T-Cell Tsira Lymphoma: Abin da ya sani

Lymphoma ƙanƙan daji yakan yi ba tare da lymph node ba

Tashin kwayoyin halitta T-cell lymphoma (HSTCL) yana da ƙananan lymphoma. An san shi a matsayin asibiti na "lymphoma lymphoma" na T-cell, "wannan rashin lafiya ne kawai ya ruwaito a cikin wallafe-wallafen kimiyya, saboda haka ba a sani ba.

HSTCL an gani sau da yawa a cikin samari, kodayake an rubuta takardu game da mata da yara. Har ila yau, akwai alamar haɗi zuwa ƙarin haɗarin HSTCL a marasa lafiya marasa rigakafi.

Bisa ga sharuɗan da aka wallafa, HSTCL ana iya ɓatar da shi a farkon, kuma yana ɗauke da ƙwarewar maras kyau.

Cutar cututtuka

• Tsarin sararin samaniya
• Wulo
• Kwayoyin cututtuka na ƙananan jini suna ƙidayar (anemia, thrombocytopenia)
  • Abun iya haifar da gajiya, gajiya
  • Thrombocytopenia zai iya haifar da zubar da jini ko zub da jini
• Tsarin tsarin mulki, wanda ya hada da:
  • Labarai maras kyau
  • Asarar nauyi ba tare da ƙoƙarin rasa nauyi ba
  • Yau da dare ka ji daɗin rigarka ko zanen gado
• Abun ciki cikakke, damuwa, ko ciwo (saboda karaɗa hanta, kara girma)
• Rashin kowane magungunan lymph mai ƙoshi
  • Ya bambanta da ƙwayoyin lympho da yawa, wannan ba shi da wani nau'in ƙwayar lymph wanda ake ganowa, ko lumps da bumps, don ku ji a karkashin fata a cikin wuyansa, ƙuƙwalwa, ko tsutsa.

Dalili na Hadarin

• Mace namiji an yi la'akari da shi a matsayin al'ada mai hadari dangane da jerin sassan da aka buga.
• Ci gaba da yin amfani da immunosuppression, ko dai a halin yanzu, ko a cikin shekaru da suka gabata:
  • Organ safar dashi
  • Tsarin tsarin jiki na cututtukan zuciya (ƙwayar Crohn ko ulcerative colitis)

• Tarihin kiwon lafiya na baya:
  • Koda dashi ko wasu kwayoyin tsabta
  • Tarihin malaria
  • Tarihin EBV-tabbatacciyar cutar Hodgkin

Kodayake an tattara labarun da aka ambata a sama, ya kamata a lura da cewa bayanin HSTCL ya zana akan wasu ƙananan ƙidodi.

HSTCL an yi la'akari da asusun na kasa da kashi 2 cikin 100 na dukkanin ƙwayar T-cell lymphomas.

Duk da rashin saninta, kimanin kashi 10 zuwa 20 cikin dari na marasa lafiya da ke fama da wannan lymphoma suna da tarihin da suka shafi tarihin maganin rikice-rikice na zamani, irin su suturar kwayoyin halitta, cututtuka na lymphoproliferative, cututtukan ƙwayar cutar ciwon jini, ciwon haɗarin B ko kuma ciwon immunosuppressive.

Yin nazarin Immunosupression

A cikin binciken da Parakkal da abokan aiki suka yi, an gano magungunan HSTCL ashirin da biyar a cikin marasa lafiya ta hanyar amfani da maganin immunosuppressive. Kashi ashirin da biyu (88 bisa dari na marasa lafiya) suna da ciwo mai jijerun jini kuma uku suna da maganin jini. Abubuwa hudu (kashi 16 cikin dari) sun kasance a cikin mata da marasa lafiya hudu a sama da shekaru 65. Shaidu ashirin da hudu (kashi 96) sun sami magungunan immunomodulator (azathioprine, 6-mercaptopurine, ko methotrexate). Biyu marasa lafiya sun karbi adalimumab kadai.

A cikin binciken da Deepak da abokan aiki suka yi, an ƙaddamar da rahotanni 3,130,267 daga tsarin FDA ta hanyar ba da rahoto (2003-2010). Sakamakon sharuɗɗan TH-cell NHL tare da masu TNF-α sun gano a cikin FDA AERS da kuma tara ƙarin lokuta da aka gano ta amfani da binciken wallafe-wallafen. Dukkan marasa lafiya 38 da suka kamu da cutar wariyar launin fata, mutane 36 sun kamu da cutar Crohn, 11 suna da psoriasis, tara sun sami ciwon zuciya, kuma shida suna da spondylitis.

Kashi sittin da takwas daga cikin shari'un (68 bisa dari) na dauke da kamuwa da magunguna TNF-α da immunomodulator (azathioprine, 6-mercaptopurine, methotrexate, leflunomide, ko cyclosporine). Tashin kwayar halitta T-cell lymphoma (HSTCL) shi ne mafi yawan rahotanni da aka fi sani da shi, yayin da yawancin mycosis / Sezary ciwo da kuma HSTCL sun kasance sun fi dacewa da tasirin TNF-α-inhibitor.

Sanin asali

Kwayar lymphoma T-kwayar cutar tazarar na iya ɗaukar lokaci mai tsawo don gano asali, tun da za'a iya la'akari da yawancin yanayi na farko. Sakamakon ganewar asali ya dogara ne akan samfurori na biopsy na kasusuwa na hakar, hanta da kuma / ko yaduwa, da kuma nazarin shafukan yanar gizo.

Review of biopsy material by gwani hematopathologist bada shawarar.

Kwayoyin da aka yi wa maniyyi suna nuna hypercellular (karin sararin samaniya) wanda ya karu saboda kwayoyin lymphoid kwayar halitta, amma an canza canje-canje a matsayin dabara. Belhadj da abokan aiki sunyi la'akari da wannan a cikin rahoto na 2003 game da jerin marasa lafiya 21 da HSTCL:

Ba a gane wannan kullun a cikin likitoci shida ba, wanda ya haifar da rashin fahimta da ɓacin jini a cikin marasa lafiya biyar da na cutar cutar sankarar jini a cikin wani mai haƙuri tare da mamaye monocytosis a farkon binciken.

Duk da haka wannan rukunin bincike ya lura da bambancin dabi'ar kirkirar haɓaka akan lalata kwayar halitta mai laushi: "... wani rarrabaccen nau'in kwayar cutar da kwayoyin halitta, wanda shine a farkon bincike, sau da yawa sau da yawa kuma yana da wuyar ganewa ba tare da jahilci ba."

Ƙwararrun gwaje gwaje-gwajen gwaje-gwaje irin su fasalin kayan shawo kan kwayoyin halitta da immunophenotyping na samfurori na biopsy sune kayan aiki masu muhimmanci don ganewar asali na HSTCL, amma masu bincike sun lura da muhimmancin samun lakabi mai mahimmanci na zato.

Binciken jiki da gwaje-gwaje na gwaje-gwaje na iya zama mai ban sha'awa. Sakamakon bincike na jiki, ciki har da ƙwararren girma da hanta zai iya kasancewa. Ƙididdigar jini cikakke zai iya nuna abubuwan da ba daidai ba kamar thrombocytopenia (lowlet count count), anemia (ƙananan jinin jini), kuma leukopenia (ƙananan jinin jini ƙidaya.

Tarihin Tarihi da Fahimci

HSTCL tana nuna haɓakar ƙananan lymphocytes masu ciwon ciki a cikin wuraren cavernous na hanta, yalwa, da kuma kasusuwan kasusuwa-duk ba tare da fadada ƙwayoyin lymph, ko lymphadenopathy ba.

Rashin mamaye daga cikin kwayoyin lymphoma zai iya haifar da fadada girma akan ƙwanƙiri da hanta. Ƙididdigar ƙananan ƙananan ba su da yawa, ba tare da la'akari da ƙananan platelet ba, wanda zai iya zama mai tsanani.

Har zuwa kashi 80 na mutanen da ke tare da HSTCL suna da alamun da ake kira B, wanda ya hada da zazzabi, shawaran dare, da asarar rashin nauyi. Kwayar da ke cikin asibiti tana da mummunar matsananciyar muni, tare da rayuwa tsakanin mutum kimanin shekara daya daga lokacin ganewar asali; Duk da haka, akwai rashin tabbas game da sakamakon da ya dace mafi kyau da ganowa da baya da kuma dacewa.

Dole ne a yi la'akari da gyare-gyare na jiki ko gyaran allogeneic kazalika da daukar ma'aikata zuwa gwaji. Kodayake bayanan da za a goyi bayan waɗannan ƙaddamar dabarun suna da iyakancewa, sakamakon ba shi da talauci tare da ilimin chemotherapy kadai.

Jiyya

Da zarar an tabbatar da ganewar asalin HSTCL kuma aikin gyaran ƙwaƙwalwa ya cika, farfesa ya kamata a fara da sauri kamar yadda cutar zata iya ci gaba sosai. Babu wata farfadowa ta hanyar wanzuwar cutar saboda wannan cuta; duk da haka, an riga an gabatar da samfurori na chemotherapy dangane da extrapolation na karatu a wasu ƙananan lymphomas. Hanyoyin kwayoyin Hematopoietic da kuma shiga cikin gwaji na asibiti na iya kasancewa cikin zabin da aka zaba.

> Sources:

> Belhadj K, Reyes F, Farcet JP, et al. Ciwon lymphoma T-cell gammadelta na T-cellular T-cell ne mai mahimmancin kwakwalwa marasa lafiya. Jinin jini. 2003; 102 (13): 4261-9.

> Brinkert F, Arrenberg P, Krech T, et al. Hoto biyu na kwayar T-cell lymphoma a cikin matasa masu kula da cutar hepatitis. Pediatrics . 2016; 138 (3) .pii: e20154245.

> Deepak P, Sifuentes H, Sherid M, et al. Tudun kwayoyin T-cell da ba Hodgkin din sun ruwaito FDA AERS tare da masu ƙwayar kwayar necrosis factor-alpha (TNF-α): sakamakon binciken na REFURBISH. Am J Gastroenterol. 2013; 108 (1): 99-105.

> Parakkal D, Sifuentes H, Semer R, et al. Tashin kwayar halitta T-cell da ke cikin marasa lafiya da ke karbar TNF-α inhibitor far: fadada kungiyoyin a hadarin. Eur J Gastroenterol Shipatol 2011; 23: 1150-6.