Gammapathy

Gammapathy

Monoclonal gammopathy (GM) an bayyana shi ta kasancewar a cikin jini da / ko fitsari na immunoglobulin monoclonal. Ana iya danganta shi da cutar ciwon daji, in ba haka ba ana kiransa monoclonal gammopathy na mahimmancin da ba a tantance ba (GMSI).

Don ganewar asali, gwaje-gwajen microbiological suna ba da damar gano immunoglobulin monoclonal a cikin yawan yawa. Bayyanar cututtuka na asibiti, ilimin halitta da na rediyo na iya nuna ciwon jini yayin da ganewar GMSI shine ganewar asali.

Menene monoclonal gammopathy?

definition

Monoclonal gammopathy (GM) an bayyana shi ta kasancewar a cikin jini da / ko fitsari na immunoglobulin monoclonal. Immunoglobulin sunadaran sunadarai ne a cikin plasma na ɗan adam waɗanda ke da kaddarorin rigakafi. An haɗa su a cikin ƙwayoyin plasma, sel na tsarin tsarin lymphoid da aka kafa a cikin ƙwayoyin cuta da ƙwayoyin lymph. GM don haka ya shaida yaduwar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar cuta.

iri

Ana iya rarraba GMs zuwa kashi 2:

  • Monoclonal gammopathies hade da hematologic malignancies
  • Monoclonal gammopathies na mahimmancin da ba a tantance ba (GMSI)

Sanadin

Gamopathies monoclonal gammopathies da ke da alaƙa da m hemopathies, manyan dalilai sune:

  • Multiple myeloma: kumburin kasusuwan kasusuwa da aka samu daga yaɗuwar ƙwayoyin plasma mara kyau
  • Macroglobulinemia (Cutar Waldenström): kasancewar a cikin ƙananan adadin a cikin plasma na macroglobulin.
  • B lymphoma

GMSI na iya haɗawa da nau'o'in pathologies marasa lahani:

  • Autoimmune cututtuka (rheumatoid polyathritis, Sjögren ta ciwo, tsarin lupus).
  • Kwayoyin cututtuka (mononucleosis, chickenpox, HIV, hepatitis C).
  • Kwayoyin cututtuka (endocarditis, osteomyelitis, tarin fuka)
  • cututtuka na parasitic (leishmaniasis, malaria, toxoplasmosis)
  • Cututtuka na yau da kullun kamar na kullum cholecystitis (kumburi na gallbladder)
  • Wasu yanayi daban-daban irin su hypercholesterolemia na iyali, cutar Gaucher, sarcoma na Kaposi, lichen, cutar hanta, myasthenia gravis (rashin watsawar jijiya daga jijiya zuwa tsoka), anemia ko thyrotoxicosis.

bincike

Ana gano GM sau da yawa ba zato ba tsammani, yayin gwaje-gwajen dakin gwaje-gwaje da aka yi don wasu dalilai.

Don gano babban wakili na monoclonal, gwaje-gwaje mafi amfani sune:

  • Electrophoresis na sunadarai na jini: dabarar da ke ba da damar ganowa da raba sunadaran jini a ƙarƙashin aikin filin lantarki.
  • Immunofixation: dabarar da ke ba da damar ganowa da buga immunoglobulins monoclonal
  • Immunoglobulin assay: tsari ne wanda ke raba sunadaran daga plasma kuma yana gano su akan abubuwan da ake iya ganowa na rigakafi da suke samarwa.

Sa'an nan kuma ganewar asali ya bi ta hanyar neman dalilin GM. Daban-daban na asibiti, nazarin halittu ko bayyanar cututtuka ya kamata su ba da shawarar myeloma da yawa:

  • Rage nauyi, kumburin kasusuwa, raunin ƙwayoyin cuta
  • Anemia, hypercalcemia, gazawar koda

Sauran bayyanar cututtuka nan da nan suna nuna ciwon jini:

  • Lymphadenopathy, splenomegaly
  • Rashin daidaituwa a cikin adadin jini: anemia, thrombocytopenia, lymphocytosis mai yawa
  • Syndrome d'hyperviskosité

GMSI an ayyana shi azaman GM ba tare da alamun asibiti ko dakin gwaje-gwaje na malignancy ba. A cikin aikin yau da kullun na asibiti, wannan shine ganewar asali na keɓewa. Sharuɗɗan da aka yi amfani da su don ayyana GMSI sune:

  • Matsakaicin bangaren monoclonal <30 g / l 
  • Dangantakar kwanciyar hankali akan lokaci na bangaren monoclonal 
  • Matsayin jinin al'ada na sauran immunoglobulins
  • Rashin lalacewar kashi mai lalacewa, anemia da rashin lafiyar koda

Abubuwan da ke faruwa na GMSI yana ƙaruwa da shekaru daga 1% a shekaru 25 zuwa fiye da 5% bayan shekaru 70.

Alamomin gammopathy na monoclonal

GMSI yawanci asymptomatic ne. Duk da haka, maganin rigakafi na monoclonal zai iya ɗaure ga jijiyoyi kuma ya haifar da raguwa, tingling, da rauni. Mutanen da ke da yanayin sun fi samun lalacewa na nama na kashi da karaya.

Lokacin da GM ke hade da wata cuta, alamun cututtuka sune na cutar.

Bugu da ƙari kuma, monoclonal immunoglobulins na iya haifar da ƙananan rikitarwa:

  • Amyloidosis: adibas na gutsuttsura sunadaran monoclonal a cikin gabobin daban-daban (koda, zuciya, jijiyoyi, hanta) wanda zai iya zama sanadin gazawar wadannan gabobin.
  • Plasma hyperviscosity ciwo: yana da alhakin matsalolin hangen nesa, alamun neurological (ciwon kai, dizziness, barci, rashin barci) da alamun jini.
  • Cryoglobulinemia: cututtuka da ke haifar da kasancewar a cikin jinin immunoglobulins wanda ke hazo lokacin da zafin jiki ya kasa 37 °. Suna iya haifar da bayyanar fata (purpura, sabon abu na Raynaud, necrosis extremity), polyarthralgia, neuritis da glomerular nephropathies.

Monoclonal gammopathy jiyya

Don IMGs, ba a ba da shawarar magani ba. Nazarin kwanan nan ya nuna cewa IMGTs tare da asarar kashi mai alaƙa na iya amfana daga jiyya tare da bisphosphonates. Kowane watanni 6 zuwa 12, marasa lafiya ya kamata su yi gwajin asibiti kuma suyi electrophoresis na serum da fitsari don tantance ci gaban cutar.

A wasu lokuta, magani shine dalilin.

Hana monoclonal gammopathy

A cikin adadin har zuwa 25% na lokuta, ana lura da juyin halitta na GMSI zuwa mummunan cutar jini. Mutanen da ke da GMSI ana biye da su tare da gwajin jiki, jini da wasu lokuta na fitsari kimanin sau biyu a shekara don bincika yiwuwar ci gaba zuwa yanayin ciwon daji. Idan an gano ci gaba da wuri, ana iya hana bayyanar cututtuka da rikitarwa ko kuma a magance su da wuri.

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