Fulminant infantile hemophagocytic syndrome associated with disseminated fungal infection – a case report

Background: Fulminant childhood hemophagocytic syndrome associated with viral infections, in particular with Epstein Barr Virus (EBV) is reported in the literature but its coexistence with infection by fungal organisms in infantile age group is not described.
Case Report: A nine month-old female infant presented with acute onset of high grade fever, cough and respiratory distress. Clinical examination suggested multisystem involvement with anemia, periorbital edema, respiratory distress and hepatosplenomegaly. Baby’s other siblings were apparently healthy. The
provisional clinical diagnosis was severe nutritional anemia with congestive cardiac failure and
septicemic pneumonia. Laboratory investigations revealed pancytopenia and a deranged renal
function. With progressive deterioration of the general condition, the baby was transferred to
pediatric intensive care unit where she bled during endotracheal intubation and died of shock
and severe respiratory distress. A post mortem bone marrow examination was carried out.
Smears revealed hypocellularity with prominent lymphohistiocytosis, striking hemophagocytosis
with a few phagocytes showing ingested fungal yeasts and hyphal structures. A final diagnosis
of fulminant infantile hemophagocytic syndrome coexisting with disseminated fungal infection
was made.
Conclusions: A case of fatal infantile hemophagocytic syndrome in association with disseminated fungal
infection is reported for its rarity. Due to its age at onset one has to rule out a possibility of familial
erythrophagocytic lymphohistiocytosis in such an instance. In the present case, a fungal culture
could not be carried out as the organisms were picked up on the postmortem bone marrow
examination.

Keywords: hemophagocytic syndrome, infantile, disseminated fungal infection