Clinical follow-up and treatment response of the 162 patients with chronic idiopathic thrombocytopenic purpura

Yüz altmış iki kronik idiyopatik trombositopenik purpura olgusunun klinik seyir ve tedavi cevabı yönünden değerlendirilmesi

Diz Reyhan KÜÇÜKKAYA, Meliha NALÇACI, Melih AKTAN, Tanju ATAMER, Deniz SARGIN, Yüksel PEKÇELEN, Kalayoğlu Sevgi BEŞIŞIK, Mesut AYER, Günçay DİNÇOL, Demirel Naciye YILDIRIM, M ustsfs Nuri YENEREL, Akif Selim YAVUZ

Abstract

Aim: In this report, we investigated the presenting clinical features, therapy indications, clinical follow-up and treatment results of the 162 patients with chronic idiopathic thrombocytopenic purpura (ITP). Material and methods: The cases that were followed-up in our outpatient clinic between years 1972 to 2003 were retrospectively analyzed.Results: A hundred and twenty-two of the patients were female, 40 of them were male and their age distribution was between 12 and 84 years (median: 35 years). Prednisolone (1mg/kg) was the first line treatment option in 138 of the patients; 24 of them was followed-up without any treatment. Complete response was achieved in 64 (46,3 %) of the patients. Platelet counts decreased to the beginning level in 26 of the complete responders when the PRD doses were decreased. Eleven of the complete responders showed relapse in between 6 months to 4 years. Platelet counts were found in the range of 5.000/mm3 to 125.000/mm3 in the patients who did not get complete response. There was not any serious bleeding problem other than bruising in none of the patients including those who had severe trombocytopenia. None of our patients died because of bleeding complication during the follow-up period.Conclusion: ITP is an autoimmune disorder characterized by muco-cutaneous bleeding. The pathogenesis of ITP is presumed to be related to platelet destruction via specific auto-antibodies. Bleeding signs and symptoms of the patients with ITP are not always obvious and not as severe as in bone marrow failure syndromes. Cytotoxic agents shouldn't be used if they are not really necessary and the patients' symptoms should be tried to be treated, not the thrombocytopenia itself.

Özet

Amaç: Bu çalışmada 162 erişkin kronik idiyopatik trombositopenik purpura (İTP)'lı hastanın tanı sırasındaki özelliklerini, tedavi endikasyonlarını, klinik seyir ve tedavi sonuçlarını araştırdık. Gereç ve yöntem: 1972-2003 yılları arasında polikliniğimize başvuran ve gözlem altına alınan olgular retrospektif olarak değerlendirildi.Bulgular: Yaşları 12 ile 84 yaş arasında (ortanca: 35 yaş) değişen hastaların 122' si kadın 40' ı erkek idi (K/E: 3,0). Prednizolon (1mg/kg) 138 olguda ilk tedavi seçeneği olarak kullanıldı; 24 olgu tedavisiz olarak takip edildi. Olguların 64'ünde (% 46,3) tam yanıt saptandı. Bu olguların 26'sında PRD dozunun azaltılmasıyla trombositler iki ay içinde tekrar eski haline geriledi. Tam yanıt sağlanan hastaların 11'inde 6 ay ile 4 yıl içinde nüks geliştiği görülmüştür. Tam yanıtın sağlanamadığı olgularda son trombosit değerleri 5.000 ile 125.000/m3 arasında (ortanca: 51.000/m3) bulundu. Ağır trombositopenisi (