Polycythemia vera

Polycythemia vera (PV) ke lefu la moko oa masapo le lebisang keketseho e sa tloaelehang ea palo ea lisele tsa mali. Lisele tse khubelu tsa mali li ameha haholo.

PV ke lefu la moko oa masapo. E baka haholo lisele tse khubelu tsa mali hore li hlahisoe. Linomoro tsa lisele tse tšoeu tsa mali le liplatelete le tsona li ka ba holimo ho feta tse tloaelehileng.

PV ke lefu le sa tloaelehang le hlahang khafetsa ho banna ho feta basali. Hangata ha e bonahale ho batho ba ka tlase ho lilemo tse 40. Bothata bona hangata bo amahanngoa le sekoli sa lefutso se bitsoang JAK2V617F. Ha ho tsejoe hore na sesosa sa sekoli sena sa lefutso ke sefe. Bofokoli bona ba lefutso hase boloetse bo futsitsoeng.

Ka PV, ho na le lisele tse khubelu tsa mali tse ngata haholo 'meleng. Sena se fella ka mali a teteaneng haholo, a sitoang ho phalla methapong e menyenyane ea mali ka tloaelo, ho lebisa ho matšoao a kang:

• Ho hema ka thata ha o paqame
• Letlalo le leputsoa
• Botsoa
• Ho ikutloa ke khathetse ka linako tsohle
• Ho tsoa mali a mangata, joalo ka ho tsoa mali letlalong
• Boikutlo bo felletseng ka mpeng e kaholimo ea leqele (ka lebaka la spleen e holisitsoeng)
• Ho opeloa ke hlooho
• Ho hlohlona, ​​haholo-holo kamora ho hlapa ka mofuthu
• Mmala o mofubelu oa letlalo, haholo sefahleho
• Phefumoloho e khutšoanyane
• Matšoao a mali a koahetsoeng ke methapo haufi le letlalo (phlebitis)
• Mathata a pono
• Ho lla litsebeng (tinnitus)
• Bohloko bo kopaneng

Mofani oa tlhokomelo ea bophelo o tla etsa tlhahlobo ea 'mele. U kanna oa ba le liteko tse latelang:

• Tlhahiso ea moko oa masapo
• Palo e felletseng ea mali ka phapang
• Phanele e akaretsang ea metabolic
• Boemo ba Erythropoietin
• Teko ea liphatsa tsa lefutso bakeng sa phetoho ea JAK2V617F
• Ho tlatsoa ha mali ka oksijene
• Boima ba lisele tse khubelu tsa mali
• Boemo ba vithamine B12

PV e kanna ea ama le litholoana tsa liteko tse latelang:

• ESR
• Lactate dehydrogenase (LDH)
• Leukocyte alkaline phosphatase
• Teko ea pokello ea Platelet
• Serum uric acid

Morero oa kalafo ke ho fokotsa botenya ba mali le ho thibela mathata a ho tsoa mali le ho koala.

Mokhoa o bitsoang phlebotomy o sebelisoa ho fokotsa botenya ba mali. Ho tlosoa yuniti e le 'ngoe ea mali (hoo e ka bang ponto e le' ngoe, kapa 1/2 litha) beke le beke ho fihlela palo ea lisele tse khubelu tsa mali e theoha. Kalafo e tsoela pele ha ho hlokahala.

Meriana e ka sebelisoang e kenyelletsa:

• Hydroxyurea ho fokotsa palo ea lisele tse khubelu tsa mali tse entsoeng ke moko oa masapo. Meriana ena e ka sebelisoa ha lipalo tsa mefuta e meng ea lisele tsa mali le tsona li phahame.
• Interferon ho theola lipalo tsa mali.
• Anagrelide ho theola lipalo tsa platelet.
• Ruxolitinib (Jakafi) ho fokotsa palo ea lisele tse khubelu tsa mali le ho fokotsa spleen e holisitsoeng. Meriana ena e laeloa ha hydroxyurea le kalafo tse ling li hloleha.

Ho nka aspirin ho fokotsa kotsi ea ho koaloa ha mali e ka ba khetho bakeng sa batho ba bang. Empa, aspirin e eketsa kotsi ea ho tsoa mali ka mpeng.

Kalafo ea ultraviolet-B e ka fokotsa ho hlohlona ho matla hoo batho ba bang ba ho utloang.

Mekhatlo e latelang ke lisebelisoa tse ntle tsa tlhahisoleseling ka polycythemia vera:

• Mokhatlo oa Naha oa Mathata a Rare - rarediseases.org/rare-diseases/polycythemia-vera
• Setsi sa Boitsebiso ba Mafu a Rare le Mafu a Rare - rarediseases.info.nih.gov/diseases/7422/polycythemia-vera

Hangata PV e hola butle. Batho ba bangata ha ba na matšoao a amanang le lefu ka nako ea tlhahlobo. Boemo bo lula bo fumanoa pele matšoao a matla a hlaha.

Mathata a PV a ka kenyelletsa:

• Lefuemia e matla ea 'mele (AML)
• Ho tsoa mali mpeng kapa likarolong tse ling tsa mala
• Gout (ho ruruha ho bohloko ha lenonyello)
• Ho hloleha ha pelo
• Myelofibrosis (pherekano ea mokoallo oa masapo moo moko o nkeloang sebaka ke lisele tsa 'mele tse bofubelu)
• Thrombosis (ho koala mali, ho ka bakang stroke, lefu la pelo kapa tšenyo e ngoe ea 'mele)

Bitsa mofani oa hau haeba matšoao a PV a hlaha.

Polycythemia ea mantlha; Polycythemia rubra vera; Lefu la Myeloproliferative; Erythremia; Splenomegalic polycythemia; Lefu la Vaquez; Lefu la Osler; Polycythemia e nang le cyanosis e sa foleng; Erythrocytosis megalosplenica; Cryptogenic polycythemia

Kremyanskaya M, Najfeld V, Mascarenhas J, Hoffman R. Polycythemias. Ka: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Melao-motheo le Tloaelo ea Motheo. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 68.

Webosaete ea National Cancer Institute. Kalafo e sa foleng ea myeloproliferative neoplasms (PDQ) - mofuta oa litsebi tsa bophelo bo botle. www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq#link/_5. E ntlafalitsoe ka la 1 Hlakola 2019. E fihlile ka la 1 Hlakubele 2019.

Tefferi A. Polycythemia vera, thrombocythemia ea bohlokoa, le myelofibrosis ea mantlha. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: khaolo ea 166.