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.