Sex: F
Vaccine: MMR
Symptoms: Haemophagocytic lymphohistiocytosis, Kawasaki's disease, Peripheral swelling, Pyrexia, Rash
Clinical narrative: Fever; Rash; swelling of the legs, feet, and fingers; Kawasaki-like symptoms; macrophage activation syndrome (MAS); This serious case was reported in a literature article and described the occurrence of fever in a 5-year-old female patient who received MMR (MMR vaccine) for prophylaxis. Literature Reference.  The patient's past medical history included gastrointestinal infection (starting at 4 months of age), respiratory infection (starting at 4 months of age), gastroenteritis bacterial (hospitalization at 14 months for), diarrhea, oral intake reduced and leukopenia. Concurrent medical conditions included fever (At 20 months of age), joint swelling (At 20 months of age), inflammatory arthritis, systemic lupus erythematosus, alopecia, pneumonia (hospitalized), immunodeficiency, inflammation, hypergammaglobulinemia, combined immunodeficiency, granuloma and upper respiratory tract infection.   On an unknown date, the patient received MMR vaccine. On an unknown date, an unknown time after receiving MMR vaccine, the patient experienced fever (Verbatim: Fever) (serious criteria hospitalization), rash (Verbatim: Rash) (serious criteria hospitalization), swelling (Verbatim: swelling of the legs, feet, and fingers) (serious criteria hospitalization), kawasaki's disease (Verbatim: Kawasaki-like symptoms) (serious criteria hospitalization and GSK medically significant) and macrophage activation syndrome (Verbatim: macrophage activation syndrome (MAS)) (serious criteria hospitalization and GSK medically significant). The patient was treated with prednisolone. The outcome of the fever, rash, swelling, kawasaki's disease and macrophage activation syndrome were not reported.   The reporter considered the fever, rash, swelling, kawasaki's disease and macrophage activation syndrome to be related to MMR vaccine.     The company considered the fever, rash, swelling, kawasaki's disease and macrophage activation syndrome to be related to MMR vaccine.   Additional Information: GSK Receipt Date: 25-Mar-2024  This case correspondence to the patient B as mentioned in the literature article.  A 5 year-old female who was born full term with no complications, but had multiple gastrointestinal and upper respiratory infections starting at 4 months of age. She required hospitalization at 14 months for bacterial gastroenteritis after presenting with high fever, diarrhea, decreased oral intake, and was subsequently treated with antibiotics. Two months later, she was hospitalized again with fever, rash, swelling of the legs, feet, and fingers, with concern for Kawasaki's disease after live MMR vaccination. Eventually, she was diagnosed with macrophage activation syndrome (MAS), successfully treated with parenteral and oral corticosteroids with good effect. Given that she was previously hospitalized with a bacterial infection and recurrent infections since 4 months of age, she also had a workup for immunodeficiency, which raised a suspicion for primary immune deficiency. At 20 months of age, she presented with another episode of fever and swelling of multiple joints with laboratory workup revealing leukopenia, elevated IgM, elevated CRP, elevated ESR, elevated Anti-Nuclear Antigens (ANA) titer (1:1,280), and positive anti-dsDNA and anti-phospholipid IgM antibodies. At this time, she was diagnosed with inflammatory arthritis, with differential diagnosis of systemic lupus erythematosus as she fulfilled the WHO classification criteria for systemic lupus erythematosus (SLE) (4/11 criteria: arthritis, leucopenia, positive ANA and positive dsDNA and APL). Her joint symptoms were controlled with NSAIDs and glucocorticoids. At 30 months of age, elevated ANA titers prompted therapy with hydroxychloroquine and glucocorticoids that resulted in a reduction in ANA titer to 1:320. Patient B was asymptomatic until 4 years of age, when she developed alopecia that was thought to be a side effect of hydroxychloroquine and therefore discontinued. Approximately 8 months later, she was hospitalized with pneumonia. This patient had hypergammaglobulinemia, but nevertheless displays a combined immune deficiency (CID) phenotype based on absent TRECs, low naive CD4 T cell count and low B cell count. Both patients share a history of arthritis and alopecia. This patient currently moderate symptoms into more severe, treatment-resistant autoimmune conditions.  This article is not available for regulatory reporting purpose due to copyright restriction.