Lymphoma e tloaelehileng e hlaha ntle le lymph node ho eketsa
Hepatosplenic T-cell lymphoma (HSTCL) ke lymphoma e sa tloaelehang haholo. Tsejoang e le "hepatosplenic γ δ T-cell lymphoma," boloetse bona bo 'nile ba tlalehoa hangata libukeng tsa saense,' me kahoo sekhahla sa 'nete sa sona ha se tsejoe.
HSTCL e atisa ho bonoa ho banna ba bacha, le hoja linyeoe tse amanang le basali le bana li ngotsoe. Hape, ho bonahala eka ho na le kamano ea kotsi e eketsehileng ea HSTCL ho bakuli ba immunocompromised.
Ho itšetlehile ka linyeoe tse hatisitsoeng, HSTCL e ka 'na ea se ke ea fumanoa hampe ka lekhetlo la pele,' me e na le mesaletsa e bobebe.
Matšoao
- Majoe a tloaelehileng
- Mokhathala
- Matšoao a palo e tlaase ea mali (mali a mali, thrombocytopenia)
- Anemia e ka hlahisa mokhathala, mokhathala
- Thrombocytopenia e ka baka ho lla kapa ho tsoa madi
- Matšoao a molao-motheo, ho kenyelletsa le latelang:
- Feberu e sa hlalosoang
- Ho lahleheloa ke tefo ntle le ho leka ho theola 'mele
- Bofufu ba bosiu bo soakang hempe kapa makhasi a hao
- Botlalo ba botšehali, boima, kapa bohloko (ka lebaka la sebete se atolositsoeng, spleen e atolositsoeng)
- Ho hloka lymph nodes leha e le efe ea ho ruruha
- Ho fapana le li-lymphomas tse ngata, ha e le hantle ha e akarelletse lymph nodes leha e le efe e ka senyang, kapa lithōle le maqhubu, ao u ka 'nang ua ikutloa u le tlas'a letlalo molaleng, mokokotlong kapa botala.
Lintho Tse Kotsing
- Botona ba banna bo 'nile ba nkoa e le kotsi e bakoang ke lihlooho tsa pele tse hatisitsoeng.
- Tšebeliso e tsoelang pele ea ho itšireletsa mafung, ebang ke hona joale, kapa lilemong tse fetileng:
- Transplant ea meriana
- Phekolo e sebetsang bakeng sa maloetse a ho ruruha (lefu la Crohn kapa lefu la ho ruruha ha ulcer)
- Histori ea bongaka e fetileng:
- Ho fetisetsa liphio kapa tse ling tse tiileng ho kenya transplant
- Histori ea malaria
- Histori ea mafu a Hodgkin a EBV
Le hoja boitsebiso bo ka holimo bo hlophisitsoe, hoa lokela ho hlokomeloa hore litlhaloso tsa HSTCL li etsa hore ho be le palo e lekanyelitsoeng ea linyeoe.
HSTCL e lumeloa hore e tlaleha karolo e ka tlase ho karolo ea 2 lekholong ea T-cell lymphomas.
Ho sa tsotellehe sesosa sa sona se sa tsejoeng, bakuli ba ka bang 10 ho isa ho 20 lekholong ba anngoeng ke lymphoma bana ba na le histori ea nakong e fetileng ea ho felisoa ha 'mele ea ho itšireletsa mafung, e kang tšoaetso ea setho se tiileng, boloetse ba lymphoproliferative, mafu a bakoang ke ho ruruha, lefuba la lefu la sebete la B, kapa phekolo ea immunosuppressive.
Ho hlahloba boitsebiso bo bobebe
Phuputsong e entsoeng ke Parakkal le basebetsi-'moho, likarolo tse mashome a mabeli a metso e mehlano tsa HSTCL li ile tsa fumanoa har'a bakuli ba sebelisang phekolo ea immunosuppressive. Batho ba mashome a mabeli a metso e 'meli (bakuli ba 88 lekholong) ba ne ba e-na le maloetse a ho ruruha' me ba bararo ba ne ba e-na le lefu la ramatiki. Maemong a mane (karolo ea 16 lekholong) ho basali le bakuli ba bane ba ne ba le ka holimo ho lilemo tse 65. Liketsahalo tse mashome a mabeli a metso e mene (96 lekholong) le tsona li ile tsa fuoa immunomodulator (azathioprine, 6-mercaptopurine, kapa methotrexate). Bakuli ba babeli ba fumane adalimumab feela.
Phuputsong ea Deepak le basebetsi-'moho, litlaleho tse 3 330 267 li ile tsa lefshoa ho tloha tsamaisong ea tlaleho ea liketsahalo tse mpe tsa FDA (2003-2010). Linyeoe tse mashome a robong a metso e 'meli tsa T-cell NHL le TNF-α inhibitors li ile tsa fumanoa FDA AERS le linyeoe tse ling tse robong tse ileng tsa fumanoa ka ho batla lingoliloeng. Bakuli ba 38 ba ne ba e-na le ramatiki ea lefu la masapo, mafu a 36 a ne a e-na le lefu la Crohn, 11 a ne a e-na le psoriasis, a robong a e-na le lefu la ulcerative, 'me a tšeletseng a na le ankylosing spondylitis.
Linyeoe tse mashome a tšeletseng a metso e robeli (karolo ea 68 lekholong) li kenyeletsa ho pepesehela TNF-α inhibitor le immunomodulator (azathioprine, 6-mercaptopurine, methotrexate, leflunomide, kapa cyclosporine). Hepatosplenic T-cell lymphoma (HSTCL) e ne e le karolo e tummeng ka ho fetisisa e tlalehiloeng, athe mycosis fungoides / Sezary syndrome le HSTCL li ile tsa fumanoa e le tse tloaelehileng ka ho hlahisa likokoana-hloko tsa TNF-α-inhibitor.
Tsejoa
Hepatosplenic T-cell lymphoma e ka nka nako e telele hore e hlahlobe, kaha maemo a mangata a tloaelehileng a ka nkoa a le pele. Tlhahlobo ena e itšetlehile ka litlhapi tsa mafu a masapo, sebete le / kapa spleen, le tlhahlobo ea cytometry ea phallo.
Ho hlahlojoa tlhahlobo ea boopsy e entsoeng ke setsebi sa hematopathologist.
Mafura a 'mele oa bone o bontša li-hypercellarlar (sebaka se eketsehileng se nkiloeng ke lisele) ka lebaka la lisele tsa atypical lymphoid, empa liphetoho li' nile tsa hlalosoa e le tse patehileng. Belhadj le basebetsi-'moho le eena ba ile ba hlokomela se latelang tlalehong ea bona ea 2003 ka letoto la bakuli ba 21 ba nang le HSTCL:
Ts'ebetsong ena e poteletseng e sa tsejoe hang-hang ho bakuli ba tšeletseng, e leng ho lebisang tlhokomelong ea meriana ea hypercellular e fokolang ho bakuli ba bahlano le lefu la lefu la myelomonocytic leukemia ho motho e mong le e mong ea nang le lefu le feteletseng la monocytosis qalong.
Leha ho le joalo sehlopha sena sa lipatlisiso se boetse se hlokometse mokhoa o khetholloang oa ho kenella ka mokhoa o tloaelehileng oa ho kenella ka mokhoa oa tloaelo oa mokotla oa masapo: "... ho aroloa ha tlhaho ea lisele tse nang le lisele tse qalang ho hlahlojoa, hangata ho thata ho lemoha ntle le immunohistochemistry."
Liteko tse khethehileng tsa laboratori tse kang cytometry ea phallo le immunophenotyping ea lipapiso tsa biopsy ke lisebelisoa tsa bohlokoa tsa ho hlahlojoa ke HSTCL, empa bafuputsi ba hlokomela bohlokoa ba ho ba le letšoao le phahameng la ho belaela.
Tlhahlobo ea 'mele le liteko tsa laboratori li ka' na tsa boela tsa e-ba tsa maikutlo. Lintho tse fumanoang tlhahlobong ea 'mele, ho kenyeletsoa le spleen e atolositsoeng le sebete li ka ba teng. Palo e feletseng ea mali e ka 'na ea bontša ho se tloaelehe ho kang thrombocytopenia (litekanyo tse tlase tsa platelet), mali a phokolo ea mali (bofubelu bo bobebe ba lisele tsa mali) le leukopenia (tekanyo e tlase ea lisele tsa mali.) Liteko tsa sebete li ka' na tsa e-ba tloaelehileng kapa tsa bontša li-enzyme tse phahameng.
Histori ea Tlhaho le Lipilisi
HSTCL e khetholloa ka ho kenngoa ha lymphocyte tse nang le kankere libakeng tsa matšoafo a sebete, spleen le bone-kaofela ha ho na le li-lymph nodes, kapa lymphadenopathy.
Ho hlaseloa ha lisele tsa lymphoma ho ka lebisa tlhokomelong e kholo ea spleen le sebete. Lintlha tse tlase tsa bohlokoa ha lia tloaeleha, ntle le lipalo tse tlaase tsa platelet, tse ka 'nang tsa e-ba matla.
Ho fihlela ho karolo ea 80 lekholong ea batho ba nang le HSTCL ba na le matšoao a bitsoang B, a kenyeletsang feberu, bosiu bosiu le ho lahleheloa ke boima ba 'mele. Koetliso ea likliniki e na le mabifi haholo, ka kakaretso ho phela nako e ka bang selemo ho tloha nakong ea ho hlahlojoa; Leha ho le joalo, ho na le khoao e kholo mabapi le liphello tse ntle tse ka 'nang tsa e-ba teng le ho fumanoa pejana le phekolo e nepahetseng.
Li-autologous kapa allogeneic transplantation li lokela ho nkoa hammoho le ho ngolisa bakuli litekong tsa bongaka. Le hoja boitsebiso bo tšehetsang mekhoa ena e mabifi bo fokolisitsoe, phello e mpe haholo ka chemotherapy feela.
Phekolo
Ha ts'oaetso ea HSTCL e tiisoa 'me mosebetsi oa ho etsa litlhapi o phethehile, phekolo e lokela ho qalisoa hang-hang ha lefu lena le ka tsoela pele ka potlako. Ha ho na phekolo e tloaelehileng e teng ka lebaka la sekheo sa lefu lena; leha ho le joalo, lik'hemik'hale tsa chemotherapy li 'nile tsa hlahisoa ho latela lithuto tse ling tsa lithuto tse ling tsa lymphomas tse mabifi. Hematopoietic stem cell cell transplant le ho kenya letsoho litekong tsa meriana e ka ba har'a tse ling tseo u ka li khethang.
> Mehloli:
> Belhadj K, Reyes F, Farcet JP, le al. Hepatosplenic gammadelta T-cell lymphoma ke ntho e sa tloaelehang ea lik'hemik'hale tse nang le liphello tse mpe: tlalehela letoto la bakuli ba 21. Mali. 2003; 102 (13): 4261-9.
> Brinkert F, Arrenberg P, Krech T, le al. Maemong a mabeli a hepatosplenic T-cell lymphoma lilemong tsa bocha li tšoaroa ka lefu la sebete sa ho ipolaea. Lingaka . 2016; 138 (3) .pii: e20154245.
> Deepak P, Sifuentes H, Sherid M, et al. Li-lymphomas tsa T-cell tse seng tsa Hodgkin li tlalehile FDA AERS tse nang le tumor necrosis factor-alpha (TNF-α) inhibitors: liphetho tsa thuto ea REFURBISH. Am J Gastroenterol. 2013; 108 (1): 99-105.
> Parakkal D, Sifuentes H, Semer R, et al. Hepatosplenic T-cell lymphoma ho bakuli ba fumanang kankere ea TNF-α inhibitor: ho eketsa lihlopha tse kotsing. Eur J Gastroenterol Hepatol 2011; 23: 1150-6.