Thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) ke lefu la mali leo ho lona liplatelet clumps li hlahang methapong e menyenyane ea mali. Sena se lebisa ho palo e tlase ea platelet (thrombocytopenia).

Lefu lena le ka bakoa ke mathata a enzyme (mofuta oa protheine) e amehang ho hoamang mali. Enzyme ena e bitsoa ADAMTS13. Ho ba sieo ha enzyme ena ho fella ka ho kopana ha platelet. Liplatelete ke karolo ea mali e thusang ho koala mali.

Ha li-platelet li kopana, li-platelet tse fokolang li fumaneha maling likarolong tse ling tsa 'mele ho thusa ka ho koala. Sena se ka lebisa ho tsoa mali ka tlasa letlalo.

Maemong a mang, bothata bona bo fetisoa ke malapa (a futsitsoeng). Maemong ana, batho ba tsoaloa ba na le maemo a tlase a enzyme ena.

Boemo bona le bona bo ka bakoa ke:

• Kankere
• Chemotherapy
• Phetisetso ea hematopoietic stem cell
• Tšoaetso ea HIV
• Phekolo ea hormone ea phetoho le li-estrogen
• Meriana (ho kenyeletsoa ticlopidine, clopidogrel, quinine le cyclosporine A)

Matšoao a ka kenyelletsa efe kapa efe ea tse latelang:

• Ho tsoa mali letlalong kapa mameneng
• Pherekano
• Mokhathala, bofokoli
• Feberu
• Ho opeloa ke hlooho
• 'Mala oa letlalo le pherese kapa o mosehla
• Phefumoloho e khutšoanyane
• Ho otla ha pelo ka potlako (ho feta likotlo tse 100 ka motsotso)

Liteko tse ka laeloang li kenyelletsa:

• Boemo ba mesebetsi ea ADAMTS 13
• Bilirubin
• Ho tlotsa mali
• Palo e felletseng ea mali (CBC)
• Boemo ba Creatinine
• Lebelo la Lactate dehydrogenase (LDH)
• Palo ea liplatelete
• Ho hlahloba urinal
• Haptoglobin
• Teko ea li-coombs

U kanna ua ba le kalafo e bitsoang phapanyetsano ea plasma. E tlosa plasma ea hau e sa tloaelehang ebe e e nkela plasma e tloaelehileng ho mofani ea phetseng hantle. Plasma ke karolo e mokelikeli ea mali e nang le lisele tsa mali le liplatelete. Phapanyetsano ea plasma e boetse e nkela enzyme e lahlehileng sebaka.

Tsamaiso e etsoa ka tsela e latelang:

• Ntlha ea pele, u hula mali a hau joalokaha eka u fana ka mali.
• Ha mali a fetisoa ka mochini o arolang mali likarolo tsa oona tse fapaneng, lero la mali le sa tloaelehang lea tlosoa ebe lisele tsa hao tsa mali lia bolokoa.
• Lisele tsa hau tsa mali li kopantsoe le plasma e tloaelehileng ho mofani, ebe li khutlisetsoa ho uena.

Kalafo ena e phetoa letsatsi le letsatsi ho fihlela liteko tsa mali li bontša ntlafatso.

Batho ba sa arabeleng kalafo ena kapa bao maemo a bona a khutlang khafetsa ba ka hloka ho:

• Etsa opereishene ho tlosa spleen ea bona
• Fumana meriana e hatellang sesole sa 'mele, joalo ka li-steroid kapa rituximab

Boholo ba batho ba kenang phapanyetsong ea plasma ba fola ka botlalo. Empa batho ba bang ba bolaoa ke lefu lena, haholo haeba le sa fumanoe hang-hang. Ho batho ba sa foleng, boemo bona bo ka ba nako e telele (bo sa foleng).

Mathata a ka kenyelletsa:

• Ho hloleha ha liphio
• Palo e tlase ea platelet (thrombocytopenia)
• Palo e khubelu ea lisele tse khubelu tsa mali (e bakoang ke ho senyeha pele ho nako ha lisele tse khubelu tsa mali)
• Mathata a tsamaiso ea methapo
• Ho tsoa mali a mangata (hemorrhage)
• Leqeba

Bitsa mofani oa hao oa tlhokomelo ea bophelo haeba u tsoa mali a sa hlalosehe.

Hobane sesosa ha se tsejoe, ha ho na mokhoa o tsebahalang oa ho thibela boemo bona.

TTP

• Lisele tsa mali

Abrams CS. Thrombocytopenia. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: khaolo ea 172.

Webosaete ea National Heart, Lung le Blood Institute. Thrombotic thrombocytopenic purpura. www.nhlbi.nih.gov/health-topics/thrombotic-thrombocytopenic-purpura. E fihliloe ka la 1 Hlakubele 2019.

Schneidewend R, Epperla N, Friedman KD. Thrombotic thrombocytopenic purpura le hemolytic uremic syndrome. Ka: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Melao-motheo le Tloaelo ea Motheo. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 134.