Title of Case(s):

PERSISTENT FEVER, HEPATOSPLENOMEGALY, RASH, GBS BACTERAEMIA AND EBV REACTIVATION IN 11 YEAR OLD GIRL

Background:

A complex case requires a multiprofessional approach to make a unifying diagnosis.

Case Presentation Summary:

An 11-year-old girl was transferred to a tertiary centre having presented to her local hospital with a 2-day history of fever, left-sided neck swelling, epistaxis, hepatosplenomegaly, and jaundice. Blood cultures grew Group B Streptococcus, and appropriate antibiotics were commenced. Significant medical history included catastrophic epistaxis four months ago requiring multiple transfusions, diagnosed as immune thrombocytopenia which had resolved at review. Old Ebstein-Barr virus(EBV) infection was evident at this time on serology, with minimal reactive viraemia.

Blood results showed low Hb(65), lymphopenia(1.04), normal platelets, positive DCT, ferritin 550, CRP 70, AST 618, total bilirubin 21 and conjugated bilirubin 12. She had a mild EBV viraemia, hypergammaglobulinaemia, undetectable C3/C4; Neck ultrasound: lymphadenitis; Blood film: changes consistent with infection, and felt to be inconsistent with autoimmune haemolysis/malignancy.

She was transferred to her local hospital on antimicrobials to be closer to family, but 3 days later she was transferred back as she remained febrile with a progressive malar rash, mouth ulcers and alopecia. Repeat infective screen was positive only for mild reactive EBV viraemia. Abdominal MRI showed inflamed/necrotic para-aortic lymph nodes, which suggested an underlying autoimmune process rather than lymphoma. Rheumatology consultation diagnosed systemic lupus erythematosus(SLE) with evolving haemophagocytic lymphohistiocytosis(HLH), and treated this with IV methylprednisolone, followed by anakinra within the first 24 hours as she developed acute pancreatitis. Her ANA and anti-dsDNA results eventually came back positive. She responded well to immunomodulatory and supportive treatment.

Key Learning Points:

When an infective process does not follow a typical course, review the diagnosis with a multiprofessional approach, and consider autoimmune aetiologies such as SLE.

ITP may be a presenting feature of evolving SLE.

Hyperinflammation(HLH) should always be considered in any sick child.