ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease. This case report describes a secondary chronic myelogenous leukemia (CML) in a nine-year-old male after treatment with a combination of etoposide (VP16), steroids and cyclosporin for HLH. VP16 can potentially induce leukemia, and single nucleotide polymorphisms may be involved in the pathogenesis of CML. We speculate that genetic backgrounds combined with chemotherapeutic drugs lead to secondary CML. This case report alerts physicians to the importance of genetic screening and the rare side effects of VP16 in children with HLH.
INTRODUCTION
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with acute onset, critical condition, and high mortality rate1. Since using the HLH protocol, mortality rates of HLH have decreased markedly2. With increased survival rates of patients with HLH, some patients who have HLH develop long-term secondary leukemia, such as secondary acute myeloid leukemia(AML), secondary acute lymphoblastic leukemia (ALL) and secondary myelodysplastic syndromes(MDS)3. Several previous cases have demonstrated clinical findings on secondary AML and ALL following diagnosed with HLH in children4, 5.
We present this case of a 9-year-old male who developed chronic myelogenous leukemia (CML) after diagnosis with HLH. He presented with a high fever and lymph node swelling after finishing HLH treatment. It is important to recognize leukemia secondary to HLH in the pediatric population.