IWaldenström macroglobulinemia

IWaldenström macroglobulinemia (WM) ngumhlaza kwiiB lymphocyte (uhlobo lweseli emhlophe yegazi). I-WM inxulunyaniswa nokuveliswa kakhulu kweeproteni ezibizwa ngokuba zii-antibodies ze-IgM.

I-WM sisiphumo semeko ebizwa ngokuba yi-lymphoplasmacytic lymphoma. Lo ngumhlaza weeseli ezimhlophe zegazi, apho iiseli zomzimba ze-B ziqala ukwahlula ngokukhawuleza. Oyena nobangela wemveliso eninzi kakhulu ye-IgM antibody ayaziwa. I-Hepatitis C inokunyusa umngcipheko we-WM. Ukutshintsha kweGene kuhlala kufumaneka kwiiseli ze-B ezimbi.

Ukuveliswa kwee-antibodies ze-IgM ezingaphaya kunokubangela iintlobo ezininzi zeengxaki:

• Hyperviscosity, ebangela ukuba igazi lityebe kakhulu. Oku kunokwenza kube nzima ukuba igazi liphume kwimithambo yegazi encinci.
• I-Neuropathy, okanye umonakalo we-nerve, xa i-antibody ye-IgM isabela kwizicubu zemithambo-luvo.
• I-anemia, xa i-antibody ye-IgM ibophelela kwiiseli ezibomvu zegazi.
• Isifo sezintso, xa i-antibody ye-IgM ifaka kwizicubu zezintso.
• I-Cryoglobulinemia kunye ne-vasculitis (ukudumba kwemithambo yegazi) xa i-antibody ye-IgM yenza izakhiwo zomzimba ezinomkhuhlane.

I-WM inqabile. Uninzi lwabantu abakule meko bangaphezulu kweminyaka engama-65 ubudala.

Iimpawu ze-WM zinokubandakanya oku kulandelayo:

• Ukopha kweentsini kunye neempumlo zeempumlo
• Ukungaboni kakuhle okanye ukunciphisa umbono
• Ulusu oluhlaza okwesibhakabhaka kwiminwe emva kokubhengeza ukubanda
• Ukuba nesiyezi okanye ukudideka
• Ukutyumka ngokulula kolusu
• Ukudinwa
• Urhudo
• Ubudenge, ukubetha, okanye iintlungu ezivuthayo ezandleni, iinyawo, iminwe, iinzwane, iindlebe, okanye impumlo
• Irhashalala
• Amadlala adumbileyo
• Ukuphulukana nobunzima ngabom
• Ukuphulukana nombono kwiliso elinye

Uvavanyo lomzimba lunokubonisa ukudumba, isibindi kunye ne-lymph node. Uvavanyo lwamehlo lunokubonisa imithambo eyandisiweyo kwi-retina okanye ukuphuma kwegazi kwithumbu (ukopha).

I-CBC ibonisa inani eliphantsi leeseli ezibomvu zegazi kunye neeplatelets. I-chemistry yegazi ingabonisa ubungqina besifo sezintso.

Uvavanyo olubizwa ngokuba yi-serum protein electrophoresis lubonisa inqanaba elonyukayo le-antibody ye-IgM. Amanqanaba ahlala ephezulu kuneemiligram ezingama-300 kwidesilitha nganye (mg / dL), okanye i-3000 mg / L. Uvavanyo lwe-immunofixation luya kwenziwa ukubonisa ukuba i-antibody ye-IgM ivela kuhlobo lweseli enye, (clonal).

Uvavanyo lwe-serum viscosity lunokuchaza ukuba igazi liye latyeba na. Iimpawu zihlala zenzeka xa igazi liphindwe kane kunesiqhelo.

Umongo wethambo uya kubonisa inani elonyukayo leeseli ezingaqhelekanga ezibonakala njengee-lymphocyte kunye neeseli zeplasma.

Uvavanyo olongezelelweyo olunokwenziwa lubandakanya:

• Iiyure ezingama-24 zeprotheyini yomchamo
• Iiproteni zizonke
• Ukuzikhusela emzimbeni kumchamo
• T (thymus etholwe) ubalo lwe-lymphocyte
• Iithambo x-reyi

Abanye abantu abane-WM abaye bonyusa ii-antibodies ze-IgM abanazo iimpawu. Le meko yaziwa njenge-smoldering WM. Akukho lunyango lufunekayo ngaphandle kokulandela ngononophelo.

Kubantu abaneempawu, unyango lujolise ekunciphiseni iimpawu kunye nomngcipheko wokukhula komzimba. Akukho lonyango lusemgangathweni lwangoku. Umboneleli wakho wezempilo unokucebisa ukuba uthathe inxaxheba kuvavanyo lweklinikhi.

I-Plasmapheresis isusa ii-antibodies ze-IgM egazini. Ikwalawula ngokukhawuleza iimpawu ezibangelwa kukuqina kwegazi.

Amayeza anokubandakanya i-corticosteroids, indibaniselwano yamayeza e-chemotherapy kunye ne-monoclonal antibody kwiiseli ze-B, i-rituximab.

Ukufakelwa kweselfowni kwi-stem kunokucetyiswa kwabanye abantu abanempilo entle.

Abantu abanenani eliphantsi leeseli ezibomvu okanye ezimhlophe zegazi okanye iiplatelets banokufuna ukutofelwa igazi okanye amayeza okubulala iintsholongwane.

Ukuphila okuphakathi malunga neminyaka emi-5. Abanye abantu baphila ngaphezulu kweminyaka eli-10.

Kwabanye abantu, ingxaki inokuvelisa iimpawu ezimbalwa kwaye iqhubeke kancinci.

Iingxaki zeWM zinokubandakanya:

• Utshintsho ekusebenzeni kwengqondo, olunokubangela ikhoma
• Ukumelwa yintliziyo
• Ukopha emathunjini okanye i-malabsorption
• Iingxaki zombono
• Imihlathi

Nxibelelana nomboneleli wakho ukuba iimpawu ze-WM ziyavela.

Waldenström macroglobulinemia; IMacroglobulinemia - ephambili; Lymphoplasmacytic lymphoma; Imonoclonal macroglobulinemia

• Waldenström
• Iintsholongwane

UKapoor P, uAnsell SM, uFonseca R, et al. Ukuchongwa kunye nokulawulwa kweWaldenström macroglobulinemia: Mayo stratification ye-macroglobulinemia kunye nonyango olulungelelanise umngcipheko (mSMART) izikhokelo ze-2016. JAMA Oncol. Ngo-2017; 3 (9): 1257-1265. IINKCUKACHA: 28056114 pubmed.ncbi.nlm.nih.gov/28056114/.

URajkumar SV. Ukuphazamiseka kweseli yePlasma. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl. 178.

I-Treon SP, uCastillo JJ, uHunter ZR, uMerlini G. Waldenström macroglobulinemia / lymphoplasmacytic lymphoma. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 87.