I-macroglobulinemia kaWaldenström, noma i-WM, uhlobo lwe-non-Hodgkin lymphoma efaka amangqamuzana e-antibody-producing. Ngokuyinhloko, amaseli abathintekayo enza ama-antibody amaningi kakhulu awaziwa ngokuthi i-immunoglobulin M noma i-IgM, futhi "i-macroglobulinemia" ibhekisela kulokhu okungaphezulu. Nakuba kubhekwa njenge-lymphoma, kuhlasela kakhulu umnyofu wethambo.
I-WM ibonakala kuphela ezimweni ezingaba yisithupha kubantu abayizigidi, futhi kuyashesha ukuthuthuka uma kuqhathaniswa nezinye izinkinga eziningi ezimbi, kodwa akukakabi khona ukwelashwa.
Abantu abanezinga eliphezulu kakhulu le-IgM egazini labo abanengozi engama-46 ephakeme yokuthuthukisa i-WM, kanti isilinganiso seminyaka yokuxilongwa siseneminyaka engama-60s.
I-Genetic Links
Ngokusho kophenyo lwakamuva, abantu abangamaphesenti angama-90 abane-WM banezakhi ezithile ezithinta isakhi esibizwa ngokuthi i-MYD88. Ngokuvamile leli gciwane lisiza amangqamuzana omzimba ukuba agxilane ukuze ahlale kahle, uwagcine ephila. Ukuguqulwa kwesigcawu kungabangela ukuthi amaselula avele ekushintsheni, ngaso sonke isikhathi, mhlawumbe avumela amangqamuzana e-WM ukuba aqhubeke. Kukhona ithemba lokuthi izindlela ezintsha zokwelapha zizophumelela ngokuphumelelayo lokhu kutholakala.
Ukuguqulwa okuvame kakhulu njengoba kutholakala ukuhlaziywa kwe-FISH kuye kwasuswa, futhi kwenzeka nge-chromosome 6. Lolu shintsho lubonakala kubantu abangaba ngu-55% abanama-WM. Abaningi abane-WM banokushintshashintsha kwezakhi zofuzo eziningi.
Izimpawu
Amaphesenti angama-25 weziguli azikho izimpawu uma efunda ukuthi anama-WM. Kodwa abantu abaningi banezibonakaliso nezibonakaliso ngesikhathi sokuxilongwa, okubangelwa ukuqoqwa kwamangqamuzana omdlavuza emangeni noma amaprotheni ajikelezayo egazini.
Izimpawu ezivame kakhulu ukukhathala nokubuthakathaka ngenxa ye-anemia.
Ezinye izimpawu ziwumkhuhlane, izithukuthuku ebusuku, izilonda ezikhulisiwe, i-pleen ekhushukile nesibindi, izinkinga zezinzwa noma i- peripheral neuropathy, ngezinye izikhathi ngobuthakathaka nobunzima noma ukugoqa ezandleni noma ezinyaweni. Abantu abane-WM nabo bangase bachaze bazizwe sengathi balwa nesifo esithathele ukuphela kwesifo.
Isibonakaliso esibonakalayo se-WM yisifo sokungabi naso okubangelwa ukuqoqwa kweprotheni Ig Ig egazini. I-Hyperviscosity syndrome ingabonakalisa njengokukhathala, ukuphuma okungavamile, ukuphefumula, ukuphathwa ikhanda, ukukhubazeka okubukwayo (umbono obonakalayo), i-vertigo, noma izinguquko esimweni sengqondo (ukudideka, ukulahlekelwa inkumbulo, ukuphazamiseka).
I-WM ithathwa kanjani?
Ayikho yokwelapha ejwayelekile ye-WM futhi njengamanye ama-lymphomas aphansi noma ebhekene ne-"smoldering", iziguli ezingenayo izimpawu ngokuvamile zigcinwa kuphela. Ukwelashwa kuxhomeke ezicini eziningi ezihlukene, zombili ngabanye - isib, ubudala, impilo yonke - kanye nesifo - isib. Izinga lokuqhubeka, izinga leprotheni ye-IgM.
Ezinye zokwelapha zihlose ukugwema izimpawu nezinkinga. I-Plasmapheresis ingokwelashwa okunjalo. Kuyinto encane efana ne-dialysis-uthola isikhumba emshinini ongasusa amanye ama-IgM egazini ukuze usize ukunciphisa ubukhulu begazi.
Amanye amanxusa ahlose ukugcina amaseli angaphandle kokuhlola. Ukwelapha kwamanje kuhlanganisa ama-alkylating agents - isib chlorambucil kanye ne-cyclophosphamide-analogs - i-fludarabine kanye ne-cladrib - i-monoclonal antibody rituximab kanye ne-proteasome inhibitor bortezomib. Ukuhlanganiswa nakho kuyasetshenziswa.
Ngeshwa, akakho okwamanje okukhethwe yi-US FDA yokwelashwa kwe-WM. Ezimweni eziningi, iziguli ezine-WM zikhuthazwa ukuba zibheke ukuthi ukuhlolwa komtholampilo kungaba yini indlela engcono kakhulu.
Ukushaya
Izinketho zokwelapha iziguli ezinezifo ezibuyiselwayo zihlanganisa olunye ukwelashwa kokuqala, ukusetshenziswa kwe-ejenti ye-line yokuqala, noma i-high-dose chemotherapy elandelwa yi-autologous hematopoietic cell transplantation (HCT).
Eminyakeni embalwa edlule, kuye kwaba nentuthuko yolwazi lwesayensi mayelana nendlela i-WM eqala ngayo, futhi izindlela zokwelashwa ezintsha ziye zaboniswa ukuthi zinomsebenzi wokulwa namaseli e-WM.
Ezinye zalezi zithunywa ezintsha zithuthukisa izimpendulo.
Amaphenyo okuphenya ngaphansi kokucwaninga ngeziguli aphinde aphinde abuyele ku-WM ahlanganisa:
- Everolimus
- I-Perifosine
- Alemtuzumab
- Imatinib mesylate
- I-Panobinostat
- Ixazomib
- Oprozomib
- Obinutuzumab
- I-Bcl-2 antisense (oblimersen, i-Genasense)
- Ibrutinib *
- Sildenafil
* Ngo-Okthoba 20, 2014, uJanssen wamemezela ukuhanjiswa kwesicelo esisha seDrug Drug Application for ibrutinib e-US Food and Drug Administration (FDA), efuna imvume yokwelashwa kwe-WM.
Yikuphi Okunye Okungahle Kube Khona?
Ukuqonda okungcono kwe-biology kulesi sifo kulindeleke ukuthi kuqhubekele phambili ukuthuthukiswa.
- I-genome yonke yokulandelana kwezifundo ingasiza ekuboneni ukuguqulwa okuqondile ezinkambeni zeziguli ezine-WM.
- Ucwaningo mayelana nokuguqulwa kwe-epigenetic ku-WM kungasiza ososayensi bafunde ukuthi nokuthi izinguquko ezithile zingase zibhekwe ngempumelelo kanjani nokuthi kanjani.
- Okokugcina, imvelo yamathambo idlala indima ebalulekile evumela amangqamuzana amathumba ukuba akhule futhi aphumelele, futhi ososayensi ukuthi yini okufanele bazi ukuthi le nsizakalo ingaqedwa kanjani.
- I-Immunotherapy esebenzisa ama-T asetshenziselwe noma aqondiswa ukuhlasela amangqamuzana omdlavuza uye wabonisa isithembiso ekwelapheni kwamanye amagciwane egazi.
Izinyathelo Ezilandelayo
Ukuze uthole ukwaziswa okwengeziwe nge-WM, cabanga ngamasayithi alandelayo:
I-International Waldenström's Macroglobulinemia Foundation International
http://www.iwmf.com
I-National Cancer Institute
http://www.cancer.gov/cancertopics/pdq/treatment/adult-non-hodgkins/Patient
> Imithombo:
> Leblebjian > H, Agarwal A, Ghobrial I. Izinketho zokwelashwa eNewvel for Waldenstrom macroglobulinemia. I-Clinical Lymphoma, i-Myeloma & Leukemia. 2013; 13 Isamba 2 >: S310-316 >.
> Inguquko ye-MYD88 L265P kuWaldenstrom macroglobulinemia.http: //www.bloodjournal.org/content/121/22/4504? Sso-checked = true.
> U-Swanepoel D, et al. Ukuhluka okuvamile ku-2q37.3, 8q24.21, 15q21.3 no-16q24.1 ithonya le-lymphocytic leukemia engozini. Imvelo yemvelo . 2010; 42 (2): 132-6.
> Fonseca R, Hayman S. Waldenstrom macroglobulinemia. I-British Journal ye-Hematology. Sep 2007; 138 (6): 700-720.
> I-IMBRUVICA ® (ibrutinib) Isicelo Sokufaka Isicelo Esidakamizwa Esisha Esidluliselwe ku-US FDA ye-macroglobulinemia yeWaldenstrom. http://www.prnewswire.com/news-releases/imbruvica-ibrutinib-supplemental-new-drug-application-submitted-to-the-us-fda-for-waldenstroms-macroglobulinemia-681133482.html. I-Ac
> I-American Cancer Society. I-Waldenstrom Macroglobulinemia.