T-cell leukemia / lymphoma na manya

**Adult T-cell leukemia** / **lymphoma** (**ATL** ko **ATLL**) wani ciwon daji ne mai ban sha'awa na tsarin rigakafin T-cells wanda ya haifar da kwayar cutar T-cell / lymphotropic virus type 1 (HTLV-1). Dukkanin ƙwayoyin ATL suna dauke da hadin gwiwar HTLV-1 provirus da ke ci gaba da tallafawa wannan rawar da kwayar cutar ke takawa a cikin dalilin neoplasm. Ƙananan mutane na HTLV-1 suna ci gaba don haɓaka ATL tare da dogon lokaci tsakanin kamuwa da cuta da ci gaban ATL. ATL an rarraba shi zuwa nau'o'i huɗu: mai tsanani, mai ƙonewa, nau'in lymphoma, da kuma na dogon lokaci. An san nau'in cututtukan cututtukat da cututtukani musamman suna da tsananin tashin hankali tare da rashin hangen nesa.

A duniya, an kiyasta retrovirus HTLV-1 don kamuwa da mutane miliyan 20 a kowace shekara tare da abin da ya faru na ATL kusan 0.05 a kowace shekara 100,000 tare da yankuna masu kama da yankuna na Japan, har zuwa 27 a kowace shekara. Koyaya, shari'o'in sun karu a yankunan da ba na kowa ba tare da mafi girman HTLV-1 a tsibirin kudancin / arewacin Japan, Caribbean, Tsakiya da Kudancin Amurka, Afirka mai zafi, Romania, arewacin Iran. ATL yawanci yana faruwa a kusa da shekaru 62 amma matsakaicin shekaru a lokacin ganewar asali ya dogara da yaduwar kamuwa da HTLV-1 a cikin wuri.

Tsarin magani na yanzu don ATL ya dogara ne akan nau'in asibiti da martani ga maganin farko. Wasu hanyoyin magani don magani bazai samuwa a duk ƙasashe ba saboda haka dabarun sun bambanta a duk faɗin duniya. Dukkanin marasa lafiya ana tura su zuwa gwaje-gwaje na asibiti idan akwai. Baya ga gwaje-gwaje na asibiti, jiyya suna tsakiya akan maganin sinadarai da yawa, zidovudine tare da interferon-a (AZT / IFN), da allogeneic hematopoietic stem cell transplantation (alloHSCT). 

## Alamomi da alamomi
ATL yawanci mai tsananin tashin hankali ne wanda ba Hodgkin's lymphoma ba tare da bayyanar histologic ba sai dai ga tsarin rarraba da kuma T-cell phenotype. Ana ganin lymphocytes masu zagayawa tare da tsarin nukiliya mara kyau (ƙwayoyin leukemic) akai-akai. Lines na shaidu da yawa sun nuna cewa HTLV-1 yana haifar da ATL. Wannan shaidar ta haɗa da keɓewar HTLV-1 akai-akai daga marasa lafiya tare da wannan cuta da kuma gano HTLV-1-proviral genome a cikin ƙwayoyin cutar sankara na ATL. ATL sau da yawa yana tare da sa hannu na ciki, hypercalcemia, cututtukan fata, da cututtuken ƙashi. Rashin mamayewar ƙashi da osteolysis, siffofi na ƙashi, yawanci suna faruwa a cikin saitin ciwon daji mai ƙarfi, kamar nono, prostate, da ciwon daji na huhu, amma ba su da yawa a cikin cututtukan jini. Koyaya, marasa lafiya tare da HTLV-1-induced ATL da myeloma mai yawa suna da alaƙa da ci gaban osteolysis da hypercalcemia. Ɗaya daga cikin siffofi masu ban sha'awa na ATL da cututtukan ƙashi masu yawa shine cewa raunin ƙashi galibi osteolytic ne tare da ƙananan aikin osteoblastic. A cikin marasa lafiya tare da ATL, matakan serum masu girma na IL-1, TGFβ, PTHrP, furotin na kumburi na macrophage (MIP-1α), da mai karɓar mai kunnawa na nukiliya-κB ligand (RANKL) an haɗa su da hypercalcemia. Mice masu ƙarancin rigakafi waɗanda suka karɓi implants tare da ƙwayoyin leukemic daga marasa lafiya tare da ATL ko HTLV-1-infected lymphocytes sun haɓaka hypercalcemia da matakan serum masu girma na PTHrP. Yawancin marasa lafiya suna mutuwa a cikin shekara guda na ganewar asali.

Cutar da ke dauke da HTLV-1, kamar kamuwa da cuta tare da wasu retroviruses, mai yiwuwa yana faruwa ne don rayuwa kuma ana iya ƙaddara shi lokacin da aka gano maganin rigakafi akan HTLV-1 a cikin serum.

## Rarraba
An yi imanin cewa yaduwar HTLV-1 tana faruwa ne daga uwa zuwa yaro; ta hanyar jima'i; kuma ta hanyar fuskantar jini mai gurbatawa, ko dai ta hanyar ƙarin jini ko raba allurar gurbatawa.

## Binciken ganewa
Binciken ya dogara ne akan siffofin asibiti da canje-canje na yanayin yanayin da immunophenotypic na ƙwayoyin cuta. Kamar yadda siffofin asibiti da hangen nesa na iya zama daban-daban, ana rarraba cutar zuwa nau'o'i huɗu bisa ga rarrabawar Shimoyama: mai tsanani, lymphoma, mai tsanani, da ƙonewa. Kullum, gano akalla kashi 5 cikin 100 na ƙwayoyin ƙwayoyin cuta a cikin jini na gefe da kuma tabbatar da kwayar cutar T-lymphotropic ta mutum nau'in 1 sun isa ga ganewar asali na nau'ikan cututtukan cututtukani masu tsanani, na yau da kullun, da masu hayaki. Ga nau'in lymphoma, ana iya buƙatar gwajin histopathologic ta hanyar biopsy na lymph nodes.

Immunophenotype na ATLL ya haɗa da alamomi masu kyau kamar CD2, CD3, CD4, CD5, da CD25, tare da alamomi marasa kyau don CD7, CD8, da alamomi na cytotoxic. Bugu da ƙari, akwai wani ɓangare na tabbatacce ga CD30, CCR4, da FOXP3. 

## Manazarta