Hemophagocytic Lymphohistiocytosis in children : Queen Rania children’s hospital experience

الملخص EN

Objectives: The aim of this study is to describe the clinical and laboratory features, etiological triggers, treatment and outcome of Hemophagocytic Lymphohistiocytosis (HLH) at single center.

Methods: Clinical features, laboratory findings, underlying causes, therapeutic options and outcomes of 42 patients with HLH who were diagnosed Queen Rania Children’s Hospital, Amman, during the period January 2010 through September 2016 are described.

Results: Total number of cases were 42; 23 males (54.8%) and 19 females (45.2%), age was ranged from one month to 12 years.

All patients met five to six criteria out of the eight HLH-2004 criteria: all patients had fever, splenomegaly, cytopenias and hemophagocytosis in the bone marrow examination; hyperferritinemia at time of presentation was seen in (95.9%), and proven CNS involvement in 10 patients (23.8%).

A significant number of our cohort had oculocutaneous albinism phenotype (18 patients; 42.9%).

Primary HLH was considered in 69%, while secondary or reactive HLH in 31%.

All patients had received corticosteroids and cyclosporine as initial therapy, etoposide (VP-16) had been used only in 6 patients, rabbit antithymocyte globuline (ATG) in 5, and 10 patients (23.8%) received 11 allogenic hematopoietic stem cell transplantations (HSCT) as a curative treatment.

The overall survival rate was 81%, HSCT group showed excellent outcome with survival rate of 90%.

Conclusion: Despite the lack of molecular diagnosis and some sophisticated tests used in diagnosis of HLH, we had diagnosed and successfully managed HLH in pediatric population.

Further studies are required to improve awareness and outcomes in children.