Dengue Virus Associated Hemophagocytic Syndrome with Neurogical Manifestations in Children: A Case Report.

Abstract 4516

Dengue fever, caused by dengue virus, can cause increased vascular permeability, which leads to a bleeding diathesis or disseminated intravascular coagulation known as Dengue Hemorrhagic Fever (DHF). Hemophagocytocytic Syndrome (HPS) with neurological manifestations is an uncommon presentation of DHF. There are no reports of virus associated with HPS and neurological manifestations in the pediatric literature. Hemophagocytic Syndrome (HPS) is a clinico-pathologic entity characterized by proliferation of T lymphocytes and macrophages leading to cytokine overproduction. HPS may be diagnosed in association with malignant, genetic, or autoimmune diseases. Dengue virus is considered non-neurotropic, however neuroinvansion has been reported. We present the case of a 10 month old female patient who developed DHF, manifested with upper GI bleeding, hypotension and pancytopenia. The infant subsequently developed dengue shock syndrome, with thrombocytopenia, intravascular hemolysis, coagulopathy, elevated transaminase, hyperbilirubinemia, and creatine kinase (CK) of 2,876U/L. She required artificial hemodynamic and blood components support. During her course of illness, she developed ecchymosed, purpuric bullae lesions of the skin in the distal upper extremities. Her IgM ELISA for Dengue virus was positive. Bone marrow aspiration and biopsy was diagnostic for HPS. The patient was initiated on IV steroids and antibiotics therapy. An improvement was noticed on day 8 of illness and was weaned off completely of all artificial support by the day 9. A complete recovery of her hematological, transaminase, billirubin, and coagulation parameters was noticed on day 12. A repeated bone marrow aspirate and biopsy examination was normal. Patient's recovery was significant by muscle weakness (MCG grade ≤3), hypotonia, reduced tendon reflex and increased CK suggestive of possible myositis. The cerebrospinal fluid and magnetic resonance imaging studies of the brain were normal. She was given IV immunoglobulin (500mg/kg) for 2 consecutive days with remarkable neurological improvement including a normal CK (56U/L).She was weaned of steroids completely on day 20.Dengue virus infection was confirmed by a positive serology result at the convalescent stage. On day 23 she had a complete clinical recovery. To our knowledge, this is the first case reported of dengue virus-associated HPS and neurological manifestations in a pediatric patient with DHF. Clinicians should consider that the occurrence of HPS and neurological manifestations in children could de due to dengue virus infection.