Lymphoplasmacytic Lymphoma ke eng?

Litaba

• LPL le li-lymphomas tse ling
• Ho etsahalang ka masole a mmele?
• Matšoao ke afe?
• E bakoa ke eng?
• E fumanoa joang?
• Khetho ea kalafo
• Shebella 'me u eme
• Chemotherapy
• Phekolo ea likokoana-hloko
• Phekolo e lebisitsoeng
• Ho fetisoa ha seleng ea stem
• Liteko tsa bongaka
• Pono ke efe?

Kakaretso

Lymphoplasmacytic lymphoma (LPL) ke mofuta o sa tloaelehang oa mofets'e o hlahang butle mme o ama boholo ba batho ba baholo. Karolelano ea lilemo tsa ho hlahlojoa ke 60.

Lymphomas ke kankere ea lisele tsa mmele, karolo ea sesole sa hau sa 'mele e thusang ho loants'a mafu. Lymphoma, lisele tse tšoeu tsa mali, ekaba li-lymphocyte tsa B kapa li-lymphocyte tsa T, li tsoa taolong ka lebaka la phetoho ea 'mele. Ho LPL, li-lymphocyte tsa B tse sa tloaelehang li ikatisa mokong oa masapo ebe li tlosa lisele tse phetseng hantle tsa mali.

Ho na le linyeoe tse ka bang 8.3 tsa LPL ho batho ba milione e le ngoe United States le Europe Bophirima. E tloaelehile haholo ho banna le ho batho ba Caucasus.

LPL le li-lymphomas tse ling

Hodgkin's lymphoma le non-Hodgkin's lymphoma li khetholloa ke mofuta oa lisele tse fetohang mofets'e.

• Li-lymphomas tsa Hodgkin li na le mofuta o itseng oa lisele tse sa tloaelehang, tse bitsoang sele ea Reed-Sternberg.
• Mefuta e mengata ea li-lymphomas tse seng tsa Hodgkin li khetholloa ke moo kankere e qalang teng le liphatsa tsa lefutso le tse ling tsa lisele tse kotsi.

LPL ke lymphoma eo eseng ea Hodgkin e qalang ho li-lymphocyte tsa B. Ke lymphoma e sa tloaelehang haholo, e nang le hoo e ka bang karolo ea 1 ho ea ho 2 lekholong ea li-lymphomas tsohle.

Mofuta o tloaelehileng haholo oa LPL ke Waldenström macroglobulinemiaemia (WM), e khetholloang ka tlhahiso e sa tloaelehang ea immunoglobulin (antibodies). Ka linako tse ling WM ka phoso e bitsoa e ts'oanang le LPL, empa ha e le hantle ke karoloana ea LPL. Batho ba ka bang 19 ho ba 20 ba nang le LPL ba na le immunoglobulin e sa tloaelehang.

Ho etsahalang ka masole a mmele?

Ha LPL e etsa hore li-lymphocyte tsa B (lisele tsa B) li hlahise haholo mongoaneng oa masapo, lisele tse fokolang tsa mali li hlahisoa.

Ka tloaelo, lisele tsa B li tloha mokong oa masapo ho ea spleen le lymph node. Moo, e ka ba lisele tsa mali a mali tse hlahisang masole a 'mele ho loantša tšoaetso. Haeba u se na lisele tse tloaelehileng tsa mali tse lekaneng, e beha sesole sa hau sa 'mele kotsing.

Sena se ka fella ka:

• khaello ea mali, khaello ea lisele tse khubelu tsa mali
• neutropenia, khaello ea mofuta oa sele e tšoeu ea mali (e bitsoang neutrophils), e eketsang kotsi ea tšoaetso
• thrombocytopenia, khaello ea liplatelete tsa mali, e eketsang likotsi tsa ho tsoa mali le ho longoa

Matšoao ke afe?

LPL ke mofetše o hōlang butle, 'me hoo e ka bang karolo ea boraro ea batho ba nang le LPL ha ba na matšoao nakong eo ba fumanoang.

Ho fihlela ho karolo ea 40 lekholong ea batho ba nang le LPL ba na le mofuta o fokolang oa phokolo ea mali.

Matšoao a mang a LPL a kenyeletsa:

• bofokoli le mokhathala (hangata bo bakoang ke khaello ea mali)
• feberu, ho fufuleloa bosiu, le ho theola boima ba 'mele (hangata bo amanang le B-cell lymphomas)
• pono e lerootho
• ho tsekela
• nko e tsoa mali
• marenene a tsoang mali
• matetetso
• beta-2-microglobulin e phahameng, letšoao la mali bakeng sa lihlahala

Hoo e ka bang karolo ea 15 ho ea ho 30 lekholong ea ba nang le LPL ba na le:

• ho ruruha lymph nodes (lymphadenopathy)
• Ho eketsa sebete (hepatomegaly)
• Ho atolosoa ha spleen (splenomegaly)

E bakoa ke eng?

Lebaka la LPL ha le utloisisoe ka botlalo. Bafuputsi ba ntse ba batlisisa menyetla e 'maloa:

• Ho ka 'na ha e-ba le karolo ea liphatsa tsa lefutso, kaha hoo e ka bang motho a le mong ho ba bahlano ea nang le WM o na le mong ka eena ea nang le LPL kapa mofuta o tšoanang oa lymphoma.
• Boithuto bo bong bo fumane hore LPL e kanna ea amahanngoa le maloetse a ikemetseng a mmele joalo ka Sjögren syndrome kapa le vaerase ea hepatitis C, empa lithuto tse ling ha li so bonts'a khokahano ena.
• Batho ba nang le LPL hangata ba na le liphetoho tse itseng tsa lefutso tse sa futsitsoeng.

E fumanoa joang?

Ho fumaneha ha LPL ho thata mme hangata ho etsoa kamora ho kenyelletsa menyetla e meng.

LPL e ka tšoana le li-lymphomas tse ling tsa B tse nang le mefuta e tšoanang ea phapang ea lisele tsa mali. Tsena li kenyelletsa:

• mantle cell lymphoma
• chronic lymphocytic leukemia / small lymphocytic lymphoma
• marginal zone lymphoma
• sele ea plasma myeloma

Ngaka ea hau e tla u hlahloba 'meleng ebe e botsa nalane ea hau ea bongaka. Ba tla laela mosebetsi oa mali mme mohlomong le moko oa masapo kapa lymph node biopsy ho sheba lisele tse tlas'a microscope.

Ngaka ea hau e kanna ea sebelisa liteko tse ling ho felisa mofetše o ts'oanang le ho tseba hore na lefu la hau le boemong bofe. Tsena li ka kenyelletsa sefuba sa X-ray, CT scan, PET scan le ultrasound.

Khetho ea kalafo

Shebella 'me u eme

LBL ke mofetše o hōlang butle. Uena le ngaka ea hau le ka nka qeto ea ho ema le ho lekola mali a hau khafetsa pele u qala kalafo. Ho ea ka American Cancer Society (ACS), batho ba liehang kalafo ho fihlela matšoao a bona a le mathata ba na le nako e telele e lekanang le ea batho ba qalang kalafo hang ha ba fumanoa.

Chemotherapy

Lithethefatsi tse 'maloa tse sebetsang ka litsela tse fapaneng, kapa metsoako ea lithethefatsi, li ka sebelisoa ho bolaea lisele tsa mofetše. Tsena li kenyelletsa:

• chlorambucil (Leukeran)
• fludarabine (Fludara)
• bendamustine (Treanda)
• cyclophosphamide (Cytoxan, Procytox)
• dexamethasone (Decadron, Dexasone), rituximab (Rituxan), le cyclophosphamide
• bortezomib (Velcade) le rituximab, e nang le dexamethasone kapa ntle le eona
• cyclophosphamide, vincristine (Oncovin), le prednisone
• cyclophosphamide, vincristine (Oncovin), prednisone le rituximab
• thalidomide (Thalomid) le rituximab

Mokhoa o khethehileng oa lithethefatsi o tla fapana, ho latela bophelo bo botle ba 'mele oa hau, matšoao a hau le kalafo e ka bang teng nakong e tlang.

Phekolo ea likokoana-hloko

Lithethefatsi tsa kalafo ea likokoana-hloko ke lintho tse entsoeng ke motho tse sebetsang joalo ka boits'ireletso ba hau ba mmele ho bolaea lisele tsa lymphoma. Lithethefatsi tsena li kanna tsa kopanngoa le kalafo tse ling.

Tse ling tsa lithibela-mafu tsena tse entsoeng ke batho, tse bitsoang `` monoclonal antibodies '' ke tsena:

• Rituximab (Rituxan)
• ofatumumab (Arzerra)
• alemtuzumab (campath)

Lithethefatsi tse ling tsa likokoana-hloko ke lithethefatsi tse itšireletsang mafung (IMiDs) le li-cytokine.

Phekolo e lebisitsoeng

Meriana e reriloeng ea kalafo e ikemiselitse ho thibela liphetoho tse itseng tsa sele tse bakang mofetše. Tse ling tsa lithethefatsi tsena li sebeliselitsoe ho loants'a mofetše o mong mme hajoale li ntse li batlisisoa bakeng sa LBL. Ka kakaretso, lithethefatsi tsena li thibela liprotheine tse lumellang lisele tsa lymphoma ho lula li hola.

Ho fetisoa ha seleng ea stem

Ena ke kalafo e ncha eo ACS e reng e kanna ea ba khetho bakeng sa bacha ba nang le LBL.

Ka kakaretso, lisele tsa bakoang tse etsang mali li tlosoa maling ebe li bolokoa li hoamisitsoe. Ebe ho sebelisoa tekanyetso e phahameng ea chemotherapy kapa radiation ho bolaea lisele tsohle tsa moko (tse tloaelehileng le tse nang le mofetše), 'me lisele tsa mantlha tse etsang mali li khutlisetsoa maling. Lisele tsa bakoang li ka hlaha ho motho ea alafshoang (autologous), kapa li ka fanoa ke motho ea haufi haholo le motho eo (allogenic).

Hlokomela hore li-transplants tsa stem li ntse li le boemong ba liteko. Hape, ho na le litla-morao tsa nakoana le tsa nako e telele tse tsoang ho li-transplants tsena.

Liteko tsa bongaka

Joalo ka mefuta e mengata ea mofetše, mekhoa ea kalafo e mecha e ntse e ntlafatsoa, ​​'me u ka fumana teko ea bongaka ea ho nka karolo. Botsa ngaka ea hau ka sena mme u etele ClinicalTrials.gov bakeng sa tlhaiso-leseling e batsi.

Pono ke efe?

LPL hajoale ha e na pheko. LPL ea hau e kanna ea tšoareloa empa hamorao ea hlaha hape. Hape, le hoja e le mofetše o hōlang butle, maemong a mang e ka ba mabifi le ho feta.

ACS e bolela hore karolo ea 78 lekholong ea batho ba nang le LPL ba phela lilemo tse hlano kapa ho feta.

Sekhahla sa pholoho ea LPL sea ntlafala ha litlhare tse ncha le kalafo e ncha li ntse li ntlafatsoa.