Kawirikawiri lymphoma imapezeka popanda kutulutsa lymph node
Hepatosplenic T-cell lymphoma (HSTCL) ndi lymphoma kwambiri. Odziwika kuti ndi "hepatosplenic γ δ T-cell lymphoma," matendawa samapezeka kawirikawiri m'mabuku a sayansi, choncho chidziwitso chake sichidziwika.
HSTCL kawirikawiri imawoneka kwa anyamata, ngakhale milandu yokhudza amayi ndi ana inalembedwanso. Komanso, zikuwoneka kuti pali kugwirizana kwa chiopsezo chowonjezeka cha HSTCL mwa odwala immunocompromised.
Pogwiritsa ntchito milandu yosindikizidwa, HSTCL ndiyomwe ingayambe kusamvetsetsedwanso poyamba, ndipo ili ndi vuto losauka.
Zizindikiro
- Zomwe zimachitika
- Kutopa
- Zizindikiro za kuchepa kwa magazi (kuchepa kwa magazi, thrombocytopenia)
- Kutaya magazi m'thupi kungabweretse kutopa, kutopa
- Thrombocytopenia ikhoza kuyambitsa kuphwanya kapena kupha magazi
- Zizindikilo za malamulo, kuphatikizapo zotsatirazi:
- Malungo osadziwika
- Kutaya thupi popanda kuyesera kuchepetsa thupi
- Kutuluka kwa usiku komwe kumawombera malaya kapena mapepala ako
- Kukwanira m'mimba, kupweteka, kapena kupweteka (chifukwa cha chiwindi chofutukuka, phala lokulitsa)
- Kulibe ma lymph node iliyonse yotupa
- Mosiyana ndi ma lymphomas ambiri, izi sizimaphatikizapo maselo amtundu uliwonse, kapena mitsempha ndi mitsempha, yomwe mungamve pansi pa khungu pamutu, phokoso, kapena kubuula.
Zowopsa
- Mwamuna wamwamuna wakhala akudziwika kuti ndi chinthu choopsa chifukwa cha zochitika zoyamba zofalitsidwa.
- Kupitiliza kugwiritsidwa ntchito kwachangu, pakalipano, kapena zaka zapitazo:
- Kupatsa mankhwala mankhwala
- Mankhwala othandiza matenda opatsirana a m'mimba (Matenda a Crohn kapena ulcerative colitis)
- Mbiri yakale ya zamankhwala:
- Impso kumuika kapena zina olimba organes kumuika
- Mbiri ya malungo
- Mbiri ya matenda a Hodgkin a EBV
Ngakhale kuti chithunzichi chapangidwa, tifunika kukumbukira kuti kufotokoza kwa HSTCL kumajambula chiwerengero chochepa.
HSTCL imakhulupirira kuti ndi yocheperapo 2 peresenti ya zonse zapakati pa T-cell lymphomas.
Ngakhale kuti palibe chifukwa chosadziwika, pafupifupi oposa 10 mpaka 20 peresenti ya odwala omwe ali ndi matendawa amakhala ndi mbiri yakale ya matenda opatsirana pogwiritsa ntchito chitetezo cha thupi, monga matenda olimbitsa thupi, matenda opatsirana, kutupa matenda a chiwindi, matenda a hepatitis B, kapena chithandizo cha immunosuppressive.
Kufufuza Kugonana kwa Anthu
Mu kafukufuku wa Parakkal ndi anzake, makumi awiri ndi asanu a HSTCL adapezeka pakati pa odwala pogwiritsa ntchito mankhwala opatsirana pogonana. Anthu makumi awiri ndi awiri (88 peresenti ya odwala) anali ndi matenda opweteka kwambiri ndipo atatu anali ndi nyamakazi. Amayi anayi (16 peresenti) anali azimayi ndi odwala anayi ali pamwamba pa zaka 65. Mavoti makumi awiri ndi anai (96 peresenti) adalandira kachilombo ka HIV (azathioprine, 6-mercaptopurine, kapena mettretrexate). Odwala awiri analandira adalimumab okha.
Mu phunziro la Deepak ndi anzake, malipoti okwana 3,130,267 adatulutsidwa kuchokera ku FDA zosokoneza zochitika zochitika zochitika (2003-2010). Matenda makumi asanu ndi anai a T-cell NHL ndi TNF-α inhibitors adadziwika mu FDA AERS ndipo milandu 9 zinazindikiritsidwa pogwiritsa ntchito kufufuza mabuku. Odwala 38 anali ndi nyamakazi yambiri, 36 anali ndi Crohn's disease, 11 anali ndi psoriasis, 9 anali ndi ulcerative colitis, ndipo asanu anali ndi ankylosing spondylitis.
Mavoti makumi asanu ndi atatu mphambu asanu ndi atatu (68 peresenti) adakhudzidwa ndi mawonekedwe a TNF-α inhibitor ndi immunomodulator (azathioprine, 6-mercaptopurine, methotrexate, leflunomide, kapena cyclosporine). Hepatosplenic T-cell lymphoma (HSTCL) inali yofala kwambiri, koma mycosis fungoides / Sezary syndrome ndi HSTCL zinadziwika kuti ndizofala ndi TNF-α-inhibitor exposure.
Kudziwa
Hepatosplenic T-cell lymphoma ingatenge nthawi yaitali kuti ipeze matenda, popeza kuti zinthu zambiri zowonjezereka zingaoneke poyamba. Matendawa amachokera ku zithunzithunzi za mafupa, chiwindi ndi / kapena ntchentche, komanso kusanthula kwa cytometry.
Kukambirana kwa chidziwitso cha akatswiri a hematopathologist akulimbikitsidwa.
Mafupa amtundu wa mafupa amasonyeza kuti magazi ambiri amakhala ndi malo enaake omwe amachotsedwa ndi maselo chifukwa cha maselo a ammicid atypical, koma kusintha kwafotokozedwa ngati kobisika. Belhadj ndi anzake adanena zotsatirazi m'chaka cha 2003 cha odwala 21 omwe ali ndi HSTCL:
Kusagwirizana kotereku sikudziwikidwe mwamsanga mwa odwala asanu ndi limodzi, zomwe zimapangitsa kuti munthu asamaganizire molakwika matenda a hypercellarlar mu odwala asanu komanso odwala matenda a khansa ya myelomonocytic ya m'magazi ena omwe ali ndi matenda oopsa omwe amawayeza.
Komabe gulu lofufuzali linanenanso zosiyana siyana zomwe zimachitika kuti munthu asalowe m'thupi mwachisawawa: "... kugawa kwapadera kwa maselo okhudzana ndi maselo omwe, poyesa kafukufuku, nthawi zambiri amakhala osabisa ndipo nthawi zambiri zimakhala zovuta kuzindikira popanda immunohistochemistry."
Kuyeza kwa ma laboratory monga ma cytometry ndi ma immunophenotyping a zizindikiro za biopsy ndizofunikira zogwiritsira ntchito HSTCL, koma ofufuzira amadziwa kufunika kokhala ndi ndondomeko yodalirika.
Kuyeza thupi ndi mayeso a labotale angakhalenso opusa. Zomwe zimapezeka pa mayeso a thupi, kuphatikizapo nthenda yotambasula ndi chiwindi zingakhalepo. Kuwerengera kwa magazi kwathunthu kungasonyeze zolakwika monga thrombocytopenia (chiwerengero chochepa cha platelet), kuchepa kwa magazi m'thupi (kuchepa kwa maselo ofiira magazi), ndi leukopenia (kuchepa kwa maselo oyera a magazi). Kuyezetsa kwa chiwindi kungakhale koyenera kapena kusonyeza ma enzyme apamwamba.
Mbiri Yachilengedwe ndi Kugonjetsa
HSTCL imadziwika ndi kulowa m'magazi a chiwindi, ntchentche, ndi mafupa onse-kulowa m'magazi a khansa ya kanseri.
Kugonjetsedwa kwa maselo a lymphoma kungayambitse kukulitsa kwakukulu kwa nthenda ndi chiwindi. Ziwerengero zochepa kwambiri sizinali zachilendo, kupatula pa chiwerengero chochepa cha platelet, chomwe chingakhale chowopsa.
Anthu 80 pa 100 alionse omwe ali ndi HSTCL ali ndi zizindikiro za B, zomwe zimaphatikizapo malungo, kutuluka usiku, komanso kutaya kwadzidzidzi. Maphunziro a zachipatala ndi okwiya kwambiri, ndipo amakhala ndi moyo wapakati pa chaka chimodzi kuchokera pa nthawi yofufuza; Komabe, pali kusatsimikizika kwakukulu pokhudzana ndi zotsatira zowonjezera zomwe zingakhale bwino ndi kuyang'ana koyambirira ndi chithandizo choyenera.
Kukonzekera kwachidziwitso kapena kutsekemera kwa magazi kukuyenera kuganiziridwa komanso kupatsidwa odwala ku mayeso a zachipatala. Ngakhale kuti deta yothandizira njira zowonongayi ndi yochepa, zotsatira zake ndizosauka ndi chemotherapy yekha.
Chithandizo
Kamodzi kafukufuku wa HSTCL atatsimikiziridwa ndipo ntchito yothetsera ntchito imatha, mankhwala ayenera kuyambitsidwa mwamsanga pamene matendawa angapite mofulumira kwambiri. Palibe mankhwala omwe alipo chifukwa cha matendawa; Komabe, mankhwala a chemotherapy akhala akuyambitsidwa pogwiritsa ntchito kufufuza kofufuza m'mabwalo ena amphamvu. Hematopoietic tsinde kusuntha maselo ndi kutenga nawo mbali m'mayesero a zachipatala angakhale pakati pa zomwe mungasankhe.
> Zotsatira:
> Belhadj K, Reyes F, Farcet JP, et al. Hepatosplenic gammadelta T-cell lymphoma ndi chinthu chosowa kwambiri cha kachipopathologic ndi zotsatira zovuta: lipoti la odwala 21. Magazi. 2003; 102 (13): 4261-9.
> Brinkert F, Arrenberg P, Krech T, et al. Matenda awiri a hepatosplenic T-cell lymphoma achinyamata amachitira matenda a chiwindi. Matenda . 2016; 138 (3) .pii: e20154245.
> Deepak P, Sifuentes H, Sherid M, et al. Mafupa a T-cell omwe si a Hodgkin amawauza FDA AERS ndi ziwalo za achule necrosis factor-alpha (TNF-α): zotsatira za kufufuza kwa REFURBISH. Am J Gastroenterol. 2013; 108 (1): 99-105.
> Parakkal D, Sifuentes H, Semer R, et al. Hepatosplenic T-cell lymphoma odwala omwe amalandira TNF-α inhibitor therapy: kuwonjezera magulu omwe ali pangozi. Eur J Gastroenterol Hepatol 2011; 23: 1150-6.