Clinical and laboratory data of primary hemophagocytic lymphohistiocytosis: A retrospective review of the Turkish Histiocyte Study Group

被引:6
作者
Fisgin, Tunc [1 ]
Patiroglu, Turkan [2 ]
Ozdemir, Akif [2 ]
Celkan, Tiraje [3 ]
Caliskan, Umran [4 ]
Ertem, Mehmet [5 ]
Yarali, Nese [6 ]
Erduran, Erol [7 ]
Vergin, Canan [8 ]
Canpolat, Cengiz [9 ]
Duru, Feride [1 ]
Bay, Ali [10 ]
Ozbek, Namik [11 ]
Karapinar, Deniz Yilmaz [12 ]
机构
[1] Ondokuz Mayis Univ, Fac Med, Dept Pediat Hematol, TR-55139 Samsun, Turkey
[2] Erciyes Univ, Fac Med, Dept Pediat Hematol & Oncol, Kayseri, Turkey
[3] Istanbul Univ, Cerrahpasa Fac Med, Dept Pediat Hematol, Istanbul, Turkey
[4] Selcuk Univ, Fac Med, Dept Pediat Hematol, Konya, Turkey
[5] Ankara Univ, Fac Med, Dept Pediat Hematol, TR-06100 Ankara, Turkey
[6] Dr Sami Ulus Childrens Hosp, Ankara, Turkey
[7] Karadeniz Tech Univ, Fac Med, Dept Pediat Hematol, Trabzon, Turkey
[8] Dr Behcet Uz Childrens Hosp, Izmir, Turkey
[9] Marmara Univ, Fac Med, Dept Pediat Hematol, Istanbul, Turkey
[10] Yuzuncu Yil Univ, Fac Med, Dept Pediat Hematol, Van, Turkey
[11] Baskent Univ, Fac Med, Dept Pediat Hematol, TR-06490 Ankara, Turkey
[12] Ege Univ, Fac Med, Dept Pediat Hematol, Izmir, Turkey
关键词
Primary hemophagocytic lymphohistiocytosis; clinical and laboratory findings; SYNTAXIN-11; MUTATIONS; DIAGNOSIS; CHILDREN;
D O I
10.5152/tjh.2010.47
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objective: This study analyzes the clinical and laboratory findings of children with primary hemophagocytic lymphohistiocytosis (HLH) followed in various referral centers of Turkey. Materials and Methods: A simple three-page questionnaire prepared by the Turkish Histiocyte Study Group was used for documentation of patient data. Results: Age at diagnosis varied from 0.6 to 78 months (median +/- SD, 16.5 +/- 26.1). Sex distribution was almost equal (F/M= 10/12). The frequencies of parental consanguinity and sibling death in the family history were 100% and 81.1%, respectively. The most common clinical findings were hepatomegaly (100%) and fever (95%). The most common laboratory findings were anemia (100%), hyperferritinemia (100%) and thrombocytopenia (90.9%). Triglyceride and total bilirubin levels in the deceased versus surviving group appear to be high (triglyceride: 394 +/- 183 mg/dl, 289 +/- 7 mg/dl; total bilirubin: 2.7 +/- 6.9 mg/dl, 0.5 +/- 1.2 mg/dl, respectively). Conclusion: We concluded that fever, hepatosplenomegaly, anemia, thrombocytopenia, and hyperferritinemia are the most common clinical and laboratory findings in primary HLH. Increased triglyceride and total bilirubin level at the time of diagnosis might be an indicator of poor prognosis in HLH. (Turk J Hematol 2010; 27: 157-62)
引用
收藏
页码:257 / 262
页数:6
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