Macrolobulinemia ya Waldenström, kana kuti WM, inoratidzira yekuti isiri Hodgkin lymphoma iyo inosanganisira masero anogadzira antibody. Kunyanya, masero anokanganisa anoita zvakawanda zvechirwere ichi chinonzi immunoglobulin M kana IgM, uye "macroglobulinemia" inoreva kuwedzera uku. Kunyange zvazvo ichionekwa seine lymphoma, inonyanya kubata mafupa emapfupa.
WM inongowanikwa chete mumatambudziko matanhatu nemamiriyoni evanhu, uye inononoka kufambira mberi ichifananidzwa nedzimwe dzakaipa, asi hapasi mushonga.
Vanhu vane zvikwereti zvisina kukodzera zveGigM muropa ravo vane njodzi yakakwirira yepamusoro makumi mana nemakumi mana ekuvandudza WM, uye muvhareji wemakore ekuongororwa uri pakati pemakore ekuma 60s.
Genetic Links
Maererano nekutsvakurudza kwemazuva ano, 90 muzana yevanhu vane WM vane mararamiro akasiyana-siyana mune rimwe jeni rinozivikanwa se MYD88. Iri jena rinowanzobatsira masero omusununguko kuti aratidzirane kuti arambe akachengetedza zvakanaka, achiita kuti vararame. Kushandurwa kwemugeni uyu kunogona kuita kuti mavhariyoni ari pamushanduri arambe aripo, nguva dzose, zvimwe achibvumira masero eWM kuti awedzere. Pane tariro yokuti mishonga mitsva inobatsira zvakanakira izvi kuwanikwa.
Mushanduko unozivikanwa zvikuru sekuonekwa kweFISH kwave kuri kubviswa, uye kunoitika pane chromosome 6. Kuchinja uku kunowanikwa kusvika 55% yevanhu vane WM. Vakawanda vane WM vane mazamu akawanda ekuchinja.
Zviratidzo
Vose vanenge 25 muzana yevarwere havana zviratidzo kana vachidzidza kuti vane WM. Asi vanhu vazhinji vane zviratidzo uye zviratidzo panguva yekuongororwa, izvo zvinowanzokonzerwa nekuunganidza kwekenza masero mumapfupa emapfupa kana mapuroteni anopararira muropa.
Zviratidzo zvinowanzoitika ndezvekuneta uye kushaya simba nekuda kwekushaya ropa.
Zvimwe zviratidzo ndeyefivha, usiku hunoti, lymph nodes yakawedzerwa, yakawedzera spleen uye chiropa, zvinetso zvepfungwa kana chirwere chepurovhesi, dzimwe nguva uine utera uye kupera kana kumira muzvanza kana tsoka. Vanhu vane WM vanogonawo kurondedzera kunzwa sokuti vari kurwisana nehutachiona husingazoperi.
Chiratidzo chinoparadzanisa cheWM ndechekuchengetedzwa kwehutano kunokonzerwa nekuunganidza kweG Ig protein muropa. Hyperviscosity syndrome inogona kuratidzika sekuneta, kubuda ropa kusina kujairika, kuperevedza kwepfungwa, kuora musoro, kuonekwa kusingaoneki (maonero asina kunaka), vertigo, kana kuchinja mumamiriro ekufunga (kuvhiringidzika, kurasikirwa mupfungwa, kuora mwoyo).
WM Wakaitwa Sei?
Hapana mishonga inowanikwa yeWM uye semamwe mabhizimusi anotsvaga kana kuti "kusvuta", varwere vasina zviratidzo vanowanzoonekwa chete. Kurapa kunoenderana nezvimwe zvinhu zvakasiyana, zvose - semuenzaniso, zera, hutano hutano-uye zvirwere-zvakananga - semuenzaniso, chiyero chekufambira mberi, chiyero cheprotein yeGMM.
Mimwe mishonga inonangwa yekudzivirira zviratidzo uye zvinetso. Plasmapheresis iri pakurapwa kwakadaro. Icho chiduku se dialysis-unosungirirwa kumashini anogona kubvisa mamwe IgM kubva muropa kuti abatsire kuderedza kuwanda kweropa.
Vamwe vamiririri vanotarisira kuchengetedza masero ekunze kwekudzivirira. Mishonga inoshandiswa ikozvino inosanganisira zvikwereti zvinokonzerwa ne-alkylating agents-eg chlorambucil uye cyclophosphamide - nucleoside analogs - fludarabine uye cladribine - monoclonal antibody rituximab uye proteasome inhibitor bortezomib. Kusanganiswa kunoshandiswa zvakare.
Zvinosuruvarisa, hapasati sarudzo yakagamuchirwa zvakananga neU.S. FDA yekurapa kweWM. Mune mamiriro ezvinhu mazhinji, varwere vane WM vanokurudzirwa kuti vaone kana kuyerwa kwekliniki kungave nzira yakanakisisa.
Kurwisana On
Kurapa kwevarwere vane zvirwere zvakadzokorora kunosanganisira imwe nzvimbo yakatanga kurapwa, kushandiswa kwemajeri ekutanga-line, kana kuti high-dose chemotherapy yakatevera ne autologous hematopoietic cell transplantation (HCT).
Mumakore anoverengeka apfuura, kwakave nekufambira mberi mune ruzivo rwezvesayenzi pamusoro pekuti WM inokura sei, uye mishonga mitsva yakaratidzwa kuva nebasa rinopesana neWM cells.
Vamwe vevatsva vatsva vanovandudza mhinduro.
Investigational agents vari pasi pekudzidza kune varwere vane WM vakadzokera zvakare vanosanganisira:
- Everolimus
- Perifosine
- Alemtuzumab
- Imatinib mesylate
- Panobinostat
- Ixazomib
- Oprozomib
- Obinutuzumab
- Bcl-2 antisense (oblimersen, Genasense)
- Ibrutinib *
- Sildenafil
* Musi waOctober 20, 2014, Janssen akazivisa kuendeswa kwechikwata cheAddingal New Drug Application yebrutinib kuU.S. Food and Drug Administration (FDA), vachitsvirwa kugamuchirwa kweWM.
Ndezvipi Zvimwe Zvingave Zvichiitika Pakutanga?
Kunzwisisa zviri nani kwehupenyu hwechirwere kunotarisirwa kuendesa mberi nekuvandudza.
- Yese genome inoratidzira zvidzidzo inogona kubatsira kuziva matanho akasiyana-siyana mumasangano evarwere vane WM.
- Kutsvakurudza pamusoro pezvigadziriswa epigenetic muWM kunogona kubatsira masayendisiti kudzidza kuti uye sei zvimwe humwe huri kuchinja hunogona kubudirira kubudirira.
- Pakupedzisira, nzvimbo yepasina yepfupa inoita basa rinoita kuti masero emuviri ave akure uye abudirire, uye masayendisiti chii chavanoziva kuti kutsigirwa uku kunogona kubviswa sei.
- Immunotherapy kushandisa T masero akave akarongedzerwa kana kuti akagadzirirwa kurwisa masero emukenza ave akaratidza chivimbiso mukurapa mamwe marariya eropa.
Zvitsva Zvitsva
Kuti uwane mamwe mashoko pamusoro peWM, fungawo nezvemasayiti anotevera:
The International Waldenström's Macroglobulinemia Foundation International
http://www.iwmf.com
National Cancer Institute
http://www.cancer.gov/cancertopics/pdq/treatment/adult-non-hodgkins/Patient
> Sources:
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> MYD88 L265P kuchinja mutsika muWaldenstrom macroglobulinemia.http: //www.bloodjournal.org/content/121/22/4504? Sso-checked = true.
> Mamwe-Swanepoel D, et al. Zvimwe zvakasiyana-siyana pa2q37.3, 8q24.21, 15q21.3 uye 16q24.1 kuchinja chronic lymphocytic leukemia ngozi. Nature genetics . 2010; 42 (2): 132-6.
> Fonseca R, Hayman S. Waldenstrom macroglobulinemia. British Journal yeHematology. Sep 2007; 138 (6): 700-720.
> IMBRUVICA ® (ibrutinib) Supplemental New Drug Application Yakatumirwa ku US FDA ye macroglobulinemia yaWaldenstrom. http://www.prnewswire.com/news-releases/imbruvica-ibrutinib-supplemental-new- drug-application-submitted-to-the-us-fda-for-waldenstroms-macroglobulinemia-681133482.html. Ac
> America Cancer Society. Waldenstrom Macroglobulinemia.