Macroglobulinemia ya Waldenström, kapena WM, ndi mtundu wa non-Hodgkin lymphoma umene umaphatikizapo maselo opanga tizilombo. Makamaka, maselo okhudzidwa amapanga anti-antibody wochuluka kwambiri wotchedwa immunoglobulin M kapena IgM, ndi "macroglobulinemia" amatanthauza kuchuluka kwa mankhwalawa. Ngakhale kuti imaonedwa ngati ndi lymphoma, imakhudza kwambiri mafupa.
WM amapezeka m'maola asanu ndi limodzi pa anthu miliyoni, ndipo ndizengereza kupitilira poyerekeza ndi ziwanda zambiri, koma palibe chithandizo.
Anthu omwe ali ndi chiwerengero chokwanira cha IgM m'magazi awo ali ndi chiopsezo chachikulu choposa 46 chokhala ndi WM, ndipo a zaka zapakati pa matendawa ali pakati pa zaka 60.
Genetic Links
Malinga ndi kafukufuku waposachedwapa, 90 peresenti ya anthu omwe ali ndi WM ali ndi kusintha koyenera mu jini yotchedwa MYD88. Geniyi imathandiza maselo ammadzi kuti agwirizane kuti akhalebe abwino, kuwasunga iwo amoyo. Kusintha kwa jinili kungayambitse ma selo pamsinkhu, nthawi zonse, mwinamwake kulola maselo a WM kuti afalikire. Pali chiyembekezo kuti njira zothandizira zatsopano zidzathandiza kwambiri kupeza izi.
Kusintha kwafala kwambiri monga momwe anawonetsera ndi kusanthula kwa FISH kwakhala kuchotsedwa, ndipo kumachitika pa chromosome 6. Kusintha uku kumawoneka kwa anthu 55% omwe ali ndi WM. Ambiri okhala ndi WM ali ndi kusintha kwa mitundu yambiri.
Zizindikiro
Odwala 25 pa 100 aliwonse alibe zizindikiro zirizonse akamaphunzira kuti ali ndi WM. Koma anthu ambiri ali ndi zizindikiro ndi zizindikiro panthawi ya matenda, zomwe zimakhala chifukwa cha kusungunuka kwa maselo a khansa m'magazi kapena mapuloteni ozungulira m'magazi.
Zizindikiro zofala kwambiri ndizokutopa ndi kufooka chifukwa cha kuchepa kwa magazi m'thupi.
Zilonda zina ndizo malungo, kutuluka usiku, ziwalo zolimbitsa thupi, nthenda yothamanga ndi chiwindi, mavuto a mitsempha kapena phokoso la mphuno, nthawi zina ndifooka ndi kufooka kapena kugwedeza m'manja kapena mapazi. Anthu omwe ali ndi WM angathenso kufotokoza ngati akulimbana ndi matenda omwe sangathe kuchoka.
Chizindikiro chodziwikitsa cha WM ndi kutengeka kwa thupi chifukwa cha kusungunula mapuloteni a Ig M m'magazi. Matenda osokoneza ubongo angasonyeze ngati kutopa, kutuluka kwa magazi, kupuma pang'ono, kupweteka mutu, kuwona maso (zosawona masomphenya), kutembenuka, kapena kusintha kwa maganizo (chisokonezo, kukumbukira, kusokonezeka).
Kodi Mwapitiridwa Motani?
Palibe mankhwala othandizira a WM komanso ngati ma lymphomas omwe ali ochepa kapena ochepa "," odwala omwe alibe zizindikiro amawoneka okha. Chithandizo chimadalira pazifukwa zosiyanasiyana, pokhapokha, msinkhu, umoyo wathanzi - komanso matenda - mwachitsanzo, mlingo wa kupita patsogolo, mlingo wa mapuloteni a IgM.
Mankhwala ena amapangidwa kuti athe kupewa zizindikiro ndi mavuto. Plasmapheresis ali pa chithandizo choterocho. Ndi pang'ono monga dialyse-mumagwiritsa ntchito makina omwe angathe kuchotsa zina mwa IgM kuchokera m'magazi kuti athandize kuchepa kwa magazi.
Ena amagwira ntchito pofuna kusunga maselo osadziletsa. Mankhwalawa akuphatikizapo alkylating agents - mwachitsanzo chlorambucil ndi cyclophosphamide - nucleoside analogs - fludarabine ndi cladribine - monoclonal antibody rituximab ndi proteasome inhibitor bortezomib. Kuphatikizana kumagwiritsidwanso ntchito.
Mwamwayi, pakadalibe chisankho chovomerezedwa makamaka ndi US FDA kuti chithandizo cha WM. Nthawi zambiri, odwala omwe ali ndi WM amalimbikitsidwa kuti aganizire ngati mayesero a chipatala angakhale njira yabwino kwambiri.
Kulimbana
Chithandizo cha chithandizo kwa odwala omwe ali ndi matenda opatsiranawo akuphatikizapo kachilombo koyambirira, kugwiritsa ntchito mankhwala osiyana siyana, kapena mankhwala apamwamba omwe amatsatiridwa ndi autologous hematopoietic transplantation (HCT).
M'zaka zingapo zapitazo, pakhala pali chitukuko mu chidziwitso cha sayansi momwe WM ikukhalira, ndipo njira zothandizira zatsopano zakhala zikuwonetsedwa kukhala ndi ntchito zotsutsana ndi maselo a WM.
Ena mwa atsopano atsopanowa akuyankhira mayankho.
Ofufuza omwe amaphunzira kwa odwala omwe ali ndi WM ophatikizidwanso ndi awa:
- Everolimus
- Perifosine
- Alemtuzumab
- Imatinib mesylate
- Panobinostat
- Ixazomib
- Oprozomib
- Obinutuzumab
- Ansikilizi a Bcl-2 (oblimersen, Genasense)
- Ibrutinib *
- Siligalafil
* Pa October 20, 2014, Janssen adalengeza kuti aperekedwe ku New Application drug drug kwa ibrutinib ku US Food and Drug Administration (FDA), kufunafuna chithandizo cha mankhwala a WM.
Kodi Zingakhale Zotani?
Kumvetsetsa bwino za chilengedwe cha matendawa kumayembekezeredwa kuyendetsa patsogolo.
- Mapulogalamu onse ogwiritsira ntchito malemba angathandize kuzindikira kusintha kwasintha kwa magulu a odwala omwe ali ndi WM.
- Kafukufuku wokhudza kusintha kwa epigenetic mu WM angathandize asayansi kudziwa ngati kusintha kwake kungakhale kovuta.
- Potsirizira pake, chilengedwe cha mafupa chimathandiza kwambiri kuti maselo otupa amakula ndi kupambana, ndipo asayansi amadziwa chomwe chithandizochi chingachotsedwe.
- Immunotherapy pogwiritsa ntchito maselo T omwe adakonzedwanso kapena opangidwira kuti awononge maselo a khansa yasonyeza malonjezano pochiza khansa zina zamagazi.
Zotsatira Zotsatira
Kuti mudziwe zambiri pa WM, onaninso malo awa:
International Waldenström's Macroglobulinemia Foundation International
http://www.iwmf.com
National Cancer Institute
http://www.cancer.gov/cancertopics/pdq/treatment/adult-non-hodgkins/Patient
> Zotsatira:
> Leblebjian > H, Agarwal A, Ghobrial I. Njira zamankhwala zotsatila za Waldenstrom macroglobulinemia. Clinical Lymphoma, Myeloma & Leukemia. 2013; 13 Suppl 2 >: S310-316 >.
> MYD88 L265P kusinthika mu Waldenstrom macroglobulinemia.http: //www.bloodjournal.org/content/121/22/4504? Sso-checked = true.
> Mmodzi-Swanepoel D, et al. Mitundu yosiyanasiyana pa 2q37.3, 8q24.21, 15q21.3 ndi 16q24.1 amachititsa matenda aakulu a khansa ya m'magazi. Chibadwa cha majini . 2010; 42 (2): 132-6.
> Fonseca R, Hayman S. Waldenstrom macroglobulinemia. British Journal ya Hematology. Sep 2007; 138 (6): 700-720.
> IMBRUVICA ® (ibrutinib) Ntchito Yothandizira Mankhwala Otsopano Operekedwa ku US FDA kwa Macroglobulinemia ya Waldenstrom. http://www.prnewswire.com/news-releases/imbruvica-ibrutinib-supplemental-new- drug-application-submitted-to-the-us-fda-for-waldenstroms-macroglobulinemia-681133482.html. Ac
> American Cancer Society. Waldenstrom Macroglobulinemia.