Akut İmmun Trombositopenili Çocukların İlk Tanı Başvuru, Klinik ve Tedavi Özelliklerinin Değerlendirilmesi

Öz

Primer immüntrombositopeni (ITP), daha önce idiyopatik trombositopenik purpura olarak bilinen, çocuklarda trombositopeninin en yaygın nedenidir ve diğer etiyolojiler olmaksızın izole bir düşük trombosit sayısı (<100,000/mm³) ile tanımlanır. Yıllık görülme sıklığı yaklaşık 100,000 çocukta 2 ila 5 arasıdır. Akut ITP, tanı anında mukozal kanama veya cilt bulguları ile kendini gösterir ve durumun süresine bağlı olarak akut, persistan ve kronik ITP olarak sınıflandırılır. Çocukların çoğu (%70-80), tanıdan sonra 12 ay içinde spontan iyileşme yaşar. Hem primer hem de sekonder ITP, immünolojik anormallikler sergiler, ancak sekonder vakalar belirgin etiyolojik faktörlerle ilişkilidir. Tedavi hedefleri aktif kanamayı durdurmaya ve gelecekteki kanamaları önlemeye odaklanmanın yanı sıra, sağlıkla ilgili yaşam kalitesini (HRQoL) artırmayı da kapsamaktadır. Kortikosteroidler, 1950'lerden beri ITP tedavisinin temel itici gücüdür ve son çalışmalar yüksek doz metilprednizolon (HDMP) kullanımını azaltma eğilimini göstermektedir. Bu retrospektif çalışma, Türkiye'de gerçekleştirildi ve akut ITP tanısı almış 40 pediatrik hastayı inceleyerek intravenöz metilprednizolon alan iki gruptaki değişen dozları analiz etti. Sonuçlar, her iki tedavi rejiminin benzer yanıt oranları elde ettiğini ve gruplar arasında relaps oranları veya kronik ITP gelişimi açısından anlamlı bir fark bulunmadığını gösterdi. Genel olarak, başlangıç tedavi yanıt oranlarının anlamlı şekilde farklılık göstermediği, bu çalışmanın akut ITP'nin etkili bir şekilde yönetimi için dozlama stratejilerini araştırma ihtiyacını vurguladığı belirtilmiştir. Bulgular, başlangıç tedavi aşaması için 'mega doz' metilprednizolon kullanımını desteklerken, daha düşük bir dozun da hasta sonuçlarını tehlikeye atmadan benzer şekilde etkili olabileceğini önermektedir.

Anahtar Kelimeler

• akut immün trombositopeni
• çocukluk çağı
• kronikleşme
• metilprednizolon.

Evaluation of Initial Diagnosis, Clinical-Treatment Features of Children with Acute Immune Thrombocytopenia

Öz

Primary immune thrombocytopenia (ITP), formerly known as idiopathic thrombocytopenic purpura, is the most common cause of thrombocytopenia in children, defined by an isolated low platelet count (<100,000/mm³) without other etiologies. It has an annual occurrence of approximately 2 to 5 per 100,000 children. Acute ITP presents with mucosal bleeding or skin manifestations at diagnosis, with classifications for acute, persistent, and chronic ITP based on the duration of the condition. Most children (70-80%) experience spontaneous recovery within 12 months. Both primary and secondary ITP exhibit immunological abnormalities, yet secondary cases are linked to identifiable etiological factors. The treatment objectives focus on halting active bleeding and preventing future bleeding, as well as enhancing health-related quality of life (HRQoL). Corticosteroids have been the mainstay of ITP treatment since the 1950s, with recent studies showing a trend toward reducing high-dose methylprednisolone (HDMP) use.This retrospective study was conducted in Turkey, analyzing 40 pediatric patients diagnosed with acute ITP who received intravenous methylprednisolone in two groups with varying doses. The results indicated that both treatment regimens achieved similar response rates, and there were no significant differences regarding relapse rates or the development of chronic ITP between the groups. Overall, while initial treatment response rates did not differ significantly, the study emphasizes the need to investigate dosing strategies for managing acute ITP effectively. The findings support the use of a ‘mega dose’ of methylprednisolone for the initial treatment phase, but suggest that a lower dose may be equally effective without compromising patient outcomes.

Anahtar Kelimeler

• acute immune thrombocytopeni
• childhood
• chronicity
• methylprednisolone

Kaynakça

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