EBV IN PID- A CASE SERIES FROM TERTIARY CARE HOSPITAL IN MUMBAI
Vijaya Gowri
Department of Immunology, BJ Wadia Hospital for Children, Mumbai
INTRODUCTION
EBV belongs γ1 Human Herpes virus family whose pathogenicity is critically dependent upon host control. Patients with PID show increased latent viral load in the blood and are at risk of fulminant Infectious Mononucleosis and EBV driven Lymphoproliferative disorder.
Clinical and Laboratory data of cases registered with BJ Wadia hospital for Children was analysed
9 cases of EBV associated disease were identified, 6 were Male and 3 Females. Mean age at diagnoses 2years - 45years. Consanguinity present in 3 cases. 9/9 positive for antibodies to EBV-VCA. Antibodies to NA and EA in 4/9 cases. EBV-PCR was positive in 5 cases. 2 cases had Selective Ig M deficiency. Lymph node Biopsy EBER positive in 5 cases. Molecular diagnosis by NGS in 4/9 cases- 1 APDS, 1 RAG1/2, 1 XLP2, 1XLP1.
1 Male+1 Female - Infectious Mononucleosis; male child had a fulminant course and expired on Day 3 of admission.
4 cases of EBV driven LPD suspected as relapse of HL- managed with Rituximab and steroids.
1 case presented with recurrent H. ZOOSTER, madarosis, lymphadenopathy, lymphomatoid papulosis of skin. Skin , L.N biopsy EBER positive.
1 case presented in DIC with ongoing sepsis. EBV-PCR positive
Knowledge regarding the varied presentation of EBV along with relevant investigation and enumeration of EBER aids in diagnosis. NGS should be sent to identify PID aiding in CAEBV proliferation