Thrombotic Thrombocytopenic Purpura: Ihe Ọ Bụ, Ihe Na-akpata na Ọgwụgwọ

Ndinaya

• Ihe na-akpata PTT
• Main ịrịba ama na mgbaàmà
• Kedu ka esi eme nchoputa
• PTT Ọgwụgwọ

Thrombotic thrombocytopenic purpura, ma ọ bụ PTT, bụ ọrịa na-arịa ọrịa hematological nke na-adịghị ahụkebe ma na-egbu egbu nke e gosipụtara site na ịmalite obere thrombi n'ime arịa ọbara na ọ na-adịkarị n'etiti ndị nọ n'agbata 20 na 40 afọ.

Na PTT enwere ọnụ ọgụgụ pụtara ìhè na ọnụ ọgụgụ platelet, na mgbakwunye na ahụ ọkụ na, n'ọtụtụ ọnọdụ, mmebi nhụjuanya n'ihi mgbanwe nke ọbara na-aga n'ụbụrụ n'ihi mkpụkọ.

Nchoputa nke PTT bu nke onye okacha mara anya ma obu onye okacha amara dika ihe nchoputa ya na ihe obula nke obara obara na ogwugwo obara na agha aghaghi amalite ya n’oge na adighi anya, ebe obu na oria a di ihe dika 95% mgbe ekwesighi.

Ihe na-akpata PTT

Ihe na - akpata Thrombotic thrombocytopenic purpura bụ isi kpatara enweghị ma ọ bụ mgbanwe mkpụrụ ndụ nke enzyme, ADAMTS 13, nke bụ ọrụ maka ịme ka ụmụ irighiri ihe nke von Willebrand pere mpe, ma na-akwado ọrụ ha. Ihe ntinye von Willebrand dị na platelet ma bụrụ ọrụ maka ịkwalite nrapado platelet na endothelium, na-ebelata na ịkwụsị ọbara ọgbụgba.

Yabụ, na enweghị enzyme ADAMTS 13, ụmụ irighiri ihe nke von Willebrand na-adịwanye ukwuu na usoro nkwụsị ọbara na-arụ ọrụ nke ọma na enwere ohere ka ukwuu nke ịkpụkọ akpụkọ.

Ya mere, PTT nwere ike ịnwe ihe nketa, nke dabara adaba ADAMTS 13, ma ọ bụ enwetara, nke bụ ndị na-eduga na mbelata ọnụ ọgụgụ platelet, dịka iji ọgwụ mgbochi ma ọ bụ ọgwụ na-agwọ ọrịa ma ọ bụ ndị na-egbochi ọrịa mgbochi, ọrịa, ụkọ nri ma ọ bụ ọrịa autoimmune, dịka ọmụmaatụ.

Main ịrịba ama na mgbaàmà

PTT na-egosipụtakarị ihe mgbaàmà na-enweghị atụ, agbanyeghị ọ bụ ndị ọrịa na-enyo enyo na PTT nwere opekata mpe 3 nke njirimara ndị a:

1. Ihe akara thrombocythemia;
2. Hemolytic anaemia, ebe ọ bụ na thrombi kpụrụ ihu ọma lysis nke mkpụrụ ndụ ọbara uhie;
3. Ahụ ọkụ;
4. Thrombosis, nke nwere ike ịpụta n'ọtụtụ akụkụ ahụ;
5. Oké mgbu mgbu n'ihi eriri afọ nke eriri afọ;
6. Mebiri emebi;
7. Mmetụta ọrịa na-adịghị mma, nke enwere ike ịghọta site na isi ọwụwa, mgbagwoju anya nke uche, ụra na ọbụna coma.

Ọ bụkwa ihe a na-ahụkarị maka ndị ọrịa a na-enyo enyo na PTT nwere ihe mgbaàmà nke thrombocytopenia, dị ka ọdịdị nke odo odo ma ọ bụ ọbara ọbara na-acha ọbara ọbara, goms ọgbụgba ma ọ bụ site na imi, na mgbakwunye na njikwa siri ike n'ịgba ọbara site na obere ọnyá. Mara ihe mgbaàmà ndị ọzọ nke thrombocytopenia.

Ọrịa na akwara na-adịghị mma bụ isi nsogbu nke PTT ma bilie mgbe obere thrombi na-egbochi nrịanrịa nke ọbara na akụrụ na ụbụrụ, nke nwere ike ịkpata ọdịda akụrụ na ọrịa strok, dịka ọmụmaatụ. Iji zere nsogbu, ọ dị mkpa na ozugbo ihe ịrịba ama mbụ pụtara, a na-akpọtụrụ onye na-eme ihe n'ozuzu ma ọ bụ ọkà mmụta ọbara ka e wee nwee ike ịmalite nyocha na ọgwụgwọ.

Kedu ka esi eme nchoputa

A na-achọpụta nchoputa nke thrombocytopenic purpura dabere na mgbaàmà nke onye ahụ gosipụtara, na mgbakwunye na nsonaazụ nke ọbara, na nke a na-ebelata ọnụ ọgụgụ platelet, nke a na-akpọ thrombocytopenia, na mgbakwunye na a hụrụ na nchịkọta nchịkọta platelet nke ọbara, nke bụ mgbe platelet na-arapara ọnụ, na mgbakwunye na schizocytes, nke bụ iberibe mkpụrụ ndụ ọbara uhie, n'ihi na mkpụrụ ndụ ọbara uhie na-agafe arịa ọbara nke obere ụgbọ mmiri na-egbochi.

Enwekwara ike inye iwu nyocha ndị ọzọ iji nyere aka nyocha nke PTT, dị ka oge ọbara ọgbụgba, nke a na-abawanye, na enweghị ma ọ bụ mbelata nke enzyme ADAMTS 13, nke bụ otu n'ime ihe kpatara mmalite nke obere thrombi.

PTT Ọgwụgwọ

A ghaghị ịmalite ọgwụgwọ maka thrombotic thrombocytopenic purpura ozugbo enwere ike, n'ihi na ọ na-egbu egbu n'ọtụtụ oge, ebe ọ bụ na thrombi e guzobere nwere ike igbochi akwara ndị ruru ụbụrụ, na-ebelata ọbara na-aga na mpaghara ahụ.

Ọgwụgwọ nke ọkachamara n'ọrịa na-ahụkarị bụ plasmapheresis, nke bụ usoro nzacha ọbara nke oke ngafe nke nwere ike ibute ọrịa a na ngafe nke von Willebrand, na mgbakwunye na nkwado nkwado, dịka hemodialysis, dịka ọmụmaatụ. , ma ọ bụrụ na enwere nsogbu. Ghọta otu esi eme plasmapheresis.

Na mgbakwunye, iji corticosteroids na ọgwụ mgbochi immunosuppressive, dịka ọmụmaatụ, enwere ike ịkwado dọkịta, iji lụso ihe kpatara PTT ọgụ ma zere nsogbu.