Neonatal Purpura Fulminans: 7 Olguluk Deneyimimiz

Abstract

Giriş: Purpura fulminans nadir görülen bir klinik tablodur. Yenidoğan döneminde sıklıkla konjenital veya kazanılmış protein C veya S eksikliğinden kaynaklanır. Sepsis ilişkili yaygın damar içi pıhtılaşma da bir diğer risk faktörüdür.Yöntem: Üçüncü basamak bir yenidoğan yoğun bakım ünitesinde Ocak 2008 ile Mayıs 2010 tarihleri arasında purpura fulminans tanısı ile izlenmiş olguların dosyaları geriye dönük olarak incelendi. Olguların faktör 5 Leiden, protrombin G20210A ve metilentetrahidrofolat redüktaz mutasyonu sonuçları, plazma protein C, S, antitrombin 3, antifosfolipid antikor ve homosistein düzeyleri ve kültür sonuçları kaydedildi.Bulgular: Toplam yedi olgunun verileri değerlendirildi. Olguların tamamının tanı anında plazma protein C, S ve antitrombin 3 düzeyi düşüktü. Metilentetrahidrofolat redüktazın homozigot mutasyonu bir olguda, heterozigot mutasyonu ise bir diğer olguda saptandı. Homosistein düzeyi metilentetrahidrofolat redüktaz mutasyonu olan olgularda hafif yüksek; diğerlerinde ise normaldi. Bir olguda tedavi sonrasında protein C, S ve antitrombin 3 düzeyi tekrarlanabildi ve sonuçlar normal bulundu. Dört olgunun kan kültüründe etken, izole edildi. İzole edilen mikroorganizmalar Pantoea agglomerans, Candida albicans, Klebsiella pneumonia ve Staphylococcus hominis’di.Sonuç: Bildiğimiz kadarıyla serimiz bugüne kadar yayınlanmış en geniş purpura fulminans tanılı yenidoğan olgu serisidir ve Pantoea agglomerans, Candida albicans ve Klebsiella pneumonia yenidoğanlarda purpura fulminans etkenleri olarak ilk kez bu seri ile gösterilmiştir. Purpura fulminans tanısı ile izlenen ve etiyolojide sepsis ilişkili yaygın damar içi pıhtılaşma düşünülen yenidoğan olgularda bu nadir patojenler de akılda tutularak tedavi ve takipler planlanmalıdır.

Keywords

• Purpura fulminans, yaygın damar içi pıhtılaşma, yenidoğan

NEONATAL PURPURA FULMINANS: OUR EXPERIENCE WITH 7 CASES

Abstract

Introduction: Purpura fulminans is a rare clinical entity. In the neonatal period it is commonly caused by inherited or acquired protein C or S deficiency. Disseminated intravascular coagulation associated with septicemia in newborns may be another risk factor for purpura fulminans. Methods: Charts of the patients followed up with the diagnosis of purpura fulminans at a tertiary level neonatal intensive care unit between January 2008 and May 2010 were retrospectively reviewed. Factor 5 Leiden, prothrombin 20210 A and methylenetetrahydrofolate reductase mutations, levels of plasma protein C, S, antithrombin III, antiphospholipid antibody and homocystein, and culture results were recorded in all the patients. Results: Results of a total of seven patients were evaluated. Plasma protein C, S and antithrombin III levels were found decreased at the time of diagnosis in all the cases. One of the cases had methylenetetrahydrofolate reductase homozygous mutation, and another had methylenetetrahydrofolate reductase heterozygous mutation. Homocystein levels were slightly elevated in cases with methylenetetrahydrofolate reductase mutation, and normal in the others cases. Plasma protein C, S and antithrombin 3 levels were studied in only one case after treatment and were normal. A pathogen was identified in blood culture in four out of the seven cases. The isolated microorganisms were Pantoea agglomerans, Candida albicans, Klebsiella pneumonia and Staphylococcus hominis.Conclusion: Pantoea agglomerans, Candida albicans, and Klebsiella pneumonia were determined as the causative agents of purpura fulminans in this series of newborns, which, to our knowledge, is the largest one reported till now. These pathogens should be remembered in infants who are followed up with the diagnosis of purpura fulminans and suspected to have disseminated intravascular coagulation associated with sepsis

Keywords

• Purpura fulminans, disseminated intravascular coagulation, newborn

References

1. Kumar L, Thapa BR, Kaushal RK, Bushnurmath SR. Purpura ful- minans. Indian J Pediatr 1987; 54(3):415-9.
2. Adcock DM, Brozna J, Marlar RA. Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis. Semin Thromb Hemost 1990;16(4):333-40.
3. Edlich R, Cross CL, Dahkstrom JJ, Long WB. Modern concepts of the diagnosis and treatment of purpura fulminans. J Environ Pathol Toxicol Oncol 2008;27(3): 191-6
4. Kavakli K, Polat A, Aydınok Y, Akisu M, Kultursay N, Cetingul N, et al. Yenidoğan Bir Bebekte Tip I Homozigot Protein-C Eksikliği ve Purpura Fulminans: Tanı, tedavi ve izlem. Turkiye Klinikleri J Pediatr 1998;7(4):194-8.
5. Sen K, Roy A. Management of neonatal purpura fulminans with severe protein C deficiency. Indian Pediatr 2006;43(6):542-5.
6. Fischer D, Schloesser RL, Nold-Petry CA., Nold MF, Veldman A. Protein C concentrate in preterm neonates with sepsis. Acta Paedi- atrica 2009;98(9):1526-9.
7. Gurgey A, Aytac S, Kanra G, Secmeer G, Ceyhan M, Altay C. Out- come in children with purpura fulminans: report on 16 patients. Am J Hematol 2005;80(1):20-5.
8. Gurses N, Ozkan A. Neonatal and childhood purpura fulminans: review of seven cases. Cutis 1988;41(5):361-3.

9. Alyamac Dizdar E, Oguz S, Nur Sari F, Erdeve O, Ozkan Ulu H, Dilmen U. Enterocutaneous fistula secondary to purpura fulminans in a preterm infant. J Pediatr Hematol Oncol. 2009;31(10): 753-5.
10. Demir Z, Yuce S, Ozdil K, Karmursel S, Velidedeoglu H, Cele- bioglu S. Bir Purpura Fulminans Olgusu: Erken Tanı ve Tedavinin Ekstremitelerin Kurtarılmasındaki Önemi. Turkiye Klinikleri J Med Sci 2005;25(4):597-9.
11. Doğan Y, Aygun D, Yilmaz Y, Kanra G, Secmeer G, Besbas N, et al. Severe protein S deficiency associated with heterozygous fac- tor V Leiden mutation in a child with purpura fulminans. Pediatr. Hematol. Oncol 2003;20(1):1-5.
12. Neonatal purpura fulminans associated with early-onset gram-neg- ative enterobacter septisemia. Int Pediatr 2003; 18: 162-3.
13. Ray JG, Shmorgun D, Chan WS. Common C677T polymorphism of the methylenetetrahydrofolate reductase gene and the risk of ve- nous thromboembolism: meta-analysis of 31 studies. Pathophysiol Haemost Throm 2002; 32(2):51-8.
14. Demirel N, Bas AY, Okumus N, Zenciroglu A, Yarali N. Severe purpura fulminans due to coexistence of homozygous protein C deficiency and homozygous methylenetetrahydrofolate reductase mutation. Pediatr Hematol Oncol 2009;26(8):597-600.
15. Darmstadt GL. Acute infectious purpura fulminans: pathogenesis and medical management. Pediatr Dermatol 1998;15(3):169-83.
16. Nolan J, Sinclair R. Review of management of purpura fulminans and two case reports. Br J Anaest 2001;86(4):581-6.
17. Warner PM, Kagan RJ, Yakuboff KP, Kemalyan N, Palmieri TL, Greenhalgh DG, et al. Current management of purpura fulminans: a multicenter study. J Burn Care Rehabil 2003;24(3): 119-26.