Kazhinji lymphoma inowanikwa pasina lymph node kuwedzera
Hepatosplenic T-cell lymphoma (HSTCL) inoratidzika kwazvo lymphoma. Anozivikanwa nekliniki se "hepatosplenic γ δ T-cell lymphoma," chirwere ichi hachiwanzozivikanwi mumabhuku ezvesayenzi, uye naizvozvo chiitiko chaicho chechokwadi hachizivikanwi.
HSTCL yakawanzoonekwa nevarume vaduku, asi nyaya dzinosanganisira vakadzi nevana dzakanyorwa zvakare. Uyewo, zvinoratidzika kunge kune hukama hwehuwandu hwehutano hweHSTCL mumakirini emunocompromised.
Kubva pane zvinyorwa zvakabudiswa, HSTCL inogona kunge isina kuongororwa zvisina kufanira pakutanga, uye inotakura maitiro asina kunaka.
Zviratidzo
- Generalized malaise
- Kuneta
- Zviratidzo zvekudzivirira ropa zvinoreva (anemia, thrombocytopenia)
- Kuora muviri kunogona kubereka kuneta, kuneta
- Thrombocytopenia inogona kukonzera nyore kupwanya kana kubuda ropa
- Zviratidzo zveMitemo-soro, kusanganisira zvinotevera:
- Tsvina isina kutsanangurwa
- Kuora muviri kurasikirwa kunze kwekuedza kuora muviri
- Usiku hukutuka hunokisa shati yako kana mapepa
- Kuzara mumimba, kusimba, kana kurwadziwa (nemhaka yechiropa chakawedzera, spleen yakakura)
- Kusava nechipi chekudzivirira kwema-lymph nodes
- Kusiyana nemarimine akawanda, izvi hazvirevi chero mishonga inoshandiswa, kana maruva uye mavhinga, kuti iwe unganzwa pasi peganda mumutsipa, mazamu, kana kugomera.
Risk Factors
- Chikadzi chevakadzi chakange chave chichionekwa sechinjodzi chinokonzerwa nenyaya yekutanga yakabudiswa nyaya.
- Kuenderera mberi kwekushandisa immunosuppression, kana ikozvino, kana mumakore adarika:
- Organ transplant medication
- Utachiona hunokonzerwa nehutachiona hwezvirwere (Crohn's disease kana ulcerative colitis)
- Nhoroondo yezvechiremba yekare:
- Kuchinja kwepyipi kana chimwe chekudzika kwomuviri wakasimba
- Nhoroondo yemarariya
- Nhoroondo EBV-positive Hodgkin chirwere
Kunyange zvazvo huwandu hwepamusoro hwave huchinyorwa, zvinofanira kuonekwa kuti tsanangudzo yeHSTCL inotora nhamba shomanana yematambudziko.
HSTCL inotendwa kuti inodarika pasi pe2 muzana yezvinhu zvose zvekunze T-cell lymphomas.
Pasinei neicho chikonzero chisingazivikanwi, anenge 10 kusvika ku20 muzana yevarwere vanokonzerwa neiyi lymphoma vane nhoroondo yekare yekusingaperi kwekudzivirira zvirwere, zvakadai sechimiro chekudzivirira muviri, lymphoproliferative disorder, chirwere chinoputika, chirwere chehepatitis B, kana kuti immunosuppressive therapy.
Kuongorora Immunosuppression
Mune chidzidzo chaParakkal nevamwe vaaishanda navo, makumi maviri nemashanu maHSTCL akaonekwa pakati pevarwere vachishandisa chirwere chekuita immunosuppressive therapy. Twenty-two (88 muzana yevarwere) vaiva nechirwere chechirwere chechirwere uye vatatu vaiva nerheatatoid arthritis. Zviitiko zvina (16 muzana) zvaive mumadzimai nevane varwere vana vaiva pamusoro pemakore makumi matanhatu nemashanu. Makumi makumi mana nemana (96 muzana) akawanawo immunomodulator (azathioprine, 6-mercaptopurine, kana methotrexate). Varwere vaviri vakagamuchira adalimumab chete.
Mukudzidza na Deepak nevamwe vaaishanda navo, nhamba yehupfumi ye3 033 267 yakadzingwa kubva kuDWD inoshuvira chiitiko chekuita chiitiko (2003-2010). Zviitiko makumi mapfumbamwe-chimwe zveT-cell NHL neTN-α inhibitors zvakasarudzwa muDFA AERS uye mimwe mapfumbamwe mamwe maitiro akawanikwa achishandisa mabhuku ekutsvaga. Avhareji 38 varwere vaiva nerheumatoid arthritis, 36 mawere aiva neCrohn's disease, 11 aiva psoriasis, mapfumbamwe aiva nechirwere chetachi, uye matanhatu aiva ankylosing spondylitis.
Makumi makumi matanhatu nemasere ematambudziko (68 muzana) aisanganisira kuonekwa kune zvose TNF-α inhibitor uye immunomodulator (azathioprine, 6-mercaptopurine, methotrexate, leflunomide, kana cyclosporine). Hepatosplenic T-cell lymphoma (HSTCL) ndiyo yainyanya kuzivikanwa pasi, asi mycosis fungoides / Sezary syndrome neHSTCL zvakaratidzwa seinojairika ne TNF-α-inhibitor exposure.
Kuziva
Hepatosplenic T-cell lymphoma inogona kutora nguva yakareba kuti iongorore, sezvo mimwe mimwe yakawanda inowanikwa inogona kuonekwa seyokutanga. Kuongororwa kwakavakirwa pamasikirwo emapfupa emapfupa, chiropa uye / kana spleen, nekuyerera kwechitometry analysis.
Kudzokorora kwezvinyorwa zvebiopsy nenyanzvi hematopathologist inokurudzirwa.
Bhoora marrow biopsies zvinowanzoratidzira hypercellular (imwe nzvimbo inotorwa nemasero) marora nemhaka yemasero eyypical lymphoid, asi kuchinja kwave kunorondedzerwa sekunze. Belhadj nevamwe vaaishanda vakaona zvinotevera mumushumo wavo we2003 pamusoro pezvikamu makumi maviri nevatatu vaine HSTCL:
Uku kubata zvisiri pachena kwakange kusati kwaonekwa mumakirini matanhatu, zvichiita kuti tisaona zviratidzo zvehutachiona hwehupenzi huri muhutano hwevashanu uye hwechirwere chechirwere chinonzi myelomonocytic leukemia mune mumwe murwere ane chirwere chinonzi monocytosis pakuongorora kwekutanga.
Kunyange zvakadaro boka iri rekutsvakurudza rakaratidzawo kusiyanisa mararamiro ekutadza kwemaitiro echirwere chepasina: "... kushandiswa kwetachiona kwemasero emuviri, izvo pakutanga, kuongororwa, kunowanzosvibiswa uye saka zvakaoma kuziva pasina immunohistochemistry."
Mamwe ma laboratory maitiro akadai sekuyerera kwe cytometry uye immunophenotyping yezviopsy specimens ndiyo inoshandiswa yekuongorora hutachiona hweHSTCL, asi vatsvakurudzi vanocherechedza kukosha kwekuve nehuwandu hwekodhiki yekufungidzirwa.
Kuongorora muviri uye laboratory tests zvinogonawo kuva mazano. Zviwanikwa pakuongorora kwemuviri, kusanganisira chirwere chakawedzerwa uye chiropa chinogona kuvapo. Nhamba yakakwana yeropa inogona kuratidza kusakanganiswa kwakadai seprombositopenia (low platelet counts), anemia (shoma tsvuku tsvuku yeropa), uye leukopenia (pasi tsvuku yakachena yeropa inoyera.) Kuongorora kwechiropa kunogona kunge kwakakosha kana kuratidza ma enzymes akareba.
Nhoroondo Yemazuva Ano uye Kurapa
HSTCL inoratidzirwa nekupinda mukati mechirwere ye lymphocyte muzvivande zvechiropa zvechiropa, spleen, uye marora-zvose pasina kuwedzera kwema-lymph nodes, kana lymphadenopathy.
Kupinda kwema-lymphoma masero kunogona kutungamirira pakuwedzera kukuru kwepleen nechiropa. Zvikamu zvakaderera zvakaderera hazviwanzoviki, kunze kweplatelet yakaverengwa, iyo inogona kuva yakaoma.
Kusvikira kuzana makumi mapfumbamwe nezana yevanhu vane HSTCL vane zvinonzi B zviratidzo, zvinosanganisira fever, usiku sweats, nekurasikirwa kwekushaya kwekuzvipira. Kliniki yekodhi yakanyanya kurwisana, nehupenyu hwehupenyu hwegore rimwe kubva panguva yekuongororwa; zvisinei, pane kusava nechokwadi kwakawanda pamusoro pemigumisiro iri nani yekutarisisa nekuonekwa kwokutanga nekurapa kwakakodzera.
Autologous kana allogeneic transplantation inofanira kufungidzirwa pamwe chete nekushandira varwere kuchipatara. Kunyange zvazvo dhigiriro inotsigira maitiro aya ane utsinye isingagumi, mhedzisiro inoshaya nechemotherapy chete.
Kurapa
Kamwe kuongororwa kweHSTCL kunosimbiswa uye kushandiswa kwemashoko kunopera, kurapwa kunofanira kutanga pakarepo sezvo chirwere chinogona kufambira mberi nokukurumidza. Hapana chirwere chinowanikwa chiripo nekuda kwekushaya kwechirwere ichi; zvisinei, chemotherapy regimens yakatangwa zvichienderana nekuwedzerwa kwezvidzidzo mune mamwe masimba ane lymphomas. Hematopoietic stem cell kushandiswa nekubatanidzwa mumatambudziko emakiriniki inogona kunge iri pakati pezvisarudzo zvinofungwa.
> Sources:
> Belhadj K, Reyes F, Farcet JP, et al. Hepatosplenic gammadelta T-cell lymphoma inoratidzika isirikiki inopathologic iyo ine nhubu isina kunaka: ichitaura pamusoro pezvikamu makumi maviri nevatano. Ropa. 2003; 102 (13): 4261-9.
> Brinkert F, Arrenberg P, Krech T, et al. Zviitiko zviviri zve hepatosplenic T-cell lymphoma muvechiri kuyaruka vanorapwa kuti vaite autoimmune hepatitis. Pediatrics . 2016; 138 (3) .pii: e20154245.
> Deepak P, Sifuentes H, Sherid M, et al. T-cell isiri yeHodgkin's lymphomas yakashuma kune FDA AERS ine tumor necrosis factor-alpha (TNF-α) inhibitors: mhinduro dzeReFURBISH yekudzidza. Am J Gastroenterol. 2013; 108 (1): 99-105.
> Parakkal D, Sifuentes H, Semer R, et al. Hepatosplenic T-cell lymphoma kune varwere vanowana TNF-α inhibitor therapy: kuwedzera mapoka ari pangozi. Eur J Gastroenterol Hepatol 2011; 23: 1150-6.