Thrombotic thrombocytopenic purpura

I-Thrombotic thrombocytopenic purpura (TTP) sisifo segazi apho iiplatelet clumps zenza kwimithambo yegazi encinci. Oku kukhokelela kwinani eliphantsi leplatelet (thrombocytopenia).

Esi sifo sinokubangelwa ziingxaki ze-enzyme (uhlobo lweprotein) oluchaphazelekayo ekunqandeni igazi. Le enzyme ibizwa ngokuba yiADAMTS13. Ukungabikho kwale enzyme kubangela ukuqhekeka kweplatelet. Iiplatelets ziyinxalenye yegazi encedisa ekunqandeni igazi.

Njengoko iiplatelets zidibana, zimbalwa iiplatelets ezikhoyo egazini kwamanye amalungu omzimba ukunceda ekunqandeni. Oku kunokukhokelela ekuphumeni kwegazi phantsi kwesikhumba.

Ngamanye amaxesha, ingxaki idluliswa kwiintsapho (ezizuzwe njengelifa). Kule meko, abantu bazalwa benamanqanaba asezantsi ale enzyme.

Le meko nayo inokubangelwa:

• Umhlaza
• Unyango ngamayeza
• Ukufakelwa kweseli ye-hematopoietic stem
• Usulelo lwe-HIV
• Unyango olungena endaweni yeHormone kunye nee-estrogens
• Amayeza (kubandakanya i-ticlopidine, i-clopidogrel, i-quinine, kunye ne-cyclosporine A)

Iimpawu zinokubandakanya nayiphi na kwezi zinto zilandelayo:

• Ukopha kulusu okanye inwebu yencindi
• Ukudideka
• Ukudinwa, ubuthathaka
• Ifiva
• Intloko ebuhlungu
• Umbala wesikhumba omdaka okanye umbala omthubi
• Ukuqhawukelwa ngumphefumlo
• Ukukhawuleza kwentliziyo (ngaphezulu kwe-100 beats ngomzuzu)

Uvavanyo olunoku-odolwa lubandakanya:

• IADAMTSI 13 inqanaba lomsebenzi
• Bilirubin
• Igazi smear
• Gcwalisa ubalo lwegazi (CBC)
• Inqanaba le-Creatinine
• Inqanaba leLactate dehydrogenase (LDH)
• Ubalo lweplatelet
• Uhlalutyo lomchamo
• IHaptoglobin
• Uvavanyo lweCombombs

Unokuba nonyango olubizwa ngokuba yi-plasma exchange. Isusa iplasma yakho engaqhelekanga kwaye iyitshintshe ibe yiplasma eqhelekileyo kumnikeli ophilileyo. IPlasma yinxalenye engamanzi yegazi equlethe iiseli zegazi kunye neeplatelets. Utshintshiselwano ngeplasma luthatha indawo ye-enzyme engekhoyo.

Inkqubo yenziwa ngolu hlobo lulandelayo:

• Kuqala, utsale igazi lakho ngokungathi unikela ngegazi.
• Njengoko igazi lidlula kumatshini owahlula igazi kwiindawo zalo ezahlukileyo, iplasma engaqhelekanga iyasuswa kwaye iiseli zakho zegazi ziyagcinwa.
• Iiseli zakho zegazi emva koko zidityaniswe neplasma eqhelekileyo evela kulowo unikeleyo, emva koko zibuyiselwe kuwe.

Olu nyango luyaphindaphindwa mihla le de uvavanyo lwegazi lubonakalise ukuphucuka.

Abantu abangayiphenduliyo le nyango okanye imeko yabo ibuya rhoqo kungafuneka:

• Yenza utyando ukuze ususe udakada
• Fumana amayeza acinezela amajoni omzimba, anje ngee-steroids okanye i-rituximab

Uninzi lwabantu abangena kutshintshiselwano ngeplasma bachacha ngokupheleleyo. Kodwa abanye abantu bayabulawa sesi sifo, ngakumbi ukuba asinakufunyanwa kwangoko. Kubantu abangafumaniyo, le meko inokuba yeyethuba elide (engapheliyo).

Iingxaki zinokubandakanya:

• Ukungaphumeleli kwezintso
• Inani eliphantsi leplatelet (thrombocytopenia)
• Inani elibomvu leeseli ezibomvu (kubangelwa kukuwohloka kwangoko kweeseli ezibomvu zegazi)
• Iingxaki zenkqubo
• Ukopha kakhulu (ukopha)
• Ukubetha

Fowunela umboneleli wakho wokhathalelo lwempilo ukuba unayo nayiphi na into eyophayo echazayo.

Kuba unobangela awaziwa, akukho ndlela yaziwayo yokuthintela le meko.

TTP

• Iiseli zegazi

Abrams CS. I-Thrombocytopenia. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Umhla wama-25. IPhiladelphia, PA: Elsevier Saunders; Ngo-2016: isahluko 172.

I-National Heart, Lung, kunye newebhusayithi yeZiko leGazi. Thrombotic thrombocytopenic purpura. www.nhlbi.nih.gov/health-topics/thrombotic-thrombocytopenic-purpura. Kufumaneka ngo-Matshi 1, 2019.

USchneidewend R, uEpperla N, uFriedman KD. I-thrombotic thrombocytopenic purpura kunye ne-hemolytic uremic syndrome. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 134.