I-lymphoma ejwayelekile ikhona ngaphandle kokukhulisa i-lymph node
I-Hepatosplenic T-cell lymphoma (i-HSTCL) iyi-lymphoma engavamile kakhulu. Eyaziwa ngokomtholampilo ngokuthi "i-hepatosplenic γ δ T-cell lymphoma," lesi sifo asikabikwa nje kuphela ezincwadini zesayensi, ngakho-ke izimo zayo zangempela aziwa.
I-HSTCL ibilokhu ibonakala emadodeni amancane, nakuba amacala abandakanya abesifazane nezingane nawo aqoshiwe. Futhi, kubonakala sengathi kunesixhumanisi sengozini eyengeziwe ye-HSTCL kuma-immunocompromised patients.
Ngokusekelwe ezimweni ezishicilelwe, i-HSTCL kungenzeka ukuthi ingalazi kahle ekuqaleni, futhi ibhekene nokuhlushwa okubi kakhulu.
Izimpawu
- I-malaise ejwayelekile
- Ukukhathala
- Izimpawu zokubala kwegazi eliphansi (i-anemia, thrombocytopenia)
- I-anemia ingaveza ukhathala, ukhathala
- I-Thrombocytopenia ingabangela ukulimala noma ukuphuma okulula
- Izimpawu zomthethosisekelo, kufaka phakathi okulandelayo:
- Amafiva angachaziwe
- Ukulahlekelwa kwesisindo ngaphandle kokuzama ukunciphisa umzimba
- Izithukuthuku zobusuku eziphoqa ihembe lakho noma amashidi
- Ukugcwala kwamathumbu, ukuqina kwesisu, noma ubuhlungu (ngenxa yesibindi esandisiwe, i-pleen ekhulisiwe)
- Ukungabi nanoma iyiphi i-lymph nodes etholakalayo
- Ngokungafani ne-lymphomas eminingi, lokhu ngokuvamile akubandakanyi nanoma iyiphi i-lymph nodes etholakalayo, noma izimpumputhe nezinsipho, ukuthi ungase uzizwe ngaphansi kwesikhumba entanyeni, emagumbini, noma ekuguleni.
Izingozi Zezingozi
- Ubulili besilisa bubhekwa njengengozi engcupheni esekelwe uchungechunge lwecala lokuqala olushicilelwe.
- Ukusetshenziswa okuqhubekayo kokugonywa kwe-immunosuppression, noma okwamanje, noma eminyakeni edlule:
- Imithi yokufakelwa komzimba
- Ukwelapha okuyisimiso sesifo sofuba esiswini (Isifo sikaCrohn noma isifo sofuba)
- Umlando wezokwelapha odlule:
- Ukufakelwa kwezinso noma okunye ukufakelwa komzimba okuqinile
- Umlando we-malaria
- Umlando we-EBV-positive Hodgkin isifo
Nakuba le phrofayili engenhla ihlanganisiwe, kufanele kuqashelwe ukuthi izincazelo zeHSTCL zidweba inani elincane kakhulu lamacala.
I-HSTCL ikholelwa ukuthi i-akhawunti engaphansi kwamaphesenti angaphansi kwangu-2 yawo wonke ama-T-cell lymphomas wendawo.
Naphezu kwesizathu esingaziwa, cishe amaphesenti angu-10 kuya ku-20 weziguli ezithintekayo ngaleli lymphoma inomlando wangaphambilini wokucindezelwa okugciwane omzimba okungapheli, njengokuguqulwa komzimba okuqinile, isifo se-lymphoproliferative, isifo sofuba esibulalayo, ukutheleleka kwesifo sofuba, noma ukwelashwa kwe-immunosuppressive.
Ukuhlola Ukuguquka Kwegazi
Esicwaningweni sikaParakkal nozakwethu, amacala angamashumi amabili nanhlanu e-HSTCL ahlonishwa phakathi kweziguli ezisebenzisa ukwelashwa okungenakuzivikela. Iziguli ezingamashumi amabili nambili (iziguli ezingu-88) zaziphethe izifo zesifo sofuba kanti ezintathu zine-arthritis ye-rheumatoid. Amacala amane (amaphesenti angu-16) ayekhona kwabesifazane kanye neziguli ezine zazingaphezu kweminyaka engu-65 ubudala. Amacala angu-24 (amaphesenti angu-96) nawo athola i-immunomodulator (azathioprine, 6-mercaptopurine, noma methotrexate). Iziguli ezimbili zithola i-adalimumab yedwa.
Esifundweni sika-Deepak nozakwabo, imibiko engu-3,130,267 yalayishwa kusukela ohlelweni lwezokubika lwesigameko se-FDA (2003-2010). Amacala angamashumi ayisishiyagalolunye nanye we-T-cell NHL ne-TNF-α inhibitors atholakala ku-FDA AERS kanye namacala angeziwe ayisishiyagalolunye ahlonishwa ngokusebenzisa ukufundwa kwezincwadi. Iziguli ezingu-38 zine-arthritis ye-rheumatoid, amacala angu-36 anesifo sikaCrohn, 11 anesifo se-psoriasis, esinesishiyagalolunye sasine-ulcerative colitis, kanti ayisithupha yayine-ankylosing spondylitis.
Amacala angamashumi ayisishiyagalombili nesishiyagalombili (amaphesenti angu-68) ahilela ukutholakala kokubili kwe-TNF-α inhibitor kanye ne-immunomodulator (azathioprin, 6-mercaptopurine, methotrexate, leflunomide, noma cyclosporine). I-Hepatosplenic T-cell lymphoma (i-HSTCL) yayiyi-subtype ebikiwe kakhulu, kanti i-mycosis fungoides / i-Sezary syndrome ne-HSTCL yabonakala njengento evame kakhulu ne-TNF-α-inhibitor exposure.
Ukuxilongwa
I-Hepatosplenic T-cell lymphoma ingathatha isikhathi eside ukuxilonga, ngoba izimo eziningi ezivamile zingase zibhekwe kuqala. Ukuxilongwa kusekelwe ezinhlobonhlobo ze-biopsy yamathambo, isibindi kanye / noma uhlangothi, nokuhlaziywa kwe-cytometry.
Ukubuyekezwa kwempahla ye-biopsy nge-hematopathologist yezobuchwepheshe kunconywa.
I-bone marrow biopsies ngokuvamile ibonisa ukuthi i-hypercellular (isikhala esengeziwe esithathwe ngamangqamuzana) umnkantsha ngenxa yamaseli e-lymphoid atypical, kodwa izinguquko zichazwe njengezicashile. UBelhadj kanye nosebenza nabo babike lokhu okulandelayo embikweni wabo ka-2003 ochungechungeni lweziguli ezingu-21 ezine-HSTCL:
Ukubandakanyeka okucashile akuzange kubonakale masinyane ngeziguli eziyisithupha, okuholela ekungenizeni kahle umongo omningi osebenzayo ezigulini ezinhlanu kanye nengculaza engapheliyo i-myelomonocytic leukemia kwesinye isiguli esine-overt monocytosis ekuqaleni kokuhlolwa.
Noma kunjalo leli qembu lokucwaninga liphinde laphawula indlela ehlukanisa ngayo isifo se-sinusal yokufaka ukungena emzimbeni wesibhakabhaka se-bone: "... ukusabalalisa okuyisipesheli kwamangqamuzana e-tumor ukuthi, ekuqaleni kokuhlolwa, kuvame ukucabangela ngakho-ke kunzima ukubona ngaphandle kwe-immunohistochemistry."
Izivivinyo ezikhethekile ze-laboratory ezifana ne-cytometry yokugeleza kanye nokuzivikela kwe-immunophenotyping ye-biopsy specimens yizinto ezidingekayo zokuxilongwa kwe-HSTCL, kodwa abaphenyi bayiqaphela ukubaluleka kokuba nenkomba ephakeme yokusola komtholampilo.
Ukuhlola ngokomzimba kanye nokuhlolwa kwelabhoratri kungase kube nesiphakamiso. Ukutholakala kokuhlolwa ngokomzimba, kuhlanganise nesipuli esikhulisiwe nesibindi kungenzeka. Ukubala okuphelele kwegazi kungabonisa okungavamile njenge-thrombocytopenia (ukubalwa okuphansi kweplatelet), i-anemia (isibalo esiphansi se-cell cell counts), ne-leukopenia (ukubala okuphansi kwe-white cell. Ukuhlolwa kwesibindi kungase kube okuvamile noma kubonise ama-enzyme aphakeme.
Umlando Wezemvelo Nokubikezela
I-HSTCL ibonakaliswa ukungena kwe-lymphocyte engumdlavuza ezindaweni ezikhungathekile zesibindi, i-spleen, nomnyobo-konke ngaphandle kokukhuliswa kwe-lymph nodes, noma i-lymphadenopathy.
Ukuhlasela kwamangqamuzana e-lymphoma kungaholela ekwandiseni okuphawulekayo kwesipere nesibindi. Izibalo eziphansi kakhulu azivamile, ngaphandle kokubala okuphansi kweplatelet, okungaba nzima.
Kuze kube ngamaphesenti angu-80 abantu abane-HSTCL banezinkomba okuthiwa yi-B, ezibandakanya umkhuhlane, ukujula ebusuku, nokulahleka kwesisindo esingenasisekelo. Inkambo yezokwelapha ihlukumezeka kakhulu, ngokusinda okuphakathi komnyaka owodwa kusukela ngesikhathi sokuxilongwa; Kodwa-ke, kunokungaqiniseki okuningi mayelana nemiphumela emihle engaba nayo kanye nokutholakala kwangaphambili kanye nokwelashwa okufanele.
Ukufakelwa kwe-Autologous noma i-allogeneic kufanele kubhekwe njengendlela yokuqashelwa kweziguli ezivivinyweni. Nakuba idatha yokusekela lezi zindlela ezinonya zilinganiselwe, umphumela awubi ngamakhemikhali kuphela.
Ukwelapha
Uma ukuxilongwa kwe-HSTCL kuqinisekisiwe futhi umsebenzi wokusebenza wesigceme uqedile, ukwelashwa kufanele kuqaliswe ngokushesha njengoba lesi sifo singathuthuka ngokushesha. Awekho ukwelashwa okujwayelekile okukhona ngenxa yokuphuluka kwalesi sifo; Kodwa-ke, amakhemikhali e-chemotherapy asetshenzisiwe ngokususelwa ekuqalisweni kwezifundo kwamanye ama-lymphomas anonya. Ukufakelwa kwe-Hematopoietic stem cell kanye nokubamba iqhaza ekuvivinyweni komtholampilo kungase kube phakathi kwezinketho ezicatshangwayo.
> Imithombo:
> Belhadj K, Reyes F, Farcet JP, et al. I-hepatosplenic gammadelta i-T-cell lymphoma iyinhlangano engavamile ye-clinicopathologic nemiphumela embi: kubika uchungechunge lweziguli ezingu-21. Igazi. 2003; 102 (13): 4261-9.
> Brinkert F, Arrenberg P, Krech T, et al. Amacala amabili we-hepatosplenic T-cell lymphoma ezinsheni ezitholwa ukubhebhetheka kwesifo sofuba. Izingane zokwelapha . 2016; 138 (3) .pii: e20154245.
> Deepak P, Sifuentes H, uSherid M, et al. Ama-lymphomas e-T-cell engekho e-Hodgkin abike ku-FDA AERS ene-tumor necrosis factor-alpha (TNF-α) inhibitors: imiphumela yesifundo se-REFURBISH. Am J Gastroenterol. 2013; 108 (1): 99-105.
> Parakkal D, Sifuentes H, uSemer R, et al. I-Hepatosplenic T-cell lymphoma kuziguli ezithola ukwelashwa kwe-TNF-α inhibitor: ukwandisa amaqembu engozini. Eur J Gastroenterol Hepatol 2011; 23: 1150-6.