Successful Treatment of Infection- Associated Hemophagocytic Syndrome with Intravenous ImmunoglobulinHale Ören, Hüseyin Gülen, Canan Uçar, Murat Duman, Gülersu İrkenDepartment Of Pediatric Hematology, Dokuz Eylül University Faculty Of Medicine, İzmir, Turkey
Hemophagocytic syndrome is a rare disorder characterized by a group of clinical, laboratory and histopathological findings such as fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, and hemophagocytosis in the bone marrow, spleen, and lymph nodes. Hemophagocytic syndrome may occur as a primary or secondary disease. Primary type of hemophagocytic syndrome is also known as familial erythrophagocytic lymphohistiocytosis and secondary type is mostly associated with an viral infection and known as infection-associated hemophagocytic syndrome (IAHS). Rapid diagnosis is very important in these patients since suggested treatment strategies for the two types have been different and mortality rate is very high. In this report we present the clinical and laboratory findings and the outcome of two children with IAHS to emphasize the importance of early diagnosis and the effectiveness of intravenous immunoglobulin therapy in these patients. Keywords: Infection-associated hemophagocytic syndrome, Immunoglobulin therapy.
İnfeksiyona Bağlı Hemofagositik Sendromun İntravenöz İmmünglobulin ile Başarılı TedavisiHale Ören, Hüseyin Gülen, Canan Uçar, Murat Duman, Gülersu İrkenDepartment Of Pediatric Hematology, Dokuz Eylül University Faculty Of Medicine, İzmir, Turkey
Hemofagositik sendrom, ateş, hepatosplenomegali, sitopeni, hipertrigliseridemi, kemik iliği, dalak ve lenf bezlerinde hemofagositoz gibi bir grup klinik, laboratuvar ve histopatolojik bulgu ile karakterize nadir bir hastalıktır. Primer veya sekonder olabilir. Primer tipine familyal eritrofagositik lenfohistiyositoz adı verilir. Sekonder tip ise sıklıkla bir viral infeksiyona eşlik eder ve infeksiyona bağlı hemofagositik sendrom (IAHS) adı da verilir. Bu hastalarda hızlı tanı önemlidir, çünkü bu iki tip arasında tedavi stratejileri farklıdır ve mortalite hızı yüksektir. Bu yazıda IAHS’li iki çocuğun klinik ve laboratuvar bulguları ve sonuçlarını sunarak, intravenöz immünglobulin tedavinin ve ekren tanının önemini vurgulamaya çalıştık. Anahtar Kelimeler: İnfeksiyona bağlı hemofagositik sendrom, Ý, mmünglobulin.
Hale Ören, Hüseyin Gülen, Canan Uçar, Murat Duman, Gülersu İrken. Successful Treatment of Infection- Associated Hemophagocytic Syndrome with Intravenous Immunoglobulin. Turk J Hematol. 2003; 20(2): 95-99
Corresponding Author: Hale Ören, Türkiye |
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