Lymphoplasmacytic Lymphoma yog dab tsi?

Zoo Siab

• LPL thiab lwm cov lymphomas
• Ua li cas rau lub cev tiv thaiv kab mob?
• Cov tsos mob zoo li cas?
• Dab tsi ua rau nws?
• Nws kuaj tau li cas?
• Cov kev kho mob xaiv
• Saib thiab tos
• Kws khomob
• Kev siv roj ntsha
• Cov hom phiaj kho
• Cov qog ua qauv hloov ntawm tes
• Cov chaw kuaj mob
• Qhov tshwm sim yog dab tsi?

Txheej txheem cej luam

Lymphoplasmacytic lymphoma (LPL) yog ib hom mob cancer uas tsis tshua pom muaj tshwm sim qeeb thiab cuam tshuam rau cov laus feem ntau. Qhov nruab nrab hnub nyoog ntawm kev kuaj mob yog 60.

Lymphomas yog cov qog nqaij hlav ntawm lub qog ua haujlwm, ib feem ntawm koj lub cev tiv thaiv kab mob uas pab tua kab mob sib kis. Hauv cov qog ntshav, cov qe ntshav dawb, tsis hais B lymphocytes lossis T lymphocytes, loj hlob tsis tuaj yeem vim muaj kev hloov pauv. Hauv LPL, txawv txav B lymphocytes yug me nyuam hauv koj cov leeg pob txha thiab ua rau cov ntshav noj qab nyob zoo.

Muaj txog 8.3 tus neeg mob LPL rau 1 lab tus tib neeg hauv Asmeskas thiab Western Europe. Nws muaj ntau rau cov txivneej thiab Caucasians.

LPL thiab lwm cov lymphomas

Hodgkin's lymphoma thiab tsis-Hodgkin's lymphoma yog qhov txawv los ntawm hom hlwb ua qog nqaij hlav.

• Hodgkin's lymphomas muaj qhov tshwj xeeb txawv txav ntawm lub ntsej muag, hu ua Reed-Sternberg cell.
• Ntau yam ntawm cov tsis yog Hodgkin's lymphomas tau txawv los ntawm qhov twg cov qog nqaij hlav pib thiab qhov muaj keeb thiab lwm yam ntawm cov hlwb tsis zoo.

LPL yog qhov tsis yog Hodgkin's lymphoma uas pib hauv B lymphocytes. Nws yog ib qhov mob ntsws tsis tshua muaj ntau, muaj li thaj tsam li 1 txog 2 feem pua ​​ntawm tag nrho cov mob ua mob qog.

Feem ntau hom LPL yog Waldenström macroglobulinemia (WM), uas yog cov cwj pwm txawv txav ntawm cov tshuaj tiv thaiv immunoglobulin (tshuaj tiv thaiv). WM qee zaus muab sau yuam kev zoo ib yam nrog LPL, tab sis nws yog qhov tseeb ntawm LPL. Kwv yees li ntawm 19 ntawm 20 tus neeg uas mob LPL muaj lub immunoglobulin txawv txav.

Ua li cas rau lub cev tiv thaiv kab mob?

Thaum LPL ua rau cov kab mob B lymphocytes (B hlwb) dhau los ua kom muaj txiaj ntsig hauv koj cov pob txha pob txha, cov ntshav tsim muaj tsawg dua tuaj.

Feem ntau, B cov hlwb txav ntawm koj tus pob txha pob txha mus rau koj tus txha caj qaum thiab cov qog ntshav. Nyob ntawd, lawv yuav dhau los ua cov ntshav ntshav tsim cov tshuaj los tiv thaiv kev kis mob. Yog koj tsis muaj cov qe ntshav txaus txaus, nws yuav ua rau koj muaj zog tiv thaiv kab mob.

Qhov no yuav ua rau:

• anemia, uas tsis txaus cov ntshav liab
• neutropenia, kev tsis txaus ntawm ib hom qe ntshav dawb (hu ua neutrophils), uas ua rau muaj kev pheej hmoo kis mob ntxiv
• thrombocytopenia, kev tsis txaus ntawm cov ntshav platelets, uas ua rau kom ntshav nce ntxiv thiab ntshav yuav tsis zoo

Cov tsos mob zoo li cas?

LPL yog mob qog noj ntshav qeeb, thiab li 1/3 ntawm cov neeg uas LPL tsis muaj mob dabtsi rau lub sijhawm lawv kuaj mob.

Txog li 40 feem pua ​​ntawm cov neeg muaj LPL muaj mob qog ua ntshav tsis huv.

Lwm cov tsos mob ntawm LPL yuav suav nrog:

• qaug zog thiab qaug zog (feem ntau tshwm sim los ntawm anemia)
• kub taub hau, tawm hws hmo ntuj, thiab poob phaus (feem ntau cuam tshuam nrog B-cell lymphomas)
• qhov muag plooj
• kiv taub hau
• los ntswg
• cov pos hniav los ntshav
• doog ntshav
• txhawb siab beta-2-microglobulin, ntshav lub cim rau qog cov hlav

Kwv yees li 15 txog 30 feem pua ​​ntawm cov neeg LPL muaj:

• cov qog ntshav o (lymphadenopathy)
• daim siab ua lub siab (hepatomegaly)
• spleen enlargement (splenomegaly)

Dab tsi ua rau nws?

Qhov ua rau LPL tseem tsis tau to taub tag nrho. Cov kws tshawb nrhiav tau tshawb nrhiav ntau yam muaj:

• Tej zaum yuav muaj cov caj ces, raws li ntawm 1 ntawm 5 tus neeg nrog WM muaj ib tug txheeb ze uas muaj LPL lossis ib hom mob qog zoo sib xws.
• Qee qhov kev tshawb fawb pom tias LPL yuav cuam tshuam nrog cov kab mob autoimmune zoo li Sjögren syndrome lossis mob siab rau tus kab mob siab C, tab sis lwm qhov kev tshawb fawb tsis tau qhia txog qhov txuas no.
• Cov neeg muaj LPL feem ntau muaj qee yam kev hloov pauv tau caj ces uas tsis tau txais cov noob caj noob ces.

Nws kuaj tau li cas?

Kev kuaj mob ntawm LPL yog qhov nyuaj thiab feem ntau yog ua tom qab tsis suav nrog lwm cov kev tuaj yeem.

LPL tuaj yeem ua tau zoo li lwm yam B-cell lymphomas nrog zoo sib xws ntawm cov ntshav ntshav sib txawv. Cov no suav nrog:

• mantle cell lymphoma
• lymphocytic mob ntsws / cov ntshav me menyuam yaus
• marginal tsam lymphoma
• ntshav cell myeloma

Koj tus kws kho mob yuav kuaj koj lub cev thiab nug koj li keeb kwm kev kho mob. Lawv yuav ua kom cov ntshav ua haujlwm thiab tejzaum cov pob txha caj qaum lossis lymph node biopsy los saib cov cell hauv lub tshuab tsom.

Koj tus kws kho mob kuj tseem yuav siv lwm yam kev kuaj los txiav txim cov qog nqaij hlav zoo sib xws thiab txiav txim siab theem ntawm koj tus kab mob. Cov no suav nrog kev xoo duab hauv siab, CT scan, PET scan, thiab ultrasound.

Cov kev kho mob xaiv

Saib thiab tos

LBL yog ib yam kabmob loj hlob qeeb. Koj thiab koj tus kws kho mob yuav txiav txim siab tos thiab saib xyuas koj cov ntshav tsis tu ncua ua ntej pib kev kho mob. Raws li American Cancer Society (ACS), cov tib neeg uas ncua sijhawm kho kom txog thaum lawv cov tsos mob muaj teeb meem muaj qhov qub qub li cov neeg uas pib kho sai li sai tau uas lawv tau kuaj.

Kws khomob

Ntau cov tshuaj uas ua haujlwm sib txawv, lossis kev sib txuas ntawm cov tshuaj, tej zaum yuav raug siv los tua cov qog nqaij hlav cancer. Cov no suav nrog:

• chlorambucil (Leukeran)
• fludarabine (Fludara)
• bendamustine (Treanda)
• cyclophosphamide (Cytoxan, Procytox)
• dexamethasone (Decadron, Dexasone), rituximab (Rituxan), thiab cyclophosphamide
• bortezomib (Velcade) thiab rituximab, nrog lossis tsis muaj dexamethasone
• cyclophosphamide, vincristine (Oncovin), thiab prednisone
• cyclophosphamide, vincristine (Oncovin), prednisone, thiab rituximab
• thalidomide (Thalomid) thiab rituximab

Qhov tshwj xeeb cov tshuaj yuav txawv, nyob ntawm koj qhov kev noj qab haus huv, koj cov tsos mob, thiab kev kho yav tom ntej.

Kev siv roj ntsha

Kev siv tshuaj lom neeg yog cov tsim tshuaj uas ua zoo ib yam li koj lub cev tiv thaiv kab mob tua cov qog ntshav. Cov tshuaj no tuaj yeem ua ke nrog lwm cov kev kho mob.

Qee cov tshuaj tiv thaiv manmade, hu ua monoclonal antibodies, yog:

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

Lwm cov tshuaj lom neeg yog cov tshuaj tiv thaiv kab mob (IMiDs) thiab cytokines.

Cov hom phiaj kho

Cov hom phiaj kho mob tsom mus rau txhawm rau hloov cov cell hloov uas ua rau mob qog nqaij hlav. Qee cov tshuaj no tau siv los tua lwm cov qog nqaij hlav cancer thiab tam sim no tau tshawb fawb rau LBL. Feem ntau, cov tshuaj no thaiv cov protein uas ua rau cov qog ua kom lub cev loj tuaj.

Cov qog ua qauv hloov ntawm tes

Nov yog kev kho mob tshiab uas ACS hais tias yog kev xaiv rau cov hluas uas muaj LBL.

Feem ntau, cov roj ntsha tsim cov qia ua ntshav tawm ntawm cov hlab ntshav thiab khaws cia khov. Tom qab ntawv cov tshuaj siab los yog hluav taws xob ntau dua yog siv los tua txhua tus pob txha hlwb (li qub thiab mob qog nqaij hlav), thiab cov keeb tsim ua ntshav rov los rau hauv cov ntshav. Cov qia ua chiv los ntawm tus neeg raug kho (autologous), lossis lawv yuav tau pub los ntawm ib tus neeg uas sib raug zoo nrog tus neeg (allogenic).

Nco ntsoov tias qog hloov ntshav ntawm tes tseem nyob hauv ib qho sim chaw. Tsis tas li, muaj cov kev mob luv luv thiab ntev ntev los ntawm cov kev hloov ntshav no.

Cov chaw kuaj mob

Zoo li ntau yam mob qog noj ntshav, kev kho mob tshiab tau tsim kho, thiab tej zaum koj yuav pom muaj kev sim kho mob los koom nrog. Nug koj tus kws kho mob txog qhov no thiab mus saib ClinicalTrials.gov yog xav paub ntau ntxiv.

Qhov tshwm sim yog dab tsi?

LPL li tseem tsis tau muaj tshuaj kho tau. Koj li LPL yuav mus rau hauv kev zam txim tab sis tom qab ntawd rov tshwm sim dua. Tsis tas li, txawm hais tias nws yog mob qog noj ntshav qeeb qeeb, qee qhov nws tuaj yeem ua rau mob hnyav.

ACS sau tseg tias 78 feem pua ​​ntawm cov neeg nrog LPL nyob 5 xyoo lossis ntau dua.

Ciaj sia nyob tus nqi rau LPL yog txhim kho raws li cov tshuaj tshiab thiab kev kho mob tshiab tau tsim.