A. Klein et al., Recurrent viral associated hemophagocytic syndrome in a child with Langerhans cell histiocytosis, J PED H ONC, 21(6), 1999, pp. 554-556
Langerhans cell histiocytosis (LCH) with subsequent viral-associated hemoph
agocytic syndrome (VAHS) or secondary hemophagocytic lymphohistiocytosis (H
LH) is extremely rare. A 15-month-old girl with disseminated LCH experience
d three episodes of VAHS during maintenance therapy. Viral infection, with
influenza A, herpes simplex, and adenovirus, respectively, was documented a
t each episode. She recovered each time after interruption of maintenance t
herapy. The occurrence of fever and pancytopenia in patients with chemother
apy-treated LCH can be associated with VAHS and not with relapsing LCH.