I-macroglobulinemia yaseWaldenström, okanye i-WM, luhlobo lwe-non-Hodgkin lymphoma equka iiseli ezivelisa abantu. Ngokukodwa, iiseli ezichaphazelekayo zenza amaninzi omzimba olwaziwa ngokuba yi-immunoglobulin M okanye i-IgM, kwaye "i-macroglobulinemia" ibhekisela kulo mfuneko. Nangona kuthathwa njenge-lymphoma, ichaphazela kakhulu umongo wethambo.
I-WM yenziwa kuphela kwiimeko ezimalunga nesithandathu ngabantu abayizigidi, kwaye ityantya ekwenzeni inkqubela ifaniswe nezinye iziganeko ezimbi, kodwa akukho nonyango.
Abantu abanamazinga aphezulu angaphantsi kwe-IgM kwigazi labo abanomngcipheko ophezulu we-WM wokuphuhlisa i-WM, kwaye umyinge oqhelekileyo wokuxilongwa uneminyaka engama-60.
Genetic Links
Ngokutsho kophando lwakutshanje, iipesenti ezingama-90 zabantu abane-WM zinokuguqulwa komzimba othile obizwa ngokuba yi-MYD88. Ngokuqhelekileyo lo mfuzo unceda amangqamuzana omzimba ukuba axelane ngomnye ukuze ahlale efanelekileyo, ahlale ephila. Ukuguqulwa komzimba kule mfuza kungabangela ukuba iselula ibe ngumtshini wokuhlala, ngexesha lonke, mhlawumbi ukuvumela iiseli ze-WM ukuba zenzeke. Kukho ithemba lokuba iindlela ezintsha zonyango ziza kuphumelela ngolu hlobo.
Utshintsho oluqhelekileyo njengoko lufunyenwe uhlalutyo lwe-FISH luye lwasuswa, kwaye lwenzeka kwi-chromosome 6. Olu tshintsho luyabonakala kwi-55% yabantu abane-WM. Abaninzi abane-WM baneenguquko ezininzi zezofuzo.
Iimpawu
Inani elinama-25 eepesenti zezigulane azikho iimpawu xa zifunda zine-WM. Kodwa abaninzi abantu banempawu kunye neempawu ngexesha lokuxilongwa, oku kubangelwa kukuqokelela kweeseli zomhlaza kwi-bone lomnatha okanye iiprotheni ezijikelezayo egazini.
Iimpawu eziqhelekileyo ziyakhathala kunye nobuthakathaka ngenxa yegazi.
Ezinye iimpawu ziyi-fever, izithukuzo zobusuku, i-lymph nodes, i-spleen kunye ne-fover, iingxaki zesibindi okanye i- peripheral neuropathy, ngamanye amaxesha unobuthathaka kunye nobunzima okanye ukubetha kwizandla okanye ezinyaweni. Abantu abane-WM nabo banokuchaza ukuvakalelwa ngathi balwa nosulelo olusayi kuhamba.
Uphawu oluphawulekayo lwe-WM luyi-hyperviscosity ebangelwa ukuqokelela kweprotein ye-Ig M egazini. I-Hyperviscosity syndrome ibonakale njengokukhathala, ukuphuma kwegazi ngokungapheliyo, ukuphefumula okufutshane, intloko yesifo, ukuphazamiseka okubonakalayo (imbonakalo ephosakeleyo), i-vertigo, okanye utshintsho kwisimo sengqondo (ukudideka, ukulahlekelwa kwememori, ukuphazamiseka).
I-WM ithathwa njani?
Akukho zonyango eziqhelekileyo ze-WM kwaye zifana nezinye i-lymphomas ezisezantsi okanye "ezigubungelayo", izigulane ezingenazo iimpawu zigcinwa kuphela. Unyango luxhomekeka kwimibandela eyahlukeneyo, bobabini ngabanye - umz., Ubudala, impilo yonke-kunye nesifo-umzekelo, izinga lokuqhubela phambili, izinga leprotheni ye-IgM.
Ezinye iindlela zonyango zijoliswe ekukhuseleni iimpawu kunye neengxaki. I-Plasmapheresis yonyango. Kancinci nje nge-dialysis-uya kufakwa kumatshini onokususa ezinye ze-IgM egazini ukwenzela ukunciphisa ubukhulu begazi.
Ezinye iinjenisi zijolise ekugcineni iiseli zangaphandle zokuhlola. Ionyango ezikhoyo ziquka ama-alkylating agents - umzekelo i-chlorambucil kunye ne-cyclophosphamide-nucleoside analogs - i-fludarabine kunye ne-cladrib - i-antioclonal antibody rituximab kunye ne-proteasome inhibitor bortezomib. Ukuhlanganiswa kusetyenziswa.
Ngelishwa, akukho ukhetho oluvunyiweyo ngqo yi-US FDA kunyango lwe-WM. Kwiimeko ezininzi, izigulane ezine-WM zikhuthazwa ukuba ziqwalasele ukuba ngaba iilingo zamakliniki zingaba yindlela efanelekileyo.
Ukubetha
Izinyango zokwelapha izigulane ezinezifo ezibuyiselwayo ziquka olunye ujikelezo lwonyango lokuqala, usebenziso lwe-ejensi yokuqala, okanye i-chemotherapy ephezulu elandelwa yi-autologous hematopoietic cell transplantation (HCT).
Kwiminyaka emininzi edlulileyo, kukho intuthuko yolwazi lwesayensi malunga nendlela i-WM ekhula ngayo, kwaye iindlela ezintsha zonyango ziye zaboniswa ukuba zinomsebenzi wokulwa neeseli zeWM.
Abanye bala majelo amatsha baphucula impendulo.
Amagosa ophando phantsi kovavanyo kwizigulane ezibuyiselwa kwi-WM ziquka:
- Everolimus
- Perifosine
- Alemtuzumab
- Imatinib mesylate
- Panobinostat
- Ixazomib
- Oprozomib
- Obinutuzumab
- I-Bcl-2 antisense (oblimersen, iGenasense)
- Ibrutinib
- Sildenafil
* Ngo-Oktobha 20, 2014, uJanssen wamemezela ukungeniswa kwesicelo esitsha sokusetyenziswa kwezidakamizwa kwi-ibrutinib kwi-US Food and Drug Administration (FDA), efuna imvume yokunyangwa kwe-WM.
Yintoni Enokuthi Ibe Ngayo?
Ukuqonda okungcono i-biology yesi sifo kulindeleke ukuba kuqhutywe phambili ukuphucula.
- I-genome yonke yokulandelelanisa izifundo kunokunceda ukuchonga utshintsho oluthile kwiinqununu zezigulane ezine-WM.
- Uphando malunga nokuguqulwa kwe-epigenetic kwi-WM lunokunceda oososayensi bafunde ukuba kunye nokuba utshintsho oluthile lunokujoliswa ngempumelelo.
- Ekugqibeleni, indawo engumongo wethambo ithatha indima ebalulekileyo ekuvumela ukuba iisilini zee-tumor zikhule kwaye ziphumelele, kwaye izazinzulu zithini ukuba zikwazi njani ukuba le nkxaso ingaqedwa.
- I-Immunotherapy esebenzisa iiseli ze-T eziye zaphinda zenziwe kwakhona okanye zenzelwe ukuhlasela iintsholongwane zomhlaza zibonise isithembiso kwonyango lwegazi lamanye.
Amanyathelo alandelayo
Ukufumana ulwazi oluthe xaxa malunga neWM, qwalasela nakwiindawo ezilandelayo:
I-International Waldenström ye-Macroglobulinemia Foundation International
http://www.iwmf.com
National Cancer Institute
http://www.cancer.gov/cancertopics/pdq/treatment/adult-non-hodgkins/Patient
> Imithombo:
> Leblebjian > H, Agarwal A, Ghobrial I. Iinketho zonyango lweNveli kwiWaldenstrom macroglobulinemia. I-Clinical Lymphoma, i-Myeloma & Leukemia. 2013; 13 iSiza 2 >: S310-316 >.
> MYD88 L265P utshintsho kwiWaldenstrom macroglobulinemia.http: //www.bloodjournal.org/content/121/22/4504? Sso-checked = nyaniso.
> Utyumba-Swanepoel D, et al. Izigaba eziqhelekileyo kwi-2q37.3, 8q24.21, 15q21.3 kunye ne-16q24.1 ithintela ingozi ye-lymphocytic leukemia engozini. Ubume bomzimba . 2010; 42 (2): 132-6.
> Fonseca R, Hayman S. Waldenstrom macroglobulinemia. IBritish Journal yeHematology. Septemba 2007; 138 (6): 700-720.
> I-IMBRUVICA ® (ibrutinib) Isicelo esongezelelweyo seMithi esitsha esithunyelwe kwi-US FDA kwi-macrolobulinemia yeWaldenstrom. http://www.prnewswire.com/news-releases/imbruvica-ibrutinib-supplemental-new-drug-application-submitted-to--da-for-waldenstroms-macroglobulinemia-681133482.html. Ac
> I-American Cancer Society. Waldenstrom Macroglobulinemia.