Objectives: Ar King Faisal Specialist Hospital and Research Centre, we retr
ospectively reviewed material from II pediatric patients to assess the most
common clinical and laboratory features of hemophagocytic syndrome, and to
evaluate the outcome of different therapy regimens.
Methods: Eleven children with the diagnosis of hemophagocytic syndrome were
reviewed. The bone marrow aspiration smears and trephine biopsies were ree
xamined under light microscopy to confirm the presence of hemophagocytosis.
The different therapeutic agents and patient outcomes are also reviewed.
Results: Fever, hepatosplenomegaly and anemia were the most consistent clin
ical findings. Bone marrow aspiration and trephine biopsy demonstrated hemo
phagocytosis in 9 out of 11 cases. Two patients were diagnosed by lymph nod
e biopsy examination and splenic aspirations. Viral infections were documen
ted in some cases. Eight patients received specific chemotherapy treatment.
Two patients received allogeneic bone marrow transplantation with 12 month
s' survival time post-bone marrow transplantation.
Conclusion: This review of 11 pediatric patients with hemophagocytic syndro
me suggests a familial type rather than an infectious cause. Allogeneic bon
e marrow transplantation might be the only curative treatment for hemophago
cytic syndrome.