I red blood cell count hi hlauhawm takin a sang em? Polycythemia Vera (PV) chungchang hi han sawi ila!

I chau fo em, a chang chuan tingling sensation i nei fo em, a nih loh leh i beng ri hriat mai mai i nei em? Chûngte chu thil pangngai angin lang mah se, a châng chuan chhan dang a awm thei a ni. Chuvangin, vawiina kan sawi tur natna hi Polycythemia Vera (PV) , kan taksaa thisen sen tam lutuk avanga lo awm a ni. Hei hi hming sei deuh mahse, awlsam takin i hrethiam ang u.

Polycythemia Vera (PV) hi eng nge ni? A awlsam zawngin...

A awlsam zawngin, Polycythemia Vera (PV) hi i taksa, a bik takin i ruhro (``Bone Marrow'' kan tih) hian thisen sen (red blood cells) a siam chhuah tur aia tam a siam chhuah theihna a ni. Traffic jam ang bawkin kawngpuiah motor a tam lutuk chuan thisen luang chu a tikhawlo thin. Hei hian thisen a ti thim a, a kal zawi zawk thin. Hei hian thisen zam a tipung a ni. Hei hian heart attack leh stroke ang chi natna khirh tak tak a thlen thei a ni .

Hei mai ni lovin, a chang chuan vun natna, beng ri (`(Tinnitus)`), pum na, hnar thisen, leh mit hmuh fiah lo ang chi symptoms te pawh a awm thei bawk.

Polycythemia Vera (PV) hi natna khirh tak a ni a, a dam kim thei lo. Mahse, lungngai suh. Damdawi lam enkawlna tha tak i neih chuan a natna lan chhuah dan i thunun thei a, a side effects pawh i ti tlem thei bawk. Mi thenkhat chuan `primary polycythemia`, `polycythemia rubra vera`, `erythema` leh `Osler-Vaquez natna` an ti bawk.

Polycythemia Vera (PV) hi cancer a ni em?

Ni e, hei hi cancer chi khat a ni tak zet a ni. Mahse cancer tih thumal kan hriat hian kan ngaihtuah angin a pawi lo va, a darh chak lo bawk. Hei hi thisen cancer a ni a, `myeloproliferative neoplasm (MPN) tih pawla tel a ni. `MPN` tih hian thisen cell chi hrang hrang siam chhuah tam lutuk thlentu natna pawl a kawk a ni.

Polycythemia Vera (PV) thlentu ber chu kan taksaa gene pakhat a inthlak (mutation) vang a ni. Hei hi kan pianpui thil a ni lo va, a chhan hriat loh avanga kan dam chhunga thil thleng a ni. He natna hi a lo vei zauh zauh a. Kum 60 hnuah hmuhchhuah a ni fo a, he cancer hian direct death a thlen tam lo hle. A hlauhawm ber chu thisen zam avanga harsatna awm atanga lo chhuak a ni. Tin, za zela tlemte chauhvin thisen cancer chi dang na tak takah a chang thei bawk.

He dinhmun hian tute nge a nghawng ber?

Polycythemia Vera (PV) hi natna hmuh tur awm lo tak a ni. United States ang ram ah chuan mi 100,000 zinga 50 vel ah a awm thin. Kum 60 chunglam ah a tam ber.

He natna hian engtin nge kan taksa a nghawng?

Kan sawi tawh ang khan Polycythemia Vera (PV) hian taksaah thisen sen (red blood cells) a siam tam lutuk a. Hetianga cell tam lutuk hian thisen chhuah, hliam leh thisen zam a tipung a ni. Thisen hi a thip a, a kal zawi zawk avangin kan taksa peng leh taksa peng hrang hrangte hian oxygen an mamawh ang an hmu lo.

Hei hian kan taksa pumpuiah hna tam tak a thawh bawk. Spleen hnathawh ber pakhat chu thisen filter leh thisen cell hlui paih chhuah hi a ni. Thisen sen (red blood cells) a pun chuan ril (spleen) pawh a thawk nasa lutuk a, chu chuan a ti na thei a, a na thei bawk. Hei hi `(Splenomegaly)` an ti a. Hun a kal zel a, he natna hian natna lan chhuah dan nuam lo chi hrang hrang a thlen thei a ni. Tin, PV hian secondary condition dang a thlen thei bawk.

Polycythemia Vera (PV) hian eng hlauhawmna leh harsatna nge a neih?

PV hi natna zual zel ni mah se, thisen cell a tam lutuk avangin thisen zam nghal mai theihna a awm. Hun kal zelah PV hian secondary condition dang a thlen thei bawk. A tlem hle a, cancer na takah a chang thei.

Thisen a chhuak thin

Hei hi PV vei theihna ber leh hlauhawm ber a ni. Thisen zam a lo awm a, thinlung emaw thluak emaw a kal chuan heart attack emaw stroke emaw a thlen thei a ni . Lung (Pulmonary Embolism - PE)-ah a kal a nih chuan lungah thisen sang (pulmonary hypertension) a thlen thei a, heart failure a thlen thei bawk. Thisen zam chu vein (Venous Thromboembolism - VTE)-ah a tlakbuak chuan tissue thihna emaw, chronic venous insufficiency emaw a thlen thei a ni. Thisen zam chu liver panna thisen kalna bulpuiah a tlakbuak (hepatic vein thrombosis) chuan liver-ah thisen zam (Budd-Chiari syndrome), jaundice leh liver failure a thlen thei a ni.

Secondary dinhmun a ni

Thisen sen (red blood cells) a tam lutuk a, a chhe chhiat chuan taksaa `uric acid` level a sang chho zel a ni. Hei hian harsatna engemaw zat a thlen thei a:

• Kidney stones: Kal chhunga uric acid a awm chuan.
• Gout: Arthritis natna hrehawm tak, ruhkawr chhunga uric acid a lo awm tam lutuk.
• Stomach ulcers: Thisen sen tam lutuk hian stomach acid a tipung bawk. Thisen sen (extra red blood cells) hian taksaah chemical histamine an tih chu a tichhuak a, chu chuan pum chhungah acid tam zawk a siam chhuak a, chu chuan natna hrik do turin a ti a ni. PV vei te hi a let li velin pum chhunga ulcer (peptic ulcer disease) an vei tam zawk a ni.

Leukemia lam hawi

Polycythemia Vera hi ruhro cancer a ni a, mahse a tlangpuiin nunna atana hlauhawm a ni lo. Enkawlna dik tak a awm chuan kum tam tak chhung chu uluk taka enkawl theih a ni. Mahse, mi thenkhat tan chuan PV hian, a tlem hle nachungin, .`Acute Leukemia` ang chi thisen cancer na takah a chang thei.

Polycythemia Vera (PV) Natna stage hrang hrang

PV natna hi hetiang hian a kal zel a:

• Early stage (`Early PV`): Hetih lai hian symptoms a tlem emaw, a awm lo emaw a ni.
• PV hmasawn: PV a na zual zel chuan natna lan chhuah dan nuam lo zawk i nei tan thei a, a nih loh leh secondary condition a lo awm thei bawk.
• Spent phase: Hei hi “spent phase” an ti a. Hetih lai hian natna thlentu thisen cell pangngai lote chu thunun theih lohvin an pung a, thisen cell siamtu bone marrow chu an block vek a ni. Heng cell pangngai lote hi an thih chuan an aiah scar tissue a lo awm ta a ni. Ruhro tam zawk chu he scar tissue hian a khat a nih chuan thisen cell hrisel a siam thei lo. Thil mak tak mai chu hei hian thisen tlakchhamna (anemia) a thlen a – thisen zam hrisel tak tak awm lohna. Thisen chhuah theihna a tipung bawk.

PV hnua thisen natna dang lo awm thei

PV a nasat chuan thisen natna dang a thlen thei a ni.

• Myelofibrosis (MF): PV leh thisen cancer chi dang ``Myelofibrosis (MF)'' , ``spent phase'' hi a danglamna a awm lo. Doctor thenkhat chuan chutiang bawk chuan an ngai a. ``Myelofibrosis'' chu ruhroah cell pangngai lo takin scar tissue an siam a, an luah khat a ni. Heng cell pangngai lote hi ruhro pawnah leh taksa peng dangah a darh thei a ni. ``MF'' hi ``myeloproliferative disorder'' a ni bawk.``MF'' vei zinga 10% vel chuan ``Acute Myeloid Leukemia'' an vei thin.
• Myelodysplastic Syndrome (MDS): A tlem hle a, PV hi Myelodysplastic Syndrome (MDS) tih ang chi natnaah a lo chang thei a ni. Hei hi thisen cell te hi a puitlin tawk lo va, a lo thang lian lo va, an thi thuai thin. Taksa hian cell hrisel, puitling a siam thei lo va, chu chuan thisen cell chi hrang hrang a tihtlem phah a ni. MDS hi PV emaw MF emaw aiin a na zawk a, Acute Myeloid Leukemia (AML) vei theihna chance 30% a nei bawk.
• Acute Myeloid Leukemia (AML): Polycythemia Vera vei zinga 3% velin Acute Myeloid Leukemia (AML) an hmuhchhuah atanga kum 10 chhungin an vei thin. AML hi thisen cancer na tak a ni a, ruhro atanga intan a ni a, rang takin thisenah a darh thei a, chumi hnuah taksa peng dangah a darh thei bawk. Enkawl vat a ngai a, mahse damchhuah theihna chance a sang hle.

Polycythemia Vera (PV) natna lan chhuah dan chu engte nge ni?

Symptoms hi a lang zawi hle fo a, a chang chuan symptoms a awm lo thei bawk. A hmasa berin natna tam takah hian a lan chhuah dan chiang lo (vague symptoms) te hi a lo lang hmasa ber a ni:

• Lu na.
• Luak chhuak thei.
• Chau.
• Thisen sang a sang.
• Blurred vision emaw double vision emaw a awm thei.
• Beng chhunga ri ri hriat (`(Tinnitus)`).

Hun a kal zel a, symptom chiang zawk a lo lang thei a:

• Thaw chhuak, a bik takin zanah.
• Thâwk harsa, a bikin mut lai.
• Vun natna, a bik takin tui lum a inbual hnuah. (Hei hi PV chhinchhiahna chiang tak a ni)
• Ke ruh a sen, a lum, a hring emaw, a kang emaw.
• Thisen chhuah tam lutuk emaw, hliam na lutuk emaw.
• Hnar leh hmui atanga thisen chhuak.
• Chhan awm lova thinrim.

PV hi a nasat a, secondary condition-ah a kal chuan a kaihhnawih symptoms pawh a awm thei bawk. Entir nan:

Splenomegaly natna lan chhuah dan:

• Thil pakhat ang maia natna chu pum veilam chunglam ah a tang a.
• A pum tikhat thei thil tawn.
• Tlemte ka ei hnu pawh hian ka chaw ei zo vek.

Pum natna (`Peptic ulcer disease`) lan chhuah dan:

• Pum natna nena lo awm natna.
• Lungchhung natna.
• Acid a rawn chhuak leh thin.

Gout natna lan chhuah dan:

• Ruh natna na tak a hring chhuak.
• Joint a nghet lo.
• Ke ruh lian tak chu a hring chhuak.

Kidney stones natna lan chhuah dan: 1.1.

• Pum hnuai lam emaw, a sir lam emaw a na.
• Zun laiin natna.
• Zun chhuah a ngai fo thin.

Budd-Chiari syndrome natna lan chhuah dan:

• Liver a hring a, pum dinglam chunglam a na a, a pum a na bawk.
• Mit leh vun a sen (jaundice).
• Ascites leh Edema te a awm bawk.

Deep Vein Thrombosis (DVT) natna lan chhuah dan:

• Kut emaw ke emaw a hring leh khawih chuan a na.
• Chu hmunah chuan a sen emaw, a lum emaw a awm.
• Vun chunglam a thisen kalna bulpui (bulging blood vessels) a lan dan.

Lung chhunga thisen zam (Pulmonary Embolism - PE) chhinchhiahna:

• A rilru a na nghal.
• Thâwk a tawi.
• Heart rate a sang chho zel.

Anemia (PV natna hnuhnung ber) chhinchhiahna: 1.1.

• Luak chhuak (vertigo).
• Vun dum (pale skin) a ni.
• Chau.

Polycythemia Vera (PV) hi eng thilin nge a thlen?

Polycythemia Vera (PV) hi kan ruh nêm tak, spongy ang maia awm, bone marrow an tih aṭangin a intan a. Hei hi thisen cell thar siamna hmun a ni. PV hi ruhro chhunga stem cell pakhata gene mutation pakhat atanga tan a ni. 90% aia tam chu he mutation hi JAK2 an tih gene-ah a awm a ni.

He mutated gene hian stem cell chu a piang chhunzawm zel turin a hrilh a ni. Cell siam chhuah apiang chu an insiam thar zel a ni. A tawpah chuan heng cell pangngai lote hian ruhro chu an luah khat a, cell pangngai tan hmun a awm tawh lo.

A tam zawkah chuan he `JAK2` gene mutation hi kan rochun thil a ni lo. Nun chhunga thil thleng a ni a, chhan hriat loh vang a ni. Mahse, a tlem hle a, chhungkaw member engemaw zatin PV an vei tih report a awm fo.

Engtin nge Polycythemia Vera (PV) hi hmuhchhuah a nih?

World Health Organization (WHO) chuan Polycythemia Vera (PV) hriat theihna tur tehfung lian pathum a rawt a. Doctor chuan criteria pathumte hi a nemnghet vek tur a ni.

Criterion 1: Thisen test-naah thisen zam (red blood cells) sang tak a awm tih hmuhchhuah a ni:

Thisen sen (red blood cells) te hi teh theih a ni:

• Hemoglobin level sang (thisen sen chhunga protein awm).
• Hematocrit level sang (thisen chhunga thisen sen awm zat).
• Thisen tam lutuk (red cell mass).

Criterion 2: Bone marrow biopsy-ah hian a hnuaia mi pakhat hi hmuh theih a ni:

• Ruh chhunga thisen cell tam lutuk, emaw
• Megakaryocyte puitling (thisen platelet siamtu cell) awm tam lutuk.

Criterion 3: Criterion pathumna chu heng pahnih zinga pakhat tal lantir hian a tlin theih a ni:

• Molecular testing hmanga JAK2 gene mutation awm leh awm loh finfiah, emaw
• Thisen test chuan thisenah `erythropoietin' (kidney-in thisen sen siam chhuahna tichaktu hormone) level a hniam hle tih a nemnghet.

"Doctor chuan heng zawng zawng hi a en ang a, Polycythemia Vera i vei leh neih loh chiang takin a hrilh ang che. Chuvangin symptom i neih chuan doctor pan a, thurawn lak a tha ber.

Polycythemia Vera (PV) hi engtin nge enkawl a nih?

PV natna bul tan tirh lamah chuan enkawlna hi a awlsam hle. Enkawlna hman tlanglawn tak takte chu:

Phlebotomy (thisen chhuahna) .

Hei hi PV enkawlna ber leh hman tlanglawn ber a ni. Thisen i pek ang bawkin tih a ni. Healthcare provider chuan i kut zungtang pakhatah needle a dah a, thisen tlemte a paih chhuak (a tlangpuiin pint khat vel, mahse i dinhmun a zirin a zat chu a danglam thei). Hei hian i thisen volume zawng zawng leh thisen cell tam zawk a tihtlem phah a ni.

Aspirin dose hniam tak a ni

Hei hi damdawi dawr atanga lei theih a ni. A tlangpuiin thisen zam awm theihna tihziaawm nan an pe thin. Ni khatah vawi khat a tlem berah aspirin ei hian thisen platelet te chu a inzawm khawm loh nan a veng a ni. Tin, taksa peng hrang hranga natna lanchhuahna te pawh a tihziaawm thei bawk. Mahse, pum tan a khauh deuh a, thisen chhuah a tipung bawk. Chuvangin hei hi pum chhunga ulcer i neih chuan a tha lo.

I natna lan chhuah dan a nasat chuan, emaw, a hmaa thisen zam (thrombosis) i neih tawh chuan, high-risk group-ah dah theih a ni a, enkawlna dang pawh rawt theih a ni bawk. Chung zinga ṭhenkhat chu:

Itch tihziaawmna atana enkawlna: 1.1.

PV a nasat chuan doctor chuan a natna enkawlna atan hetiang damdawi hi a pe thei a:

• Antihistamines: Hengte hi allergy tan damdawi hman tlanglawn tak a ni.
• Phototherapy: Ultraviolet (UVA) light leh chemical psoralen (organic compound, vun light therapy laka sensitize tu) te inzawmkhawma enkawlna a ni.
• SSRIs (Selective Serotonin Reuptake Inhibitors): Hengte hi lungngaihna damdawi chi khat a ni a, a tlangpuiin lungngaihna damdawi chi khat a ni. Mahse, a tlem lutuk a pek a nih chuan taksa lama natna langsar tak tak, itching ang chi tan pawh a tangkai hle tih hmuhchhuah a ni bawk.

Thisen zam (red blood cell count) tihtlem thei damdawi: 1.1.

Chungte chu amah chauhin emaw, a rualin emaw hman theih a ni. Entirnan:

• `Hydroxyurea` tih a ni
• `Interferon alfa` tih a ni
• `Ruxolitinib` tih a ni
• `Busulfan` tih a ni

Clinical trial neih a ni

Chûngte chuan mite chu enkawlna thar ber berte enchhin theihna hun remchâng a pe a, damdawi lam zir chiannaah pawh an thawhhlawk hle. Tuna zirchianna kalpui mek damdawi thenkhat chu:

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

Bone marrow thlak danglam a ni

A then phei chuan ruhro thlak (bone marrow transplant) pawh a tha thei bawk. Doctor chuan he thutlukna hi thil engemaw zat a zirin a siam ang a, chu chu i natna nasat dan leh i taksain a dam theih dan te hi a ni.

Supportive care

Enkawlna dawng chung pawhin i PV a na zual zel a nih chuan i doctor chuan i symptoms enkawl dan tur a ngaihtuah ber ang. PV hnuhnung zawkah chuan thisen tlakchhamna (anemia) leh spleen lian zawk i nei thei bawk. Enkawlna hrang hrang pek i ni thei bawk a, chungte chu:

• Natna tihdamna damdawi.
• Thisen pek a ni.
• Radiation dose hniam tak hmanga enkawl a ni.

Polycythemia Vera (PV) vei dam rei zawng chu engzat nge ni ang?

Tun hnaia zirchianna hrang hrangah Polycythemia Vera (PV) hmuhchhuah hnua dam rei zawng chu kum 20 vel a ni. Thih kum zat chu kum 77 vel a ni. Thihna thlentu ber chu thisen zam avanga harsatna (33% vel) a ni. A chhan lian ber pahnihna chu cancer (15% vel) a ni.

"Hetiang statistics hi hlau suh. Hengte hi value pangngai a ni. Enkawlna dik tak i hmuh a, nunphung i thlak danglam chuan rei tak i hrisel thei a ni."

Engtin nge ka in enkawl ang a, ka symptoms te hi ka enkawl ang?

Polycythemia Vera (PV) natna vei i nih chuan doctor nena inbiak reng a pawimawh. Hei hian i doctor-in i dinhmun a kal zel dan a enfiah theih nan a pui ang. I doctor hnenah enkawlna i dawn dan leh side effect i neih leh neih loh pawh i hrilh thei bawk. Symptom na tak nei lovin rei tak i dam thei.

Test leh enkawlna pangngai bakah hian i doctor chuan thisen zam leh harsatna dangte venna atana pui thei nunphung tihdanglam awlsam tak tak a rawn rawt bawk ang. Entir nan:

• Insawizawi. Exercise tlemte pawh hian thisen kal dan a titha thei a ni.
• Meizial zuk loh tur. Meizial in hian i thisen kalna kawng a ti zim a.
• Oxygen level hniamna hmun chu pumpelh rawh. Hmun sang takah chuan thisenah oxygen level a tlahniam thei a ni.
• I thisen sang control rawh. Hemi atan hian taksa rihna hrisel tak neih hian a pui dawn a ni.

Polycythemia Vera (PV) hi cancer chi khat ni mah se, mi tam tak chu he natna an vei tih hmuhchhuah a nih hnuah kum sawm tam tak chhung chu an dam tha hle. He cancer hi cell te hi control theih loha siam chhuah a nih vang a ni. Mahse, thisen zam (red blood cells) control loha siam chhuah hian a nghawng tan theih nan hun rei tak a ngai a ni. Chutianga i tih pawhin i thisen tam lutuk ṭhenkhat chu paih chhuakin hun rei tak chhung chu i dinhmun chu i enkawl thei a ni.

Polycythemia Vera (PV) atanga hlauhawm ber chu cancer ngei ni lovin, thisen zam a awm theihna a ni. PV natna vei i nih chuan doctor pan fo thin ang che. Tin, "thil tenawm" - chu chu nunphung hrisel tak tak hmanga in enkawl pawh theihnghilh suh. Hengte hian i cardiovascular system hriselna tlangpui vawng reng turin a pui a, hei hi Polycythemia Vera damdawi zawng zawng aiin a pawimawh zawk mai thei.

He thawnthu atang hian eng thuchah nge in lama kan hruai duh?

Okay, chuvang chuan Polycythemia Vera (PV) chungchang hi kan sawi nasa hle. Hriat reng tur pawimawh tak tak thenkhat chu hetiang hi a ni:

• PV hi thisen natna a ni a, taksain thisen sen (red blood cells) a siam tam lutuk a, a lo thang lian lo hle. Cancer pawl pakhat `myeloproliferative neoplasm (MPN)` an tih zingah a tel a ni.
• A hlauhawm ber chu thisen thim leh thisen zam atanga lo chhuak a ni a, hei hian heart attack leh stroke ang chi natna khirh tak tak a thlen thei a ni.
• A lan chhuah dan chu vun natna (a bik takin inbual hnuah), rilru hah, lu na, leh luak chhuak te a ni thei.
• Dam kim theih ni lo mah se, enkawl dan dik, nunphung thlak danglam leh damdawi hmanga enkawl that theih a ni. Phlebotomy (bloodletting) leh aspirin dose tlem te hi enkawlna bulpui ber a ni.
• `JAK2` gene mutation vang hian a awm fo thin.
• Heng symptoms te hi i neih chuan doctor pan ngei ngei tur a ni. Hriat hmasak hian harsatna i pumpelh theihna leh nun hrisel tak i neih theihna a tipung a ni.

Hlau suh, mahse awlsam takin la ngai suh. I hriselna enkawl rawh. Doctor nen inbia fo la, a hun takah test leh enkawlna tul tak tak la rawh. Chuti a nih chuan hetiang dinhmunah pawh hian hrisel takin i awm thei ang!

` Polycythemia Vera, Thisen sen, Thisen zam, MPN, JAK2, Phlebotomy, Cancer