohia he iti noa te toto (tata ki te paina, engari ka rerekē te nui i runga i tō āhua). Ka whakaitihia e tēnei te nui o tō toto katoa me te maha o ngā pūtau toto tāpiri.

Āpirini horopeta iti

He rongoā tēnei ka taea te hoko mai i te whare rongoā. He mea whakarite kia whakaitihia te tūponotanga o te toto totoka. Ina tangohia he horopeta iti kotahi i te rā, ka ārai te aspirin i te piri o ngā pereti toto. Ka āwhina hoki ki te whakaiti i ngā tohu pēnei i te pupuhi o ngā peka. Heoi, he paku kino ki te puku, ā, ka nui ake te toto. Nō reira, ehara tēnei i te whakaaro pai mēnā he mate puku tōu.

Mena he kino rawa ō tohu, he hītori toto toto kua pāngia koe e te toto totoka (thrombosis), tera pea ka whakanohoia koe ki tētahi rōpū mōrearea nui, ā, tera pea ka tūtohutia ētahi atu kōwhiringa maimoatanga. Ko ētahi o ēnei ko:

Ngā maimoatanga hei whakaiti i te mākūkū:

Ina kaha rawa te PV, ka tohua pea e te tākuta ēnei rongoā hei rongoā i te mākūkū:

• Ngā rongoā ātete pāwera: He rongoā noa ēnei e hoatu ana mō ngā mate pāwera.
• Whakaora-ata: He maimoatanga e whakakotahi ana i te mārama ultraviolet (UVA) me te matū e kiia nei ko te psoralen (he matū waro e whakaoho ana i te kiri ki te whakaora mārama).
• Ngā SSRI (Ngā Aukati Whakahoki Anō o te Serotonin): He momo rongoā ēnei e tohua ana mō te pouri. Heoi, ina hoatu i roto i ngā horopeta tino iti, kua kitea hoki he whai hua mō ngā tohu tinana pumau pēnei i te mākūkū.

Ngā rongoā hei whakaiti i te maha o ngā toto toto whero:

Ka taea te whakamahi takitahi i ēnei, te whakamahi tahi rānei. Hei tauira:

• `Hairokiurea`
• Interferon alfa
• `Ruxolitinib`
• `Busulfan`

Ngā whakamātautau haumanu

Mā ēnei ka whai wāhi te tangata ki te whakamātau i ngā maimoatanga hou me te whai wāhi atu ki ngā rangahau hauora. Ko ētahi o ngā rongoā kei roto i te wāhanga rangahau i tēnei wā ko:

• `Peginterferon alfa-2a``
• `Givinostat`
• `Idasanutlin`

Te whakawhitinga wheua

I ētahi wā, ka tūtohutia pea he whakawhitinga wheua. Ka whakatauhia tēnei e te tākuta i runga i ētahi āhuatanga, pērā i te kaha o tō mate me te kaha o tō tinana ki te whakaora.

Manaaki tautoko

Ki te haere tonu te kino o tō PV ahakoa te maimoatanga, ka arotahi tō tākuta ki te whakahaere i ō tohu. I ngā wāhanga whakamutunga o te PV, ka pāngia pea koe e te anemia me te whēkau nui. Tērā pea ka tukuna atu ngā maimoatanga pēnei i:

• Ngā rongoā whakamamae.
• Ngā whakawhiti toto.
• Te rongoā irahiko iti-hopu ki te ate.

He aha te roanga o te oranga o te tangata e pāngia ana e te Polycythemia Vera (PV)?

E ai ki ngā rangahau hou , ko te roanga o te oranga i muri i te tātaritanga o te Polycythemia Vera (PV) he tata ki te 20 tau. Ko te tau toharite o te mate he tata ki te 77 tau. Ko te tino take o te mate ko ngā raruraru mai i ngā toto totoka (tata ki te 33%). Ko te tuarua o ngā tino take ko te mate pukupuku (tata ki te 15%).

"Kaua e mataku i ēnei tatauranga. He uara noa ēnei. Ki te whiwhi koe i te maimoatanga tika, ā, ki te huri i tō momo noho, ka taea e koe te noho hauora mō te wā roa."

Me pēhea taku tiaki i ahau anō me te whakahaere i aku tohumate?

Mena kei te noho tahi koe me te Polycythemia Vera (PV), he mea nui kia noho tonu koe ki te whakapā atu ki tō tākuta. Mā tēnei ka āwhina i tō tākuta ki te aroturuki i te ahunga whakamua o tō mate. Ka taea hoki e koe te kōrero ki tō tākuta mō tō urupare ki te maimoatanga me te mea kei te pāngia koe e ētahi pānga taha. Ka taea e koe te ora mō te wā roa me te kore he tohu kino.

Haunga ngā whakamātautau me te maimoatanga auau, ka tūtohu anō tō tākuta i ētahi whakarerekētanga ngāwari o te noho hei āwhina i te ārai i te toto totoka me ētahi atu raruraru. Hei tauira:

• Whakakorikori tinana. Ahakoa he iti noa te whakakorikori tinana ka pai ake te rere o te toto.
• Kaua e kai hikareti. Ka whakawhāitihia ō uaua toto e te kai hikareti.
• Āta karohia ngā taiao he iti te taumata o te hāora. Ka iti pea te taumata o te hāora i roto i te toto i ngā teitei.
• Whakahaerehia tō toto toto. Mā te pupuri i tō taumaha hauora ka āwhina i tēnei.

Ahakoa he momo mate pukupuku te Polycythemia Vera (PV), he maha ngā tāngata e ora pai ana mō ngā tekau tau i muri i te tātaritanga kua pāngia rātou e te mate. Ka puta tēnei mate pukupuku nā te mea ka puta ngā pūtau i waho o te mana whakahaere. Heoi, ka roa te wā ka tīmata te pānga o te whakaputanga kore mana whakahaere o ngā pūtau toto whero ki a koe. Ahakoa ka pērā koe, ka taea e koe te whakahaere i te mate mō te wā roa mā te tango i ētahi o ō toto taikaha.

Ko te tūmomo mōrearea nui rawa atu o te Polycythemia Vera (PV) ehara i te mate pukupuku tonu, engari ko te mōrearea o te toto totoka. Mena kei te noho tahi koe me te PV, kia kite tonu koe i tō tākuta. Kaua hoki e wareware ki te tiaki i a koe anō me ngā "mea iti" - arā, ngā tikanga noho hauora. Ka āwhina ēnei ki te pupuri i te hauora whānui o tō pūnaha manawa, he mea nui ake pea i tētahi maimoatanga hauora mō te Polycythemia Vera.

He aha te karere e hiahia ana tātou ki te kawe ki te kāinga mai i tēnei pūrākau?

Āe, kua maha ā tātou kōrero mō te Polycythemia Vera (PV). Anei ētahi o ngā mea nui hei maumahara:

• He mate toto te PV e nui rawa ai ngā toto whero e whakaputaina ana e te tinana, ā, ka tipu rerekē. Nō tētahi rōpū mate pukupuku e kiia nei ko te `myeloproliferative neoplasm (MPN)`.
• Ko te mōrearea matua e ahu mai ana i te matotoru o te toto me te totoka, ka arahi pea ki ngā mate kino pēnei i te whakaeke ngakau me te whiu.
• Ko ētahi o ngā tohu ko te mākūkū o te kiri (ina koa i muri i te kaukau), te ngoikore, te ānini, me te whanoke.
• Ahakoa kāore e taea te whakaora katoa, ka taea te whakahaere pai mā te maimoatanga tika, ngā huringa momo noho, me te rongoā. Ko te tango toto (phlebotomy) me te aspirin iti te horopeta ngā maimoatanga matua.
• Ko te whakarerekētanga ira 'JAK2' te tino take o tēnei.
• Ki te pā mai ētahi o ēnei tohu ki a koe, me toro atu ki te tākuta mō ētahi tohutohu. Mā te kitenga wawe ka nui ake te tūponotanga ka karohia ngā raruraru me te noho ora.

Kaua e wehi, engari kaua hoki e whakahawea. Kia tiakina tō hauora. Kōrero ki tō tākuta i ngā wā katoa, me whiwhi i ngā whakamātautau me ngā maimoatanga e tika ana i te wā tika. Kātahi ka taea e koe te noho hauora ahakoa tēnei mate!

` Polycythemia Vera, Ngā Pūtau Toto Whero, Ngā Pukupuku Toto, MPN, JAK2, Phlebotomy, Matepukupuku