| Abstract | Imunotrombocitopenija (ITP), se definira kao broj trombocita manji od 100 x 109/L, uz isključenje sekundarnih uzroka trombocitopenije. Neki čimbenici rizika za razvoj ITP su virusne infekcije, infekcija bakterijom Helicobacter pylori, drugi autoimuni poremećajii lijekovima poput heparina i kininskih alkaloida. Klinička slika je izrazito varijabilna te uključuje simptome poput mukokutanih krvarenja i umora, dok je nekolicina bolesnika u potpunosti asimptomatska. ITP se smatra hemoragijskom i tromboembolijskom bolešću, s incidencijom arterijske tromboze u rasponu od 1 do 2,8%. Po trajanju bolesti dijeli se na akutnu, perzistentnu i kroničnu ITP. U dijagnostičkom algoritmu potrebno je isključiti sekundarne uzroke bolesti. ITP je dijagnoza isključenja i ne postoji "zlatni standard" za postavljanje dijagnoze. Najuvjerljiviji dokaz prisutnosti bolesti je odgovor na specifičnu terapiju za ITP. U prvu liniju liječenja bolesnika s ITP spadaju glukokortikoidi poput deksametazona, metilprednizolona i prednizona te intravenski imunoglobulini.
Kortikosteroidi povećavaju adipocitnu hipertrofiju unutar kosti, te mogu dovesti do ekstravaskularne kompresije nutritivnih žila epifize i metadijafize. Trombofilni učinak bolesti i terapija kortikosteroidima povećavaju rizik od aseptične nekroze (ASN) kostiju. MR je najosjetljivija i najspecifičnija dijagnostička metoda za ASN. MR omogućuje najraniju dijagnozu i treba se naručiti kod sumnje na ASN ako je rendgenski nalaz normalan.
ASN u bolesnika s ITP je rijetka ali izrazito debilitirajuća pojava. ASN najčešće zahvaća proksimalne epifize femura, međutim opisani su i slučajevi ASN koljena i ramena. Postoje značajni izazovi u liječenju bolesnika s ASN i ITP. Rezultati konzervativnih ortopedskih zahvata i artroplastika pokazali su se zadovoljavajući u tih bolesnika, međutim nužna je adekvatna hematološka skrb u perioperacijskom periodu. Nužno je pratiti razinu trombocita prije zahvata, tokom i u poslijeoperacijskom periodu i prema njoj prilagođavati terapiju. |
| Abstract (english) | Immune thrombocytopenia (ITP) is defined as a platelet count below 100 x 109/L, with secondary causes of thrombocytopenia being excluded. Some risk factors for the development of ITP include viral infections, Helicobacter pylori bacterial infection, other autoimmune disorders, and medications such as heparin and quinine alkaloids. The clinical presentation is highly variable and includes symptoms such as mucocutaneous bleeding and fatigue, while some patients are completely asymptomatic. ITP is considered both a hemorrhagic and thromboembolic disease, with an incidence of arterial thrombosis ranging from 1 to 2.8%. Depending on the duration of the disease, ITP is classified as acute, persistent, or chronic. In the diagnostic algorithm, secondary causes of the disease should be excluded. ITP is a diagnosis of exclusion, and there is no "gold standard" for its diagnosis. The most compelling evidence for its presence is the response to specific ITP therapy. Glucocorticoids such as dexamethasone, methylprednisolone, and prednisone, as well as ntravenous immunoglobulins, are included in the first-line treatment of patients with ITP.
Corticosteroids increase the hypertrophy of adipocytes within the bone, which can lead to extravascular compression of the epiphyseal and metaphyseal nutrient vessels. The thrombophilic effect of the disease and corticosteroid therapy increase the risk of aseptic necrosis (ASN) of the bone. Magnetic resonance imaging is the most sensitive and specific diagnostic method for ASN. MRI enables the earliest diagnosis and should be ordered when ASN is suspected if the X-ray findings are normal.
ASN in patients with ITP is a rare but extremely debilitating event. ASN most commonly affects the proximal epiphysis of the femur, but cases of ASN have also been described affecting the knee and shoulder. There are significant challenges in the treatment of patients with ASN and ITP. Conservative orthopedic surgeries and joint arthroplasty have shown satisfactory results in these patients, but adequate hematological care is necessary in the perioperative period. It is necessary to monitor platelet levels before, during, and after the orthopedic procedure and adjust the therapy accordingly. |
