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Open Access Case report

Pancytopenia as an early indicator for Stevens-Johnson syndrome complicated with hemophagocytic lymphohistiocytosis: a case report

Zhi-Dan Fan, Xiao-Qing Qian and Hai-Guo Yu*

Author Affiliations

Department of Rheumatology and Immunology, Nanjing Children’s Hospital Affiliated to Nanjing Medical University, No. 72 Guangzhou Road, Nanjing, Jiangsu Province 210008, China

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BMC Pediatrics 2014, 14:38  doi:10.1186/1471-2431-14-38

Published: 10 February 2014

Abstract

Background

Stevens-Johnson syndrome (SJS) is a severe skin and mucosal bullous disease. When complicated with Hemophagocytic lymphohistiocytosis (HLH), the condition is especially life-threatening.

Case presentation

Here we report the case of a 4-year-old boy suffering from SJS with extensive erythema multiforme and bulla. Despite active intervention and supportive care, the boy experienced increased skin lesions and a higher fever. Meanwhile, decreases in white blood cell count and hemoglobin were observed. Hyperferritinemia, increased soluble CD25 level, decreased NK cell activity and hemophagocytosis in the boy’s bone marrow confirmed the diagnosis of HLH. After high-dose intravenous immunoglobulin and methylprednisone pulse therapy, the boy was discharged in good condition.

Conclusion

Simultaneous occurrence of HLH and SJS is very uncommon and the condition is life-threatening. Pancytopenia can be a precocious indicator and enables to start a prompt diagnosis and treatment.

Keywords:
Pancytopenia; Early diagnosis; Stevens-Johnson syndrome (SJS); Hemophagocytic lymphohistiocytosis (HLH)