Gammapathy

Gammapathy

A na-akọwa monoclonal gammopathy (GM) site na ọnụnọ na serum na / ma ọ bụ mmamịrị nke immunoglobulin monoclonal. Enwere ike ijikọ ya na hemopathy na-adịghị mma, ma ọ bụghị ya, a na-akpọ ya monoclonal gammopathy nke ihe a na-achọpụtaghị (GMSI).

Maka nchoputa ahụ, nyocha microbiological na-eme ka o kwe omume ịchọpụta immunoglobulin monoclonal na oke ibu. Ngosipụta ụlọ ọgwụ, usoro ndu na redio nwere ike ịtu aka na hemopathy ebe nchoputa nke GMSI bụ nchoputa dị iche.

Gịnị bụ monoclonal gammopathy?

definition

A na-akọwa monoclonal gammopathy (GM) site na ọnụnọ na serum na / ma ọ bụ mmamịrị nke immunoglobulin monoclonal. Immunoglobulin bụ protein dị na plasma mmadụ nwere ihe ndị na-egbochi ya. A na-ejikọta ha na sel plasma, mkpụrụ ndụ nke usoro lymphoid nke e guzobere na splin na lymph nodes. Ya mere, GM na-agba akaebe na mmụba nke clone nke mkpụrụ ndụ plasma na-emepụta immunoglobulin monoclonal.

ụdị

Enwere ike kewaa GM n'ime ụzọ abụọ:

  • Monoclonal gammopathies metụtara hematologic malignancies
  • Monoclonal gammopathies nke a na-achọpụtaghị uru (GMSI)

Eme

Maka monoclonal gammopathies jikọtara ya na hemopathies dị njọ, isi ihe kpatara ya bụ:

  • Otutu myeloma: etuto ụmị ọkpụkpụ nke etolite site na mmụba nke mkpụrụ ndụ plasma na-adịghị mma
  • Macroglobulinemia (ọrịa Waldenström): ọnụnọ na-adịghị mma na plasma nke macroglobulin.
  • B lymphoma

Enwere ike jikọta GMSI na ọrịa dị iche iche na-adịghị njọ:

  • Ọrịa autoimmune (ọrịa rheumatoid polyathritis, ọrịa Sjögren, lupus systemic).
  • Ọrịa nje (mononucleosis, chickenpox, HIV, ịba ọcha n'anya C)
  • Ọrịa nje bacteria (endocarditis, osteomyelitis, ụkwara nta)
  • Ọrịa parasitic (leishmaniasis, ịba, toxoplasmosis)
  • Ọrịa na-adịghị ala ala dị ka cholecystitis na-adịghị ala ala (mbufụt nke gallbladder).
  • Ọnọdụ ndị ọzọ dị iche iche dị ka hypercholesterolemia ezinụlọ, ọrịa Gaucher, sarcoma Kaposi, lichen, ọrịa imeju, myasthenia gravis (ọgbaghara nke nnyefe nke mkpali akwara site na akwara na akwara), anaemia ma ọ bụ thyrotoxicosis.

diagnostic

A na-achọpụtakarị GM na mberede, n'oge ule ụlọ nyocha emere maka ihe ndị ọzọ.

Iji chọpụta ndị nnọchi anya monoclonal karịrị akarị, ule kacha baa uru bụ:

  • Electrophoresis nke protein serum: usoro na-enye ohere ịchọpụta na ikewapụ protein nke serum n'okpuru ọrụ nke mpaghara eletrik.
  • Immunofixation: usoro na-enye ohere ịchọpụta na dee immunoglobulins monoclonal
  • Immunoglobulin assay: usoro na-ekewa protein na plasma ma na-achọpụta ha na ndabere nke mmeghachi omume immunological nke a na-achọpụta.

Mgbe ahụ, nchoputa na-aga site na-achọ ihe kpatara GM. Ngosipụta ụlọ ọgwụ dị iche iche, usoro ndu ma ọ bụ redio kwesịrị ịtụ aro otutu myeloma:

  • Ịdị arọ, mgbu ọkpụkpụ na-egbuke egbuke, ọkpụkpụ pathological
  • Anaemia, hypercalcemia, ọdịda akụrụ

Ihe ngosi ndị ọzọ na-arụtụ aka ozugbo na hemopathy:

  • Lymphadenopathy, splenomegaly
  • Ọdịiche dị na ọnụọgụ ọbara: anaemia, thrombocytopenia, oke lymphocytosis
  • Syndrome d'hyperviskosité

A kọwapụtara GMSI dị ka GM na-enweghị akara ụlọ ọgwụ ma ọ bụ ụlọ nyocha ọ bụla nke malignancy hematologic. Na usoro ụlọ ọgwụ, nke a bụ nchoputa nke mwepu. Usoro eji kọwaa GMSI bụ:

  • Ọnụego akụrụngwa monoclonal <30 g / l 
  • Nkwụsi ike dị n'etiti oge nke akụkụ monoclonal 
  • Ọkwa serum nkịtị nke immunoglobulins ndị ọzọ
  • Enweghị mmebi ọkpụkpụ na-emebi emebi, anaemia na ọrịa akụrụ

Mmetụta nke GMSI na-abawanye site na 1% na afọ 25 ruo ihe karịrị 5% gafere afọ 70.

Mgbaàmà nke monoclonal gammopathy

GMSI na-abụkarị asymptomatic. Otú ọ dị, mgbochi monoclonal nwere ike jikọta na irighiri akwara ma mee ka ahụ erughị ala, nrịanrịa, na adịghị ike. Ndị nwere ọnọdụ ahụ na-enwekarị mbibi nke anụ ahụ na mgbaji.

Mgbe GM jikọtara ya na ọrịa ọzọ, ihe mgbaàmà bụ nke ọrịa ahụ.

Ọzọkwa, monoclonal immunoglobulins nwere ike ịkpata nsogbu dị ụkọ:

  • Amyloidosis: nkwụnye ego nke iberibe protein monoclonal dị iche iche (akụrụ, obi, irighiri akwara, imeju) nke nwere ike ịbụ ihe kpatara ọdịda nke akụkụ ndị a.
  • Plasma hyperviscosity syndrome: ọ na-akpata nsogbu ọhụụ, akara akwara (isi ọwụwa, dizziness, iro ụra, nsogbu ịmụrụ anya) na akara hemorrhagic.
  • Cryoglobulinemia: ọrịa nke ọnụnọ n'ime ọbara nke immunoglobulins na-akpalite mgbe okpomọkụ dị n'okpuru 37 Celsius. Ha nwere ike ime ka akpụkpọ ahụ pụta ìhè (purpura, Raynaud's phenomen, extremity necrosis), polyarthralgia, neuritis na glomerular nephropathies.

Ọgwụgwọ monoclonal gammopathy

Maka IMG, ọ nweghị ọgwụgwọ akwadoro. Nnyocha e mere n'oge na-adịbeghị anya na-egosi na IMGT nwere ọkpụkpụ ọkpụkpụ jikọtara nwere ike irite uru na ọgwụgwọ na bisphosphonates. Kwa ọnwa 6 ruo 12, ndị ọrịa kwesịrị ịga nyocha ụlọ ọgwụ ma mee serum na protein protein electrophoresis iji chọpụta ọganihu ọrịa.

N'ọnọdụ ndị ọzọ, ọgwụgwọ bụ nke kpatara ya.

Na-egbochi monoclonal gammopathy

N'ihe ruru 25% nke ikpe, a na-ahụ mgbanwe nke GMSI maka ọrịa hematologic na-adịghị mma. A na-esochi ndị nwere GMSI jiri nyocha anụ ahụ, ọbara na mgbe ụfọdụ mmamịrị ihe dị ka ugboro abụọ n'afọ iji lelee maka ọganihu enwere ike na ọnọdụ ọrịa kansa. Ọ bụrụ na achọpụtara ọganihu n'oge, enwere ike igbochi mgbaàmà na mgbagwoju anya ma ọ bụ gwọọ ya na mbụ.

Nkume a-aza