Macroglobulinemia o Waldenstrom: tohumate, ai, waitohunga

He onge tuku mate toto, engari e tino kino ki te tangata. Ko tētahi taua mate ko Vandelstrema macroglobulinemia.

Atu i te āhuatanga reira?

I raro i tenei mate i roto i te rongoā hou e pā ana ki te syndrome hematological tuku tahi e te aroaro o te macroglobulin toto. i hanga tēnei pūmua e te mahi o monoclonal pūtau toto B-lymphoid (B-lymphocytes).

Tikanga, He kawenga mo te kōtuitui o toto-M globulin enei pūtau. A, no te tetahi mahi o te hinu wheua wheua (maha i roto i te whanaketanga o te tukanga puku) ko te huringa i roto i te hanganga waikawa amino me te raupapa o te ira, ārahi ki te kōtuitanga macroglobulin, i.e. whakawhanake macroglobulinemia o Waldenstrom.

Ko te tino onge, e pā ana ki 3 take ia miriona iwi te mate. whakawhanake te reira i te nuinga i roto i nga tangata i runga i te tau o 55 tau. tenei māuiuitanga te tata kore e puta ake ki te 40 tau.

Pehea, Na, faaite mai te mate i te taumata pūtau me te kiko? E ngā hanganga e pāngia i te wahi tuatahi, me te e rite te hua i reira?

Ko te pathogenesis o te mate

I te ngakau o patogenza te mate ko te tini mārere o ngā pūtau wē ki tou pūtau taea o te whakawhanake immunoglobulin M. enei pūtau whakaekea te wheua wheua, ate, kōateate, te wahi timata ratou ki mātātoa proliferate ka huna macroglobulin pathogenic. Ko te kupu whakawhanake macroglobulinemia o Waldenstrom.

Ko te haaputuputuraa o te pūmua pathogenic i roto i te toto arata'i ki te whanaketanga o tona pokey. Ka rite ki te hua o heke auau rere toto i roto i te oko, e arata'i ki tona stagnation. I tua atu, leva āhuatanga coagulation wē (e tika ana ki te tākai, ka Tautauā ratou macroglobulins). No te mea o tenei raru te hanganga katinga noa, e te mutunga arata'i ki nui haere toto.

Whatu, ka te uiui i raro i te karu whakarahi, te pūtau wheua wheua, e taea te kite i reira initareti e "plazmatizirovannyh" lymphocytes, he maha nui o pakeke B-lymphocytes me secreting takawaenga inflammatory o ngā pūtau rewa. Katoa o enei pūtau fokotuu e reira ko te macroglobulinemia Waldenstrom.

tohu

Pehea e taea hauora te whakapae o tenei mate?

Ka amuamu nga tūroro tuatahi o tohu nonspecific - ngoikore whānui, iti-māka kirika mo te wa roa, sweating, mate taimaha hoki kahore take kitea.

Ko te tohu matua, i runga i te pūtake o e taea tatou whakapae e macroglobulinemia Vandelstrema tipu, kua nui te toto i te ihu, ngā pae me kiriuhi mucous. He nui iti pinepine kitea karawarawa subcutaneous me karawarawa.

Neke atu i te wa, kia whakawhanake hepatosplenomegaly me lymphadenopathy (taikiri tetere). Ko taea ano hoki te whanaketanga o pōauautanga macroglobulinemia.

A, no te ki te kore whakarato i te atawhai hauora wā i reira ko te mōrea o te manawanui coma, a tae noa te mate (engari mate tūroro e te nuinga o kore pera nui i runga i te mea e ratou e mate ana i macroglobulinemia o Waldenstrom). Ko te take o ratou matenga he te hua o pōauautanga whakawhanakehia.

fifi o te mate

Ka taea e te taua te uaua e te mate?

Matua whai wāhi i roto i te tukanga pathological o te pūnaha Cardiovascular, ara nga oko iti - tōiti, arterioles me venules. Na roto i te whakaiti rere toto kitea te whanaketanga o te retinopathy, nephropathy. kino tākihi. I roto i tenei take āhuatanga e te whanaketanga o glomerular occlusion iaia me te urolithiasis ki waipara o urate.

Macroglobulinemia Waldenstrom āhuatanga e wātea i te mate iti, me te hono mate kapo te fifi me tātaritanga mo te mate whāriki.

Ka rite ki te ahunga whakamua o te tukanga ko te huna i tohua o tata iroriki katoa o hematopoiesis me te whanaketanga o te pancytopenia. Amyloidosis āhuatanga e te whanaketanga me pāpaka pathological rite pūmua ate me oko kōateate toto, e atu worsens me pera ratou mahi waimaero.

tātaringa

He aha inenga e tokoni ke mahino koe e tipu macroglobulinemia?

Tuatahi, kia tatou e whakarongo ki te CBC. Ka whakanui i nga tohu matua o te mate i roto i te ngeniwhero auau te parataiao me te hanganga o motuhake "rouleaux" - pipiki tahi o ngā pūtau toto whero. WBC nuinga tohu te nui haere te maha o lymphocytes i te tātari me te ahua o leukocytes puka teeki.

Hei whakaū i te tātaritanga i te whakahaere immuno. I muri te whakahaere i tenei ako e kore e taea te tautuhi i te nui nui haere i roto i te immunoglobulin toto M

kua oti te whakarite immunoglobulins Monoclonal i roto i te toto i muri i te tua o te sera tapanga.

Atu, engari e kore e motuhake tohu ngā he ate rahi me te kōateate, rapunga o amyloid i roto i te unuhanga kappillyarah whatukuhu, me te whakaiti i te maha o ngā āhuatanga wē coagulation (i roto i ngā, take 8).

maimoatanga

I roto i te tīmatanga o te mate, te kore i reira e nui te whai wāhitanga okana ā, e kore e tohu maimoatanga motuhake. E mea pinepine, e i raro i te tirotiro hauora i te hematologist rohe enei tūroro.

A, no te whakau nga tohu tuatahi, me te e te mate - Waldenstrom macroglobulinemia, timata therapy motuhake ki cytostatics. Hoki te whakamahi maimoatanga taero pērā i "hlorbutin", "Cyclophosphamide". E ratou he pānga cytostatic me pehi te mahi o te tou mārere o te B-lifotsitov.

Whakaritea i roto i te wahi tuatahi "hlorbutin" ki te 6 mg ia waha hoki 3-4 wiki. Te whakanui ake i te horopeta, he mahi wehe ki te whanaketanga o te wheua wheua aplasia. I te mutunga o te akoranga o te maimoatanga i whakaritea pota tiaki o te tarukino (4.2 mg) atu nga ra.

Ki te whakapai ake i te tawhā rheological toto whakamahia plasmapheresis. kawea maimoatanga plasmapheresis i roto mo te whakamahi o te raau taero cytotoxic. Hoki te tukanga tango whakaaturia i roto i te 2 rita wē ki kaituku whakauru i runga i ona inactivated.

ārai

No te mea he tuku te mate, he uaua i roto i tetahi ara pānga tona whanaketanga reira, mai te kua whakatakotoria ki raro i roto i te ira. Ko te ara anake ki te karo i te whanaketanga o te tātaritanga wā o te mate, me te mahere pai-unu o maimoatanga.

Autaki pā taea te mate kia ngohengohe ki te āhua noho hauora, hoatu ake kai paipa, me te waipiro, rohe paowa me rihi raukikini.

Mai te he ira mate, e taea tīmata te reira tona whanaketanga me etahi tikanga kino - mahi ki tae aniline, peita, varnishes.

E taea e ki te huri i te genome tangata hoki auau mate viral. ka tauturu i te maimoatanga wawe o enei mate ārai huringa i roto i te ira me te ārai i te whanaketanga o te macroglobulinemia.

Ka taea te pēnei āraihia atu ki te waia tūroro ki te āhuatanga o te mate, me te whakatairanga o ratou mahi ki te ū ki te āhua noho hauora.

waitohunga

He aha tatari hoki tūroro e kua whakawhanakehia Waldenstrom macroglobulinemia? Waitohunga o te mate tei runga i te pakeke o te mate e kua whakawhanakehia pōauautanga me maimoatanga wā.

Ki te ko te mate i roto i tona atamira wawe o te whanaketanga, i roto i te nuinga o te wā e taea ana ki te ārai i tona ahunga whakamua. Taua tūroro ara i te tupono noa mo te rongoa oti, te whakamahi o pota tiaki o cytostatics tonu tino e taea faarahi i to ratou tümanako ora.

He kino nui mo te hunga ki te macroglobulinemia uaua o Waldenstrom. Tohu, Ko tino matekiri te waitohunga o nei - ate amyloid me whatukuhu, toto me te whanaketanga auau pōroa paraproteinemic. Ki te kore e wa te āwhina i taua tūroro, he tūponotanga tiketike o te mate.

Ko te tümanako ora toharite mō ngā tūroro ki te mate, ko te pā ana ki 4-5 tau. A, no te tika te tuhi ake ai te mahere o te maimoatanga ake i reira ki te 9-12 tau.

Risk o mate

Hoatu e puta te mate uaua, he uaua ki te whakapae ia o te whanaketanga o te tangata e tata kore tutaki ki a ia. Hoatu e te whakapā tuatahi ki kaihaumanu rohe o te manawanui, ko reira ki runga ki o ratou pokohiwi, a ka he haepapa mō te whakarite i whakapae wā, ka pāngia ki te macroglobulinemia o Waldenstrom. He aha te mea tenei mate, ka e maharatia, e kore kaiwhakaako katoa, Heoi, ki te whakaaro e kua tika te manawanui homai māuiuitanga, tango ki pūkete ona faaiteraa haumanu katoa, kia tetahi kaihaumanu.

I roto i te heke mai, tenei manawanui ka arahi hematologists, engari kei te tātaritanga tuatahi anake i runga i nga pokohiwi o tākuta haumanu.

tāutu Late o te mate arata'i ki mau hopearaa kino ina he kore te hahau whai hua. Ko Ko te aha kia mohio koutou e pā ana ki tenei mate katoa e kore e ki te mahue i reira, a e kore e kawea ki te āhua o te tau'a ore.