Waldenström macroglobulinemia

Waldenström macroglobulinemia (WM) ndi khansa ya ma B lymphocyte (mtundu wamaselo oyera amwazi). WM imagwirizanitsidwa ndi kuchuluka kwa mapuloteni otchedwa IgM antibodies.

WM ndi chifukwa cha matenda otchedwa lymphoplasmacytic lymphoma. Ichi ndi khansa yamagazi oyera, momwe maselo amthupi a B amayamba kugawanika mwachangu. Zomwe zimayambitsa kupanga kwambiri mankhwala a IgM sizikudziwika. Hepatitis C imatha kuwonjezera ngozi ya WM. Kusintha kwa majini kumapezeka m'maselo oopsa a B.

Kupanga ma antibodies owonjezera a IgM kumatha kuyambitsa mitundu ingapo yamavuto:

• Hyperviscosity, yomwe imapangitsa kuti magazi akule kwambiri. Izi zitha kupangitsa kuti kukhale kovuta kuti magazi azidutsa m'mitsempha yaying'ono.
• Matenda a m'mimba, kapena kuwonongeka kwa mitsempha, anti-IgM ikachita ndi mitsempha.
• Kuchepa kwa magazi m'thupi, anti-IgM ikamangirirana ndi maselo ofiira.
• Matenda a impso, anti-IgM ikamayika mu minofu ya impso.
• Cryoglobulinemia ndi vasculitis (kutupa kwa mitsempha ya magazi) pamene anti-IgM imapanga malo achitetezo amthupi ozizira.

WM ndiyosowa kwambiri. Anthu ambiri omwe ali ndi vutoli ali ndi zaka zopitilira 65.

Zizindikiro za WM zitha kuphatikizira izi:

• Kutuluka magazi m'kamwa ndi m'mphuno
• Maso osawona kapena ochepa
• Khungu labuluu m'zala mutatha kuzizira
• Chizungulire kapena kusokonezeka
• Kuvulaza kosavuta kwa khungu
• Kutopa
• Kutsekula m'mimba
• Kunjenjemera, kumva kulasalasa, kapena kupweteka m'mimba, mapazi, zala, zala zakumapazi, makutu, kapena mphuno
• Kutupa
• Zotupa zotupa
• Kuchepetsa mwangozi
• Kutaya masomphenya m'diso limodzi

Kuyezetsa thupi kumatha kuwonetsa nthenda zotupa, chiwindi, ndi ma lymph node. Kuyezetsa diso kumatha kuwonetsa mitsempha yotakuka m'maso kapena m'maso (zotaya magazi).

CBC imawonetsa kuchuluka kwama cell ofiira ndi ma platelets. Chemistry yamagazi imatha kuwonetsa umboni wa matenda a impso.

Chiyeso chotchedwa serum protein electrophoresis chikuwonetsa kuchuluka kwa anti-IgM. Mulingo nthawi zambiri umakhala wokwera kuposa mamiligalamu 300 pa deciliter (mg / dL), kapena 3000 mg / L. Kuyesedwa kwa ma immunofixation kudzachitika kuwonetsa kuti antibody wa IgM amachokera ku mtundu umodzi wa cell, (clonal).

Kuyezetsa magazi kwa seramu kumatha kudziwa ngati magazi akula. Zizindikiro nthawi zambiri zimachitika magazi akakhala owirikiza kanayi kuposa momwe zimakhalira.

Mafupa a m'mafupa adzawonetsa kuchuluka kwa maselo osadziwika omwe amawoneka ngati ma lymphocyte ndi maselo am'magazi.

Mayeso owonjezera omwe angachitike ndi awa:

• Mapuloteni a maola 24 a mkodzo
• Mapuloteni onse
• Kuthamangitsidwa kwa mkodzo mumkodzo
• T (thymus yotengedwa) kuchuluka kwa ma lymphocyte
• Mafupa x-ray

Anthu ena omwe ali ndi WM omwe awonjezera ma antibodies a IgM alibe zizindikiro. Vutoli limadziwika kuti WM yoyaka. Palibe chithandizo chofunikira kupatula kutsatira mosamalitsa.

Mwa anthu omwe ali ndi zizindikilo, chithandizo chimalimbikitsa kuchepetsa zizindikilozo komanso chiwopsezo chotenga ziwalo zosawonongeka. Palibe chithandizo chamakono chamakono. Wothandizira zaumoyo wanu angakuuzeni kuti mutenge nawo mbali pazachipatala.

Plasmapheresis amachotsa ma antibodies a IgM m'magazi. Imayang'aniranso mwachangu zomwe zimayambitsa kukhathamira kwa magazi.

Mankhwala atha kuphatikizira corticosteroids, kuphatikiza mankhwala a chemotherapy ndi anti monoclonal antiB B, rituximab.

Autologous stem cell cell ingalimbikitsidwe kwa anthu ena omwe ali ndi thanzi labwino.

Anthu omwe ali ndi kuchuluka kochepa kwa maselo ofiira kapena oyera kapena magazi am'magazi angafunike kuthiridwa magazi kapena maantibayotiki.

Ambiri amakhala ndi zaka pafupifupi 5. Anthu ena amakhala zaka zoposa 10.

Kwa anthu ena, vutoli limatha kukhala ndi zizindikilo zochepa ndikukula pang'onopang'ono.

Zovuta za WM zitha kuphatikiza:

• Kusintha kwa magwiridwe antchito, mwina kumabweretsa chikomokere
• Mtima kulephera
• Kutuluka m'mimba kapena malabsorption
• Mavuto masomphenya
• Ming'oma

Lumikizanani ndi omwe amakupatsani ngati zizindikiro za WM zikuyamba.

Waldenström macroglobulinemia; Macroglobulinemia - pulayimale; Lymphoplasmacytic lymphoma; Monoclonal macroglobulinemia

• Waldenström
• Ma antibodies

Kapoor P, Ansell SM, Fonseca R, ndi al. Kuzindikira ndikuwunika kwa Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) malangizo 2016. JAMA Oncol. 2017; 3 (9): 1257-1265. PMID: 28056114 pubed.ncbi.nlm.nih.gov/28056114/.

Rajkumar SV. Matenda a m'magazi. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 178.

Treon SP, Castillo JJ, Hunter ZR, Merlini G. Waldenström macroglobulinemia / lymphoplasmacytic lymphoma. Mu: Hoffman R, Benz EJ, Silberstein LE, et al, olemba. Hematology: Mfundo Zoyambira ndi Zochita. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 87.