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Lymphoma Hodgkin Classical: sclerosis nodular

sclerosis nodular - he momo histological o te mate o Hodgkin, āhuatanga e te tupu tangotango o kiko tūhonohono, wehewehe i te papatipu o ngā pūtau kōhikohikotia āhua me lobules. Kei roto ratou mea lymphoid tupuria ki te maha nui o ngā pūtau Berezovsky-Sternberg. tīmata te mate ki te hua o ngā kōpuku. Tenei māuiuitanga Ko tetahi o nga rerekē o lymphoma puāwaitanga o Hodgkin.

whakaaro mate o Hodgkin te te ma'i tino e pā te pūnaha lymphatic. kia hanga i te mate ki hea i roto i te tinana, e kua te kopa lymphoid (thymus, tonsils, kōateate me adenoids al.).

Nodular sclerosis tohu

lymphoma o Hodgkin taea e te tangata, ki te he ia taua tohu rite:

  • mate taimaha;
  • taikiri tetere (pinepine i roto i te rohe o te kaki);
  • mate o te hiahia;
  • hāhā o te manawa;
  • po sweats pāmahana ranei;
  • mamae pouaka;
  • ate rahi (5% o tūroro) ranei kōateate (30% o tūroro);
  • pouri mamae ranei i roto i te kopu (i roto i ngā tamariki);
  • kiri oranga (1/3 anake o te iwi e mate ana ma'i);
  • manawa uaua;
  • mare.

take

Ka taea te kirimana mate o Hodgkin i tetahi tau, engari te nuinga maha puta te reira i roto i nga tane taitamariki i roto i te kāwai tau o 16 ki te 30 tau o te tau ranei i roto i te iwi paari i runga i 50 tau. Tamariki ki runga ki te 5 tau, tata kore e whiwhi mate. He aha rite pāngia e tenei mate ko tonu unknown. Heoi, i reira he he whakaaro e te puna - wheori. Kei te whakapono te reira e taea te tīmata i te take o tenei raruraru:

sclerosis nodular taea lymphoma o Hodgkin haere tonu, muri i te 3 ki te 6 marama, whakamutunga hoki 20 tau ranei.

He aha te wā e te mate?

whakaritea tohu hodzhinskoy lymphoma i rangahau taiwhanga me i te kaupapa o te tohu e whai ake nei:

  • te maha o taikiri pāngia, me te ratou wāhi;
  • wātea o ngā kōpuku raraunga i roto i ngā wāhi rerekē o te HWM;
  • pukupuku i roto i te tahi atu whekau (hei tauira, ate kōateate ranei).

Ko te wāhanga tuatahi. I roto i tenei take pāngia kotahi anake kōpuku waitinana okana lymphoid (kōateate, mowhiti Pirogov-Valdera) ranei.

Ko te wāhanga tuarua. He nuinga o taikiri miharo i runga i ngā taha e rua o nga whekau pouaka, HWM, me lymphoid.

Ko te atamira tuatoru. Tenei Hodgkin tohu whakauaua rerekē i te atamira tuarua. Heoi, kua reira e rua momo o sclerosis nodular atamira tuatoru:

  • i roto i te take tuatahi e pāngia whēkau kei raro te HWM (kōpuku celiac taikiri, kōateate);
  • i roto i te tua ki te wāhi i tabulahia i roto i te momo tuatahi, miharo tonu, me ētahi atu wāhi ki te taikiri kei tata te HWM.

tuawha te atamira. pānga te reira i te wāhanga e kore anake, engari ano hoki whekau kore-lymphoid - wheua wheua, ate, wheua, pūkahukahu, me te kiri.

Iku'anga nekehanga lymphoma o Hodgkin

tohua taupū te āhuatanga haumanu me te pakeke o te akoranga mate o te tahi atu te kopa me whēkau te ki pukapuka.

A - nui tohu whānui o te mate e kore.

B - te aroaro o tetahi atu ranei tohumate (kiko ake te pāmahana, sweats po, tere mate taimaha).

te tono aronga ki te kopa me whēkau kei tata te taikiri kua pā - E.

S - Kua lesion splenic.

X - i reira ko te puku nui rahi nui o.

momo Histological o mate

No nia i te hanganga pūtau o te mate o Hodgkin e 4 puka o ma'i.

  1. Nodular sclerosis lymphoma o Hodgkin - te puka tino noa o te mate, e pā ana ki 40-50% o te wā katoa. whiwhi ratou mate atu pinepine atu i te taitamariki wahine, e e te nuinga pāngia taikiri o te mediastinum. Te rauemi unuhanga, i roto i te tua ki ngā pūtau Berezovsky-Sternberg e hoki nui pūtau lacunar ki foamy cytoplasm, me te taimaha karihi. Ko te tikanga pai matapae i roto i tenei mate.
  2. Lymphohistiocytic lymphoma i hanga e 15% o nga tauira. Nuinga o ngā wā e taea e kitea taua mea i roto i nga tangata taitamariki i raro i 35 tau. He he-tau e rima tere ora pai o ngā pūtau, me he limfaitov pakeke me Shtrenberga. Tenei ahua o te mate ki te mārere iti a mauherea reira i roto i te wāhanga tuatahi.
  3. momo ngā Kei te nuinga o te wā pāngia i roto i te paari, me ngā tamariki. Rerekē āhuatanga pikitia haumanu angamaheni me te fakahehema ki ahuwhānui te mahi. whakakitea whakamātautau Histological putanga rerekē o hononga pūtau, tae atu Sternberg. Kitea i roto i te 30% o tūroro ki lymphoma. sclerosis nodular i roto i tenei take he waitohunga ngā pai, a ki te whakaritea te maimoatanga wa te, puta te murunga u, kahore raruraru.
  4. granuloma mōrearea tuhia pinepine te whakangaromanga o te kiko lymphoid, anake 5% o te mau hi'oraa (te nuinga i roto i te paari). He āhuatanga āhuatanga i konei ko e reira he kahore pūtau me te wā e pūtau Sternberg. Tenei puka o te lymphoma āhuatanga e te ōrau i raro o-tau e rima oranga.

tātaringa

tautuhia te tātaritanga o "lymphoma" Kei anake e whakamātautau histological o nga taikiri, me te kia whakataetae anake i roto i te take i reira i kitea nga pūtau motuhake maha-Sternberg rite te hua o tenei ako. I roto i te wā kino e hiahia immunophenotyping. tātari Cytological o te tīpona waitinana whatukuhu ranei te tikanga poka iti ki te whakaū i sclerosis nodular, momo 1. He aha Me ki kia meatia ki te whakatū i te tātaritanga o te mate:

  • whakamātautau toto whānui, me te matüora;
  • X-hihi o te pūkahukahu (whakahauanga i roto i te taha, me te ngä frontal);
  • waitinana kōpuku unuhanga;
  • tirohanga ultrasonic o nga mea katoa o piringa, me te intraabdominal taikiri, thyroid, ate, me kōateate;
  • CT scan ki te whakakore i äta taikiri mediastinal i Radiography tikanga;
  • unuhanga trephine o iliac wheua ki te ārai i wheua wheua;
  • karapa me X-hihi o wheua.

therapy

Oku i ai e radiation therapy, pokanga me te hahau. Te tohu i te ara ki te whakaturia te wā o mate, me te aroaro o āhuatanga prognostic pai kino ranei. Ko te āhuatanga pai ngā:

  • patapata i histological whakamātautau nodular sclerosis, me te momo lymphohistiocytic;
  • Tau iti iho i te 40 tau;
  • rōrahi waitinana kōpuku kore e e nui 6 cm te whānui;
  • kore o faaiteraa noa o te whai huatanga koiora (whanaketanga kuputohu matüora o toto);
  • te aroaro o kore wāhi lesion neke atu i te 3.

Ki te mahi koe i te kore i te iti rawa tetahi o enei take, ka te tuku te manawanui ki te rōpū ki te waitohunga rawakore.

therapy radiation

Full iraruke rite te whakamahia he tikanga takitahi mō ngā tūroro ki atamira Tuhinga ka IIA, whakau i laparotomy, a ka he āhuatanga prognostic pai. Kei te hanga te reira i mara noa ki te rongo ki ngā momo katoa o taikiri pāngia me irava waitinana pana.

Wahi o te katoa whenumi i roto i te metastases lesion he 40-45 g hoki 4-6 wiki i roto i te mara o te irradiation prophylactic - 30-40 g ia 1-4 wiki. Hoki ki ki tikanga widefield te whakamahi i irradiation maha-mara o te tahi mau pāpaka ārai sclerosis nodular ns1.

Ka taea e te maimoatanga radiation meinga pōauautanga pērā i kakakīrehe kiko subcutaneous, radiation pneumonitis me pericarditis. Ino puta i roto i te wā rerekē - i te 3 marama ki te 5 tau i muri i te maimoatanga. Tei ratou matatini i runga i te whakamahi o te horopeta.

ngā mahi

Kei te uaua maimoatanga hāparapara whakamahia anake onoobychno he wahi o te therapy matatini. Te mahi splenectomy, me te ngā mahi i runga i te pukorokoro, te pū kai, te puku, me te tahi atu whēkau (ki te mōrea o te asphyxia, turaki te wahi o te kai). Huraina i roto i rerengia chlamydia hapūtanga tino kia haukotia.

hahau

whakamahia ana tēnei puka te rite tetahi wāhanga o te maimoatanga matatini. Ki te hamani sclerosis nodular, whakamahia taka rerekē:

  • pāhare ( "Vinblastine" ranei "rozevin", "Etoposide" ranei "Vincristine," Oncovin ");
  • Alkire ranunga ( "Mustargen", "Cyclophosphamide" ranei "embihina", "nitrosomethylurea" ranei "hlorbutin");
  • āpiha waihanga ( "Natulan" ranei "procarbazine," "Dacarbazine" ranei "Imidazole-carboxamide");
  • paturopi antitumor ( "Bleomycin", "adriablastin").

monochemotherapy

Kei te whakamahia te reira i anake i roto i ngā take motuhake ki te whakaaro o te heheu mai. Tikanga rapu atu medicaments therapy ki te tikanga rerekē o te mahi (te hahau). I roto i te wha te atamira i roto i te tūroro ki te mate ate E ruia ana wheua wheua taua maimoatanga ranei he anake te ara - ko te lymphoma Hodgkin puāwaitanga tenei. sclerosis nodular tukinotia ki taua kaupapa:

  • ABVD ( "Bleomycin", "Dacarbazine", "adriablastin" "Vinblastine");
  • MOTAPMs ( "Oncovin", "prednisolone", "Mustargen", "procarbazine");
  • TSVPP ( "Vinblastine", "prednisolone", "Cyclophosphamide", "procarbazine").

Therapy he poto (2, 7, 14 ra) akoranga ki wā e rua-wiki. Ko te maha o huringa rerekē e tika ana ki te nui o te lesion tuatahi, me te susceptibility ki te maimoatanga. Ko te tikanga tutuki te murunga katoa ki whakahaunga 2-6 akoranga. I muri i taua, e tūtohu ana ki te mahi i te atu 2 huringa o therapy. Ki te ko te hua murunga wāhanga, te mea ke te talena maimoatanga te, me te maha o piki akoranga.

Reception he hematopoiesis rongoā pressurized, alopecia, tohu dyspeptic, e waiho i te mutunga o te maimoatanga. sclerosis nodular tonu arata'i ki te mutunga o nga fifi, pērā i pākoko, leukaemias me ētahi atu malignancies (pukupuku tuarua).

outlook

meinga mate o Hodgkin e te āhuatanga rere, atamira haumanu o te mate, tau o te manawanui, me ētahi atu momo histological. Ki te akoranga koi me te subacute o te mate e kore te mea he waitohunga pai: tūroro te tikanga mate i roto i te wā o te 1-3 marama ki te 1 tau. Otiia i roto i te matapae chlamydia ma'i herenga pai. Ko te mate e taea muri mo te wa rawa te roa, ki runga ki te 15 tau (i roto i ētahi wā nui roa).

I roto i te 40% o nga iwi huaketo katoa, ina koa i roto i te wā 1 me te 2, me te he take prognostic pai, hoki i runga i te 10 tau me te ake auau i tuhia. e kore e whati kaha ki te mahi rite ki te hua o te roanga o te murunga.

ārai

Ka rite ki te tikanga, kua whakahaua ai ki te ārai o relapses. Tūroro ki Hodgkin kia tirotirohia matepukupuku rānei ma'io te. I roto i te ako, i te mea te 3 tau tuatahi e hiahiatia ana ki te kia kawea i roto i nga marama e ono, a ka kotahi te tau, e hiahia ana koe ki te arotahi ki runga i te whai huatanga o ngā tohu koiora maha he tohu tuatahi o te hohokitanga (hua i roto i te taumata o te pūtaitoto me globulins, ake i pops). Tūroro ki te mate i tua o Hodgkin kino waiariki, pukā me insolation tika. hua whakapumautia i roto i te maha o relapses tika ki te hapūtanga.

Na e matau he pono maha iwi e lymphoma o Hodgkin - ki te sclerosis nodular kē, i te mea he tino kino, me te uaua hamani mate.

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