Dengue Associated Hemophagocytic Lymphohistiocytosis: A Case Series.

Hemophagocytic lymphohistiocytosis is a rare complication of dengue. We present 8 cases of dengue associated hemophagocytic lymphohistiocytosis diagnosed in our hospital during the dengue outbreak of 2012. All the cases were treated with a short (4 weeks) course of steroids along with supportive measures, and showed an excellent response.

Factors Associated With Delay in Diagnosis of Kawasaki Disease in India.

A retrospective analysis was carried out to identify factors associated with delay in diagnosis of Kawasaki disease in a tertiary care pediatric hospital setting in Chennai, India. Over a period of 2 years, a total of 37 cases were studied. The cases were divided into Early Diagnosis Group (EDG) and Delayed Diagnosis Group (DDG) with the cut-off for early diagnosis being ten days. A greater proportion of cases in the EDG presented primarily to our institution (P=0.004). In the DDG group greater number of cases had received medical attention from practicing pediatricians prior to referral. There was greater interval in onset of individual symptoms in the DDG group. There was no difference between the two groups with regard to age, gender, total blood counts, CRP, liver enzymes, urine analysis or serum albumin values. Platelet counts were higher in the DDG compared to the EDG (P=0.004).Coronary abnormalities were more common in the DDG (P=0.05). Our findings suggest that children presenting primarily to a tertiary care centre with symptoms of Kawasaki disease are more likely to be associated with early diagnosis and delay in onset of neck swelling or oral lesions may be associated with delayed diagnosis. There is a need for creating more awareness about Kawasaki disease among practicing pediatricians in India.

Profile of Hemophagocytic Lymphohistiocytosis in Children in a Tertiary Care Hospital in India.

Objective: To describe the epidemiology, clinical features, laboratory findings, outcome and the difficulties in diagnosis and management of children with Hemophagocytic Lymphohistiocytosis (HLH) in a tertiary children’s hospital in India. Study Design: Retrospective analysis of case records of all the children with a diagnosis of HLH from December 2006 to December 2008. Setting: Tertiary care children’s teaching hospital in Chennai, India. Results: 43 children had a diagnosis of hemophagocytosis, of who only 33 (19 male, mean age 46 months, range 50 days-14 years) met the inclusion criteria based on the HLH-2004 protocol of the Histiocyte Society. The predominant presenting features included prolonged fever and hepatosplenomegaly. CNS symptoms were present in 36%. Anemia (Hb <9gm/dL), and thrombocytopenia (platelets <1,00,000/mm3) were present in 97% and 72%, respectively. Among the biochemical markers, hyperferritinemia was present in 97%, and hypofibrinogenemia and high LDH in 92%. Bone marrow examination showed hemophagocytosis in 84%. Infectious agents were identified in 42% children, with viruses accounting for 2/3 of them (5 Dengue virus, 3 EBV, 1 CMV, 1 TB and 5 bacterial agents). The mean duration between the onset of symptoms and the diagnosis was 16 days. Corticosteroids were the most commonly used immunomodulatory agents (67%), followed by IVIg (64%). Cyclosporine was used in 33% and Etoposide in 15%. Improvement of laboratory parameters was noticed within 5-7 days of starting treatment. Overall survival rate was 76%. Conclusion: HLH should be considered in the differential diagnosis of children with prolonged fever, hepatosplenomegaly and cytopenia. Prompt recognition and appropriate therapy may result in good outcome, particularly in Infection associated HLH.