Yintoni iLymphoplasmacytic Lymphoma?

Umxholo

• I-LPL kunye nezinye ii-lymphomas
• Kwenzeka ntoni kumajoni omzimba?
• Ziintoni iimpawu?
• Ibangelwa yintoni?
• Kufunyaniswa njani?
• Izinketho zonyango
• Bukela ulinde
• Unyango ngamayeza
• Unyango lwebhayoloji
• Unyango ekujoliswe kulo
• Ukutshintshwa kweseli
• Uvavanyo lwezonyango
• Yintoni imbonakalo?

Ushwankathelo

I-Lymphoplasmacytic lymphoma (LPL) luhlobo olunqabileyo lomhlaza olukhula kancinci kwaye luchaphazele ikakhulu abantu abadala. Umyinge weminyaka yobudala ekuxilongweni ngama-60.

IiLymphomas ngumhlaza wesixokelelwano se-lymph, yinxalenye yamajoni akho omzimba anceda ukulwa izifo. Kwi-lymphoma, iiseli ezimhlophe zegazi, nokuba ziiL lymphocyte okanye iiL lymphocyte, zikhula ngaphandle kolawulo ngenxa yenguquko. Kwi-LPL, ii-lymphocyte ezingaqhelekanga ze-B zivelisa umongo wakho kwaye zisuse iiseli zegazi ezisempilweni.

Kukho amatyala ayi-8.3 e-LPL kwisigidi esinye sabantu eMelika naseNtshona Yurophu. Kuqheleke ngakumbi kumadoda nakwiiCaucasians.

I-LPL kunye nezinye ii-lymphomas

I-Hodgkin's lymphoma kunye ne-non-Hodgkin's lymphoma yahlulahlulwa luhlobo lweeseli eziba ngumhlaza.

• Ii-lymphomas zeHodgkin zinohlobo oluthile lweseli engaqhelekanga ekhoyo, ebizwa ngokuba yiseli yeRed-Sternberg.
• Iindidi ezininzi zee-lymphomas ezingezizo ze-Hodgkin zahlulwa apho umhlaza uqala khona kunye nemfuza kunye nezinye iimpawu zeeseli ezimbi.

I-LPL yi-non-Hodgkin's lymphoma eqala kwii-lymphocyte ze-B. I-lymphoma enqabileyo kakhulu, equka malunga neepesenti ezi-1 ukuya kwezi-2 zazo zonke i-lymphomas.

Olona hlobo luqhelekileyo lwe-LPL yiWaldenström macroglobulinemiaemia (WM), ebonakaliswa yimveliso engaqhelekanga ye-immunoglobulin (antibodies). I-WM ngamanye amaxesha ibizwa ngempazamo njengefana ne-LPL, kodwa eneneni iseti esezantsi ye-LPL. Phantse abantu abangama-19 kwabangama-20 abane-LPL bane-immunoglobulin engaqhelekanga.

Kwenzeka ntoni kumajoni omzimba?

Xa i-LPL ibangela ii-lymphocyte B (iiseli ze-B) ukuba zivelise kakhulu kumongo wakho wethambo, ziveliswa iiseli zegazi eziqhelekileyo ezimbalwa.

Ngokuqhelekileyo, iiseli ze-B zihamba ukusuka kumnkantsha wethambo ukuya kwi-spleen kunye ne-lymph nodes. Apho, banokuba ziiseli zeplasma ezivelisa ii-antibodies zokulwa usulelo. Ukuba awunazo iiseli zegazi eziqhelekileyo ngokwaneleyo, iyawonakalisa amajoni akho omzimba.

Oku kunokubangela:

• i-anemia, ukunqongophala kweeseli ezibomvu zegazi
• i-neutropenia, ukunqongophala kohlobo lweeseli ezimhlophe zegazi (ezibizwa ngokuba zii-neutrophils), ezonyusa umngcipheko wosulelo
• i-thrombocytopenia, ukunqongophala kweeplatelets zegazi, ezonyusa ukopha kunye nokulimala komngcipheko

Ziintoni iimpawu?

I-LPL ngumhlaza okhula kancinci, kwaye malunga nesinye kwisithathu sabantu abane-LPL abanazo iimpawu ngexesha lokuchongwa.

Ukufikelela kuma-40 ekhulwini abantu abane-LPL banefom ye-anemia.

Ezinye iimpawu zeLPL zingabandakanya:

• ubuthathaka kunye nokudinwa (kuhlala kubangelwa yi-anemia)
• umkhuhlane, ukukhupha ebusuku, kunye nokwehla kobunzima (ngokubanzi kunxulunyaniswa ne-B-cell lymphomas)
• ukungaboni kakuhle
• isiyezi
• ukuphuma kwempumlo
• iintsini ezophayo
• ukugruzuka
• i-beta-2-microglobulin ephezulu, uphawu lwegazi kwizidumbu

Phantse iipesenti ezili-15 ukuya kuma-30 zalabo abane-LPL bane:

• i-lymph nodes (i-lymphadenopathy)
• Ukwandiswa kwesibindi (hepatomegaly)
• Ukwandiswa kwepleen (splenomegaly)

Ibangelwa yintoni?

Isizathu se-LPL asiqondakali ngokupheleleyo. Abaphandi baphanda iindlela ezininzi:

• Kukho ukuba ne-genetic component, malunga ne-1 kubantu aba-5 abane-WM banesihlobo esineLPL okanye uhlobo olufanayo lwe-lymphoma.
• Olunye uphononongo lufumanise ukuba i-LPL inokunxulunyaniswa nezifo ezizimela njenge-Sjögren syndrome okanye intsholongwane ye-hepatitis C, kodwa ezinye izifundo azibonisanga eli khonkco.
• Abantu abane-LPL bahlala benemfuza ethile engazalwanga njengelifa.

Kufunyaniswa njani?

Ukuchongwa kwe-LPL kunzima kwaye kuhlala kwenziwa emva kokukhetha amanye amathuba.

I-LPL inokufana nezinye i-B-cell lymphomas eneentlobo ezifanayo zokwahluka kweseli yeplasma. Oku kubandakanya:

• Ingubo yeseli lymphoma
• I-lymphocytic leukemia engapheliyo / i-lymphoma encinci ye-lymphocytic
• ummandla osemdeni we-lymphoma
• Iseli yeplasma myeloma

Ugqirha wakho uya kukuvavanya ngokwasemzimbeni aze abuze imbali yakho yonyango. Baya kuyala umsebenzi wegazi kwaye mhlawumbi umongo wethambo okanye i-lymph node biopsy ukujonga iiseli phantsi kwemicroscope.

Ugqirha wakho unokusebenzisa olunye uvavanyo ukulawula umhlaza ofanayo kunye nokujonga inqanaba lesifo sakho. Oku kunokubandakanya i-X-ray yesifuba, ukuskena kwe-CT, ukuskena kwe-PET kunye ne-ultrasound.

Izinketho zonyango

Bukela ulinde

I-LBL ngumhlaza okhula kancinci. Wena kunye nogqirha wakho unokugqiba ekubeni ulinde kwaye ujonge igazi lakho rhoqo ngaphambi kokuba uqale unyango. Ngokwe-American Cancer Society (ACS), abantu abalibazisa unyango de iimpawu zabo zibe yingxaki banobude obufanayo nabantu abaqala unyango kwakamsinya nje ukuba bafumaneke.

Unyango ngamayeza

Amachiza aliqela asebenza ngeendlela ezahlukeneyo, okanye indibaniselwano yamachiza, anokusetyenziselwa ukubulala iiseli zomhlaza. Oku kubandakanya:

• I-chlorambucil (iLeukeran)
• fludarabine (Fludara)
• bendamustine (iTreanda)
• icyclophosphamide (ICytoxan, iProcytox)
• i-dexamethasone (i-Decadron, i-Dexasone), i-rituximab (i-Rituxan), kunye ne-cyclophosphamide
• i-bortezomib (Velcade) kunye ne-rituximab, kunye okanye ngaphandle kwe-dexamethasone
• cyclophosphamide, vincristine (Oncovin), kunye ne-prednisone
• cyclophosphamide, vincristine (Oncovin), prednisone, kunye ne-rituximab
• thalidomide (Thalomid) kunye ne-rituximab

Uhlobo oluthile lwamachiza luya kwahluka, kuxhomekeke kwimpilo yakho ngokubanzi, iimpawu zakho, kunye nonyango olunokwenzeka kwixesha elizayo.

Unyango lwebhayoloji

Amachiza onyango lwebhayoloji zizinto ezenziwe ngabantu ezisebenza njengamajoni omzimba wakho ukubulala iiseli ze-lymphoma. La machiza anokudityaniswa nolunye unyango.

Ezinye zezi zintsholongwane ezenziwe ngabantu, ezibizwa ngokuba yi-monoclonal antibodies, zi:

• rituximab (Rituxan)
• ofatumumab (iArzerra)
• i-alemtuzumab (inkampu)

Ezinye iziyobisi zebhayiloji ngamachiza okuchasana nomzimba (ii-IMiDs) kunye nee cytokines.

Unyango ekujoliswe kulo

Ukunyanga okujolise kunyango kujolise ekuthinteleni utshintsho oluthile lweseli olubangela umhlaza. Amanye ala machiza asetyenziselwa ukulwa neminye imihlaza kwaye ngoku kuphandwa nge-LBL. Ngokubanzi, la machiza athintela iiproteni ezivumela iiseli ze-lymphoma ukuba ziqhubeke nokukhula.

Ukutshintshwa kweseli

Olu lonyango olutsha athi i-ACS inokuba lukhetho kubantu abancinci abane-LBL.

Ngokubanzi, iiseli zeziqu ezenza igazi ziyasuswa kwigazi kwaye zigcinwe ngumkhenkce. Emva koko kusetyenziswa idosi ephezulu yokunyanga ngamayeza okanye ngemitha ukubulala onke amaseli omongo wethambo (aqhelekileyo kunye nomhlaza), kwaye iiseli zokuqala zokwenza igazi zibuyiselwa kwigazi. Iiseli zeziqu zinokuvela emntwini onyangiweyo (kwi-autologous), okanye zinokunikezelwa ngumntu ohambelana nomntu (i-allogenic).

Yazi ukuba ukufakelwa kweeseli ezitshisayo kukwinqanaba lokulinga. Kananjalo kukho iziphumo ebezingalindelekanga zexesha elifutshane kunye nexesha elide kwezi zinto zifakelweyo.

Uvavanyo lwezonyango

Njengakwiintlobo ezininzi zomhlaza, iindlela zonyango ezintsha ziphantsi kophuhliso, kwaye unokufumana uvavanyo lonyango ukuba uthathe inxaxheba. Buza ugqirha wakho malunga noku kwaye undwendwele iClinicalTrials.gov ngolwazi oluthe kratya.

Yintoni imbonakalo?

I-LPL okwangoku ayinanyango. I-LPL yakho inokuya kuxolelo kodwa iphinde ivele kwakhona. Kwakhona, nangona ingumhlaza okhula kancinci, kwezinye iimeko unokuba ndlongondlongo.

I-ACS ithi iipesenti ezingama-78 zabantu abane-LPL bahlala iminyaka emihlanu okanye ngaphezulu.

Amaxabiso okusinda kwi-LPL ayaphucuka njengoko amachiza amatsha kunye nonyango olutsha luphuhliswa.