Byline: Baijayantimala. Mishra, Neelam. Varma, Suma. Appannanavar, Pankaj. Malhotra, Mrinalini. Sharma, Anil. Bhatnagar, Radha. Ratho, Subhash. Varma
Background : Hemophagocytic syndrome (HPS) is a rare clinicopathological condition characterized by the activation of macrophages with prominent hemophagocytosis in bone marrow and other reticulo-endothelial systems. HPS can be familial or secondary to infections including viruses. Aim : To study the viral markers in patients with HPS. Materials and Methods : Serum samples of patients with HPS and control group were screened for anti EBV VCA IgM, and IgG, anti-Parvo B19 IgM, and anti-CMV IgM antibodies using commercially available ELISA kits and CMV and ParvoB19 DNA by polymerase chain reaction (PCR). Results and Discussion : The present prospective study reports the profile of viral markers in HPS cases from north India. Among the 14 HPS cases 43% (6/14) were positive for at least one viral marker tested, of which EBV was found to be the most prevalent (3/6: 50%) followed by parvovirus B19(2/6: 33%) and cytomegalovirus (1/6: 17%). Mortality was noted in 33% of virus associated HPS patients. Our study highlights the higher association of Epstein-Barr virus (EBV) with HPS as compared to other viruses along with higher rate of mortality in both parvovirus B 19 and EBV associated HPS.
Hemophagocytic syndrome (HPS) is a clinicopathological condition characterized by the activation of histiocytic macrophages with prominent hemophagocytosis in bone marrow and other reticulo-endothelial systems. [sup] Clinically, the disease is characterized by fever, pancytopenia, splenomegaly, and features of hemophagocytosis in reticulo endothelial cells. Besides familial etiology, various infectious agents have been reported in HPS. Among the viruses, agents like Epstein-Barr virus (EBV), cytomegalovirus (CMV), human herpesvirus 6 (HHV6) and parvovirus B19 (PVB19) have been implicated. [sup],, The present study was planned to find out the profile of viral markers in patients with HPS in a well controlled prospective case series.
The study was carried out over a period of three years in a tertiary care hospital that caters mostly to the Northern and North Western states of India. Fourteen consecutive patients with clinical and biochemical markers suggestive of HPS were recruited in the study group following the criteria of Henter et al. [sup] Nineteen patients with aplastic anemia with no evidence of hemophagocytosis in bone marrow were included...