Gammapathy

Gammapathy

Monoclonal gammopathy (GM) imatanthauzidwa ndi kupezeka mu seramu ndi / kapena mkodzo wa monoclonal immunoglobulin. Itha kulumikizidwa ndi matenda oopsa a hemopathy, apo ayi amatchedwa monoclonal gammopathy of undetermined significance (GMSI).

Kuti muzindikire, kuyezetsa kwa tizilombo tating'onoting'ono kumapangitsa kuti tizindikire kuchuluka kwa monoclonal immunoglobulin. Mawonetseredwe azachipatala, biological and radiological angaloze ku hemopathy pomwe kuzindikira kwa GMSI ndikosiyana.

Kodi monoclonal gammopathy ndi chiyani?

Tanthauzo

Monoclonal gammopathy (GM) imatanthauzidwa ndi kupezeka mu seramu ndi / kapena mkodzo wa monoclonal immunoglobulin. Immunoglobulins ndi mapuloteni omwe ali m'madzi am'magazi a anthu omwe ali ndi chitetezo chamthupi. Amapangidwa m'maselo a plasma, maselo a lymphoid system omwe amapangidwa mu ndulu ndi ma lymph nodes. Chifukwa chake GM imachitira umboni kufalikira kwa maselo a plasma omwe amapanga monoclonal immunoglobulin.

mitundu

Ma GM amatha kugawidwa m'magulu awiri:

  • Monoclonal gammopathies yokhudzana ndi matenda a hematologic
  • Monoclonal gammopathies of undetermined significance (GMSI)

Zimayambitsa

Kwa ma monoclonal gammopathies okhudzana ndi matenda oopsa a hemopathies, zifukwa zazikulu ndi izi:

  • Multiple myeloma: chotupa cha m'mafupa opangidwa kuchokera ku kuchuluka kwa maselo achilendo a plasma
  • Macroglobulinemia (matenda a Waldenström): kupezeka kwachilendo mu plasma ya macroglobulin
  • B lymphoma

GMSI imatha kulumikizidwa ndi ma pathologies osiyanasiyana osawopsa:

  • Matenda a Autoimmune (rheumatoid polyathritis, Sjögren's syndrome, systemic lupus)
  • Matenda a virus (mononucleosis, nkhuku, HIV, hepatitis C)
  • Matenda a bakiteriya (endocarditis, osteomyelitis, chifuwa chachikulu)
  • Matenda a parasitic (leishmaniasis, malungo, toxoplasmosis)
  • Matenda osatha monga cholecystitis (kutupa kwa ndulu)
  • Zinthu zina monga banja hypercholesterolemia, matenda a Gaucher, sarcoma ya Kaposi, lichen, matenda a chiwindi, myasthenia gravis (kusokonezeka kwa kufalikira kwa mitsempha kuchokera ku mitsempha kupita ku minofu), kuchepa magazi kapena thyrotoxicosis.

matenda

GM nthawi zambiri imapezeka mwangozi, pakuyesa kwa labotale komwe kumachitika pazifukwa zina.

Kuti mudziwe kuchuluka kwa monoclonal wothandizira, mayeso othandiza kwambiri ndi awa:

  • Electrophoresis of serum proteins: njira yomwe imalola kuzindikira ndikulekanitsa mapuloteni a seramu pogwiritsa ntchito gawo lamagetsi.
  • Immunofixation: njira yololeza kuzindikira ndi kulemba kwa monoclonal immunoglobulins
  • Immunoglobulin assay: njira yomwe imalekanitsa mapuloteni kuchokera ku plasma ndikuzindikiritsa iwo potengera momwe thupi limagwirira ntchito.

Ndiye matenda amadutsa kufunafuna chifukwa cha GM. Mawonetseredwe osiyanasiyana azachipatala, biological kapena radiological ayenera kuwonetsa myeloma angapo:

  • Kuchepa thupi, kutupa mafupa ululu, pathological fractures
  • Anemia, hypercalcemia, aimpso kulephera

Mawonetseredwe ena nthawi yomweyo amawonetsa hemopathy:

  • Lymphadenopathy, splenomegaly
  • Zolakwika m'magazi: kuchepa magazi, thrombocytopenia, lymphocytosis yambiri
  • Syndrome d'hyperviscosité

GMSI imatanthauzidwa ngati GM popanda zizindikiro zachipatala kapena za labotale za matenda a hematologic. M'chizoloŵezi chachipatala, uku ndikuzindikiritsa kuti palibe. Njira zomwe zimagwiritsidwa ntchito potanthauzira GMSI ndi:

  • Monoclonal gawo mlingo <30 g / l 
  • Kukhazikika kwachibale pakapita nthawi ya gawo la monoclonal 
  • Normal seramu mlingo wa ma immunoglobulins ena
  • Kupanda kuwonongeka kwa mafupa owononga, kuchepa kwa magazi m'thupi ndi matenda a impso

Chiwopsezo cha GMSI chikuwonjezeka ndi zaka kuchokera pa 1% pazaka 25 mpaka kupitirira 5% kupitirira zaka 70.

Zizindikiro za monoclonal gammopathy

GMSI nthawi zambiri imakhala yopanda zizindikiro. Komabe, anti-monoclonal antibody imatha kumangirira ku minyewa ndikupangitsa dzanzi, kumva kuwawa, komanso kufooka. Anthu omwe ali ndi vutoli amatha kuwonongeka kwa minyewa ya fupa ndi fractures.

Pamene GM ikugwirizana ndi matenda ena, zizindikiro ndizo za matendawa.

Kuphatikiza apo, ma monoclonal immunoglobulins angayambitse zovuta zina:

  • Amyloidosis: ma depositi a zidutswa za mapuloteni a monoclonal mu ziwalo zosiyanasiyana (impso, mtima, mitsempha, chiwindi) zomwe zingakhale chifukwa cha kulephera kwa ziwalo izi.
  • Plasma hyperviscosity syndrome: imayambitsa kusokonezeka kwa masomphenya, minyewa (mutu, chizungulire, kugona, kusokonezeka kwa maso) ndi zizindikiro zamagazi.
  • Cryoglobulinemia: matenda omwe amayamba chifukwa cha kukhalapo kwa ma immunoglobulins m'magazi omwe amalowa pamene kutentha kuli pansi pa 37 °. Zitha kuyambitsa mawonetseredwe a khungu (purpura, chodabwitsa cha Raynaud, extremity necrosis), polyarthralgia, neuritis ndi glomerular nephropathies.

Chithandizo cha monoclonal gammopathy

Kwa ma IMG, palibe mankhwala omwe akulimbikitsidwa. Kafukufuku waposachedwa akuwonetsa kuti ma IMGT omwe ali ndi kuwonongeka kwa mafupa ogwirizana angapindule ndi chithandizo ndi ma bisphosphonates. Miyezi 6 mpaka 12 iliyonse, odwala ayenera kukayezetsa kuchipatala ndikuchita seramu ndi mkodzo mapuloteni electrophoresis kuti awone momwe matenda akuyendera.

Nthaŵi zina, chithandizo ndicho chimene chimayambitsa.

Pewani gammopathy ya monoclonal

Mu gawo la 25% ya milandu, kusintha kwa GMSI kupita ku matenda oopsa a hematologic kumawonedwa. Anthu omwe ali ndi GMSI amatsatiridwa ndi kuyezetsa thupi, magazi komanso nthawi zina mkodzo pafupifupi kawiri pachaka kuti awone momwe angapitirire ku matenda a khansa. Ngati matendawa apezeka msanga, zizindikiro ndi zovuta zake zitha kupewedwa kapena kuchiritsidwa msanga.

Siyani Mumakonda