Romdị thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) bụ ọrịa ọbara nke platelet clumps na-etolite na obere arịa ọbara. Nke a na - ebute ọnụ ọgụgụ platelet dị ala (thrombocytopenia).

Ọrịa a nwere ike ibute site na nsogbu enzyme (ụdị protein) nke metụtara mkpụkọ ọbara. A na-akpọ enzyme a ADAMTS13. Enweghị enzyme a na-ebute platelet clumping. Platelet bụ akụkụ nke ọbara na-enyere aka na ịkpụkọ ọbara.

Ka platelet ahụ na-atakọkọta ọnụ, obere platelet dị n'ọbara n'akụkụ ndị ọzọ nke ahụ iji nyere aka n'ịkpụkọ. Nke a nwere ike ibute ọbara ọgbụgba n'okpuru anụ ahụ.

N'ọnọdụ ụfọdụ, ọrịa ahụ na-esite n'ezinụlọ (nke e ketara eketa). N’okwu ndị a, a mụrụ ndị nwere oke ogo nke enzyme a.

Ọnọdụ a nwekwara ike kpatara site na:

• Ọrịa cancer
• Ọgwụ
• Hematopoietic sel sel transplantation
• Nje HIV
• Ọgwụ na-agbanwe ọgwụ na estrogens
• Ọgwụ (gụnyere ticlopidine, clopidogrel, quinine, na cyclosporine A)

Mgbaàmà nwere ike ịgụnye nke ọ bụla n'ime ihe ndị a:

• Ọbara ọgbụgba n'ime akpụkpọ ahụ ma ọ bụ akpụkpọ ahụ
• Mgbagha
• Ike ọgwụgwụ, adịghị ike
• Ahụ ọkụ
• Isi ọwụwa
• Icha akpụkpọ anụ ma ọ bụ acha odo odo
• Iku ume ọkụ ọkụ
• Obi ngwa ngwa (ihe karịrị 100 na-eti kwa nkeji)

Ule ndị a pụrụ inye iwu gụnyere:

• ADAMTS 13 ọkwa ọrụ
• Bilirubin
• Smeju ọbara
• Ọnụọgụ ọbara zuru ezu (CBC)
• Creatinine larịị
• Lactate dehydrogenase (LDH) ọkwa
• Mbadamba platelet
• Urinalysis
• Haptoglobin
• Ule nwale

Nwere ike ịnwe ọgwụgwọ akpọrọ mgbanwe plasma. Ọ na - ewepu plasma gị na - adịghị mma ma dochie ya na plasma nkịtị site na onye nyere aka dị mma. Plasma bụ mmiri mmiri nke nwere mkpụrụ ndụ ọbara na platelet. Mgbanwe Plasma na-anọchi enzyme na-efu efu.

A na-eme usoro a dị ka ndị a:

• Nke mbu, i weputara obara gi dika aga enye gi obara.
• Ka ọbara na-esi n’igwe na-ekewa ọbara gaa n’akụkụ ya dị iche iche, a ga-ewepụ plasma ahụ na-adịghị mma, a na-echekwa mkpụrụ ndụ ọbara gị.
• Ejikọtara mkpụrụ ndụ ọbara gị na plasma nkịtị site na onye nyere onyinye, wee nyeghachi gị ya.

A na-emegharị ọgwụgwọ a kwa ụbọchị ruo mgbe ule ọbara na-egosi mmelite.

Ndị na-anaghị anabata ọgwụgwọ a ma ọ bụ ndị ọnọdụ ha na-alaghachikarị nwere ike:

• Mee ịwa ahụ iji wepụ splin ha
• Nweta ọgwụ na-egbochi usoro ahụ ji alụso ọrịa ọgụ, dị ka steroid ma ọ bụ rituximab

Imirikiti ndị na-enweta mgbanwe plasma na-agbake kpamkpam. Mana ụfọdụ mmadụ na-anwụ n’ọrịa a, tụmadị ma ọ bụrụ na achọpụtaghị ya ozugbo. Na ndị na-agbakeghị, ọnọdụ a nwere ike bụrụ ogologo oge (adịghị ala ala).

Nsogbu nwere ike ịgụnye:

• Akụrụ ọdịda
• Obere platelet (thrombocytopenia)
• Obere ọbara ọbara uhie (nke kpatara mkpụmkpụ nke ọbara ọbara uhie)
• Nsogbu usoro nsogbu
• Ọbara siri ike (ọbara ọgbụgba)
• Ọkụ

Kpọọ onye nlekọta ahụike gị ma ọ bụrụ na ịnwe ọbara ọgbụgba ọ bụla na-akọwaghị ya.

N'ihi na amaghi ihe kpatara ya, ọ nweghị ụzọ mara ama iji gbochie ọnọdụ a.

TTP

• Mkpụrụ ndụ ọbara

Abrams CS. Thrombocytopenia. Na: Goldman L, Schafer AI, eds. Ọgwụ Goldman-Cecil. Nke iri abụọ na ise. Philadelphia, PA: Elsevier Saunders; 2016: isi 172.

Nnukwu mkpokọta National Heart, Lung, na Blood Institute. Romdị thrombotic thrombocytopenic purpura. www.nhlbi.nih.gov/health-topics/thrombotic-thrombocytopenic-purpura. Nabata March 1, 2019.

Schneidewend R, Epperla N, Friedman KD. Thrombotic thrombocytopenic purpura na hemolytic uremic ọrịa. Na: Hoffman R, Benz EJ, Silberstein LE, et al, ndị ọzọ. Hematology: ickpụrụ na Omume. Nke 7. Philadelphia, PA: Elsevier; 2018: isi 134.