Türkiye’de Batı Akdeniz Bölgesindeki Edinsel Trombotik Trombositopenik Purpura Hastalarının Klinik Özellikleri ve Relaps/Refrakter Hastalık Risk Faktörleri

Year 2024, Volume: 46 Issue: 4, 618 - 626, 16.07.2024

Ünal Ataş , Sevgi Gülşen , Lütfullah Zahit Koç , Orhan Kemal Yücel , Utku Iltar , Ozan Salim , Erdal Kurtoğlu , Levent Ündar , Volkan Karakuş

https://doi.org/10.20515/otd.1478471

Abstract

Güncel yaklaşımlarla yanıt ve genel sağkalım oranları %90’ın üzerine çıkan trombotik trombositopenik purpurada (TTP), relapsı öngördürücü ve engelleyici parametrelerin önemi artmıştır. Bu açıdan Batı Akdeniz bölgesindeki immun TTP (iTTP) hastalarının klinik prezentasyonlarını, laboratuvar bulgularını, tedavilerini, tedavi yanıtlarını, relaps/refrakter hastalık durumlarını ve genel sağkalım oranlarını inceledik. Son 10 yılda tanı almış, iTTP tanılı 35 erişkin hasta çalışmaya dahil edildi. Hastaların ortanca takip süresi 46 (2-118) ay olup, 32 hasta (%91.4) hayattaydı. Birinci basamak tedavide 20 (%57.1) hastada klinik remisyon sağlanırken, relaps/refrakter hastalık nedeniyle ikinci sıra tedavi verilen 21 hastanın 20 sinde klinik remisyon sağlanmıştı. Birinci basamakta sadece 4 hastada kullanılan rituksimab ikinci sıra tedavi alan 14 hastaya verilmişti. Relapslar nedeni ile 5 hasta üç basamak, 2 hasta ise dört basamak tedavi almıştı. Yaş, cinsiyet, klinik prezentasyon, laboratuvar bulguları ve plazmaferez sayısı ile hem ADAMTS13 inhibitör düzeyleri hem de relaps/refrakter hastalık arasında bir ilişki yoktu. Geçmişte yaş, ADAMTS13 aktivasyonunun düşüklüğü, yüksek laktat dehidrogenaz gibi bazı parametreler prognostik olarak bildirilse de, %90’ın üzerinde yanıt ve genel sağkalım sağlayan güncel tedavi yaklaşımları ile bu değerlendirmelerin yeniden ele alınması gerektiğini düşünüyoruz. Çalışmamızda hem relaps/refrakter hastalık öngördürücü bir faktör hem de ADAMTS13 inhibitör düzeyinin etkilediği bir klinik yansıma saptamadık.

Keywords

İmmun Trombotik Trombositopenik Purpura, von Willebrand Faktör, ADAMSTS13

Features and Relapse/Refractory Disease Risk Factors of Patients with Acquired Thrombotic Thrombocytopenic Purpura in the Western Mediterranean Region of Turkey

Abstract

The importance of parameters that predict and prevent relapse has increased in thrombotic thrombocytopenic purpura (TTP), where response and overall survival rates exceed 90% with current approaches. In this respect, we examined the clinical presentations, laboratory findings, treatments, treatment responses, states of relapsed/refractory disease and overall survival rates of immune-mediated TTP (iTTP) patients in the western Mediterranean region. 35 adult patients who were diagnosed with iTTP in the last 10 years were included in the study. The median follow-up period of the patients was 46 (2-118) months, and 32 patients (91.4%) survived. While clinical remission was achieved in 20 (57.1%) patients in the first-line treatment group, clinical remission was achieved in 20 of 21 patients who received second-line treatment due to relapsed/refractory disease. Rituximab, which was used as the first-line treatment in only 4 patients, was given to 14 patients as the second-line treatment. Due to relapse, 5 patients received third-line treatment, and 2 patients received fourth-line treatment. There was no relationship between age, sex, clinical presentation, laboratory findings, the number of plasmapheresis treatments, and either ADAMTS13 inhibitor levels or relapsed/refractory disease. Although several parameters, such as age, low ADAMTS13 activation, and high lactate dehydrogenase, have been reported to be prognostic in the past, we believe that these findings should be reconsidered with current treatment approaches that provide a greater than 90% response and overall survival. In our study, we did not detect either a predictive factor for relapsed/refractory disease or a clinical indicator influenced by ADAMTS13 inhibitor levels.

Keywords

Immune Thrombotic Thrombocytopenic Purpura, von Willebrand Factor, ADAMSTS13

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