Usoro ọgwụ na-emepụta ọgwụ na thrombocytopenia (CAMT) bụ otu n'ime ìgwè buru ibu nke ụbụrụ ọkpụkpụ ọkpụkpụ ọkpụkpụ, dịka Fanconi anemia ma ọ bụ dyskeratosis congenital. Ọ bụ ezie na okwu ahụ bụ ọnụ, ọ kachasị nghọta site n'ịlele okwu ọ bụla n'aha ya. Mkpuru okwu pụtara na a mụrụ onye ahụ na ọnọdụ ahụ. Thrombocytopenia bụ okwu ahụike maka ọnụ ala platelet.
Amegakaryocytic na-akọwa ihe kpatara thrombocytopenia. A na-eme platelets n'ime ụmị ọkpụkpụ site na megakaryocytes. Usoro thrombocytopenia amegakaryocytic pụtara na ọnụ ala platelet bụ nke abụọ na enweghị mgakaryocytes.
Mgbaàmà nke Thrombocytopenia Amegakaryocytic Na-ahụ Maka Ahụhụ
A na-amata ọtụtụ ndị nwere CAMT n'oge ha bụ nwata, na-adịkarị obere oge amuchara ha. Dị ka ọnọdụ ndị ọzọ na thrombocytopenia, ọbara ọgbụgba na-abụkarị ihe mgbaàmà mbụ. Ọkụ na-emekarị na akpụkpọ ahụ (a na-akpọ purport), ọnụ, imi, na eriri afọ. Ihe fọrọ nke nta ka ọ bụrụ ụmụaka nile a chọpụtara na CAMT nwere ihe nchọpụta akpụkpọ ahụ. Ihe kachasị banyere ọbara ọgbụgba bụ ọbara ọgbụgba (ụbụrụ), mana ekele na anaghị eme mgbe mgbe.
Ihe ka ọtụtụ ụbụrụ ọkpụkpụ nke ọkpụkpụ ọkpụkpụ dịka Fanconi anaemia ma ọ bụ dyskeratosis congenita nwere nsogbu ntụpọ doro anya. Ụmụaka nwere CAMT anaghị enwe nsogbu ntụpọ kpọmkwem nke metụtara ya.
Nke a nwere ike inyere aka ịmata ọdịiche dị na CAMT site na ọnọdụ ọzọ nke na-eweta mgbe a mụrụ ya na thrombocytopenia nke a na-akpọ thrombocytopenia na-enweghị radius syndrome (TARS). Ọnọdụ a nwere nnukwu thrombocytopenia, mana ọ na-eji njedebe dị mkpirikpi.
Nchoputa
Ọnụ ọgụgụ ọbara zuru ezu (CBC) bụ ụdị ọbara a na-ahụkarị mgbe mmadụ na-agba ọbara n'ihi ihe a na-amaghị.
Na CAMT, CBC na-egosi oké thrombocytopenia na platelet ọnụ na-abụkarị ihe na-erughị sel 80,000 na microliter na-enweghị anaemia (obere ọbara ọbara ọbara ọbara) ma ọ bụ mgbanwe n'ọgụ ọbara ọbara ọcha. Enwere ọtụtụ ihe mere nwatakịrị a mụrụ iji nwee thrombocytopenia nke mere na ọrụ ahụ ga-agụnye ịchịkwa ọtụtụ ọrịa ndị dị ka rubella, cytomegalovirus (CMV), na sepsis (nnukwu ọrịa nje). Thrombopoietin (nke a na-akpọkwa megakaryocytic growth and development factor) bụ protein nke na-emepụta ihe ngosi platelet. A na-ebuli ọkwa nke thrombopoietin na ndị nwere CAMT.
Mgbe enwere ihe ndị ọzọ na-akpata thrombocytopenia, a pụrụ ịchọpụta ọkpụkpụ ụbụrụ nke ọkpụkpụ iji nyochaa mmepụta platelet. Ọkpụkpụ ọkpụkpụ nke ọkpụkpụ na CAMT ga-ekpughe ihe fọrọ nke nta ka ọ bụrụ enweghị megakaryocytes, ọbara ọbara nke na-emepụta platelets. Nchikota nke ala ala platelet buru ibu na enweghi megakaryocytes bu nyocha nke CAMT. A na - eme ka CAMT site ngbanwe na ngalaba MPL (thrombopoietin receptor). A na-eketa ya na ejiji oge na-eme ka ndị nne na nna na-eburu àgwà maka nwa ha ịzụlite ọnọdụ ahụ. Ọ bụrụ na ndị nne na nna na-ebu, ha nwere 1 n'ime 4 ohere nke ịmị nwa na CAMT.
Ọ bụrụ na ịchọrọ, a ga-eziga nyocha mkpụrụ ndụ iji chọpụta ngbanwe na gene MPL, ma a gaghị achọ nyocha a iji mee nyocha.
Ọgwụgwọ
A na-eduzi ọgwụgwọ mbụ na ịkwụsị ma ọ bụ gbochie ọbara ọgbụgba na transfusion. Mgbapụta Platelet nwere ike ịdị irè dị mma, ma ihe ize ndụ na abamuru kwesịrị iji nlezianya tụlee dị ka ụfọdụ ndị na-enweta ọtụtụ okpukpu platelet nwere ike ịmepụta ọgwụ nje platelet ịbelata irè ọgwụgwọ a. Ọ bụ ezie na ụdị ọgwụgwọ nke thrombocytopenia nwere ike ịgwọ thrombopoietin, n'ihi na ndị nwere CAMT anaghị ezuru megakaryocytes iji mee platelets n'ụzọ zuru ezu, ha anaghị emeghachi omume na ọgwụgwọ a.
Ọ bụ ezie na naanị platelet na-emetụta na mbụ, na oge oge anaemia na leukopenia (obere ọbara blood count) nwere ike ịzụlite. A na-akpọlata ụdị ọkpụkpụ ọbara atọ a pancytopenia ma nwee ike ibute mmepe nke anaemia aplastic siri ike . Nke a na - emekarị n'agbata afọ 3 - 4, ma ọ nwere ike ime mgbe ọ dị nká na ụfọdụ ndị ọrịa.
Nanị ọgwụgwọ ọrịa na-eme ugbu a bụ eriri mkpụrụ (ma ọ bụ ụmị ọkpụkpụ) transplantation. Usoro a na - eji sel snoo na - enye onyinye (na - abụkarị nwanne ma ọ bụrụ na ọ dị) iji maliteghachi mkpụrụ ndụ ọbara n'ime ụmị ọkpụkpụ.
Okwu Site
Ịchọta na nwa gị nwere nsogbu ahụike na-adịghị ala ala n'oge na-adịghị anya mgbe amuchara nwa nwere ike bụrụ ihe na-agbawa obi. N'ụzọ dị mma, a pụrụ iji mmịnye nke platelet mee ihe iji gbochie njupụta ọbara ọgbụgba na nsị cell transplant nwere ike ịgwọ ọrịa. Gwa dọkịta nwa gị gbasara nchegbu gị ma jide n'aka na ị ghọtara usoro nhọrọ niile.
> Isi mmalite:
> Olson TS. Anemia aplastic e nwetara na ụmụaka na ndị na-eto eto. Na: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA.
> Ee DL. Ihe kpatara thrombocytopenia na ụmụ. Na: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA.