Waldenstrom si Macroglobulinemia: mgbaàmà, na-akpata, prognosis

Butere n'aka ọbara ọrịa ndị obere, ma dị nnọọ ize ndụ ụmụ mmadụ. One dị otú ahụ ọrịa Vandelstrema macroglobulinemia.

Than ya e ji?

N'okpuru ọrịa a na nkà mmụta ọgwụ ọgbara na-ezo aka a butere n'aka hematological syndrome so site ọnụnọ nke ọbara macroglobulin. Nke a protein a kpụrụ site ọrụ nke monoclonal B-lymphoid sel ọbara (B-lymphocytes).

Nọmalị, ndị a na mkpụrụ ndụ na-ahụ maka njikọ nke ọbara-M globulin. Mgbe ọ bụla ọrụ nke ụmị ọkpụkpụ (mgbe na mmepe nke akpụ usoro) bụ a gbanwee amino acid mejupụtara na usoro nke mkpụrụ ndụ ihe nketa, na-eduga njikọ macroglobulin, i.e. emepe emepe Waldenstrom si macroglobulinemia.

The ọrịa bụ nnọọ obere, banyere 3 ikpe kwa nde mmadụ. Ọ na-amalite tumadi na ndị ikom n'ebe dị afọ 55 na afọ. a daa ọrịa na-fọrọ nke nta adịghị ime ruo afọ 40.

Olee, mgbe ahụ,, na-egosipụta na ọrịa na cellular na anụ ahụ larịị? Nke owuwu-emetụta na akpa ebe nakwa na dị ka a n'ihi na e nwere?

The pathogenesis nke ọrịa

Na obi patogenza ọrịa bụ a ajọ proliferation nke plasma mkpụrụ ndụ mmepụta oyiri mkpụrụ ndụ ike nke na-emepe emepe immunoglobulin M. A mkpụrụ ndụ wakporo ụmị ọkpụkpụ, imeju, splin, ebe ha na-amalite ifịk ifịk proliferate na secrete pathogenic macroglobulin. N'ihi ya na-emepe emepe Waldenstrom si macroglobulinemia.

The ìgwè pathogenic protein n'ime ọbara-eduga ná mmepe nke ya viscosity. Dị ka a n'ihi nke belata ọbara eruba ọnụego site na arịa, nke na-eduga ná ya ọnọdụ na-achọghị. Ke adianade do, iji gbochie ha ịkpa plasma coagulation ihe (n'ihi na wrapping na inactivate ha macroglobulins). N'ihi nke a akwusila nkịtị clot guzobere, nke n'ikpeazụ na-eduga ná ụba ọbara ọgbụgba.

Anya, mgbe enyocha n'okpuru a microscope, ụmị ọkpụkpụ sel, nwere ike hụrụ e Mkpokota "plazmatizirovannyh" lymphocytes, a ọnụ ọgụgụ buru ibu nke ndị tozuru okè B-lymphocytes na secreting mkpali ogbugbo nke poolu mkpụrụ ndụ. All ndị a na mkpụrụ ndụ na-egosi na e nwere a Waldenstrom macroglobulinemia.

mgbaàmà

Olee otú clinically ike-enyo enyo na nke ọrịa a?

The mbụ ọrịa ka ga-eme mkpesa na nonspecific mgbaàmà - general adịghị ike, ala-ọkwa fever maka a ogologo oge, sweating, arọ ọnwụ n'ihi na ọ dịghị ihe doro anya kpatara.

Isi mgbaàmà, na ndabere nke anyị nwere ike chee na ghọrọ usoro Vandelstrema macroglobulinemia, na-mụbaa, na-agba ọbara si imi, goms na mucous membranes. Ọtụtụ obere ugboro ugboro hụrụ subcutaneous bruises na bruises.

N'ime oge ahụ, nwere ike ịzụlite hepatosplenomegaly na lymphadenopathy (aza Lymph). Ọ dịkwa ike mmepe nke nsogbu macroglobulinemia.

Mgbe ọdịda inye adọ ọgwụ e nwere ihe ize ndụ nke a amaghị onwe-enwe ndidi, na ọbụna ọnwụ (ma na-anwụ anwụ ọrịa bụ ndị na-emekarị bụghị nke ukwuu na ihe ha na-ata ahụhụ site na Waldenstrom si macroglobulinemia). Na-akpata ọnwụ ha bụ n'ihi nke mepụtara nsogbu.

nsogbu nke ọrịa

E nwere ike mgbagwoju anya site na ọrịa?

N'ụzọ bụ isi so na-enweghị nchịkwa usoro nke obi usoro, ya bụ obere arịa - capillaries, arterioles na venules. Site na mbenata ọbara hụrụ mmepe nke retinopathy, nephropathy. Akụrụ mebiri. Na nke a ji na mmepe nke glomerular vaskụla occlusion na urolithiasis na deposition nke urate.

Macroglobulinemia Waldenstrom ji ala ọgụ na isonye opportunistic efe efe nsogbu na nchọpụta nsogbu maka kpatara ọrịa.

Dị ka progression nke usoro bụ nnọọ suppression nke fọrọ nke nta niile germs nke hematopoiesis na mmepe nke pancytopenia. Amyloidosis ji mmepe na-enweghị nchịkwa na ọnya Dị protein umeji splin ọbara arịa, nke ozokwa njọ na otú ha mkpọchị ọrụ.

nchọpụta nsogbu

Gịnị metrics ga-enyere gị na-aghọta na ghọrọ usoro macroglobulinemia?

Nke mbụ, anyị kwesịrị ntị ka CBC. Isi egosi nke ọrịa ga-amụba na erythrocyte sedimentation ọnụego na guzobe kpọmkwem "rouleaux" - ịrapagidesi nke mkpụrụ ndụ ọbara uhie. Wbc a na-egosi ọnụ ọgụgụ ka elu nke lymphocytes na analysis na ọdịdị ọmụmụ akabeghị aka leukocytes iche-iche.

Iji gosi nchoputa site eduzi immuno. Mgbe na-eduzi ọmụmụ a na-apụghị ịmata ihe ibu na ọbara immunoglobulin M

Monoclonal immunoglobulins na-kpebisiri ike na ọbara mgbe na mgbakwunye na nke kpọrọ sera.

Ọzọ, ma ọ bụghị kpọmkwem ihe mgbaàmà na-agụnye otu ikpọ imeju na splin, nchọpụta nke amyloid na biopsy kappillyarah akụrụ, nakwa dị ka mbenata ọnụ ọgụgụ nke plasma coagulation ihe (akpan akpan, na-akpata 8).

ọgwụgwọ

Ná mmalite nkebi nke ọrịa, ma ọ bụrụ na e nwere ịrịba esịtidem ngwa aka, kpọmkwem ọgwụgwọ na-adịghị gosiri. Ọtụtụ mgbe, ndị ọrịa a na-n'okpuru nlekọta nke ndị ọrụ na ndị district hematologist.

Mgbe mbụ mgbaàmà na-egosi na ọrịa - Waldenstrom macroglobulinemia, amalite kpọmkwem ọgwụ na cytostatics. Maka ọgwụgwọ eji ọgwụ ọjọọ eme otú ahụ dị ka "hlorbutin", "Cyclophosphamide". Ha nwere a cytostatic mmetụta na-ebelata ọrụ nke a ajọ mmepụta oyiri nke B-lifotsitov.

Kara Aka na akpa ebe "hlorbutin" 6 mg kwa ụbọchị ọnụ maka 3-4 izu. Amụba dose bụ fraught na mmepe nke ụmị ọkpụkpụ aplasia. Mgbe ọ gụchara na ọgwụgwọ kenyere mmezi doses nke ọgwụ (4.2 mg) kwa ụbọchị.

Ka iwelie mma ọbara rheological parameters eji plasmapheresis. plasmapheresis ọgwụgwọ a rụrụ maka ojiji nke cytotoxic ọgwụ ọjọọ. Maka iwepụ usoro e gosiri na 2 lita plasma na-enye onyinye nnọchi na ya inactivated.

mgbochi

N'ihi na ọrịa bụ butere n'aka, ọ bụ ike n'ụzọ ọ bụla na-emetụta ya mmepe, ebe ọ na-ama tọrọ ala na mkpụrụ ndụ ihe nketa. The naanị ụzọ iji zere mmepe nke a na-adọ nchoputa nke ọrịa na-ọma-mmasị plan ọgwụgwọ.

Udiozi emetụta ọrịa nwere ike ịbụ na isiokwu a ike ndụ, ịkwụsị ịṅụ sịga na mmanya, ịgba na-ese siga na-atọkwa ụtọ efere.

Ebe ọ bụ na ọrịa bụ mkpụrụ ndụ ihe nketa, ya nwere ike ịkpalite ya mmepe na ụfọdụ ọnọdụ ọjọọ - na-arụ ọrụ aniline dyes, akọwa, varnishes.

Ugboro malitere ịrịa nje nwekwara ike ịgbanwe chromosome mmadụ. Early ọgwụgwọ nke ọrịa ndị ga-enyere gbochie mgbanwe mkpụrụ ndụ ihe nketa ma gbochie mmepe nke macroglobulinemia.

Mgbochi nwere ike ekewet mara ọrịa na atụmatụ nke ọrịa na nkwalite nke ha na-eme ime a ike ndụ.

prognosis

Gịnị chere maka ọrịa ndị mepụtara Waldenstrom macroglobulinemia? Prognosis nke ọrịa na-adabere na ogo nke ọrịa na mepụtara nsogbu na-adọ ọgwụgwọ.

Ọ bụrụ na ọrịa ahụ bụ na ya na n'oge ogbo nke mmepe, ọtụtụ mgbe ọ bụ ike iji gbochie ya na-enwe ọganihu. Ndị dị otú ahụ ọrịa ọbụna nwere ohere maka a zuru ezu na-agwọ, na ojiji nke mmezi doses nke cytostatics ka nwere ike budata dịkwuo ha ndu ndimmadu.

Ọ bụ nnọọ njọ maka ndị na-mgbagwoju anya Waldenstrom si macroglobulinemia. Mgbaàmà, ndị prognosis nke nke dị oké akụda - amyloid umeji akụrụ, na-agba ọbara na Ugboro development paraproteinemic amaghị onwe ya. Ọ bụrụ na oge anaghị enyere ndị dị otú ahụ ọrịa, a elu puru nke ọnwụ.

The nkezi ndu ndimmadu maka ọrịa na ọrịa bụ banyere 4-5 afọ. Mgbe kwesịrị ekwesị na-eru elu atụmatụ nke ọgwụgwọ nwere ike na-amụba ya na 9-12 afọ.

Ize ndụ nke ọrịa

Nyere na ọrịa emee adịkarịghị, ọ bụ ike na-enyo ya nke mmepe nke a nwoke na-fọrọ nke nta mgbe zutere ya. Nyere na mbụ na kọntaktị na ọrịa obodo na-agwọ ọrịa, ọ bụ n'ubu ha, na bụ maka n'ịhụ na-adọ na-enyo enyo na chọpụtara na Waldenstrom si macroglobulinemia. Gịnị bụ ọrịa a, a ga-echeta, ọ bụghị ihe niile ndị dọkịta, Otú ọ dị, na-eche na onye ọrịa ka nyere daa ọrịa, na-echebara ya niile na-adakarị ngosipụta, ga bụla therapist.

N'ọdịnihu, a ọrịa ga-eduga hematologists, ma ihe bụ isi nchoputa dabeere nanị na n'ubu dọkịta clinics.

Mbubreyo njirimara nke ọrịa na-eduga ná oké pụta mgbe ọgwụ bụ agaghịkwa irè. Ọ bụ ya mere i kwesịrị ịma banyere ọrịa a niile ghara uche ya na-adịghị eweta na a ala nke na-eleghara anya.