I-Waldenström macroglobulinemia

I-Waldenström macroglobulinemia (WM) ingumdlavuza wama-lymphocyte B (uhlobo lweseli elimhlophe). I-WM ihlotshaniswa nokukhiqizwa ngokweqile kwamaprotheni abizwa ngama-antibody e-IgM.

I-WM ingumphumela wesimo esibizwa nge-lymphoplasmacytic lymphoma. Lona ngumdlavuza wamangqamuzana amhlophe egazi, lapho amaseli omzimba B aqala ukuhlukana ngokushesha. Imbangela ngqo yokukhiqizwa okuningi kwe-antibody ye-IgM ayaziwa. I-Hepatitis C ingandisa ingozi ye-WM. Ukuguquka kwezakhi zofuzo kuvame ukutholakala kumaseli abulalayo akwa-B.

Ukukhiqizwa kwama-antibody we-IgM amaningi kungadala izinhlobo eziningana zezinkinga:

• I-Hyperviscosity, edala ukuthi igazi likhulu kakhulu. Lokhu kungenza kube nzima ukuthi igazi ligeleze ngemithambo yegazi emincane.
• I-Neuropathy, noma ukulimala kwemizwa, lapho i-antibody ye-IgM iphendula ngezicubu zomzimba.
• I-anemia, lapho i-antibody ye-IgM ibopha kumaseli abomvu egazi.
• Isifo sezinso, lapho i-antibody ye-IgM ifaka izicubu zezinso.
• I-Cryoglobulinemia ne-vasculitis (ukuvuvukala kwemithambo yegazi) lapho i-antibody ye-IgM yakha izakhiwo zokuzivikela ezifweni ezibandayo.

I-WM ayivamile kakhulu. Iningi labantu abanalesi simo lineminyaka engaphezu kwengama-65 ubudala.

Izimpawu ze-WM zingafaka noma yikuphi okulandelayo:

• Ukopha kwezinsini namakhala ekhaleni
• Umbono ofiphele noma owehlisiwe
• Isikhumba se-Bluish eminweni ngemuva kokuchayeka kwamakhaza
• Isiyezi noma ukudideka
• Ukulinyazwa okulula kwesikhumba
• Ukukhathala
• Uhudo
• Ukuba ndikindiki, ukudikiza, noma ukuvutha kobuhlungu ezandleni, ezinyaweni, eminweni, ezinzwaneni, ezindlebeni noma ekhaleni
• I-Rash
• Izindlala ezivuvukile
• Ukwehla kwesisindo ngokungenhloso
• Ukulahleka kombono esweni elilodwa

Ukuhlolwa komzimba kungaveza ubende obuvuvukele, isibindi nama-lymph node. Ukuhlolwa kwamehlo kungakhombisa imithambo ekhulisiwe ku-retina noma ukopha kwe-retinal (hemorrhages).

I-CBC ikhombisa inani eliphansi lamaseli abomvu nama-platelets. I-chemistry yegazi ingakhombisa ubufakazi besifo sezinso.

Isivivinyo esibizwa nge-serum protein electrophoresis sikhombisa izinga lenyukile le-antibody ye-IgM. Amaleveli avame ukuba ngaphezulu kwama-milligram angama-300 nge-deciliter (mg / dL), noma i-3000 mg / L. Kuzokwenziwa ukuhlolwa kwe-immunofixation ukukhombisa ukuthi i-antibody ye-IgM itholakala ohlotsheni olulodwa lweseli, (clonal).

Ukuhlolwa kwe-serum viscosity kungatshela ukuthi igazi selishubile yini. Izimpawu zivame ukwenzeka lapho igazi liba mkhulu ngokuphindwe kane kunokujwayelekile.

I-bone marrow biopsy izokhombisa inani elandayo lamaseli angajwayelekile abukeka njengama-lymphocyte namaseli e-plasma.

Ukuhlolwa okungeziwe okungenziwa kufaka phakathi:

• Amaprotheni angamahora angama-24
• Ingqikithi yamaprotheni
• I-immunofixation emchameni
• T (thymus etholakala) ukubalwa kwe-lymphocyte
• Amathambo ama-x-ray

Abanye abantu abane-WM abaye bakhulisa ama-antibody e-IgM abanazo izimpawu. Lesi simo saziwa njenge-smoldering WM. Akukho ukwelashwa okudingekayo ngaphandle kokulandelwa ngokucophelela.

Kubantu abanezimpawu, ukwelashwa kuhlose ukunciphisa izimpawu kanye nengozi yokulimala komzimba. Akukho ukwelashwa okujwayelekile njengamanje. Umhlinzeki wakho wokunakekelwa kwezempilo angaphakamisa ukuthi ubambe iqhaza esivivinyweni somtholampilo.

I-Plasmapheresis isusa ama-antibodies e-IgM egazini. Iphinde ilawule ngokushesha izimpawu ezidalwa ukuqina kwegazi.

Imithi ingafaka i-corticosteroids, inhlanganisela yemithi yokwelapha ngamakhemikhali kanye ne-antibody monoclonal kumaseli we-B, i-rituximab.

Ukufakelwa kwe-stem cell stem kunganconywa kwabanye abantu abanempilo enhle ngenye indlela.

Abantu abanenani eliphansi lamaseli abomvu noma amhlophe egazi noma ama-platelets bangadinga ukumpontshelwa noma ama-antibiotics.

Ukusinda okujwayelekile cishe kuyiminyaka emi-5. Abanye abantu baphila iminyaka engaphezu kwengu-10.

Kwabanye abantu, lesi sifo singaveza izimpawu ezimbalwa futhi siqhubeke kancane.

Izinkinga ze-WM zingafaka:

• Izinguquko ekusebenzeni kwengqondo, okungenzeka ziholele ku-coma
• Ukwehluleka kwenhliziyo
• Ukopha emathunjini noma i-malabsorption
• Izinkinga zombono
• Isifuba

Xhumana nomhlinzeki wakho uma kuvela izimpawu ze-WM.

Waldenström macroglobulinemia; I-Macroglobulinemia - eyinhloko; Lymphoplasmacytic lymphoma; I-monoclonal macroglobulinemia

• Waldenström
• Amasosha omzimba

UKapoor P, u-Ansell SM, uFonseca R, et al. Ukuxilongwa nokuphathwa kwe-Waldenström macroglobulinemia: Mayo stratification ye-macroglobulinemia kanye nemihlahlandlela yokwelashwa eguqulelwe engozini (mSMART) 2016. JAMA Oncol. 2017; 3 (9): 1257-1265. I-PMID: 28056114 pubmed.ncbi.nlm.nih.gov/28056114/.

URajkumar SV. Ukuphazamiseka kwamangqamuzana e-Plasma. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 178.

UTreon SP, uCastillo JJ, uHunter ZR, uMerlini G. Waldenström macroglobulinemia / lymphoplasmacytic lymphoma. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 87.