ABSTRACT
A 57-year old woman with a history of multiple sclerosis, treated with interferon beta-1a in the last 5 months, was referred for hyperpyrexia (>40°C) that persisted for 15 days. At admission, there was elevation of transaminases, anemia (hemoglobin 8.9 g/dL), thrombocytopenia (platelet 135,000/mm3), and hypofibrinogenemia (fibrinogen 1.26 g/L). C-reactive protein was 10.7 mg/dL, lactate dehydrogenase 1270 U/L and ferritin 2380 ng/ mL, with hepatosplenomegaly and linfoadenomegaly. Hemophagocytic lymphohistiocytosis induced by direct stimulation of macrophages by interferon (IFN) was suspected. IFN was withdrawn as only measure and onemonth later signs and symptoms disappeared, with complete normalization of laboratory examinations.
Subject(s)
Adjuvants, Immunologic/adverse effects , Interferon-beta/adverse effects , Lymphohistiocytosis, Hemophagocytic/chemically induced , Female , Humans , Interferon beta-1a , Middle Aged , Multiple Sclerosis/drug therapyABSTRACT
Familial Mediterranean fever is an autosomal recessive hereditary disease characterised by recurrent fever, poliserositis, chest and/or abdominal pain. Up to date diagnosis is based on clinical symptoms, familial anamnesis and response to colchicine. It is an inflammatory reaction affecting serosal tissues but until recently different hypotheses have been suggested to explain the greatly increased chemotactic activity of the polymorfonuclear leucocytes. Identification of the function of the MEFV gene on chromosome 16 and its protein allows us to understand the pathogenesis of familial Mediterranean fever as well as provides a new diagnostic test and therapeutic measures. We describe a case of an young patient and review the literature.
