Waldenström's macroglobulinemia, ama WM, waa nooca non-Hodgkin lymphoma oo ku lug leh unugyada soo saara unugyada. Gaar ahaan, unugyada ay saameeyeen waxay sameeyaan waxyaabo badan oo ka mid ah unugyada loo yaqaan 'immunoglobulin M' ama IgM, iyo "macroglobulinemia" waxa loola jeedaa xadigaas. Inkasta oo loo tixgeliyo lymphoma, waxay badanaa saameeysaa dhuuxa lafta.
WM waxay ku dhacdaa qiyaastii lix xaaladood halkii malyan oo qof, waana ay gaabis u tahay horumarka marka loo eego cuduro kale oo badan, laakiin weli ma jirto daawo.
Dadka qaba heerarka aan caadiga ahayn ee IgM ee dhiigooda waxay leeyihiin 46-kiiba halis dheeraad ah oo ah inay ku dhacdo WM, iyo celceliska da'da cudurka lagu ogaado waa bartamihii 60aad.
Xidhiidhada Hiddaha
Sida laga soo xigtay cilmi-baaristii dhawayd, boqolkiiba 90 dadka qaba WM waxay leeyihiin isbeddel gaar ah oo ku saabsan hiddaha loo yaqaan MYD88. Hivkani caadi ahaan wuxuu ka caawiyaa unugyada difaaca jirka si uu u muujiyo midba midka kale si uu ugu noolaado qaab wanaagsan, isaga oo nooleynaya. Isku-bedelashada hiddo-gashigan waxaa laga yaabaa in ay sababto unugyada taleefanka si ay u joogaan, mar kasta, laga yaabo inay u oggolaadaan unugyada WM in ay sii kordhaan. Waxaa jira rajo laga qabo in daaweyn cusubi ay si wax ku ool ah uga faa'iidaysato helitaankan.
Isbedelka ugu caansan sida lagu ogaado falanqaynta FISH ayaa la tirtiray, waxayna ku dhacdaa koromosoom 6. Isbadalkan waxaa lagu arkay 55% dadka WM. Qaar badan oo leh WM waxay leeyihiin isku-dhafan kala duwan.
Astaamaha
Qiyaas ahaan boqolkiiba 25 dadka bukaanka ah ma laha astaamo marka ay bartaan inay haystaan WM. Laakiin dadka intooda ugu badani waxay leeyihiin astaamo iyo calaamado xilliga cudurka, taaso badanaa waxaa sabab u ah unugyada kansarka ee lafaha lafaha ama borotiinada wareega dhiigga.
Astaamaha ugu caansan waa daal iyo daciifnimo sababta oo ah dhiig-yaraanta.
Calaamadaha kale waa qandho, dhidid habeenkii ah, qanjidhada casriga ah, maqaarka iyo beerka, dhibaatooyinka dareemayaasha ama neuropathy xannuunsan, marmarka qaarkood oo daciif ah iyo kabuubyo ama xoqid gacmaha ama lugaha. Dadka qaba WM waxay sidoo kale sharxi karaan dareenka sida ay ula dagaallamayaan cudurka oo kaliya ma tagi doono.
Calaamadaha kala duwan ee WM waa ba'an xumada ay keento sumoobidda borotiinka 'Ig M' ee dhiigga. Cudurka hyperviscosity syndrome wuxuu u muuqan karaa daal, dhiig-bax aan caadi ahayn, neefta oo gaabisa, madax-xanuun, arag-darro arag-arag ah (aragga cillad-darrada ah), vertigo, ama isbedelka xaaladda maskaxda (jah wareer, xusuus-darro, xusuusin la'aan).
Sidee WM Daaweyneysaa?
Ma jiro daweyn caadi ah oo loogu talagalay WM iyo sida lafdhabarka kale ee hooseeya ama "smoldering lymphomas", bukaanada aan lahayn calaamadaha ayaa guud ahaan la arkay. Daaweyntu waxay kuxirantahay arrimo badan oo kala duwan, labadaba - sida, da'da, caafimaadka guud - iyo cudur-gaar - tusaale, heerka uureysiga, heerka unugyada IgM.
Daawooyinka qaarkood waxaa loogu talagalay inay ka fogaadaan calaamadaha iyo dhibaatooyinka. Plasmapheresis waxay ku jirtaa daaweyntaas. Waa wax yar oo la mid ah sifeynta - waxaad ku xiran tahay mashiinka kaas oo ka saari kara qaar ka mid ah IgM dhiiga si loo yareeyo dhumucda dhiigga.
Qaar ka mid ah wakiilada ayaa ujeedadoodu tahay inay ilaaliyaan unugyada ka baxsan xakameynta jeegga. Daawooyinka hadda jira waxaa ka mid ah walxaha alkyling - sida chlorambucil iyo cyclophosphamide - analogs-nucleoside - fludarabine iyo cladribine - rituximab antibody monoclonal iyo bortezomib inhibitor proteasome. Isku darka ayaa sidoo kale loo isticmaalaa.
Nasiib darro, ma jiro wali doorasho gaar ah oo ay ansixisay FDA-da Mareykanka ee daaweynta WM. Xaalado badan, bukaanka leh WM waxaa lagu dhiirigelinayaa inay tixgeliyaan in tijaabooyinka kiliinikada ay noqon karaan waddada ugu fiican.
Dagaalka On
Fursadaha daaweynta ee bukaanka qaba cudurka dib u noqoshada waxaa ka mid ah wareega kale ee daaweynta bilowga ah, isticmaalka siyaabaha kale ee hore, ama daaweynta qotodheerta dheer oo ay ku xigto unugyada hormoonka ah ee unugyada dhiig-mareenka (hematopoietic transplantation) (HCT).
Sanadihii ugu dambeeyay, waxaa jiray horumarin aqoonta sayniska ee ku saabsan sida loo horumariyo WM, iyo daaweynta cusub ayaa lagu muujiyay in ay leeyihiin waxqabadyo ka dhan ah unugyada WM.
Qaar ka mid ah wakiilada cusub waxay hagaajiyaan jawaabaha.
Wakiilada baadhitaanka ah ee laga wado waxbarashada dadka bukaanka ah ee WM ku soo noqotey waxaa ka mid ah:
- Everolimus
- Perifosine
- Alemtuzumab
- Imatinib mesylate
- Panobinostat
- Ixazomib
- Oprozomib
- Obinutuzumab
- Baaskiilka 2-aad ee Bcl-2 (Kiimiko, Genasense)
- Ibrutinib *
- Sildenafil
* Oktoobar 20-keedii, 2014, Janssen ayaa ku dhawaaqay soo gudbinta Codsi Cusub oo Cusub oo cusub oo loogu talagalay mid ka mid ah Maamulka Cusub ee Maamulka Cuntada iyo Dawooyinka ee Maraykanka (FDA), isagoo raadinaya oggolaanshaha daaweynta WM.
Maxay Else Ka Jirto U Horoon?
Fahfin fiican oo ku saabsan bayoolajiyada cudurka ayaa la filayaa in ay wado hagaajin dheeraad ah.
- Daraasooyinka isdaba-saarida ee genome waxay kaa caawin karaan inaad ogaato isku-dhafnaanta gaarka ah ee koox-hoosaadyada bukaanada leh WM.
- Cilmi-baaris ku saabsan isbeddelada epigenetic ee WM ayaa ka caawin kara saynisyahannada inay bartaan in iyo sida isbeddelada qaarkood lagu beegsan karo si guul leh.
- Ugu dambeyntii, jawiga lafaha lafaha ayaa kaalin muhiim ah ka ciyaara unugyada burooyinka si ay u koraan uguna barwaaqoobaan, iyo saynisyahanno waxay ogaanayaan sida taageeradaas loo tirtiri lahaa.
- Immunotherapy ayadoo la adeegsanayo unugyo T-ka oo dib loo habeeyay ama loo qorsheeyay in ay weeraraan unugyada kansarku waxay muujiyeen ballan qaadka daaweynta kansarka dhiigga qaarkood.
Talaabooyinka Xiga
Wixii macluumaad dheeraad ah oo ku saabsan WM, sidoo kale tixgelin goobaha soo socda:
International Waldenström Foundation Macroglobulinemia International
http://www.iwmf.com
Machadka Kansarka Qaranka
http://www.cancer.gov/cancertopics/pdq/treatment/adult-non-hodgkins/Patient
> Ilo:
> Leblebjian > H, Agarwal A, Gobrial I. Doorashooyinka daweynta ee loo yaqaan 'Waldenstrom macroglobulinemia'. Lymphoma, Myeloma & Leukemia. 2013; 13 Qalabka 2 >: S310-316 >.
> MYD88 L265P isbeddel ku yaal Waldenstrom macroglobulinemia.http: //www.bloodjournal.org/content/121/22/4504? Sasser-checked = true.
> Crowther-Swanepoel D, et al. Noocyada caadiga ah ee 2q37.3, 8q24.21, 15q21.3 iyo 16q24.1 saameynaya halista lymphocytic leukemia. Hiddaha dabiiciga ah . 2010; 42 (2): 132-6.
> Fonseca R, Hayman S. Waldenstrom macroglobulinemia. British Journal of Hematology. Sep 2007; 138 (6): 700-720.
> IMBRUVICA ® (ibrutinib) Codsi Dheeraad ah oo Dheeraad ah oo loo soo gudbiyay FDA ee loogu talagalay macruufulinemia Waldenstrom. http://www.prnewswire.com/news-releases/imbruvica-helutinib-supplemental-new-drug-application-submitted-to-the-us-fda-for-waldenstroms-macroglobulinemia-681133482.html. Ac
> Bulshada Mareykanka ee Kansarka. Waldenstrom Macroglobulinemia.