Gammapathy

Gammapathy

Monoclonal gammopathy (GM) waxaa lagu qeexaa joogitaanka serum iyo/ama kaadida immunoglobulin-ka monoclonal. Waxaa lala xiriirin karaa dhiig-bax xun, haddii kale waxaa loo yaqaan monoclonal gammopathy ee muhiimadda aan la go'aamin (GMSI).

Si loo ogaado cudurka, baaritaannada microbiological waxay suurtogal ka dhigayaan in la aqoonsado monoclonal immunoglobulin ee xad-dhaafka ah. Calaamadaha kiliinikada, bayoolojiga iyo shucaaca waxay tilmaami karaan dhiig-baxa halka ogaanshaha GMSI uu yahay ogaanshaha kala duwan.

Waa maxay gammopathy monoclonal?

Qeexid

Monoclonal gammopathy (GM) waxaa lagu qeexaa joogitaanka serum iyo/ama kaadida immunoglobulin-ka monoclonal. Immunoglobulin waa borotiinno ku jira balasmaha dadka kuwaas oo leh sifooyin difaac. Waxay ku dhex abuurmaan unugyada balasmaha, unugyada nidaamka lymphoid ee lagu sameeyay beeryarada iyo qanjidhada. GM sidaas darteed waxay ka marag kacaysaa badinta unugyada balasmaha ee soo saara monoclonal immunoglobulin.

Noocyada

GM-yada waxaa loo kala saari karaa 2 qaybood:

  • Gammopathies monoclonal ee la xidhiidha malignancies hematologic
  • Gammopathies monoclonal gammopathies ee muhiimadda aan la go'aamin (GMSI)

Sababaha

Gammopathies monoclonal ee la xidhiidha hemopathies malignant, sababaha ugu waaweyn waa:

  • Multiple myeloma: buro dhuuxa lafta ah oo ka dhalatay kororka unugyada balaasmaha ee aan caadiga ahayn
  • Macroglobulinemia (Cudurka Waldenström): joogitaanka xaddi aan caadi ahayn ee balasmaha macroglobulin
  • B lymphoma

GMSI waxa lala xidhiidhin karaa cuduro kala duwan oo aan halis ahayn:

  • Cudurada difaaca jirka (rheumatoid polyathritis, Sjögren's syndrome, lupus systemic)
  • Caabuqyada fayraska (mononucleosis, busbus, HIV, cagaarshow C)
  • Caabuqyada bakteeriyada (endocarditis, osteomyelitis, tuberculosis)
  • Caabuqyada dulin (leishmaniasis, malaria, toxoplasmosis)
  • Cudurada daba-dheeraada sida cholecystitis-ka daba-dheeraada (bararka xameetida)
  • Xaalado kale oo kala duwan sida hypercholesterolemia qoyska, cudurka Gaucher, Kaposi's sarcoma, lichen, cudurka beerka, myasthenia gravis ( khalkhalka gudbinta dareenka dareemayaasha ee dareemayaasha ilaa muruqa), dhiig-yarida ama thyrotoxicosis

cudurka

GM waxaa badanaa lagu ogaadaa si kadis ah, inta lagu jiro shaybaarka shaybaarka oo lagu sameeyay sababo kale.

Si loo aqoonsado wakiilka aadka u badan ee monoclonal, imtixaanada ugu faa'iido badan waa:

  • Electrophoresis ee borotiinka serum: farsamo u oggolaanaysa in la aqoonsado oo la kala saaro borotiinnada serum ee hoos yimaada ficilka beer koronto
  • Immunofixation: waa farsamo u oggolaanaysa ogaanshaha iyo qoritaanka immunoglobulins monoclonal
  • Immunoglobulin assay: waa hab ka sooca borotiinnada balasmaha oo lagu gartaa iyada oo lagu salaynayo falcelinta difaaca jirka ee la ogaan karo

Kadibna cilad-sheegashadu waxay maraysaa raadinta sababta GM. Xaalado caafimaad oo kala duwan, bayooloji ama shucaac ah ayaa soo jeedin kara myeloma badan:

  • Miisaanka oo yaraada, xanuunka bararka ee lafaha, jabka cudurada
  • Dhiig-yaraan, hypercalcemia, kelyaha oo hawlgab

Calaamadaha kale waxay isla markiiba tilmaamayaan dhiig-baxa:

  • Lymphadenopathy, splenomegaly
  • Waxyaalaha aan caadiga ahayn ee tirada dhiigga: dhiig-yarida, thrombocytopenia, lymphocytosis xad-dhaaf ah
  • Syndrome d'hyperviscosité

GMSI waxaa lagu qeexaa sida GM iyada oo aan lahayn wax calaamado caafimaad ama shaybaar ah oo ah malignant hematologic. Jadwalka joogtada ah ee kiliinikada, tani waa ogaanshaha ka saarida. Shuruudaha loo isticmaalo in lagu qeexo GMSI waa:

  • Heerka ka kooban monoclonal <30 g / l 
  • Deganaanshiyaha qaraabada ah ee wakhtiga ka kooban qaybta monoclonal 
  • Heerka serum caadiga ah ee immunoglobulins kale
  • Maqnaanshaha burburka lafaha, dhiig-yarida iyo cilladda kelyaha

Dhacdooyinka GMSI waxay ku kordhaan da'da laga bilaabo 1% 25 sano ilaa in ka badan 5% wixii ka dambeeya 70 sano.

Calaamadaha gammopathy monoclonal

GMSI inta badan waa asymptomatic. Si kastaba ha ahaatee, antibody-ka monoclonal wuxuu ku xidhi karaa neerfayaasha wuxuuna sababi karaa kabuubyo, xiirid, iyo daciifnimo. Dadka qaba xaaladdan waxay aad ugu dhow yihiin inay burburaan unugyada lafaha iyo jabka.

Marka GM lala xiriiriyo cudur kale, calaamaduhu waa kuwa cudurka.

Intaa waxaa dheer, monoclonal immunoglobulins waxay keeni kartaa dhibaatooyin dhif ah:

  • Amyloidosis: kaydka jajabyada borotiinka monoclonal ee xubnaha kala duwan ( kelyaha, wadnaha, neerfaha, beerka) taas oo noqon karta sababta fashilka xubnahaas.
  • Plasma hyperviscosity syndrome: waxay mas'uul ka tahay cilladaha aragga, calaamadaha neerfaha (madax-xanuun, dawakhaad, lulmo, cillado feejignaan) iyo calaamadaha dhiigbaxa
  • Cryoglobulinemia: cudurada ay keento joogitaanka dhiigga ee immunoglobulins kuwaas oo soo dada marka heerkulku ka hooseeyo 37 °. Waxay keeni karaan maqaarka maqaarka (purpura, ifafaale Raynaud, necrosis xag jirka ah), polyarthralgia, neuritis iyo nephropathies glomerular.

Daawaynta monoclonal gammopathy

IMG-yada, wax daaweyn ah laguma talinayo. Daraasadihii ugu dambeeyay waxay muujinayaan in IMGT-yada leh lafo luminta la xidhiidha laga yaabo inay ka faa'iidaystaan ​​daaweynta bifosphonates. 6 ilaa 12 bilood kasta, bukaanku waa in ay maraan baaritaan caafimaad oo ay sameeyaan serum iyo protein borotiinka kaadida si loo qiimeeyo horumarka cudurka.

Xaalado kale, daaweyntu waa tan sababta.

Ka hortagga gammopathy monoclonal

Qiyaas ahaan ilaa 25% kiisaska, horumarka GMSI ee ku wajahan cudurka dhiig-baxa ee xun ayaa la arkay. Dadka qaba GMSI waxaa lagu raacaa baaritaan jireed, dhiig iyo marmar kaadi qiyaastii laba jeer sannadkii si loo hubiyo horumarka suurtagalka ah ee xaalad kansar ah. Haddii horumarka hore loo ogaado, calaamadaha iyo dhibaatooyinka waa laga hortagi karaa ama hore ayaa loo daweyn karaa.

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