BackgroundInfectious mononucleosis is one of the main manifestations of Epstein – Barr virus, which is characterized by fever, tonsillar-pharyngitis, lymphadenopathy and atypical lymphocytes. Although 60% of patients with IMN develop cold type antibodies, clinically significant hemolytic anemia with a high ferritin level is very rare and validity of serum ferritin as an important biomarker has not been used frequently.Case presentation18-year-old girl presented with fever, malaise and sore throat with asymptomatic anemia, generalized lymphadenopathy, splenomegaly and mild hepatitis. Investigations revealed that she had cold type autoimmune hemolysis, significantly elevated serum ferritin, elevated serum lactate dehydrogenase level with serological evidence of recent Epstein Barr infection. She was managed conservatively and her hemoglobin and serum ferritin levels normalized without any intervention following two weeks of the acute infection.ConclusionCold type autoimmune hemolytic anemia is a rare manifestation of infectious mononucleosis and serum ferritin is used very rarely as an important biomarker. Management of cold type anemia is mainly supportive and elevated serum ferritin indicates severe viral disease.
BackgroundLymphadenopathy is not an uncommon presentation of adult onset Still’s disease: it is present in up to two thirds of patients with adult onset Still’s disease. The characteristic appearance of lymphadenopathy is described as intense, paracortical immunoblastic hyperplasia. Changes in light microscopy may resemble lymphoma, but immunohistochemistry reveals a benign polyclonal B cell hyperplasia.Case presentationWe describe a 67-year-old Sri Lankan woman who manifested relapsing prolonged fever, raised inflammatory markers, arthralgia, myalgia, transient skin rash, and cervical lymphadenopathy histologically characterized by noncaseating granulomatous adenitis with central suppuration. Due to the fact of high prevalence of tuberculosis in the region, an extensive diagnostic evaluation was done to exclude the possibility of extrapulmonary tuberculosis; unsuccessful therapeutic trials of complete antituberculosis regime reliably excluded the possibility of tuberculosis and strengthened the diagnostic validity. Disease flares were characterized by systemic inflammatory response syndrome with immediate clinical and laboratory response to corticosteroids. After systematic diagnostic workup which ruled out possible malignant, rheumatic, or autoimmune diseases and infections previously described as causes of granulomatous adenitis, our patient was diagnosed as having adult onset Still’s disease based on Yamaguchi criteria. She required a trial of indomethacin followed by methylprednisolone pulse therapy and long-term maintenance steroid therapy without steroid-sparing immunosuppressive agents or biological disease-modifying antirheumatic drugs. She achieved full disease remission in 3 months. Reevaluation after 6 months and 1 year did not reveal residual disease activity.ConclusionsTo the best of our knowledge this is the first report of suppurative noncaseating granulomatous lymphadenitis attributed to adult onset Still’s disease among Asian or South Asian ethnicities and it is also rarely reported among Europeans and North Americans. It is an extremely challenging situation to diagnose Still’s disease with granulomatous lymphadenitis where tuberculosis is highly prevalent. This case highlights the importance of consideration of adult onset Still’s disease as a potential diagnosis in a compatible clinical context in the presence of noncaseating granulomatous adenitis and indicates that one should not be misled into a diagnosis of tuberculosis by the fact of the high prevalence of tuberculosis, however, the exclusion of other diagnoses is a prerequisite.
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