Trombotik trombositopenik Purpura Tanılı Hastalarımızın Klinik, Laboratuvar ve Tedavi Sonuçlarının Değerlendirilmesi

Öz

Trombotik trombositopenik purpura (TTP) klinik olarak ADAMTS-13 proteazının eksik aktivitesinden kaynaklanır. TTP’nin klasik pentadı mikroanjiyopatik hemolitik anemi (MAHA), trombositopeni, nörolojik bulgular, ateş ve böbrek yetmezliğidir. Tedavide plazma değişimi (PD), kortikosteroidler, rituksimab kullanılmakta ve son yıllarda kaplasizumab tedavi kombinasyonunda yer almaktadır. Çalışmamızın amacı yılda milyonda 3,7 ile 11 arasında yeni vakanın görüldüğü TTP hastalığına ait gerçek yaşam verilerine ulaşıp sonuçları tespit ederek literatürdeki az sayıdaki gerçek yaşam verilerine ait çalışmalar ile karşılaştırıp literatüre katkı sağlamaktır. Çalışmamızda hastanemizin İç Hastalıkları ABD, Hematoloji BD’da 1.Ocak.2008-1.Ocak.2023 tarihleri arasında 18 yaş ve üzerinde iken immün (i) TTP tanısı konulan 31 hastanın klinik ve laboratuvar bulguları ile tedavi sonuçları retrospektif olarak değerlendirildi. 31 hastanın 7’si erkek (%22,6) idi. Ortalama yaş 45,13 ±19,07 (16-83) yıl idi. Hastaların tamamında MAHA ve trombositopeni tespit edildi. Tedavide tüm hastalara PD uygulandı. Hastaneye başvurudan sonra PD’ye başlama süresi 24 (12-24) saat idi. Hastaların 16’sında (%57,1) 1.basamak tedaviye direnç görülmezken 12’sinde (%42,9) direnç mevcuttu. Direnç durumunda hastaların 10’una (%83,3) 4 doz 375 mg/m2/hafta rituksimab tedavisi verildi. Refrakter 12 hasta (%42,9) ile non-refrakter 16 hasta (%57,7) arasında yapılan değerlendirme sonucunda tanı anındaki total bilirubin (p=0.019) ve direkt bilirubin (p=0.031) değerleri arasında anlamlı fark saptandı. Mortal 7 hasta (%22,6) ile mortal seyretmeyen 24 hasta (%77,4) arasındaki değerlendirme sonucunda tanı anındaki yaş açısından anlamlı fark saptandı (p=0.034). Sonuç olarak TTP’nin kadınlarda erkeklere göre daha sık izlendiği MAHA ile trombositopeninin tanıda olmazsa olmaz bulgular olduğu; tanı için verilerin kapsamlı analizinin gerektiği saptandı. Plazma ADisintegrin and Metalloprotease with a ThromboSpondin type 1 motif, member 13 (ADAMTS-13) aktivite testi tanının doğrulanmasında yardımcı olup TTP tedavisinin köşe taşını PD oluşturmaktadır.

Anahtar Kelimeler

• Trombotik trombositopenik purpura
• terapötik plazma değişimi
• hemoliz

Evaluation Of Clinical, Laboratory and Treatment Results Of Our Patients With Thrombotic Thrombocytopenic Purpura.

Öz

Thrombotic thrombocytopenic purpura (TTP) is clinically caused by severely deficient activity of the ADAMTS-13 protease. The classical pentad of TTP, microangiopathic hemolytic anemia (MAHA), is thrombocytopenia, neurologic findings, fever, and renal failure. Plasma exchange (PEX), corticosteroids, rituximab are used in the treatment and in recent years, caplasizumab has been included in the treatment combination. The aim of our study is to contribute to the literature by reaching the real-life data of TTP disease, which has between 3.7 and 11 new cases per million, and by determining the results and comparing them with the studies of real-life data in the literature. In our study, clinical and laboratory findings and treatment results of 31 patients who were diagnosed with immune (i) TTP when they were 18 years of age or older between 1.Jan.2008 and 1.Jan.2023 in hospital. Department of Internal Medicine, Department of Hematology were evaluated retrospectively. Of 31 patients, 7 (22.6%) were male. The mean age of the patients at the time of diagnosis was 45.13 ±19.07 (16-83) years. MAHA and thrombocytopenia were detected in all patients. In the treatment, PEX was applied to all patients. The time to start PEX after admission to the hospital was 24 (12-24) hours. While 16 (57.1%) of the patients did not show resistance, 12 (42.9%) patients had resistance. In case of resistance, 4 doses of 375 mg/m2/week rituximab treatment were given to 10 (83.3%) of the patients. As a result of the evaluation made between 12 refractory patients (42.9%) and 16 non-refractory patients (57.7%), a significant difference was found between total bilirubin (p=0.019) and direct bilirubin (p=0.031) values at the time of diagnosis. As a result of the evaluation between 7 mortal patients (22.6%) and 24 non-mortal patients (77.4%), a difference was found in terms of age at the time of diagnosis (p=0.034). In conclusion, TTP is seen more frequently in women than in men, and MAHA thrombocytopenia is an indispensable finding in the diagnosis, and comprehensive analysis of the data is required for diagnosis. The plasma ADisintegrin and Metalloprotease with a ThromboSpondin type 1 motif, member 13 (ADAMTS-13) activity test is helpful in confirming the diagnosis. PEX is the basis of TTP treatment.

Anahtar Kelimeler

• Thrombotic thrombocytopenic purpura
• therapeutic plasma exchange
• hemolysis

Kaynakça

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