Published: 2021-10-25

Study of secondary hemo-phagocytic lympho-histiocytosis in children at a tertiary centre

Usha B. Kantharajanna, Pragalatha Kumar A., Sanjay K. S., Pooja Gujjal Chebbi, Anil Kumar Tenneli


Background: Hemophagocytic lympho-histiocytosis (HLH) is fatal condition characterised by aggressive immune activation, dysregulation and up regulation of inflammatory cytokines leading to uncontrolled activation of T-cells, defective granule mediated cytotoxicity. HLH is classified into primary and secondary HLH.

Methods: This is retrospective study of case-records of children who fulfilled criteria of HLH-2004 guidelines from November 2016 to November 2020.

Results: Total number of patients with HLH was 33, excluding 4 cases of primary HLH, 29 cases met the inclusion criteria. 19 (65.5%) were children less than 5 years of age at the time of diagnosis. Six (20.7%) children were between 6 to 10 years and the rest 4 (13.8%) ten years. Average age at presentation was 4.93 years. Male to female ratio was 0.8 to 1. Fever was the chief complaint present in 29 (100%) of cases. Laboratory parameters showed anaemia, thrombocytopenia, hyper-ferritinemia and hypertriglyceridemia in all 29 (100%) cases. CRP elevated in 28 (96.6%) cases. Infections found in 29 (100%) of cases, bacteria in 19 (65.5%), viral in 10 (34.5%). Mortality seen in 11 (37.9%) cases. Average time of diagnosis from admission was 5.6 days. 4 children out of 11 had dengue as cause for secondary HLH and 4 had bacterial sepsis one each CMV, scrub typhus and SLE. Following factors affected the outcome by multivariate analysis, coagulopathy, elevated liver enzymes, bone marrow biopsy and immunosuppressive therapy.

Conclusions: HLH should be considered in differential diagnosis of children with sepsis.  High index of suspicion for HLH with early initiation of treatment should be considered for better outcome.


Infection, Secondary HLH, Children, Bacterial, Viral

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