소아에서 발생한 혈구탐식증후군의 예후인자로서 혈청 빌리루빈의 의의
Significance of serum total bilirubin as a prognostic factor for hemophagocytic lymphohistiocytosis in childhood
2
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. Despite of proper treatment and improving treatment regimens, HLH patients still show a fatal prognosis. Therefore the evaluation of prognostic factor is important and there are many studies about hyperbilirubinemia as a prognostic factor in HLH. So we studied the prognostic value of hyperbilirubinemia in HLH children. Methods: A retrospective analysis was performed about 33 patients who were diagnosed with HLH at Pusan National University Hospital and Yangsan Pusan University Hospital between January 2000 to December 2012. We reviewed the clinical characteristics, laboratory findings, and results of treatment to identify hyperbilirubinemia as a prognostic factor in HLH patients. Results: The median age of patients at diagnosis was 32 months. Most of patients presented with fever, pale appearance, abdominal pain and jaundice. Forty-eight point five percentage of patients showed normal serum bilirubiln level (<2.0 mg/dL) and 51.5% showed hyperbilirubinemia (≥2.0 mg/dL). In normal serum bilirubin group, 1 patient (6.3%) was relapsed and 1 patient (5.9%) was relapsed in hyperbilirubinemia group. In the hyperbilirubinemia group, the mortality was higher than the normal bilirubin group but, there was no statistical significance. Conclusion: As a prognostic factor serum bilirubin at diagnosis in HLH patients, there was no significant correlation between hyperbilirubinemia and poor outcome. But, our study has a limitation that the number of patients is too small and almost showed good prognosis.
Abstract
Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. Despite of proper treatment and improving treatment regimens, HLH patients still show a fatal prognosis. Therefore the evaluation of prognostic factor is important and there are many studies about hyperbilirubinemia as a prognostic factor in HLH. So we studied the prognostic value of hyperbilirubinemia in HLH children. Methods: A retrospective analysis was performed about 33 patients who were diagnosed with HLH at Pusan National University Hospital and Yangsan Pusan University Hospital between January 2000 to December 2012. We reviewed the clinical characteristics, laboratory findings, and results of treatment to identify hyperbilirubinemia as a prognostic factor in HLH patients. Results: The median age of patients at diagnosis was 32 months. Most of patients presented with fever, pale appearance, abdominal pain and jaundice. Forty-eight point five percentage of patients showed normal serum bilirubiln level (<2.0 mg/dL) and 51.5% showed hyperbilirubinemia (≥2.0 mg/dL). In normal serum bilirubin group, 1 patient (6.3%) was relapsed and 1 patient (5.9%) was relapsed in hyperbilirubinemia group. In the hyperbilirubinemia group, the mortality was higher than the normal bilirubin group but, there was no statistical significance. Conclusion: As a prognostic factor serum bilirubin at diagnosis in HLH patients, there was no significant correlation between hyperbilirubinemia and poor outcome. But, our study has a limitation that the number of patients is too small and almost showed good prognosis.
참고문헌 (19)
- Henter JI, "HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis", Pediatr Blood Cancer, 48, pp.124~131 (2007)
- Fujiwara F, "Hypercytokinemia in hemophagocytic syndrome", Am J Pediatr Hematol Oncol, 15, pp.92~98 (1993)
- Filipovich AH, "Hemophagocytic lymphohistiocytosis(HLH)and related disorders", Hematology Am Soc Hematol Educ Program, pp.127~131 (2009)
- Gürgey A, "Primary hemophagocytic lymphohistiocytosis in Turkish children", Pediatr Hematol Oncol, 20, pp.367~371 (2003)
- Janka GE, "Familial and acquired hemophagocytic lymphohistiocytosis", Annu Rev Med, 63, pp.233~246 (2012)
- Henter JI, "Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society", Semin Oncol, 18, pp.29~33 (1991)
- Trottestam H, "Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol", Blood, 118, pp.4577~4584 (2011)
- Trottestam H, "Risk factors for early death in children with haemophagocytic lymphohistiocytosis", Acta Paediatr, 101, pp.313~318 (2012)
- Imashuku S, "Hemophagocytic lymphohistiocytosis in infancy and childhood", J Pediatr, 130, pp.352~357 (1997)
- Horne A, "Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis", Br J Haematol, 140, pp.327~335 (2008)
- Lin TF, "Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality", Pediatr Blood Cancer, 56, pp.154~155 (2011)
- Bell RJ, "Familial haemophagocytic reticulosis", Arch Dis Child, 43, pp.601~606 (1968)
- Aricò M, "Hemophagocytic lymphohistiocytosis. Report of 122children from the International Registry. FHL Study Group of the Histiocyte Society", Leukemia, 10, pp.197~203 (1996)
- Henter JI, "Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis", Acta Paediatr Scand, 80, pp.428~435 (1991)
- Allen M, "Familial hemophagocytic lymphohistiocytosis:how late can the onset be?", Haematologica, 86, pp.499~503 (2001)
- Janka G, "Infection- and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis", Hematol Oncol Clin North Am, 12, pp.435~444 (1998)
- 서지영, "급성 A형간염에 의해 발생한 혈구포식증후군 1예", Clinical and Molecular Hepatology, 16(1), pp.79~82 (2010), 대한간학회
- 조은윤, "The Spectrum of Epstein-Barr Virus-Associated Lymphoproliferative Disease in Korea: Incidence of Disease Entities by Age Groups", Journal of Korean Medical Science, 23(2), pp.185~192 (2008), 대한의학회
- Kogawa K, "Prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children: report of the Japan Histiocytosis Study Group", Pediatr Blood Cancer, 61, pp.1257~1262 (2014)