Nilgun USTUN, Alper Murat ULAŞLI, Tanju ÇELİK, Erkan YULA, Abdullah Erman YAGİZ, Hayal GULER

Mean Platelet Volume in Children with Familial Mediterranean Fever and the Relationship with Attack Status, Colchicine Treatment and Gene Mutation

Artmış ortalama platelet hacmi (OPH) platelet fonksiyonları ve aktivasyonunun bir göstergesidir ve kardiovaskuler hastalalığı olanlar için bir prognostik faktor olarak kabul edilmektedir. Pediatrik Ailesel Akdeniz Ateşi (AAA) hastalarında OPH düzeylerinin atak sırasında ve atak dışı dönemde incelemeyi ve kolşisin tedavisi ile M694v mutasyonunun varlığının OPH üzerine etkisini incelemeyi amaçladık. Çalışmaya retrospektif olarak 35 pediatrik yaş grubu AAA hastası ile 38 yaş ve cinsiyet eşleştirilmiş sağlıklı control grubu alındı. Çalışmaya alınan hastaların 11’inde (%31) devam eden atak mevcut iken, 24’ü (%69) atak dışı dönemdeydi. 26 (%74) hasta kolşisin tedavisi alırken, 16 (%45.7) hasta M694V gen mutasyonu taşıyordu. Hastalar ile kontrol grubu arasında platelet ve OPH bakımından anlamlı farklılık yoktu. (sırasıyla p=0.196 ve p=0.167). Ortalama platelet sayısı ile OPH değerleri atak sırasında ve atak dışı dönemde de anlamlı farklılık göstermedi (sırasıyla p=0.355 ve p=0.118). Bununla birlikte, OPH düzeyleri AAA atağı sırasında sağlıklı kontrol grubuna göre anlamlı olmayan düzeyde daha düşüktü. (p=0.08). Kolşisin tedavisi alan hastalarda platelet ve OPH düzeyleri daha yüksek idi fakat istatistiksel olarak anlamdı düzeyde değildi. (p=0.097 ve p=0.446). M694V mutasyonu taşıyan AAA’li hastaların OPH düzeyi ortalaması kontrollerden farklı değildi (p=0.773). Sonuç olarak bu çalışma pediatrik AAA hastaları M694V mutasyonu varlığında dahi sağlıklı bireylerle benzer OPH düzeylerine sahip olduğunu göstermiştir. Atherosklerozun erken bir belirteci olarak OPH bu hasta kohortunda anlamlı düzeyde artış göstermemektedir. Düzenli kolşisin tedavisi ve genç yaş AAA hastalarında platelet aktivasyonunun sağlam kalmasında rol oynayabilir

Anahtar Kelimeler:

Ailesel akdeniz ateşi, MEFV geni, ortalama platelet hacmi, kolşisin

Mean Platelet Volume in Children with Familial Mediterranean Fever and the Relationship with Attack Status, Colchicine Treatment and Gene Mutation

Increased mean platelet volume (MPV) is a manifestation of platelet functions and activation, and accepted as a prognostic biomarker in patients with cardiovascular disease. We aimed to investigate MPV levels in pediatric Famillial Mediterranean Fever (FMF) patients during the attack and attack-free periods, and the effect of colchicine treatment and presence of M694V mutation. Thirty-five pediatric patients with FMF and 38 age-sex-matched healty controls were enrolled retrospectively into the study. Of the patients 11 (31%) had an ongoing attack, and 24 (69%) were in attack-free period. 26 (74%) patients were receiving colchicine and 16 (45.7%) had M694V gene mutation. There was no significant difference in platelet (PLT) and MPV between patients and healthy controls (p=0.196 and p=0.167 respectively). Mean PLT and MPV values of the patients during attack and attack-free period were also not significantly different (p=0.355 and p=0.118 respectively). However, MPV levels during an FMF attack were non-significantly lower than healthy control group (p=0.08). PLT and MPV levels were higher in patients receiving colchicine but the differences were not significant (p=0.097 and p=0.446 respectively). Mean MPV value of the FMF patients with M694V mutation was not significantly different than controls (p=0.773). In conclusion, this study reveals that pediatric FMF patients have similar MPV levels with healthy individuals even in the presence of M694V mutation. MPV as an early atherosclerosis marker, is not significantly elevated in this patient cohort. Regular treatment with colchicine and younger age may have a role in non-impaired platelet activation in FMF patients

Keywords:

Familial Mediterranean fever, MEFV gene, Mean platelet volume, Colchicine,

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