Sézary Syndrome: Kwayar cututtuka da Tsammani na rayuwa

Wadatacce

• Menene alamun da alamun?
• Hoton erythroderma
• Wanene ke cikin haɗari?
• Me ke kawo shi?
• Yaya ake gane shi?
• Yaya ake gudanar da ciwo na Sézary?
• Yaya ake magance ta?
• Psoralen da UVA (PUVA)
• Extracorporeal photochemotherapy / hotunan hoto (ECP)
• Radiation far
• Chemotherapy
• Immunotherapy (ilimin ilimin halittu)
• Gwajin gwaji
• Outlook

Menene Ciwon Sézary?

Ciwon Sézary wani nau'i ne na cututtukan T-cell lymphoma. Kwayoyin Sézary wani nau'in farin jini ne. A wannan yanayin, ana iya samun kwayar cutar kansa a cikin jini, fata, da ƙugiyoyin lymph. Ciwon kansa kuma na iya yaduwa zuwa wasu gabobin.

Ciwon Sézary ba shi da yawa, amma ya kai kashi 3 zuwa 5 na cututtukan cututtukan T-cell. Hakanan zaka iya jin shi ana kiransa Sézary erythroderma ko lymphoma na Sézary.

Menene alamun da alamun?

Babban alamar cutar Sézary shine erythroderma, ja, mai kumburi wanda zai iya rufe kusan kashi 80 cikin dari na jiki. Sauran alamu da alamomi sun haɗa da:

• kumburin fata
• alamun fata da marurai
• kara narkarda lymph
• kaurin fata a tafin hannu da tafin kafa
• rashin daidaiton farce da ƙusoshin hannu
• ƙananan fatar ido wanda ke juya waje
• asarar gashi
• matsalar daidaita yanayin zafin jiki

Ciwon Sézary kuma na iya haifar da faɗaɗa ƙwaro ko matsaloli tare da huhu, hanta, da hanjin ciki. Samun wannan nau'in cutar kansa yana ƙara haɗarin kamuwa da wasu cututtukan kansa.

Hoton erythroderma

Wanene ke cikin haɗari?

Kowa na iya kamuwa da cutar Sézary, amma mai yiwuwa ya shafi mutane sama da shekaru 60.

Me ke kawo shi?

Dalilin da ya sa ba a bayyana ba. Amma yawancin mutanen da ke fama da cutar Sézary suna da cututtukan chromosomal a cikin DNA na ƙwayoyin kansa, amma ba a cikin ƙwayoyin lafiya ba. Waɗannan ba lahani ba ne na gado, amma canje-canje da ke faruwa a tsawon rayuwarsu.

Abubuwan da suka fi dacewa sune asarar DNA daga chromosomes 10 da 17 ko kuma ƙarin DNA zuwa chromosomes 8 da 17. Duk da haka, ba a tabbata cewa waɗannan abubuwan rashin lafiyar suna haifar da ciwon daji ba.

Yaya ake gane shi?

Binciken jiki na fata zai iya faɗakar da likita game da yiwuwar cutar Sézary. Gwajin bincike na iya haɗawa da gwajin jini don gano alamomi (antigens) a saman ɗakunan cikin jini.

Kamar yadda yake tare da sauran cututtukan daji, biopsy shine hanya mafi kyau don isa ga ganewar asali. Don nazarin halittu, likita zai dauki karamin samfurin fatar jiki. Kwararren likitan kwalliya zai bincika samfurin a ƙarƙashin microscope don neman ƙwayoyin kansa.

Hakanan ƙwayoyin lymph da kasusuwan ƙashi suma za a iya yin biopsied. Gwajin hoto, kamar su CT, MRI, ko PET scans, na iya taimakawa wajen tantance ko kansar ta bazu zuwa ƙwayoyin lymph ko wasu gabobin.

Yaya ake gudanar da ciwo na Sézary?

Yin kallo yana faɗi yadda nisan kansa ya bazu kuma menene mafi kyawun zaɓuɓɓukan magani.Ciwon Sézary an tsara shi kamar haka:

• 1A: Kasa da kashi 10 cikin 100 na fatar an rufe ta da jan faci ko alamu.
• 1B: Fiye da kashi 10 cikin 100 na fatar ja ne.
• 2A: Duk adadin fata yana da hannu. Lymph node sun kara girma, amma ba na daji ba.
• 2B: Oraya ko ƙari ƙari fiye da santimita 1 sun samu akan fata. Lymph node sun kara girma, amma ba na daji ba.
• 3A: Mafi yawan fatar ja ne kuma yana iya samun ciwace-ciwace, alamu, ko faci. Lymph node na al'ada ne ko na faɗaɗa, amma ba na kansa ba. Jinin na iya ko ba zai ƙunshi wasu ƙwayoyin Sézary ba.
• 3B: Akwai raunuka akan mafi yawan fatar. Lymph node na iya fadada ko ba za'a fadada ba. Adadin ƙwayoyin Sézary a cikin jini ba su da yawa.
• 4A (1): Raunin fata yana rufe kowane ɓangare na fuskar fata. Lymph node na iya fadada ko ba za'a fadada ba. Adadin ƙwayoyin Sézary a cikin jini yana da yawa.
• 4A (2): Raunin fata yana rufe kowane ɓangare na fuskar fata. Akwai kumburin kumburi na lymph kuma ƙwayoyin suna kama da mahaukaci sosai a ƙarƙashin gwajin microscopic. Kwayoyin Sézary na iya zama ko a'a cikin jini.
• 4B: Raunin fata yana rufe kowane ɓangare na fuskar fata. Lymph nodes na iya zama na al'ada ko na al'ada. Kwayoyin Sézary na iya zama ko a'a cikin jini. Kwayoyin Lymphoma sun bazu zuwa wasu gabobin ko kyallen takarda.

Yaya ake magance ta?

Yawancin dalilai suna tasiri wane magani zai iya zama mafi kyau a gare ku. Daga cikinsu akwai:

• mataki a ganewar asali
• shekaru
• sauran matsalolin lafiya

Wadannan suna daga cikin magungunan cutar Sézary.

Psoralen da UVA (PUVA)

Wani magani da ake kira psoralen, wanda ke neman tattarawa a cikin kwayoyin cutar kansa, ana masa allura a cikin jijiya. Yana zama yana aiki lokacin da aka fallasa shi zuwa hasken ultraviolet A (UVA) wanda aka doshi fata. Wannan aikin yana lalata ƙwayoyin cutar kansa tare da cutar kaɗan kawai ga ƙoshin lafiya.

Extracorporeal photochemotherapy / hotunan hoto (ECP)

Bayan karɓar magunguna na musamman, ana cire wasu ƙwayoyin jini daga jikinka. An bi da su da hasken UVA kafin a sake dawo da su cikin jikinku.

Radiation far

Ana amfani da rayukan-kuzari masu ƙarfi don lalata ƙwayoyin kansa. A cikin fitilun katako na waje, wata inji tana aika haskoki zuwa wuraren da ake niyya na jikinku. Radiation na iya taimakawa jin zafi da sauran alamun. Jimlar katangar lantarki (TSEB) mai amfani da fitila ta waje tana amfani da inji na waje don nufar wutar lantarki a fatar jikin ku duka.

Hakanan zaka iya samun maganin UVA da ultraviolet B (UVB) ta hanyar amfani da haske na musamman wanda yake nufin fata.

Chemotherapy

Chemotherapy magani ne na yau da kullun wanda ake amfani da magunguna masu ƙarfi don kashe ƙwayoyin kansa ko dakatar da rarrabuwarsu. Akwai wasu magungunan ƙwayoyi da ke cikin ƙwayar kwaya, kuma wasu dole ne a ba su ta iska.

Immunotherapy (ilimin ilimin halittu)

Ana amfani da ƙwayoyi kamar su interferons don faɗakar da tsarin garkuwar ku don yaƙi da cutar kansa.

Magungunan da ake amfani da su don magance cututtukan Sézary sun haɗa da:

• alemtuzumab (Campath), wani maganin rigakafi ne na monoclonal
• bexarotene (Targretin), mai sake ganowa
• brentuximab vedotin (Adcetris), mai haɗa kwayar antibody
• chlorambucil (Leukeran), magani ne na kula da cutar kanjamau
• corticosteroids don taimakawa bayyanar cututtuka na fata
• cyclophosphamide (Cytoxan), magani ne na magance cutar sankara
• denileukin difitox (Ontak), mai sauya martanin ilmin halitta
• gemcitabine (Gemzar), antimetabolite jiyyar cutar sankara
• interferon alfa ko interleukin-2, masu kara kuzari na rigakafi
• lenalidomide (Revlimid), mai hana angiogenesis
• liposomal doxorubicin (Doxil), magani ne na maganin cutar sankara
• methotrexate (Trexall), maganin ƙwaƙwalwar antimetabolite
• pentostatin (Nipent), wani maganin antimetabolite
• romidepsin (Istodax), mai hana maganin histamine deacetylase
• vorinostat (Zolinza), mai hana maganin histamine deacetylase

Likitanku na iya ba da umarnin hada magunguna ko magunguna tare da sauran hanyoyin kwantar da hankali. Wannan zai dogara ne akan matakin cutar kansa da yadda kuka amsa wani magani.

Jiyya don mataki na 1 da na 2 na iya haɗawa da:

• Topical corticosteroids
• retinoids, lenalidomide, histone deacetylase masu hanawa
• PUVA
• radiation tare da TSEB ko UVB
• ilimin halittu da kansa ko kuma tare da maganin fata
• maganin kansar jiki
• chemotherapy na tsarin, mai yiwuwa haɗe shi da maganin fata

Mataki na 3 da na 4 za'a iya bi dasu tare da:

• Topical corticosteroids
• lenalidomide, bexarotene, histone deacetylase masu hanawa
• PUVA
• ECP shi kaɗai ko tare da TSEB
• radiation tare da TSEB ko UVB da UVA
• ilimin halittu da kansa ko kuma tare da maganin fata
• maganin kankara na waje
• chemotherapy na tsarin, mai yiwuwa haɗe shi da maganin fata

Idan jiyya basa aiki, dasawar kwayar halitta zai iya zama zaɓi.

Gwajin gwaji

Bincike a cikin jiyya don cutar kansa yana gudana, kuma gwaji na asibiti ɓangare ne na wannan tsari. A cikin gwaji na asibiti, ƙila ku sami dama ga hanyoyin kwantar da hankalin ƙasa wanda ba a samun shi ko'ina. Don ƙarin bayani game da gwaji na asibiti, tambayi likitan ku ko ziyarci ClinicalTrials.gov.

Outlook

Ciwon Sézary cuta ce mai saurin tashin hankali. Tare da magani, ƙila ku iya rage ci gaban cutar ko ma shiga cikin gafara. Amma raunana tsarin garkuwar jiki na iya barin ku cikin saukin kamuwa da cuta da sauran cututtukan daji.

Matsakaicin rayuwa ya kasance shekaru 2 zuwa 4, amma wannan ƙimar yana inganta tare da sababbin jiyya.

Ganin likitan ku kuma fara farawa da wuri-wuri don tabbatar da kyakkyawan fata.