Macroglobulinemia ea Waldenström, kapa WM, ke mofuta oa non-Hodgkin lymphoma e akarelletsang lisele tse hlahisang likokoana-hloko. Ka ho khetheha, lisele tse amehileng li etsa hore ho be le motho ea nang le likokoana-hloko tse ngata tse tsejoang e le immunoglobulin M kapa IgM, le "macroglobulinemia" e bolelang ho feteletseng. Le hoja e nkoa e le lymphoma, e ama haholo moko oa masapo.
WM e hlaha feela ka linyeoe tse ka bang tse tšeletseng ho batho ba limilione, 'me e lieha ho tsoela pele ho bapisoa le mekhoa e meng e mebe, empa ha e e-s'o fumane pheko.
Batho ba nang le likarolo tse sa tloaelehang tsa IgM maling a bona ba na le kotsing e ka holimo ho 46 ea ho hlahisa WM, 'me ka karolelano lilemo tsa ho hlahlojoa li bohareng ba lilemo tse 60.
Genetic Links
Ho ea ka lipatlisiso tsa morao-rao, karolo ea 90 lekholong ea batho ba nang le WM ba na le phetoho e itseng ka liphatsa tsa lefutso tse bitsoang MYD88. Ka tloaelo liphatsa tsa lefutso li thusa lisele tsa 'mele tsa ho itšireletsa mafung hore li bontsane hore li lule li le ntle, li li boloke li phela. Ho fetoha ha liphatsa tsena lefatseng lena ho ka etsa hore lisele tsa selefouno li lule li le teng, ka linako tsohle, mohlomong li lumella lisele tsa WM hore li ate. Ho na le tšepo ea hore mekhoa e mecha ea phekolo e tla ntlafatsa ka katleho ho sibolloa hona.
Phetoho e tloaelehileng ka ho fetisisa kamoo e fumanoeng ke tlhahlobo ea FISH e bile ho tlosoa, 'me e etsahala ka chromosome 6. Phetoho ena e bonoa ho batho ba ka etsang 55% ba nang le WM. Ba bangata ba nang le WM ba na le liphetoho tse ngata tsa lefutso.
Matšoao
Ba ka bang karolo ea 25 lekholong ea bakuli ha ba na matšoao leha e le afe ha ba ithuta hore ba na le WM. Empa batho ba bangata ba na le matšoao le lipontšo nakong ea ho hlahlojoa, e leng haholo-holo ka lebaka la ho bokella lisele tsa kankere mongobo kapa liprotheine tse potolohang maling.
Matšoao a tloaelehileng ke mokhathala le bofokoli ka lebaka la phokolo ea mali.
Matšoao a mang ke feberu, ho ruruha bosiu, li-lymph node tse atolositsoeng, spleen e eketsehileng le sebete, mathata a methapo kapa meriana ea boloetse ba pelo, ka nako e 'ngoe e e-na le bofokoli le ho fokotsa kapa ho otla matsohong kapa maotong. Batho ba nang le WM le bona ba ka hlalosa ho ikutloa eka ba loantša tšoaetso e ke keng ea fela.
Matšoao a khethollang a WM ke hyperviscosity e bakoang ke ho bokella liprotheine tsa Ig M maling. Lefu la Hyperviscosity le ka 'na la bonahala joaloka mokhathala, mali a sa tloaelehang, phefumoloho e khutšoanyane, hlooho ea maikutlo, ho se bone hantle habonolo (pono e fokolang), vertigo kapa liphetoho tsa boemo ba kelello (pherekano, ho lahleheloa ke mohopolo, ho sithabela).
U tšoaroe joang ho WM?
Ha ho na phekolo e tloaelehileng bakeng sa WM le joaloka lymphomas tse ling tse tlaase kapa tse "fokolang", bakuli ba se nang matšoao ka kakaretso ba hlokomeloa feela. Kalafo e itšetlehile ka mabaka a mangata a fapaneng, ka bobeli - mohlala, lilemo, bophelo bo botle - le mefuta e itseng ea mafu - mohlala, tekanyo ea tsoelo-pele, boemo ba protheine ea IgM.
Liphekolo tse ling li reretsoe ho qoba matšoao le mathata. Plasmapheresis e le kalafo e joalo. Ho hlile ho tšoana le dialysis-o kenngoa mokokotlo ho mochine o ka tlosa tse ling tsa IgM maling ho thusa ho fokotsa ho teba ha mali.
Mahlahana a mang a ikemiselitse ho boloka lisele tse sa tsoa laola li hlahloba. Lithethefatsi tsa morao-rao li kenyeletsa mahlahana a alkylating - mohlala chlorambucil le cyclophosphamide - li-nucleoside analogs - fludarabine le cladribine - rituximab ea monoclonal antibody, le proteasome inhibitor bortezomib. Likhohlano li boetse li sebelisoa.
Ka bomalimabe, ha ho e-s'o be le khetho e amohelehang ka ho khetheha ke US FDA bakeng sa phekolo ea WM. Maemong a mangata, bakuli ba nang le WM ba khothalletsoa ho nahana hore na liteko tsa litliniki e ka ba tsela e ntle ka ho fetisisa.
Ho hlasela
Khetho ea phekolo bakeng sa bakuli ba nang le lefu lena le khutlisitsoeng e akarelletsa lekhetlo le le leng la phekolo ea pele, tšebeliso ea moemeli oa mefuta e fapaneng ea li-line, kapa chemotherapy e phahameng e amanang le autologous hematopoietic cell transplantation (HCT).
Lilemong tse 'maloa tse fetileng, ho bile le tsoelo-pele ea tsebo ea saense mabapi le hore na WM e tsoela pele joang,' me mekhoa e mecha ea phekolo e bontšitsoe hore e na le tšebetso khahlanong le lisele tsa WM.
Tse ling tsa mekhatlo ena e ncha li ntlafatsa likarabo.
Litsebi tsa ho etsa lipatlisiso tlas'a liphuputso bakeng sa bakuli ba nang le boipheliso ba WM li kenyeletsa:
- Everolimus
- Perifosine
- Alemtuzumab
- Imatinib mesylate
- Panobinostat
- Ixazomib
- Oprozomib
- Obinutuzumab
- Antisense ea Bcl-2 (oblimersen, Genasense)
- Ibrutinib *
- Sildenafil
* Ka la 20 October, 2014, Janssen o phatlalalitse ho fana ka ts'ebetsong ea K'homphieutha e Ncha bakeng sa ibrutinib ho US Food and Drug Administration (FDA), e batlang tumello ea phekolo ea WM.
Ke Eng E ka 'nang ea ba Lebelong?
Kutloisiso e molemo ea biology ea lefu lena ho lebeletsoe ho khanna ntlafatso e eketsehileng.
- Genome eohle e tsamaisang liphuputso e ka thusa ho khetholla liphetoho tse itseng ka lihlotšoana tsa bakuli ba nang le WM.
- Lipatlisiso mabapi le liphetoho tsa epigenetic ho WM li ka thusa bo-rasaense ho ithuta hore na liphetoho tse itseng li ka 'na tsa etsoa joang ka katleho.
- Qetellong, tikoloho ea mofu e phetha karolo ea bohlokoa e lumella lisele tse nang le lisele hore li hōle le ho atleha, 'me bo-rasaense ba tsebe hore na tšehetso ena e ka felisoa joang.
- Immunotherapy ka lisele tsa T tse 'nileng tsa hlophisoa kapa tsa etsoa lihlaseli tsa kankere li bontšitse tšepiso ea ho phekola kankere ea mali.
Mehato e latelang
Bakeng sa tlhahisoleseling e eketsehileng ka WM, hape nahana ka libaka tse latelang:
International Waldenström's Macroglobulinemia Foundation International
http://www.iwmf.com
National Cancer Institute
http://www.cancer.gov/cancertopics/pdq/treatment/adult-non-hodgkins/Patient
> Mehloli:
> Leblebjian > H, Agarwal A, Ghobrial I. Mekhoa ea phekolo ea morao-rao bakeng sa Waldenstrom macroglobulinemia. Clinical Lymphoma, Myeloma & Leukemia. 2013; 13 Tlatsetso ea 2 >: S310-316 >.
> MYD88 L265P phetoho ea Waldenstrom macroglobulinemia.http: //www.bloodjournal.org/content/121/22/4504? Sso-checked = e le 'nete.
> Monghali-Swanepoel D, et al. Mefuta e sa tloaelehang ho 2q37.3, 8q24.21, 15q21.3 le 16q24.1 tšusumetso ea mafu a sa foleng a lefu la kankere ea lymphocytic leukemia. Tlhaho ea lefutso . 2010; 42 (2): 132-6.
> Fonseca R, Hayman S. Waldenstrom macroglobulinemia. British Journal ea Hematology. Sep 2007; 138 (6): 700-720.
> IMBRUVICA ® (ibrutinib) Tlhahiso ea Meriana e Mecha ea Lithethefatsi e fuoang US FDA bakeng sa Macroglobulinemia ea Waldenstrom. http://www.prnewswire.com/news-releases/imbruvica-ibrutinib-supplemental-new- drug-application-submitted-to-the-us-fda-for-waldenstroms-macroglobulinemia-681133482.html. Ac
> Mokhatlo oa Kankere oa Amerika. Waldenstrom Macroglobulinemia.